key: cord-0034088-n03zk9ni
authors: nan
title: 15(th) Congress of the International Pediatric Nephrology Association: 29 August – 2 September, 2010 New York, NY, USA
date: 2010-06-22
journal: Pediatr Nephrol
DOI: 10.1007/s00467-010-1577-z
sha: 02e359329ec03045275aaf6a153b97dc4e0866af
doc_id: 34088
cord_uid: n03zk9ni

nan

All sessions will take place at the Hilton New York Hotel unless otherwise stated.

The 15 None of the children underwent biopsy in the country. Three patients received a biopsy abroad. All patients have been treated with cortisone orally. Two patients with frequent recurrences were treated with levamisole.

The data shows that the prevalence of steroid resistant nephrotic syndrome is lower if compared to other series in black population. We recommend another study for a larger population of nephrotic patients, to appreciate their evolution and the infl uence of nephrotic syndrome in chronic renal failure.

Demographic data and outcome AZA (n=8) MMF (n=9) Total (n=17) Age 6.4 ± 1.5 7.4 ± 2 7.4 ± 2 ISKDC Histologic lesion Class I 1 (11%) 1(6%) Class II 4(50%) 2 (22%) 6(35%) Class III 4(50%) 6 (67%) 10(59%) Baseline Creatinine clearance (ml/min/ Immunosuppressive protocol included basiliximab, prograf or cyclosporine and prednisone. We emphatize that all patients were addressed to KTx with a signifi cant fl uid overload (as much as 3% above optimal body weight) obtained with plasma infusion. Results: Over a cumulative observation period of 61 mos., we only observed 2 recurrences (in 2 different patients) which were managed with PLE and Eculizumab with immediate recovery of the recurrence. Conclusions: Our therapeutic approach to TKx in FHD-HUS represents a less aggressive solution in the meantime that Factor H becomes available for maintenance treatment.

The main reason for switching was chronic allograft nephropathy. For nine patients we analyzed EGFR and Proteinuria before and after switch with a follow-up more than 12 months: 7 showed stabilization or improvement of EGFR. Treatment was stopped in two patients because proteinuria closes to nephrotic rate. For the other 11 patients : seven needed to stop treatment due to secondary effects. Conclusions: mTor inhibitor treatment seems to be associated with a GFR stabilization. Nevertheless, these results must be confi rmed and validated by prospective studies in bigger cohorts. One patient (ID 1) was switched one year post transplantation to sirolimus, prednisone and low dose of Tinmun because of secondary effects of tacrolimus (severe tremor). Initial doses of T-Inmun were 0.15 mg/kg/d, and were adjusted to maintain trough plasma levels between 10-15 ng/mL in the fi rst year after transplantation and 5-10 ng/mL thereafter. To date, no rejection episodes have been observed and all patients have a stable renal function. Two have controlled arterial hypertension. None has developed proteinuria, hyperglicemia or hyperkalemia. Conclusions: T-Inmun has been a safe and effi cient immunosuppressive drug in this little group of children with renal transplant.

Objectives: Anatomical malformations of the kidney and urinary tract (UT) account for 17% of pediatric renal transplantations (Tx). HSPs activates innate immunity through toll-like receptor (TLR)4. HSPA1B A(1267)G polymorphism leads to lower HSP72 mRNA and is associated with kidney diseases in adults and children. TLR4 A(896)G polymorphism is associated with a higher risk of infections. Methods: HSPA1A G(190)C, HSPA1B A(1267)G and TLR4 A(896)G polymorphisms were analyzed in 41 pediatric Tx recipients, 103 children with recurrent UT infections and 235 healthy controls (CON) . Clinical data were also evaluated. Results: HSPA1B (1267)GG genotype and (1267)G allele were more frequent in Tx (p=0.014; p=0.011) and in UTI (p=0.0001) vs. CON. HSPA1B (1267)GG and (1267)G allele were associated with higher risk of renal scarring after UTI (p=0.012 and 0.049). VUR was associated with a higher incidence of HSPA1B (1267)GG genotype in Tx (p=0.007). TLR4 (896)AG genotype and (896)G allele were more frequent in UTI vs. CON (p=0.031; 0,041), while there was no difference in Tx. TLR4 (896)G was more often in UTI without VUR vs. UTI with VUR (p=0.03). Conclusions: HSPA1B (1267)G allele might be a risk for UTI, VUR and infl uences the development of renal scarring and ESRD leading to Tx.TLR4 (896)G allele might be associated with UTI independently of other renal abnormalities. These fi ndings raise further questions about the clinical relevance of these polymorphisms in pediatric nephrology. Supported by OTKA, ETT,TAMOP.

Native Nephrectomy (Nx) in Children Prior to Kidney Transplant (KT) F. Ghane Sharbaf, 1,2 L. Bell, 1 I. Gupta, 1 G.-P. Capolicchio, 3 M. Bitzan. 1 1 Pediatrics, McGill Univ, Montreal, QC, Canada; 2 Mashhad Univ of Med Sciences, Mashhad, Islamic Republic of Iran; 3 Urology, McGill Univ, Montreal, QC, Canada. Objectives: To review indications, complications, urine output (UO) & laboratory changes pre/post-Nx in a pediatric KT recipient cohort. Methods: From our database (1992) (1993) (1994) (1995) (1996) (1997) (1998) (1999) (2000) (2001) (2002) (2003) (2004) (2005) (2006) (2007) (2008) (2009) ), we identifi ed all patients (pts) with uni-(U) or bilateral (B) native pre-KT Nx and extracted pertinent data (mean, SD, paired t-test). Results: 82 kidneys were removed from 52 pts, age 8.5 yrs, 77% male (22 U, 30 B Nx; 75% retroperitoneoscopically). Main indications: polyuria (PU;13), proteinuria (PrU;13), PU&PrU (7), frequent UTI (9), hypertension (HTN;2), Denys Drash syndrome (2) . 8/22 UNx were performed at KT, one 4 mo post KT. 12/30 with BNx had a 2-stage procedure. Complications were rare: peritoneal laceration requiring temporary PD to HD switch (1), subcutaneous emphysema (1), fl uid overload/HTN (5) , hyperkalemia (2) . UTI decreased in 7/9. Objectives: Chronic antibody-mediated rejection (CAMR) of renal transplant is now a defi ned nosologic entity. Some success has been described with the use of IVIg and Rituximab, but there is no established treatment protocol for this condition. Methods: Herein we describe our experience with 12 young patients (18+5.6yrs) with CAMR treated with IVIg and Rituximab, associated to Plasmapheresis in two cases. The median follow up post-transplantation at diagnosis of CAMR was 14 months (10-27). All patients but one presented a GFR reduction. Transplant glomerulopathy and C4d positivity were present in all biopsies. Donor specifi c antibodies were positive in 6/12 patients. Results: After the treatment only one patient improved GFR; in this patient the diagnosis of CAMR was performed after a protocol biopsy and without laboratory tests suggestive for GFR impairment. One patient had a progressive graft dysfunction, 5 needed dialysis treatment after 18 months and the last 5 maintained a stable renal function. At the end of follow up a mean GFR reduction of 8.4+9.2 ml/min was observed. Conclusions: CAMR is confi rmed to be a clinical condition with a severe outcome. The currently available treatments are not always able to reverse the CAMR. A better outcome is obtained when the treatment is well-timed and in patients with mild tubular and interstitial damages. Objectives: Childhood end-stage renal disease (ESRD) is associated with an increased risk for early adulthood cardiovascular (CV) morbidity and mortality. Increased left ventricular mass (LVM) is an early indicator of this high risk. Previous studies suggested that in children LVM decreases after renal transplantation however, trends have been inconsistent. Our objective was to evaluate the longitudinal effect of transplantation on LVM, in children with ESRD. Methods: In our centre, children routinely undergo echocardiograms prior to transplant and yearly thereafter. LVMI values from these measurements, starting pre-transplant and annually post-transplant, were averaged among included patients. Results: Twenty seven children were followed for a median period of 5 years.

Mean LVMI values graphically represented in the attached fi gure, decreased after renal transplant for up to a maximum of 10 years.

A sustained decline in LVMI following renal transplantation was observed in a cohort of children post-renal transplantation. We plan to investigate the correlation between blood pressure (BP) control and the observed decline in LVMI, however since BP targets in this cohort, before and after transplant, remained similar; we speculate that other factors associated with renal transplantation may have been responsible for this observation.

S. Gulati, P.N. Gupta, V. Kher, R. Ahlawat. Neprhology, Fortis Institute of Renal Sciences adn Transplantation, New Delhi, Delhi, India. Objectives: The aim of this study was to assess the safety of steroid free protocol in Asian pediatric renal transplant recipients receiving Tacrolimus based immunosuppression. Methods: We evaluated three children who received live related kidney transplant and were on steroid free (SF) protocol. All of them received 2 doses of IL2RA as induction. They were given IV Methylprednisolone in an initial dose 500 mg followed by oral prednisolone which was stopped on day 5. All of them received Tacrolimus (0.1 mg/kg) and MMF (1100 mg/m2) as maintenance immunosuppression. We compared these 3 children with three other children who received steroid based protocol during the same time period. The outcome measures that were evaluated were number of acute rejections, graft loss, PTDM, antihypertensive medications, S. Cholestrol, eGFR, height velocity and height SDS.

The study group comprised of two boys and one girl. The mean age was 11.7 years and mean follow up was 14 months (5-24 months) . We compared these 3 children (Group 1) with 3 age matched controls who were on steroid protocol (Group 2). None of these 6 children has any episode of acute rejection. We observed that the growth velocity in group 1 (6.8 cm/yr) was greater than group 2 (4.7 cm/yr). Similarly the improvement in Z-score was greater in group 1 as compared to group 2 (1.48 vs 1.07). The mean cholesterol level in group 1 was lower as compared to group 2 (149 mg/dl vs 248 mg/dl). None of the children in Gp 1 had PTDM while transient PTDM was seen in 1 child in Gp 2. Conclusions: Complete steroid free immunosuppression is effi cacious and safe in this selected group of children with no early clinical acute rejection episodes.

Results: Mean age of recipients was 13,7±4,2 years old. Mean follow-up time after transplantation was 3,2±2,7 years. There was no signifi cant difference for age, donor type, follow-up time between 2 groups. Early proteinuria was signifi cantly higher for the cadaver donor group (mean 14,25mg/m 2 /hour versus 7,08mg/m 2 /hour, p=0,002). Median proteinuria of the rejection group (11,25 mg/m 2 /hour, min-max:2,3-101,8) was signifi cantly higher than these of nonrejection group (4,13mg/m 2 /hour, min-max:0,13-39,42 ) (p<0,05). Signifi cant positive correlation between early proteinuria and acute rejection was shown (r=0,34, p=0,005) . Another positive correlation between early proteinuria and increased serum creatinine on 3 rd year, as a marker of graft injury was shown (r=0,36, p=0,045) . This kind of relationship was not valid for graft loss, GFR and immunosuppressive regiment. Conclusions: Early proteinuria can be used for prediction of the posttransplant acute rejection and graft injury.

The First Case of MMF Administration to a Pediatric Renal

Conclusions: Linear growth in children on SRL was similar to those maintained on CNI. The potential inhibitory growth effects of SRL may be offset by avoidance of CNI nephrotoxicity and better graft function. PATIENT Conclusions: More potent immunosuppressive agents have improved graft outcomes but led to an increase in infections in the paediatric renal transplant population. Our 5 patients failed to respond to conventional antibiotic therapy for recurrent or persistent respiratory infections that required extensive investigation, prolonged antimicrobial treatment, and MMF withdrawal. Medicine, Tokyo, Japan; 2 Pathology, Saitama Children's Medical Center, Saitama, Japan. Objectives: The therapeutic benefi ts of Cyclosporine A (CsA) are often limited by the chronic nephrotoxicity of its long-term use.Chronic nephrotoxicity is manifested by renal function impairment and progressive histopathological kidney lesions characterized by tubular vacuolization, tubular necrosis, interstitial fi brosis, and afferent arteriolopathy. This study tested the hypothesis that the concurrent administration of Mizoribine (MZR) may improve chronic CsA nephrotoxicity. Methods: Sprague-Dawley male rats were divided into the following four groups: group 1, control (n = 6); group 2, treated with CsA alone (n = 5); group 3, treated with CsA and MZR (n = 4); and group 4, treated with MZR alone (n = 6). The anti-infl ammatory and antifi brotic effects of MZR were studied by evaluating the concentrations of the infl ammatory mediator, osteopontin, renal function, and histopathology. The interstitial fi brosis was stained blue with Elastica-Massontrichrome and the sections were quantifi ed. Results: The CsA-treated rats showed decreased renal function and increased histologic parameters in comparison with the control rats and also showed signifi cantly increased interstitial fi brosis area and macrophage in comparison with the control rats. The CsA MZR treatment signifi cantly improved the interstitial fi brosis area and macrophage in comparison with the CsA-treated rats. Conclusions: On the basis of these fi ndings, we suggest MZR effectively attenuates renal macrophage accumulation and the progression of interstitial fi brosis. Objectives: Nephronophthisis (NPHP) is the most common genetic cause of ESRD in children and adolescents. The most frequently affected gene is NPHP1 encoding for the ciliary protein nephrocystin-1. Mutations of the NPHP4 gene encoding for nephrocystin-4 account for a smaller proportion of NPHP cases, yet the clinical presentation of patients with NPHP1 or NPHP4 mutations is quite similar. The cellular function and regulation of the proteins nephrocystin-1 and nephrocystin-4 remain poorly understood. We have recently shown that nephrocystin-1 interacts with the focal adhesion kinase Pyk2. Here we show that Pyk2 induces phosphorylation of nephrocystin-1 at three defi ned tyrosine residues and that this phosphorylation regulates binding of nephrocystin-1 to the trans-golgi sorting protein PACS-1. Interestingly, fullength nephrocystin-4, but not different patient mutations, abolishes the Pyk2-induced tyrosine phosphorylation of nephrocystin-1. Knockdown of nephrocystin-4 leads to changes of the subcellular localisation of nephrocystin-1 in ciliated epithelial cells.

Our data suggest a model in which nephrocystin-4 acts upstream of nephrocystin-1 and modulates its tyrosine phosphorylation state. Thus nephrocystin-4 dynamically regulates the composition of the nephrocystin protein complex and defi nes its subcellular localisation.

Clinical Situation of Patients wth Cystinosis in Germany K. Latta, A. Latta, C. Selbsthilfe e.V. Clementine Kinderhospital, Frankfurt, Germany. Objectives: It was the aim to assess the current situation of cystinosis patients in Germany. Methods: A questionnaire was sent to all patients of the German cystinosis group (n=89). 59 responded. Results: There were 23 adults, and the children had a mean age of 10.9 years. In 39, diagnosis was made before age of two, while 7 were diagnosed beyond 3 years of age. Cysteamine treatment was started at over 2 years of age in 23 patients, and in 15 even after the age of 6 years. Glycosuria Figure) and pediatric patients (91%). Other detected virus species comprised Dobrova virus (adult 1.4%, pediatric 1.8%), Hantaan virus (adult 0.8%, pediatric 0.9%) and Belgrad virus (adult 0.03%, none pediatric). All these virus species are associated with HFRS. Andes virus infections, the only hantavirus transmitted by person-to-person contact, as well as species causing the HCPS did not occur.

The hantavirus species most frequently observed in Germany is the Puumala virus causing HFRS via contact to mice. The distribution of various hantavirus species in pediatric patients in Germany is comparable to adults.

Objectives: Lysinuric proteine intolerance (LPI) is an autosomal recessive metabolic disorder characterized by defective transport of dibasic amino acids at the basolateral membrane of epithelial cells in intestine and kidney tubule. The dysfunction leads to low plasma and high urine concentration of dibasic amino acids. Hyperammonaemia is caused by functional impairment of the urea cycle. Clinical symptoms are variable and non specifi c leading often to misdiagnosis. Methods: We report the case of a 13 year old boy, seen in paediatric nephrology consultation after one episode of macroscopic haematuria with mild proteinuria. Results: He had no positive family history and was born after an uneventful pregnancy. Because of cataract, hepatosplenomegaly and hyperferritinemia, he had had lots of explorations for suspicion of galactosemia, Gaucher disease, Niemann-Pick disease, spherocytosis and haemochromatosis. Urinary amino acid chromatography revealed increased excretion of lysine, arginine, ornithine and citrulline, subsequently plasma analysis has shown low concentration of them and urinary organic acid chromatography elevated acid orotic. Elevated levels of ferritin, liver transminases and mild leucopenia with anemia were also present. Detailed dietary history revealed aversion to protein diet. Conclusions: The treatment has been started consisting in strict avoidance of protein rich food and supplementation of citrulline to replenish the urea cycle.

The renal function will be carefully monitored since chronic renal failure has been reported in LPI. Conclusions: Compared with C, K defi ciency reduces body weight gain and increases kidney size associated with low expression of GHR and IGF-I and upregulation of IGF-IR. Treatment normalized weight gain of DK animals but did not modify the exaggerated renal growth in spite of further increasing cell proliferation. GH also normalized GHR expression, intensifi ed IGF-IR upregulation and did not modify low IGF-I mRNA levels caused by K depletion. -2,8 to -1,4 and renal function remained stable in 86% of patients. GFR changed only slightly during this period of observation.

The most frequent causes of NC were hereditary tubulopathies and vitamin D intoxication in childhood in Albania. Our results show that the treatment of the underlying conditions is associated with catch-up growth and stabilization of renal function in many children, but not with reduction in the degree of NC in the majority of cases.

Objectives: Plasmatherapy (PT) is the fi rst line treatment in atypical hemolytic uremic syndrome (aHUS), but little is known on its effi ciency to prevent end stage renal disease (ESRD). We report the follow up of 10 children treated by long term PT. Results: Median age at diagnosis was 6 months (0.5 to 51 months). 8 patients have factor H (CFH) mutations: homozygous in 3 (quantitative defi ciency), heterozygous in 4 (type 1 -quantitative-in 1; type 2 -functional-in 3), compound (type 1 and 2) in 1. One patient has a heterozygous factor B mutation and 1 patient a heterozygous factor I (CFI) mutation (type 2), combined to an unknown risk factor. All patients received PT (fresh frozen plasma infusions in 6, plasma exchanges in 4) within the fi rst month after onset and were maintained on long term prophylactic PT. Median follow up is 31 months (5 to 67 months). All patients except 2 have had relapses during infections or PT tapering, rescued by treatment intensifi cation. All patients have a normal glomerular fi ltration rate at last follow up, except 1 with hybrid CFH (type 2) mutation. Another patient (CFI mutation) had persistent anemia after 1 year on PT, and has been switched to eculizumab therapy. Conclusions: Prophylactic PT appeared to be effi cient to prevent ESRD in 8/10 patients. Therefore it should be recommended in aHUS although the complement blocker eculizumab may be another option in near future.

Nephritis T. Pereira, C.L. Abitbol, W. Seeherunvong, J. Chandar, M. Freundlich, G. Zilleruelo. Pediatric Nephrology, University of Miami/ Holtz Children's Hospital, Miami, FL, United States. Objectives: To compare renal survival in a cohort of pediatric patients with childhood onset systemic lupus erythematosus (cSLE) and severe lupus nephritis (LN) with progressive treatment regimens that included pulse solumedrol, cyclophosphmide (CYC), mycophenolate mofetil (MMF) and rituximab (RTX). Methods: A retrospective analysis was performed on 117 patients with cSLE. Of the initial cohort, 79 (63 female; 80%) had ≥ WHO class 3 and were designated as having severe LN. The end point for renal survival was progression to end stage kidney disease (ESKD). Results: Average age of onset was 12.5 ± 3.0 years with a mean follow-up of 5.6 ± 3.5 years. There was a nonwhite racial predominance of 92%. Twenty-six (33%) progressed to ESKD. During the fi rst 2 decades, all patients received corticosteroids and CYC. When MMF was added, renal survival improved from a median of 6 to 12 years (p<0.003). Patients treated with MMF alone or with RTX have experienced no patient or renal demise.

Renal survival in childhood onset LN has improved during the past 3 decades with the addition of MMF to the treatment regimen. Continued experience with this as an induction agent with or without the use of RTX requires further investigation. Collaborative trials in pediatric patients are very much warranted.

Objectives: Henoch shonlein purpura (HSP) is a systemic vasculitis characterized by tissue deposition of IgA containing immune complexes. Although associated renal involvement is common it is usually mild and of favorable outcome. The tetraspan molecule CD81 is widely expressed on immune cells, but also on most stromal and epithelial cells. In B-cells it is required for signaling upon antigen recognition. Its functions on other cells are still unclear. Methods: We herein report the case of a 7-year-old girl who presents an association of severe and uncontrollable IGA glomerulonephritis, profound hypogammaglobulinemia, immunopathology and a CD81 mutation. Results: The patient is the fi rst child of consanguineous parents. At the age of 3.5 years she developed HSP. Renal biopsy revealed diffuse mesangial proliferation, crescent formation and strong deposition of IgA and C3.She also presented recurrent episodes of thrombocytopenia associated with anti-platelet antibodies and seric levels of IgG were persistently low.She was treated with repeated full course immunosuppressive therapy but progressed to end stage renal failure. Flow cytometric immunophenotyping of blood showed no expression of CD19 on the patient's B-cells. Additional studies showed that all cells lacked CD81 expression. Sequencing of the CD81 gene showed a homozygous G>A substitution. Conclusions: We present the fi rst case of immunodefi ciency syndrome, due to a CD81 mutation. The nephropathy is likely an indirect consequence of the CD81 defect.

Varicella Zoster Virus (VZV) Infection in Immunocompromised Patients: Treatment with Specific Human VZV Polyclonal Immunoglobulins A.-L. Sellier-Leclerc, 1 B. Aoun, 2 V. Baudouin, 1 G. Deschênes, 1 T. Ulinski. 2 1 Pediatric Nephrology, Robert Debré Hspital, Paris, France; 2 Pediatric Nephrology, Armand Trousseau Hospital, Paris, France. Objectives: Infection with the varicella-zoster virus (VZV) is more dangerous in immunocompromised patients than it is in the general population. High doses of acyclovir and immediate reduction of immunosuppression may improve the prognosis of severe forms, but multiorgan failure and death may occur despite early antiviral treatment.

Here, we report four cases of VZV infection in children receiving steroid therapy and immunosuppressive drugs for renal allograft, idiopathic nephrotic syndrome, and systemic lupus. Methods: The only clinical manifestation in three patients was general malaise, fever, and disseminated vesicular rash, whereas one patient also showed severe and diffuse visceral involvement with multiorgan failure. Results: Adjuvant treatment with specifi c human varicella zoster polyclonal immunoglobulins led to a dramatic improvement of VZV infection in all four patients within two days after administration. Simultaneous acyclovir therapy may jeopardize interpretation, but there was a rapid clinical improvement 24 to 48 hours after administration of specifi c human varicella zoster polyclonal immunoglobulin, suggesting its favourable role for disease outcome in these four patients. Conclusions: Specifi c human varicella zoster polyclonal immunoglobulin is probably a useful adjuvant therapy to acyclovir in declared and severe varicella in immunocompromised children.

Regulatory T Cells (Treg) and Interleukin 17 Producing T Cells (TH17) in Iga Nephropathy (IgAN) R. Camilla, 1 V. Daprà, 1 E. Loiacono, 1 L Peruzzi, 1 C. Rollino, 2 G. Beltrame, 2 M. Ferro, 2 R. Gallo, 1 L Garassino, 1 A. Amore, 1 R. Coppo. 1 1 Nephrol Dial Transplant, R Margherita Hosp, Turin, Italy; 2 Nephrol, Dial, G Bosco Hosp, Turin, Italy. Objectives: We aimed at investigating Treg/TH-17 producing cells balance in IgAN and the correlation with TLRs expression, as marker of innate immunity. Methods: Circulating mononuclear cells were isolated from 28 IgAN patients, 82% males, e-GFR 92.6±46.5 ml/min, median protein/creatinine 0.2, and10 healthy controls (HC). mRNA expression of Foxp3, TH17-related factors (IL-17 and retinoid orphan nuclear receptor (RORc), TFG-beta1 and TLR 2, 4 and 7 were assessed by Taqman and normalized to Abelson gene (Abl). Results: Transcriptional levels of Foxp3 were signifi cantly lower in IgAN vs HC (0.82±0.30 vs 1.05±0.37, p= 0.041), while those of IL-17 and of its regulatory factor RORc were slightly, but not signifi cantly increased (IL-17 1.17±1.07 vs 1.05±0.41 in HC, RORc 1.25±0.85 vs 1.14±0.71 in HC). A signifi cant correlation was found between IL-17 and RORc mRNAs (p<0.0001). Transcriptional levels of TGFβ1 were similar to HC in IgAN patients and directly correlated with RORc (p=0.0015) and IL-17 (p=0.031) mRNAs. Patients with urinary protein/creatinine >1 (median 1.62) had Foxp3 levels lower than those with protein/creatinine <1, (median 0.18), p=0.06. ROR-c levels had a trend inverse correlation with e-GFR (p=0.06). Of interest, a signifi cant inverse correlation was found between Foxp3 and TLR4 expression (p= 0.022). Conclusions: These results indicate in IgAN a functional defect in Tregs, which correlated with signs of hyperactive innate immunity. TGFβ1 seems to favour the expansion of TH17 cells. A trend correlation with proteinuria was observed.

Slit2 Impairs Neutrophil Adhesion in Ischemia Reperfusion Injury S. Chaturvedi, I. Mukovozov, S. Patel, Y. Huang, G.Y. Liu, L. Robinson. Nephrology, The Hospital for Sick Children, Toronto, ON, Canada. Objectives: Acute Kidney Injury (AKI) occurs in approximately 5% of all hospitalized patients and leads to signifi cant morbidity, mortality and fi nancial costs. Infl ammation marked by recruitment of circulating leukocytes particularly neutrophils into the injured kidney is a key component of AKI caused by ischemiareperfusion injury (IRI). Recruited neutrophils exacerbate injury by releasing infl ammatory mediators. The neuronal guidance cue, Slit2 and its transmembrane receptor roundabout (Robo) prevent axonal migration during development of central nervous system. Recently, we showed that Slit2 also inhibits neutrophil chemotaxis towards diverse chemoattractants. The objectives of this study were to determine whether Slit2 affects neutrophil adhesion in an in vitro model of IRI. Methods: Human umbilical endothelial cells (HUVECs) grown to confl uence were exposed to 1% oxygen (hypoxia), followed by variable periods of reperfusion. HUVEC were then incubated with fl uorescent labelled human neutrophil in presence or absence of Slit2. Nonadherent neutrophils were removed by washing and adherent neutrophils were counted using a fl uorescent plate reader. Results: Slit2 reduced neutrophil adhesion in a cell culture model of IRI ranging from 30 minutes to 3 hrs of re-perfusion (p<0.05). Conclusions: Our fi ndings suggest that in IRI, Slit2 inhibits neutrophil adhesion. Thus Slit2 may have a potential role in prevention and treatment of AKI.

Objectives: IL7 and BAFF serum levels are elevated in adults with SLE. These cytokines act synergistically to control survival, proliferation and differentiation of B cells. Aims of this study were to assess IL7 and BAFF serum levels in children with SLE and to compare with adults. Methods: IL-17 and BAFF were measured by ELISA in a prospective crosssectional cohort of 20 children (3 boys, mean±SD for age 14.7±1.9 years) and 52 adults with SLE, 12 children (3 boys, 14.2±3.3 years) and 38 adult controls. 13/20 children presented lupus nephritis (active in 6). Statistic Objectives: The present study was designed to estimate serum (sIL2R) levels and MDR-1 gene expression on lymphocytes in NS to elucidate relationship between there level and clinical relevance to corticosteroids therapy. Methods: We examined 40 patients with NS group A; they were 15 cases with recent onset NS and 25 known cases of NS and 20 healthy children as a control group B. We examined every patient twice 1 st with activity and 2 nd within one week of remission. Results: we found a signifi cant increase in sIL-2R level and MDR1 gene expression in patients group in comparison to control group whether in activity or remission and also they were signifi cantly higher in activity than in remission as sIL-2R was (1197.2183.3) Iu\L in group A in activity, (791192.6) Iu\L in remission and (44927.3) Iu\L in control group B and MDR1 gene was (8.70.86) % in group A in activity, (6.31.4) % in remission and (2.80 .87) % in control group and p value was <0.05 in comparing groups. Levels of sIL-2R and MDR1 gene expression in different subgroups are higher in old cases than new ones both in activity and remission and relatively higher levels in steroid resistant NS than steroid sensitive ones. Conclusions: We propose using sIL2R and /or MDR1gene expression levels as early predictors of steroid resistance in NS to promote early control of disease, and as this is the fi rst report providing new insight into use of sIL2R as a predictor of steroid resistance a wide scale study is needed to determine a cut off level of sIL2R above it cytotoxic drugs are introduced.

Acute and Chronic Renal Failure Objectives: We showed that cachexia in CKD is associated with infl ammation and maladaptive energy homeostasis (Cheung et al JCI 2005) . Vitamin D(VD) defi ciency is prevalent in CKD. We studied the effect of VDRA in a mouse model of CKD. Methods: 8-wk old c57BL/6J mice underwent 5/6 nephrectomy(N) or sham operation(S). N mice received either vehicle or paracalcitol(PC)(N-PC) (0.15 mg/ kg ip 3X per week). S mice received vehicle. N mice were fed ad lib, the other 2 groups of mice were pair-fed for 2 weeks. Results: Serum creatinine was higher in N and N-PC than in S mice(p<0.01). Serum 25-VD3 and 1,25-VD3 were lower in N mice than in S mice(p<0.01). S & N-PC mice gained more weight than N mice (p<0.01) with equal food intake. Basal metabolic rate was higher in N than S and N-PC mice(p<0.01). Effi ciency of food consumption was lower in N mice than S and N-PC mice. N mice lost both lean body mass and fat mass whereas S and N-PC mice gained lean body mass and fat mass. mRNAs of uncoupling proteins(UCP)-1 and 2, which control energy expenditure, were upregulated in both skeletal muscle and adipose tissue in N compared with S and normalized in N-PC mice. mRNAs of myogenic pathway genes, IGF-I, MyoD and PAX3, were all downregulated in the skeletal muscles in N compared with S and normalized in N-PC mice. IL-6 mRNAs in skeletal muscle and adipose tissue was upregulated in N compared with S and normalized in N-PC mice. Conclusions: VDRA ameliorated cachexia and reversed cytokine over-expression in a mouse model of CKD. VD defi ciency may be an important factor in the pathogenesis of infl ammation and cachexia in CKD.

To determine lipid profi le, carotid intima media thickness (CIMT) and brachial artery fl ow mediated dilatation (FMD) in children with CKD and compare them with controls. Methods: Cases included 80 children with CKD III-IV of >2 yr duration while 42 age and sex matched healthy children served as controls. All children underwent CIMT and brachial artery FMD determination using high resolution ultrasonography. Total cholesterol (Tc), low density lipoprotein (LDL), very low density lipoprotein (VLDL), high density lipoprotein (HDL) and triglycerides (Tg) were estimated. Results: The mean age of cases was 9.5±3.4 yr. 45% were malnourished and 87% had hypertension. 35% cases had at least one abnormal lipid parameter. The mean maximum CIMT was signifi cantly higher and brachial artery FMD was signifi cantly lower in cases than in controls. Tc, Tg, LDL, VLDL were signifi cantly higher in cases than controls. Substances identifi ed as renal toxins a priori and those associated with ≥50% incidence of AKI were compared to other substances in the database. A chisquared analysis identifi ed agents associated with AKI. Results: 5332 pediatric exposures were coded with a related renal effect. The case fatality rate was >500 times greater in pediatric exposures with related renal effects than those without. There were 2711 single-substance exposures, with AKI occurring in 26% (males 56%; females 43%). The highest incidence of AKI was in 13-19 year olds (42%). The vast majority (94%) of exposures in ≤12 year olds were unintentional while for 13-19 year olds the most common reason was intentional/suspected suicide (41%). Of single-substance medication exposures with related renal effects, the highest rates of AKI occurred with acetaminophen (84%), lithium (52%), non-steroidal anti-infl ammatories (74%), and salicylates (81%). Conclusions: Substances in the database associated with high rates of AKI among children are known nephrotoxins. This is the fi rst large database study associating AKI with acetaminophen. This data will direct future areas of research aimed at prevention and early intervention of kidney injury resulting from pediatric toxic exposures. We did not fi nd any improvement in renal function nor side effects.

Conclusions: RU appears to be safe and effi cacious in the treatment of hyperuricemia in low birth weight neonates.

Low Objectives: Report low rates of peritonitis in our center over past 5 years and discuss characteristics and outcomes. Methods: Retrospective chart review of children on PD between 2004 -2008. Episodes of peritonitis, laboratory data reviewed. Number of Peritonitis Episodes per Patient Months of PD recorded for each year. Peritonitis was diagnosed >100 WBC x 10^6/litre, >50% polymorphs ± organisms on Gram stain, supported by history and examination. Results: 23 patients included in study. All on cycler dialysis. Age 8.8 ± 5.7 years. Mean Time on PD 18 ± 13 months, range 1 -54 months. All received intravenous antibiotics at time of Double cuff Tenckhoff catheter insertion. Over 5 year period mean number of Peritonitis Episodes was 1 per 73 Patient Months of PD, range 1 per 14.3 to 1 per 95.5 months.10 episodes of peritonitis over 5 years. Mean number of peritonitis episodes per patient was 1.3 (range 1-3). Median time to onset of peritonitis post insertion of catheter was 1 month (range 2 days to 29 months).Median number of WCC was 1,050 (range 160-100,000). In 5 no organism was cultured. Staphylococcus aureus accounted for 4 ( 1 was relapse) and Staphylococcus epidermidis in 2 episode, 1 by Pantoea spp.2 Tenckhoff catheters removed because of Staphylococcus aureus peritonitis. Mean duration of treatment with intraperitoneal antibiotics 12 days (range 3-21). Vancomycin and ciprofl oxacin are antibiotics used. Conclusions: Low rate of peritonitis due to intense training of parents by dedicated trained nursing staff. No case of gram negative peritonitis and only 2 catheters lost over 5 years. Objectives: Diabetic (DM) nephropathy leads to end-stage renal failure. Its pathophisiology is unclear, but the role of the RAAS is presumptive. Previously we found elevated expression and translocation of the renal Na/K ATPase (NKA) in a DM rat model, which worsens renal function. Heat-shock protein (HSP) 72 provides a stable, membrane associated NKA. Methods: We induced DM in male Wistar rats with iv. streptozotocin (60 mg/ kg). After 5 weeks of DM we daily treated them with ACE inhibitor enalapril (40 mg/kg/day), renin inhibitor aliskiren (30 mg/kg/day) and aldosteron antagonist spironolactone (50 mg/kg/day) for 2 weeks p.o. Untreated DM, and treated non-DM animals served as controls (n=6/group). To evaluate protein levels and intrarenal localization of NKA and HSP72 we used Western blot, and immunofl uorescent staining. Results: DM rats had higher NKA and HSP72 protein expressions than the controls. Aliskiren, and spironolactone reduced the elevated NKA and HSP72 protein levels in DM rats (p<0.05 vs. DM). NKA moved from the basolateral membrane to the cytoplasm in tubular cells in DM group vs controls, which was prevented only by aliskiren treatment. Conclusions: Higher glucose levels might be the reason for altered protein expression and distribution in DM nephropathy. Various RAAS inhibitors infl uence the expression and distribution of NKA, which could explain their various clinical effi cacies in moderating DM nephropathy.

Intestinal Damage in Enterohemorrhagic Escherichia coli (EHEC) Infection Z.D. Békássy, 1 C. Calderon Toledo, 1 G. Leoj Objectives: The RIFLE classifi cation provides standardized defi nitions of acute kidney injury (AKI). The pediatric adaptation (pRIFLE) defi nes risk (R) as a decrease in estimated Schwartz-GFR by 25%, incipient (I) by 50% and failure (F) by 75%. This corresponds to a rise in creatinine (Crea) by 1.33, 2 and 4 x baseline concentration. Aim: To develop a corresponding defi nition for cystatin C (Cys), an alternative marker of GFR, using a pharmacokinetic model. Methods: Simulation of Cys and Crea concentrations during AKI using a onecompartment model with zero-order input. Baseline GFR entered as 100 ml/ min/1.73m 2 , volume of distribution as total body water (Crea) or extracellular fl uid (Cys), production rate as 20 mg/kg/day and 0.117 mg/min/1.73m 2 , respectively. Clearance taken linear with GFR. In a second analysis, extrarenal elimination of Cys (i.e. 22.7 ml/min/1.73m 2 ) taken into account. The corresponding relative rise in Cys concentrations calculated using Cys-based GFR-estimation equations by Grubb and by Filler for comparison. Results: Following an acute decrease in GFR, Cys concentrations reach a new steady state after 4 (R), 6 (I) and 13 (F) vs. 13, 19 and 39 hours (Crea). Taking only renal elimination into account, the relative rise (RR) of Cys is 1.33 (R), 2 (I) and 4 (F) x baseline concentration. If extra-renal elimination is included, RR is only 1.28, 1.79 and 2.97. The latter corresponds better with in vivo data (Grubb 1.19, 1.51, 2.28; Filler 1.29, 1.85, 3.44) .

Conclusions: Owing to the lower volume of distribution, Cys reaches a new steady state earlier than Crea. The RR in Cys is attenuated in severe AKI as a result of constant extrarenal elimination. DISCLOSURE: Bökenkamp, A. : Grant/Research Support, Siemens Healthcare Paid Lectures. sepsis/septic shock(OR=14.76, P<0.01), severe acidosis(OR=11.38, P<0.01), and white blood cells(WBC) count more than 20×10 9 /L(OR=8.51, P<0.01). Conclusions: Infectious and autoimmune diseases, kidney diseases and circulatory disturbance were the most common causes of AKI in children. The important risk factors for death in children with AKI were need for mechanical ventilation, sepsis/septic shock, severe acidosis, and WBC count more than 20×10 9 /L.

Cause 44.16% represented hypertension. Hyperphosphatemia, hyperpotassium, hypocalcemia, and anemia were found in 77.27%, 16.88%, 58.44% and 96.10% respectively. The mean level of BUN and Cr was 40.18+20.57mmol/L and 987.67+381.46µmol/L. Within patients who followed up, 50% died. The survival cases who accepted kidney transplantation was 9 in 12, and the longest living time was 4.5 years post transplantation.

Most of patients with CRF were referred to the nephrologists at the advanced stage. The high mortality and low kidney transplant rate was mostly due to the costly expense of treatment and renal source. Lack of systematic monitoring might be a major reason of missed diagnosis or diagnostic errors.

Tumor Objectives: As a cause of acute renal failure, tumor lysis syndrome (TLS), a serious complication of highly proliferating malignancy, is one of the important underlying conditions. The strategy against TLS had been hyperhydration, urine alkalinization, and allopurinol to protect the kidney. Recently, rasburicase was added to the armament against this life-threatening condition. In Korea, rasburicase is used as a rescue therapy for cases with allopurinol-resistant hyperuricemia due to the restriction by the National Health Insurance. We reviewed our experiences throughout the last decades to re-assess the risk factors of childhood TLS and the effi cacy of rasburicase in our country. Methods: Medical records were retrospectively reviewed for 396 children who were diagnosed as acute leukemia and non-Hodgkin lymphoma (NHL) at our center between 2000 and 2009. The risk factors for TLS were analyzed statistically, and those before and after the availability of rasburicase were compared. Results: Sixty eight patients (17.2%) had TLS with no mortality. Multivariate analysis showed that pre-chemotherapy hypophosphatemia was a signifi cant independent risk factor for TLS, in addition to the known risk factors of hyperuricemia and high LD concentration. The availability of rasburicase as a rescue therapy did not negate the importance of uric acid as a risk factor of TLS. Conclusions: Rasburicase as a second line treatment for intractable hyperuricemia was not effective in reducing the incidence of TLS. Since prechemotherapy hypophosphatemia was a signifi cant independent risk factor for TLS in addition to the traditional risk factors, hypophosphatemia may be a warning sign for emergency dialysis.

Hemolytic Uremic Syndrome D. Karpman, L. Sartz, A.-l. Ståhl. Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden. Objectives: Hemolytic uremic syndrome (HUS) is commonly associated with Shiga toxin (Stx)-producing Escherichia coli O157:H7. This study examined patient samples for complement activation on leukocyte-platelet complexes and microparticles as well as donor samples for Stx and lipopolysaccharide (O157LPS)-induced complement activation on platelet-leukocyte complexes and microparticles.

Methods: Whole blood and plasma analyzed by fl ow cytometry. Results: Whole blood from a child with EHEC-associated HUS showed surface-bound C3 on 27% of platelet-monocyte complexes compared to 3% of platelet-monocyte complexes from a healthy volunteer. Plasma samples from 10 patients with EHEC-induced HUS were analyzed for the presence of microparticles derived from platelets, monocytes and neutrophils. Acute phase samples exhibited high levels of platelet microparticles and, to a lesser extent, monocyte microparticles, both bearing C3 and C9. Levels decreased signifi cantly at recovery. Stx or O157LPS incubated with whole blood from healthy donors increased the population of platelet-monocyte and platelet-neutrophil complexes with surface-bound C3 and C9. Simultaneous incubation with Stx and O157LPS enhanced C3 and C9 deposition on complexes. Both Stx and O157LPS induced the release of microparticles, mostly from platelets and monocytes, which had C3 and C9 deposited on their surfaces. Conclusions: Complement deposition on leukocytes and platelets would promote their activation, and the presence of complement on platelet-leukocyte complexes, and microparticles-derived from these cells, suggests a role in the infl ammatory and thrombogenic events occurring during HUS.

Factor H Antibody of Japanese Children with Atypical Hemolytic Uremic Syndrome K. Maekawa, T. Shibano, N. Takagi, J. Sawaki, H. Mae, M. Hattori, T. Tanizawa, F. Kawashima, M. Nishimura. Department of Pediatrics, Hyogo College of Medicine, Nishinomiya, Japan. Objectives: Typical hemolytic uremic syndrome (HUS) is a self-limited disorder, but atypical disease, not associated with diarrhea, has signifi cant acute morbidity and high rates of recurrence and progression to end-stage renal disease. Multiple complement pathway mutations are thought to predispose to atypical HUS. The purpose of this study was to determine the incidence and the effi cacy of anti complement factor H (CFH) antibody related atypical HUS. Methods: We retrospectively analyzed CFH and anti CFH antibody in 13 children with atypical HUS from 2005 to 2008. Age ranged from 2-11(mean age of 5.3), 5 boys and 8 girls. They were divided two groups; anti CFH antibody positive group and anti CFH antibody negative group. We compared two groups. These character were positive group (2 boys and 1 girl) and negative group (3 boys and 7girls). Results: Three children have high titer of anti CFH antibody (positive group), case 1 has 2094.6ug/ml, case 2 has 1403.4ug/ml, case 3 has 427.6ug/ml. Negative group(other ten children) have low titer of anti CFH antibody mean 96.4ug/ml. Positive group was performed plasma pheresis or hemodialysis or peritoneal dialysis. Now they were administered immunosuprresive therapy and low anti CFH antibody and normal complement, renal function was almost normal. Familial hypocomplementaemia was not found in two groups.

We consider that analysis of anti CFH antibody is effective and necessary for atypical HUS. Atypical HUS patients who found to have autoimmune antibody may receive benefi ts from treatments with corticosteroids with or without immunosuprresive therapy, and plasma exchange.

A Objectives: In the context of a project aimed at developing pediatric nephrology in Nicaragua, data on children with CRF were collected by 7 Departmental Hospitals, covering 61% of the pediatric population, with the aim of asessing the epidemiology of CRF and using such data to improve the allocation of resources. Methods: Between 2002 Between -2008 patients (< 19 yrs old) with CRF (gfr <75 ml/ min/1,73 m²) were entered in the database. Results: Comparison of Nicaraguan vs Italian (ItalKid) data shows a lower incidence and prevalence of CRF in Nicaragua: 9.3 vs 12.1 new cases/yr/million age-related population (marp) and 42.9 vs 74.7 marp. These data are repectively due to the under-diagnosis of kidney diseases in particular of VUR (0,8 vs 25,8 % of causes of CRF) and to the higher mortality rate (39,1 vs 1,4%). The high grade of CRF at fi rst diagnosis (IV-V NKF-CKD in 56% of pts) and the high rate of unknown causes of CRF (30.8 vs 3,3%) indicate late referral. A higher percentage of neurogenic bladder (15 vs 3.7%), lupus nephritis (4,2 vs 1,1%) and glomerulopathies (19,2vs 5.8%) suggests different etiology of CRF due to geographic/genetic reasons and the need of improving primary and secondary care over the territory. Conclusions: The database played an important role to gather epidemiological information and allocate project resources over the territory for a bettere diagnosis and prevention of CRF.

Incidence of Acute Kidney Injury (AKI) in Sick, Hospitalized Children: A Prospective Observational Study P. Mehta 

Modifi ed RIFLE Criteria in Critically Ill Children with Acute Kidney Injury A. Mehta, Y.S. Sudan, U. Acharya. Pediatrics, SMS Medical College, Jaipur, India. Objectives: Modifi ed RIFLE criteria(pRIFLE)to describe the epidemiology and clinical course of Acute Kidney Injury (AKI) in critically ill children.

Methods: In this prospective study 100 critically ill children admitted in Pediatric Intensive Care Unit (PICU) either on mechanical ventilation and/or on vasopressor medications were included.CKD or pre-existing renal disorders were excluded.Prism III score was applied for severity of illness.Serum creatinine and urine output were measured for initial 3 days.Estimated creatinine clearence( eCCL)was calculated using Schwartz formula. Results: 80 patients developed AKI by pRIFLE criteria,28(35%)patients reached pRIFLEmax for risk,29(36.2%) for injury and 23(28.7%) for failure. AKI occurred early in the course of PICU stay (First day 62 cases, second day 12 and third day 6 cases).Common admitting diagnoses were encephalitis(23%) septicemia(20%)and congestive heart failure(19%).Mean PRISM III score was 11.01±8.30with a maximum of 18.35±6.93 in failure group.61.2% patients with AKI required mechanical ventilation.Mean duration of ventilation was 4.02±1.88 days.91.2%AKI patients were on vasopressors.Mean number of medications was 2.55±77. Serum creatinine showed stronger association than urine output alone.5(6.2%) AKI patients required renal replacement therapy and all belonged to failure group.Mean PICU length of stay and hospital stay in AKI group were5.92±2.64 days and 7.72±4.66 days respectively.A signifi cant mortality of 61.25% was observed in AKI patients.AKI was independent risk factor irrespective of admitting diagnosis. Conclusions: pRIFLE criteria is an important method to detect AKI for early intervention.The score is comparable to other ICU scores in predicting the outcome.

Views of Adults Who Presented in Infancy with CKD 4/5 D. Mekahli, A. Gulett, S.E. Ledermann, L Rees. Renal Unit, Gt Ormond St Hospital, London, United Kingdom. Objectives: Renal replacement therapy for infants began in 1980s.Before this, nephrologists believed treatment was unjustifi ed because of ethical dilemmas and uncertain outcome.Data on long-term outcome of these young children is emerging, but there little is known about psychosocial aspects.We have previously reported the outcome of 101 infants who presented with CKD stage 4/5 after1986 (Mekahli et al. CJASN2010; 5:10-7) .We now describe the psychosocial outcome of those who are now young adults. Methods: Forty patients now aged >16 years were sent a 71-item questionnaire:36 from the Rand-36 Health survey and 35 about environment and social life.Patient scores were compared according to gender,duration of dialysis and/or transplant, number and treatment modality changes and compared with general UK population. Results are given as median(range). Results: All 40 patients(12 females) completed the questionnaire at age 19. 2(16.3-23.4) years.Five were on dialysis and 35 were transplanted.Ages at diagnosis of CKD and start of RRT were 0.1(0.0-1.4) and 4.5(0.0-15.6) years and cumulative times on dialysis or transplant were 1.4(0.0-7.7) and 12.8(1.0-21.3) years respectively.Numbers of changes of modality were 2(1-7).Compared with the general UK population our cohort fared worse in physical and social functioning and general health.Patients who had more than one transplantation and/or period of dialysis had a worse health score(p<0.05). 28(70%) were still living at home and 16(40%) of the patients' parents were separated or divorced. Conclusions: Long term outcome of infant with CKD is continuously improving, however,we need to pay more attention to their social and emotional well-being and encourage parents to stimulate social contacts and autonomy. Objectives: The aim of the study was an assessment of the concentrations of the early markers of AKI in intensive care unit children.

In 47 children during 6 consecutive days the following urine (u) and serum (s) concentrations were determined: uNGAL and sNGAL, uIL-6 and sIL-6, uIL-8 and uIL-18. Results: The study group was divided into two AKI(+) (N=26) and AKI (-) (N=21) subgroups according to RIFLE criteria. In AKI(+) subgroup only the average results of sNGAL and uNGAL were statistically signifi cantly higher that in AKI(-) group: (sNGAL: 3,84 ± 5,5 vs 1,66 ± 2, 14 ng/ml; p<0, 00001 and uNGAL:13, 43 ± 29, 6 vs 5 ± 18, 52 ng/ml; p<0, 00001) . This difference [between AKI(+) and AKI(-) subgroup] was also noted for the average sNGAL values in the 1-st (4,31 vs 1,6 ng/ml, p=0,038), 2-nd (4,99 vs 1,72 nl/ml, p = 0,009) and 3-rd (4,29 vs 1,49 ng/ml, p = 0,011) day of screening, respectively. The mean concentrations of other cytokines did not vary, however the higher average values of sIL-6 and uIL-6 were noted in AKI (+) patients. In group of children with fatal outcome (N=11) mean level of sNGAL concentration was statistically higher than in survivors' group (N=36). sNGAL levels survivors vs non-survivors was: 2,52 ± 4,12 vs 4,20 ± 5,5 ng/ml; p<0,00001. Conclusions: In AKI (+) pediatric ICU patients the uNGAL and sNGAL levels were statistically higher than in AKI (-) group, unlike the studied interleukins. The uNGAL and sNGAL may be useful markers of AKI in ICU pediatric patients.

Non-Invasive Urinary Markers of Renal Dysfunction in Sickle Cell Disease D. Mohtat, 1 R. Thomas Objectives: Renal dysfunction is a major complication of sickle cell disease (SCD). We hypothesized that subjects with SCD will have increased urinary excretion of Transforming Growth Factor β-1 (TGFβ1) and Neutrophil Gelatinase Associated Lipocalin (NGAL) as compared to healthy controls (CTR). Methods: 51 Patients (42 with hemoglobin SS, 8 with hemoglobin SC/SD) and 21 controls (CTR) had spot urine samples collected. ELISA was used to detect urinary TGFβ1/NGAL. Exclusion criteria: subjects who received blood transfusion in the last 90 days, estimated glomerular fi ltration rate (eGFR) of <60ml/min/1.73m², or presence of any other glomerular disease. Results: Mean age, gender, and race were not statistically different between SCD and CTR. Urinary excretion of TGFβ1was 26.4 pg/mg Cr +/-1.5 in SCD vs 14.9 pg/mg Cr +/-2.4 in CTR, (p<0.00001). TGFβ1was higher in both SS and SC subjects (SS vs CTR, p=0.001, and SC vs CTR, p=0.01), but there was no statistical difference between SS and SC TGFβ1 levels. SCD patients with hemoglobin less than 9gm/dl had higher urinary TGFβ1 than SCD patients with milder anemia, (p=0.002). There was no difference in urinary NGAL between the SCD subjects vs CTR. There were no correlation between urinary TGFβ1 and levels of urinary microalbumin (p=0.15) or estimated glomerular function. Conclusions: Urinary TGFβ1, a biomarker of renal injury, may serve as a better guide than microalbuminuria to monitor progression of renal disease. This proposition needs to be tested prospectively.

Thrombotic Microangiopathy in Allogenic Bone Marrow Transplantation in Acute Leukemia Y. Motoyoshi Objectives: A ten year old boy with high risk AML received cord-blood transplantation using CY 120mg/kg + kidney uncovered TBI 12Gy as conditioning regimen on July 2008. Leukemic relapse was developed on March 2009 and he received second transplantation with allogenic unrelated HLA DRB1 mismatched bone marrow. After 2nd BMT, serum creatinine and cystatin C had elevated to 0.40 mg/dl and 2.11 mg/l, respectively, and although there was no hematuria observed, his urinary protein increased to 1g/day. Results: By the symptoms of GVHD appeared in September 2009, the dose of FK506 was increased although his serum Cre level was 2.97 mg/dl. FK506 improved GVHD paralleled with serum Cre level to 2.0 mg/dl. Interestingly, tapering of FK506 was accompanied with relapse of renal dysfunction. Kidney biopsy on November 2009 showed diffuse interstitial fi brosis and edema, tubular atrophy, remarkable glomerular mesangiolysis and features of TMA those of which seemed responsible for renal dysfunction. Immunofl uorescence study was negative for IgA, IgG, IgM, C3, C4 and C1q. Presence of TMA was confi rmed by electron microscopy. Conclusions: Clinical profi les and kidney pathology suggested that both interstitial fi brosis and TMA by radiation nephropathy as well as GVHD might be the base of kidney dysfunction of the patient. Re-elevation of serum Cre after reduction of FK506 administration is likely explained by remedy of renal circulation including attenuation of interstitial edema and improvement of TMA changes via modulation of systemic GVHD. Results: The incidence of paediatric chronic kidney disease native to Hanoi City was estimated to be 5.1 per million-child population (pmcp). Median age was 11.29 years; 60.5% were boys and 39.5% were girls; 65% of patients were in endstage renal disease. Cause of chronic kidney disease included glomerulonephritis (66.4%) and congenital/hereditary anomalies (13%). In 19.8% of children, the aetiology was unavailable. During hospitalization, 5 patients died and 76 patients (50%) refused the treatment although benefi ciary of health insurance. Thirty patients (19.74%) received peritoneal dialysis and hemodialysis, 7 patients received renal transplantation with a familial living donor. Conclusions: Late referral and limited facilities for renal replacement therapy explain the poor outcome in this study. We need a program to delineate the burden of chronic kidney disease and improve primary health care for health promotion and prevention of paediatric chronic kidney disease.

Clinical Objectives: Clinical characteristics of hospital-acquired acute kidney injury (hAKI) including 60-day outcome were determined. Methods: Clinicolaboratory data of hAKI patients were retrospectively analysed. The AKI Network Workgroup's serum creatinine (Scr) crteria were used. hAKI was staged both at onset and peak (highest recorded Scr) of AKI. Results: Annual hAKI incidence and prevalence rates were 0.17% (or 3.7 per million children population [pmcp] /year) and 0.84% (or 18.3 pmcp), respectively. Male (20): female (8) ratio was 2.5: 1. Median age was 5 (0.063-15.0) years. At hAKI onset there were 14 (50.0%), 5 (18.0%) and 9 (32.0%) patients in AKI stages 1, 2, and 3, respectively. At peak of injury there were 4 (14.3%), 7 (25.0%), and 17 (60.7%), respectively in AKI-1, -2 and -3. None progressed to permanent kidney function loss. AKI-3 was most anuric with high dialysis requirement (p=0.0329). Nephrotoxics (42.87%): frusemide, cytotoxics, captopril and lisinopril caused hAKI most. All deaths were in AKI-2 and -3. 75% of deaths were in the fi rst 28 hAKI days. Median survival time was 23.5 admission (11-52) days. Maximum Scr means for survivors (486.0 ± 382.0 µmol/L or 5.5 ± 4.3 mg/ dL) and non-survivors (353.0 ± 160.0 µmol/L or 4.0 ± 1.8 mg/dL) were similar (p>0.20). The 60-day cumulative mortality was 36.7%. Conclusions: Progression from one AKI stage to the next severe stage occurred commonly. Scr level was not a reliable mortality determinant. Maximal mortality in the fi rst 28 hAKI days and high mortality rate called for high level of clinical vigilance and informed therapeutic interventions to limit AKI progression during this period.

Acute Kidney Injury in a Pediatric Intensive Care Unit Z.B. Ozcakar, 1 A. Kavaz, 1 T. Kendirli, 2 S. Altugan, 1 M. Ekim, 1 F. Yalcinkaya. 1 1 Pediatric Nephrology, Ankara University Medical School, Ankara, Turkey; 2 Pediatric Intensive Care Unit, Ankara University Medical School, Ankara, Turkey.

Objectives: Acute kidney injury (AKI) is a common complication in the intensive care unit (ICU)s. However, clinical information about the characteristics of AKI in the pediatric population is limited. The aim of this sudy was to determine the incidence and outcome of AKI in a tertiary pediatric ICU in Turkey.

Methods: An analysis of all patients admitted to our ICU (with 7 beds) between the dates of July 2009-January 2010 was performed. Patients were classifi ed according to proposed staging system for AKI (modifi ed from RIFLE). Results: Ninety two patients (46 M; 46 F-mean age 56.25 ± 63.33 months) were enrolled. Acute kidney injury developed in 17 (18.5%) patients. Eight of them had stage I and 9 had stage III AKI. Twelve patients with AKI had underlying chronic diseases and 2 had renal diseases. Acute kidney injury developed at the fi rst week of ICU admission in all patients. Major cause of AKI was circulatory failure ± nephrotoxic medications. Peritoneal dialysis was performed in 5 patients. Overall mortality rate in the ICU was 18% during this period. Mortality rate was 41% in patients with AKI and 13% in patients without AKI. Three patients with stage I AKI and 4 patients with stage III AKI were died. 76% of the patients with AKI were mechanically ventilated whereas, 28% of the patients without AKI were mechanically ventilated. Intensive care unit length of stay did not differ between patients with and without AKI. Conclusions: Acute kidney injury developed in a signifi cant number of ICU patients. There was a close association between AKI and ICU outcome in pediatric patients.

Study of AKIN Criteria in Nonventilated Neonates in NICU S.K. Patnaik, A.K. Patra, N.K. Biswas. Pediatrics, Command Hospital (Eastern Command) , Kolkata, West Bengal, India. Objectives: To assess the applicability of AKIN criteria for Acute Kidney Injury (AKI) in neonates admitted to Neonatal Intensive Care Unit (NICU) not requiring mechanical ventilation. Methods: Prospective followup of all consecutive admissions to a level II NICU with serial serum creatinine and urine output monitoring till discharge and at 3 months of age. Neonates were classifi ed for AKI as per AKIN criteria as well as using a serum Creatinine cutoff of >1.5 mg/dl as per previously published studies. Results: 125 neonates were recruited (28-42 wks;median 36 wks) over 18 months. Comorbidities: MAS (9.6%), sepsis (35.2%), asphyxia (23.2%), RDS (8.8%), shock (2.4%), neonatal jaundice (40%) and antibiotics in 79 (63.2%) babies -aminoglycosides 34.4%; vancomycin 12%. 67(53.6%) neonates showed a difference of >=0.3 mg/dl between peak and baseline creatinine values with a median time gap of 120 hours. Peak creatinine values ranged from 0.3to 4.2 mg/dl; baseline values ranged from 0.2 to 2.3 mg/dl. Oliguria of >= 6 hours was observed in 16 (12.8%) cases; 11 in fi rst 24 hours of life; 10 had serum creatinine rise of at least 0.3 mg/dl (range 0.3-1.4 mg/dl). Serum creatinine cutoff of > 1.5 mg/dl to defi ne AKI led to a prevalence of 7/125 (5.6%). Using AKIN criteria, 10/125 (8%) cases met the serum creatinine criteria and 05 (4%) cases met urinary output criteria leading to AKI prevalence of 12% (stage I -10; II-3; III-2). Mortality was 2.4%; progression to CKD was observed in 04 cases (3.2%) ; all had oliguria and serum creatinine > 0.9 mg/dl at end of 02 weeks. Conclusions: AKIN criteria detected twice the number of cases than a cutoff of >1.5 mg/dl serum creatinine. Majority of neonatal renal failure remains nonoliguric.

Causes and Outcomes of Acute Renal Failure in 1998-2008. Data of Kaunas Medical University Hospital Clinic of Children Diseases B. Pundziene, D. Dobiliene, S. Rudaitis. Pediatric Nephrology Department, Kaunas Medical University Hospital, Kaunas, Lithuania. Objectives: To determine causes of acute renal failure in children and to compare outcomes between periods: 1998-2003 and 2004-2008. Methods: Retrospective analysis of medical records data of all children treated for acute renal failure (ARF) in Kaunas Medical University Hospital between 1998-2008 years was made. Causes of ARF, sex and age at the onset of the disease, the need for renal replacement therapy (RRT), duration and methods of RRT were evaluated. Comparison between periods (1998-2003 and 2004-2008) was made. Results: 179 children were included in the study. The age range of patients was 1 month to 18 years. 75 were treated in year 1998-2003 (I group) and 104 in 2004 -2008 . 124 (69.3%) children were treated in PICU. Basic characteristics (according to child sex and age) between the groups didn't differ. Causes of ARF between the groups did't differ (p>0.05): primary kidney disease 22 (29.3%) in the I group vs 27 (26.0%) in the II group, sepsis 11 (14.7%) vs 28 (26.9%), HUS 7 (9.3%) vs 8 (7.7%) and other causes. Multi organ failure (MOF) was diagnosed in 33 (44.0%) vs 40 (38.5%) patients (p<0.05). 55 (30.7%) childred needed RRT: 29 .3% in the I group and 30.8% in the II group. RRT procedures performed: HD 37 (67.3%), PD 13 (23.6%) and CRRT 5 (9.1%). 28 (37.3%) children with ARF died in the period of 1998-2003 and 15 (14.4%) -in 2004-2008 (p<0.05) . The most common cause of the death was sepsis with MOF: 78.8% in the I group and 37.5% in the II group (p<0.001). Objectives: Cardiovascular events are important causes of morbidity in pediatric patients with chronic kidney disease (CKD). This study aimed at determining factors present during CKD stages 2-5 that may affect cardiovascular status at the time of end-stage renal disease (ESRD). Methods: 46 pediatric patients, mean age 9.70±6.48 years at diagnosis of CKD and mean CKD duration of 3.65±3.74 years were retrospectively reviewed.

The following parameters were studied: age, sex, time-averaged systolic and diastolic blood pressure index (SBPi and DBPi), hemoglobin (Hb), serum calcium, phosphate, uric acid and intact parathyroid hormone levels. 2-D echocardiographic parameters obtained at time of ESRD included: left ventricular mass index (LVMI), ejection fraction (EF) and fractional shortening (FS). Pearson's correlation and multivariate linear regression analysis were performed. Results: 54.3% of patients had at least one echocardiographic abnormality at onset of ESRD. Severe LVMI (>51g/m 2 ) was present in 21.7%. There was signifi cant correlation between SBPi and LVMI (r=0.33, p=0.027), DBPi and both FS (r=-0.40, p=0.006) and EF (r=-0.42, p=0.004), and uric acid and LVMI (r=0.46, p=0.001). Only time-averaged uric acid (p=0.002) was a signifi cant predictor of LVMI on multivariate linear regression. Conclusions: Time-averaged serum uric acid independent of elevated blood pressures, appeared to be an important predictor of LVMI at onset of ESRD. This may be related to long-term proinfl ammatory effects on vascular cells. Control of uric acid in CKD patients may have a role in improving long-term cardiovascular outcomes.

Application of the Criteria of Acute Kidney Injury Networt and the Criteria pRIFLE in Critically Ill Children M.C. Riyuzo, C.S. Macedo, H.D. Bastos, J.R. Fioretto. Pediatrics, Faculdade de Medicina de Botucatu, UNESP-Univ Estadual Paulista, Botucatu, Sao Paulo, Brazil. Objectives: The objective was to apply the classifi cations criteria proposed by the Acute Kidney Injury Network (AKIN) and the criteria pRIFLE in ctitically ill children and to determine the clinical characteristics, laboratory features and outcomes of AKI. Methods: We retrospectively studied children with sepsis and AKI admitted to Pediatric Care Intensive Unit (PICU) (Faculdade de Medicina de Botucatu,UNESP-Univ Estadual Paulista) in a tertiary hospital. Results: Seventy seven patients (47 male; 30 female) were enrolled. Median age at the time of AKI was 4 months (range 1 to 132 months). Twenty six patients (33,8%) received invasive mechanical ventilation and 76 received vasoactive medications. Mean PICU lenght of stay was 7,33±0,16days and 66 patients (85,7%) had the diagnosis od AKI at the fi rst day of hospitalization in the PICU.Twenty patients (25,9%) presented oligo-anuria and peritoneal dialysis was perfomed in 33 (42,8%). Patients ' AKI classifi cation according to the AKIN criteria was: 11(14,3%) stage 1; 23(29,9%) stage 2 and 43 (55,8%) stage3. The patients' pRIFLE was 4(5,2%) had I (injury) and 73(94,8%) had F (failure). Mortality rate was 33,7%. Hypoalbuminemia, metabolic acidosis, thrombocitopenia, PICU length of stay, invasive mechanical ventilation, dialysis need and stage 2 and stage 3 patients' AKI were independent risk factors of mortality. Admission creatinine and urea were 7.1 +/-3.41 and 121.8 +/-47 mg/dl, respectively. Proteinuria and hematuria were universally found. Urine cultures were positive in 6, negative in 10 and not done in 7. Admission Hb was 5.8 +/-1.6 gm/dl; platelets 188000 +/-126 /mm3. Fragmented RBCS were seen in all but 2 cases. Treatment included antihypertensives in 14, steroids in 3, blood transfusion in 18, plasma transfusion in 15, plasmapheresis in 3, acute PD in 15 and HD in 8. Four recovered, 5 died and 14 ended up with CKD. Conclusions: HUS is still a major cause of morbidity and mortality in developing countries. Objectives: Background Three patients with diabetic nephropathy with nephrotic syndrome due to juvenileonset type 1 diabetes mellitus were treated with a high-dose angiotensin II receptor blocker (ARB) and erythropoietin (EPO) combination therapy, which resulted in the remission of nephrotic syndrome and long-term suppression of renal impairment.

Patients are 37∼40-year-old female who developed type 1 diabetes mellitus at the age of 12∼17. They developed nephrotic syndrome at the age of 29∼33 and received candesartan at a dose gradually increased up to 11∼16 mg/day with concomitant EPO. All 3 patients achieved remission of nephrotic syndrome after combination therapy, and the progression of renal impairment was retarded in two patients. Since subsequent reduction of candesartan dose in one patient resulted in a relapse of nephrotic syndrome, she is currently undergoing an escalated dose of candesartan again. Results: Discussion All 3 patients were at risk for early transition to end-stage renal disease. Dose escalation of ARB up to double the maximum dose for treatment of renal impairment with careful monitoring of changes in serum electrolyte and renal clearance function aimed at elimination of proteinuria resulted in remission of nephrotic syndrome and suppressed progression of renal impairment. It should be noted that this high-dose ARB therapy has the potential to suppress progression of Stage IV diabetic nephropathy.

The high-dose ARB/EPO combination therapy has the potential to serve as an effective treatment strategy for chronic kidney disease (CKD). Objectives: To gain understanding on the underlying mechanism of both the abnormal catch-up growth described in young uremic rats (Kidney Int 2006; 70,1955-61) and the normalizing effects of GH treatment on this process, the growth plate expression of factors potentially involved in the endochondral growth regulation was analyzed during a catch-up growth period. Methods: IGF-1, IGFBP5, GH receptor, BMP2, TGFβ, PTHrP, caspase 3, VEGF, chondromodulin 1 and collagen X expressions were explored by immunohistochemistry, in situ hybridization and western blot in proximal tibiae of uremic rats either untreated or GH treated and control animals, also treated or untreated, fed ad libitum or pair-fed with the former ones. Animals were sacrifi ced when the uremic rats exhibited catch-up growth after food restriction (Kidney Int 2006; 70,1955-61) . Results: Consistent quantitative differences were not found in any of the analyzed peptides, although protein expressions of BMP2 and VEGF showed a tendency towards repression by GH treatment. The immunohistochemical distribution pattern of IGF-I, IGFBP5, TGFβ, PTHrP and caspase 3 was patched in the widened hypertrophic zones of uremic rats with GH instead of being uniform as in the other groups.

The catch-up phenomenon in uremia is not accompanied by relevant changes in the growth plate expression of the analyzed factors. Normalization of catch-up growth induced by GH treatment is not associated with consistent quantitative differences on the analyzed peptides. The pathophysiological meaning of these fi ndings deserves further investigation.

Conclusions: Nadir serum creatinine is the main risk factor affecting renal outcome in patients with RHD. A value of nCr > 1.0 mg/dl at three years of age predicts a high probability of progression to ESRD before the age of 16 years in patients with RHD.

Acute Kidney Injury Following Cardiopulmonary Bypass for Congenital Heart Disease S.K. Sethi, 1 D. Yadav, 1 D. Goyal Objectives: There is paucity of data on incidence, epidemiology and risk factors for the development of acute kidney injury (AKI) in children post cardiopulmonary bypass for cardiac surgery. The objective was to investigate the incidence, implicating factors and outcome of AKI (AKIN criteria) after cardiopulmonary bypass. Methods: Design: Retrospective review study. Patients: One hundred and twenty fi ve children (less than 18 years of age) admitted to the cardiothoracic intensive care unit following cardiopulmonary bypass between January 2007 and December 2009. Methods: Age, sex, diagnosis, Jenkins score, duration of cardiopulmonary bypass, ischemia time, Baseline and post surgery: biochemical investigations, urea (mg/dl), creatinine (mg/dl), urine output (ml/kg/hr), frusemide dose, inotrope requirement, and systemic dysfunctions (cardiac, renal, hepatic and neurological) were recorded. Results: Results: Seven (5%) children developed AKI I, 5 (4%) developed AKI II and 2 developed AKI stage III (2%). The patients with AKI stage II and III had a longer ICU stay and increased mortality. Two children required dialysis and none developed chronic renal impairment. All patients with AKI III died during the ICU stay. Using stepwise regression, younger age (< 1 year), low cardiac output, sepsis and duration of cardiopulmonary bypass were the signifi cant risk factors identifi ed for developing AKI. Conclusions: Conclusions: AKI is common and occurred in 11% of children following cardiac surgery, but AKI requiring renal replacement therapy is uncommon.

Chronic Objectives: Many uncontrolled studies and a meta-analysis based on these studies led to the opinion that diarrhea positive (D+) hemolytic uremic syndrome (HUS) is associated with a signifi cant risk for chronic renal sequelae. Two recent controlled studies that followed children with D+ HUS after an E coli O157:H7 outbreak, with controls selected from the population exposed in the outbreak, have confl icting results. To clarify this apparent difference in renal outcome, we evaluated renal sequelae after sporadic D+HUS with unexposed case matched controls. Methods: Thirty children after sporadic D+ HUS were compared in long-term follow-up with 30 children unexposed to E coli O157:H7 that were age and gender matched. Both groups had measurements of albuminuria, blood pressure (BP) and estimated GFR (Cystatin C). For children, BP measurements were converted to z scores and percentiles. Results: In the follow-up (median 6.2 years, range 3.3-20.1), 40% of the HUS patients showed albuminuria, and one-third of albuminuric subjects had macroalbuminuria. In contrast, microalbuminuria was present in only 3% of the matched controls, and none had macroalbuminuria. The proportion of children with hypertension and prehypertension was three times higher and eGFR was 30 ml/min/1.73 m 2 lower in the HUS group versus the controls. Conclusions: Unlike the interpretation in two controlled studies on D+HUS with controls exposed in the outbreaks, children with sporadic D+ HUS compared to unexposed controls had a higher prevalence of chronic renal sequelae.Future studies with unexposed controls can expand on this issue.

Optic Objectives: We report a case of 3-year-old patient with optic neuritis after H1N1 Infl uenza vaccination. Methods: A 3-year-old boy with chronic renal failure secondary to bilateral hypoplastic kidneys became ill tempered. He had been on peritoneal dialysis (PD) since 10-month-old of age. Two days later, his parents noticed his visual impairment and he was hospitalized the next day. It was found he had received a vaccination of H1N1 fl u ten days before the admission. Results: On examination, his general condition was good; however, his pupil was dilated and didn't respond to light stimulation in both eye. On fundus examination bilateral papilledema was observed. He had no other abnormal neurological fi ndings including ocular movement. A brain magnetic resonance imaging and an electroencephalogram were normal. We made a diagnosis of optic neuritis and employed methylprednisolone pulse (MP) therapy three times and supplementation of oral vitamins with continuing PD. However, he was left with severe visual impairment one month later. He had transient hyponatremia during the MP therapy. Conclusions: Reports of optic neuritis after vaccination are infrequently found; however, the relation between optic neuritis and chronic renal failure has not been pointed out specifi cally. We will report because of an unusual and serious presentation.

Characterization of an Experimental Model of Progressive Renal Disease in Rats N.V. Baracho Objectives: This study aimed to characterize functional changes produced by progressive nephrectomy in rats. Methods: Male Wistar rats were anesthetized and submitted to progressive degrees of nephrectomy. According to surgical procedure, animals were allocated into experimental groups and placed in individual metabolic cages for two weeks: control(sham operated animals, n=10), unilateral nephrectomy(n=10), 3/4 nephrectomy(n=10) and 5/6 nephrectomy(n=10). Daily measurements of urinary output, water and food intake were performed. At the end of experiment, blood and 24-hour urine samples were collected to determine serum and urinary concentrations of creatinine, urea, Na, K and microalbuminuria. Results: Unilateral nephrectomy did not change water intake and urinary output, but increased serum creatinine and urea and reduced creatinine clearance(p<0.05). 3/4 nephrectomy also did not change water and food intake. However, this procedure increased urinary output, microalbuminuria and serum levels of urea, creatinine, Na and K(p< 0.05). 5/6 nephrectomy did not change food intake, but increased water intake and urinary output and produced the highest serum levels of urea, creatinine, Na, K and microalbuminuria and the lowest creatinine clearance(p< 0.05). Conclusions: Progressive degrees of nephrectomy seem to be a helpful model for evaluating chronic renal disease.

The Objectives: This study aimed to estimate the prevalence of mental disturbances and to analyze the quality of life (QL) in a pediatric population with chronic kidney disease (CKD). Methods: This is a transversal study with 136 pediatric CKD patients regularly assisted in our Pediatric Nephrology Center under various types of treatment: conservative (n=75), dialysis (n=38) and transplant (n=23). The Strengths and Diffi culties Questionnaire (SDQ) and PedsQL were applied to both patients and caregivers, in their specifi c versions. An instrument was also standardized to analyze sociodemographic and economic characteristics of the studied population. Clinical control data were collected from medical charts. Results: There was a high prevalence of mental disturbances (60.2%) and impairment of quality of life in all aspects, with lower scores in the physical and educational aspects. There were no statistically signifi cant alterations between therapies. Better QL was associated with: Catholic religion, non-Caucasian race and age under 10 years. Emotional disturbances predominate among Caucasians over the age of 10, and among those in the initial stages of CKD.

The present study stresses the various emotional and social repercussions of CKD and its treatment. In the follow up, urinary and serum NGAL did not signifi cantly correlate with other markers. Conclusions: We conclude that urinary and serum NGAL are highly spesifi c and sensitive predictors of ARF in critially ill children. They are found to be useful markers for detecting ARF in the early period, however, they are not in the follow up.

Effects Objectives: MELAS is one of a group of clinically syndromes defi ned mitochondrial dysfunction. Common kidney involvements in MELAS are chronic clinical patterns including Fanconi syndrome, renal tubular acidosis and FSGS, but a few reports with acute renal failure including ATN. Here we report a case who had diagnosed with MELAS developed irreversible acute renal failure. Rapidly evolving from normal renal function to ESRF meant relationship with mitochondrial dysfunction, histo-and ultrastructural analysis was performed. Methods: We performed renal biopsy and analyzed histo-and ultrastructural pathology of the renal tissue, especially mitochondria in glomeruli and renal tubules.

Histological fi ndings showed features of ATN, extensive tubular dilation, degeneration of tubular structure, necrosis. Severe interstitial fi brosis and infl ammatory cell infi ltration were observed. In some glomeruli sclerotic changes were observed. Ultrastructural changes in proximal renal tubules included proliferation of enlarged mitochondria with irregular abnormal cristae were observed. Most of mitochondria in glomeruli and distal renal tubules are intact. Conclusions: Histological analysis suggested that the acute renal failure may have been secondary to acute renal ischemic event, but features of chronic renal injury, FSGS and interstitial fi brosis may have been caused by continuous mitochondrial dysfunction. Most of ATN cases with normal renal function prior to ischemic event recover suffi cient renal function, but our case rapidly developed to ESRF. This irreversible acute renal failure is thought to be related to chronic renal injury and regional mitochondrial abnormalities in proximal tubules. 

There was a trend toward higher levels of homocysteine with less risk,95%CI= 0.7 to 1.02,OR= 0.8, P=0.08). Higher factor VIII associated with greater risk,OR=1,95%,CI= 0.99 to1.02,P = 0.15

There is a linear relation between factor VIII level and risk of thrombosis. Prophylactic anticoagulants should be considered in pts with high factor VIII.

Study Objectives: of this work was to demonstrate the effect of rHu EPO therapy on LVH and LV systolic function in patients with end stage kidney disease.

Methods: Thirty two patients were enrolled in this study, 14 females and 18 males. Their age ranged from 5 to 17 years along with 15 age and sex matched healthy subjects as controls. The inclusion criteria were; the presence of renal anemia, adequate serum iron status with serum ferritin level of 100 ng/ml or more and a transferrin saturation of >20%, normotension or controlled hypertension and no history of valid heart disease or other systemic illness. We analyzed the laboratory and echocardiographic data before starting EPO treatment and after treatment in period of follow up ranged between 4 and 9 months with a mean of 5.8+/-1.5 months. Results: Hb level increased from 8.5+/-1.87 to 9.3+/-1.7 gm/dl, Hct level increased from 25.78+/-6.59% to 28.88+/-5.5%, LVMI showd reduction from 108.8+/-41.97 to 97.13+/-43.9 g/m 2 , SV decreased from 59.58+/-21.17 to 53.9+/-18.49 ml and fi nally CO decreased from 5.74+/-2.2 to 5+/-1.5 L/minute. No signifi cant change was detected regarding the HR, EDV, &ESV. LV systolic function was normal at the start of the work and remained so in the follow up examination.

We concluded that in patients with ESRD on chronic hemodialysis, LVH regression can be obtained after partial correction of anemia with rHu EPO which can be also associated with reduction of the high CO encountered in these cases. Weather this regression would improve outcome in haemodialysis patients remain to be established.

Acute Objectives: CKD triggers acceleration of atherosclerosis, pictured by cardiovascular complications seen already in childhood. MMP/TIMP system, by the impact on matrix accumulation and endothelial destruction, may play a key role in atherogenesis. However, the data on its role in children with CKD is lacking. The aim of our study was to evaluate serum concentrations of MMP-9, MMP-2, TIMP-1 and TIMP-2, as well as their correlations with known markers of endothelial dysfunction (sE-selectin) and infl ammation hsCRP) , in CKD children treated conservatively. Methods: 37 CKD children were divided into two groups (gr.I -20 patients with CKD stage II-III, gr.II -18 children with CKD stage IV-V). 24 healthy subjects served as controls. Serum concentrations of MMP-9, MMP-2, TIMP-1, TIMP-2, sE-selectin and IL-4 were assessed by ELISA. Kidney function and serum hsCRP were also evaluated. Results: Median values of MMP-9, MMP-2, TIMP-1 and TIMP-2 were signifi cantly higher in CKD children than in controls. Moreover, the concentrations of all parameters were increased in patients with CKD stage IV-V when compared to the group with CKD stage II-III. All parameters correlated with: GFR, sE-selectin and IL-4 in serum.

Conclusions: Chronic kidney disease in children is characterized by MMP/ TIMP system dysfunction, aggravated by the disease progression. The presence of correlations between examined parameters and markers of endothelial damage and infl ammation suggests the possibility of their application as novel markers of vascular changes characteristic for atherogenesis in children with CKD on conservative treatment. Objectives: The aim of our study was to investigate whether urine Endothelin-1 (uET-1) could represent a novel biomarker of renal scarring and to determine the optimal cut-off level for uET-1 for prediction of renal scar. Methods: Fourty four patients with renal scar and 44 patients without renal scar on dimercaptosuccinic acid (DMSA) scan were enrolled in the study. Serum urea and creatinine levels were normal in all children in the study. Urine ET-1 was measured by ELISA. Results: Mean uET-1 level was signifi cantly higher in children with scar than in without scar (2.65 fmol/ml vs 0.50 fmol/ml, p=0.001). According to receiver operating curve (ROC) analysis, the optimal cut-off level was 1.1 fmol/ml for uET1 to predict renal scar. Using a cut-off 1.1 fmol/ml for uET-1, sensitivity and specifi city were 93.18% and 93.18% respectively. Area under curve (AUC) was found 0.975 (%95 CI 0.945-1.005) for uET-1. Mean uET-1/creatinine (uET-1/cr) level was also signifi cantly higher in children with scar than in without scar (3.36 fmol/mg vs 1.03 fmol/mg, p=0.001).

According to ROC analysis, the optimal cut-off level was 2 fmol/mg for uET1/ cr to predict renal scar. Using a cut-off 2 fmol/mg for uET1/cr, sensitivity and specifi city were 86.36% and 90.91% respectively. Area under curve was found 0.946 (%95 CI 0.901-0.990) for uET-1/cr. Conclusions: Urine ET-1 and urine ET-1/creatinine can be used as a sensitive marker for prediction of renal scar in patients with normal renal function. 1-15 year). The most common urinary symptoms were dysuria (15%), urinary frequency (12.5%), and dribbling (4.1%). The frequency of nocturnal enuresis and daytime enuresis were 22.5% and 3.3%, respectively. Pyurias were seen in 11 (9.2%) patients and urine cultures were positive in 7 (5.8%) cases, that all of them were female. E.coli was the most common organism (71.4%) followed by S.aureus and Enterobacter each in 14.3%. All organisms were sensitive to ciprofl oxacin. Conclusions: Nocturnal enuresis was found in a signifi cant number of children who had chronic constipation but UTI is not more prevalent than the general population. Therefore, we suggest that nocturnal enuresis should be questioned in children with chronic functional constipation, but screening for UTI is not mandatory in these patients.

The Of all 20, 12 caes were on prophylaxis at the onset of recurrent UTIs. Of all, 3 patients had neither VUR nor lower urinary tract abnormalities. Two of them were under one-year-old boys while the rest was seven-year-old girl whose toilet training was already completed. One male infant was on prophylaxis, on the other hand another infant and seven-year-old girl were not. Conclusions: Recurrent UTIs can occur not only in children with some urinary tract abnormalities but also in those without any urological abnormalities. Therefore, once a child experiences a UTI, a close attention should be paid to them until a certain age not to overlook another UTI episode.

The Objectives: Despite its drawbacks, bag remains a widely used device to collect urine in non toilet trained children suspected of urinary tract infection (UTI), mostly because of practical matters and concern about the level of induced pain. We conducted a prospective study in order to compare, from this point of view, bag and catheter urine collection. Methods: In our centers, every positive dipstick performed on bag obtained urine samples are confi rmed with an urethral catheterization before the child is treated. Therefore we conducted a prospective study in two phases to compare these two methods in the same children. The fi rst part aimed to compare the duration of the procedure between the application of the two devices and the collection of urines. The second part aimed to compare, with a FLACC scale, the pain level induced by each of the two devices. Results: 56 children were involved in the fi rst part of the study. Mean (± SD) duration of the procedure was 35.7 ± 28. Objectives: We studied the frequency of bacterial meningitis in infants with primary diagnosis of febrile urinary tract infection.

The study comprised neonates and infants, age 0-60 days, admitted to the pediatric department with febrile UTI from 1999 to 2008. Inclusion in this study was confi rmed by a diagnosis of UTI based on positive urine culture. All these patients also underwent a lumbar puncture for cerebrospinal fl uid examination as a part of fever workout guidline. The study was based on the computerized medical record. Results: During 1999 During -2008 neonates and infants 0-60 days of age have been hospitalized in pediatric department with the diagnosis of febrile UTI. There was no case of bacterial meningitis in all patients included in our study.

The study has shown that the frequency of bacterial meningitis in young infants with febrile UTI is almost zero. This fact raises a question about expendiency of lumbar pubcture and possibility of revising a fever workup guidlines in this group of patients. The most common causative organism was E.Coli in both groups. 5) On the sonographic fi ndings, the most lesions were seen on the upper lobe of the kidney, more frequent on the left side. The lesions of 29 cases showed globular or wedge shaped increased echogenecity compared with the adjacent normal renal cortex. 6) 99mTc-DMSA scan could detect earlier lesion of AFBN which was not seen on ultrasonography at initial diagnosis. It showed the complete coincidence of the location, size and shape in all cases compared to the fi ndings of renal sonography. 6) 14 of 18 cases who had radiologic follow up showed improvement by antibiotics therapy alone. Conclusions: Ultrasonography is excellent as an initial detection tool in diagnosing AFBN. Since the degree of infection in AFBN is more severe than other UTIs and evolution into a renal abscess is possible, early diagnosis and appropriate antibiotics therapy is essential.

Features of Lysosome Enzymuria in Children with Pyelonephritis Depending on Age I. Bagdasarova, L. Korol, L. Migal, O. Lavrenchuk. SI "Institute of Nephrology AMS of Ukraine", Kyiv, Ukraine; SI "Institute of Nephrology AMS of Ukraine", Kyiv, Ukraine.

Objectives: The purpose of workis to study the age features of changes in activity of enzymes lysosomal origins: Ν-acetylβ-D-glukosaminidase (NAG), its isoenzyme is NAG B and galaktosidase (Gal) in children with PN. Methods: 186 children at the age from 2 till 16 years (mainly girls) with an active stage of infl ammatory process in kidneys and without infringement of a functional condition of kidneys which were derided in groups depending on the activity degree of PN (1-3), forms PN (obstructed -OPN and nonobstructed -NPN), character of a disease course (acute, chronic) and age features of patients (till 6 years-82, after 6 years-104 patients) are surveyed. Control group -was practically healthy 30 children.

Results: It was shown, that in all children with 3-rd degree of activity of infl ammatory process in kidneys and practically in all children with the 2-nd degree, in patients with acute PN, and chronic OPN and NPN activity levels NAG, NAG B and Gal in children till 6 years statistically authentically exceeded similar indicators in children in above 6 years old. We noticed, that the maximum indicators of activity of lysosomal enzymes of urine among children are elderly till 6 years, and also among children above 6 years are registered in patients with chronic OPN with 2-nd and 3-rd degree of activity of infl ammatory process in kidneys in comparison with patients with acute and chronic NPN. Conclusions: So, we registered the dependence of changes in levels of activity of lysosomal enzymuria on the degree of activity of infl ammatory process in kidneys and on the age of PNH children. Objectives: Probiotics, benefi cial living microorganisms, were deemed to be an alternative to antibiotics for prophylaxis but the preventive effect against UTI is inconclusive. To compare the effect of probiotic prophylaxis with antibiotic prophylaxis, a prospective randomized controlled study was done in infants with primary VUR. Methods: Methods: 132 infants (age 5.2+4.5 months), who were diagnosed to have primary VUR after their fi rst UTI, were randomly allocated into a probiotic (Antibio300 R Lactobacillus acidophilus 10 8 CFU/g 1g twice daily, n=64) or an antibiotic (Septrin R trimethoprim/sulfi methoxazole (TMP-SMX) 2/10 mg/kg once at night, n=64) prophylaxis groups and were followed for one year. Four infants were dropped out and 128 infants were analyzed. Methods: This is a cross-sectional study, including 739 school children aged 6 to 12 years from 3 public schools, being 239 from school 1 (socioeconomic class C), located in Belo Horizonte, and two social class D schools; school 2 with 267 students and school 3 located in the metropolitan area of high social risk, with 233 children. We carried out an interview in the school environment, with questions about the voiding during the night. NE was considered when the child had at least 1 episode of bedwetting per month. Children with NE received an educational booklet on the function of the lower urinary tract and also a referral for medical evaluation. Results: NE was detected in 110 (14.8%) children and classifi ed as monosymptomatic enuresis (MNE) in 63 (57.2%), being more prevalent in boys; nonmonosymptomatic enuresis (NMEN) were detected in 47 (42.7%) children, with higher prevalence in girls (34). Both were more common in younger children and in schools with more socially disadvantaged.

Although NE is a condition associated with multiple causes, has several consequences for the psychosocial and behavioral development of children. The individual interviews at the school helped select the children with NE. Early detection can prevent complications related to lower urinary tract, and improve the emotional aspects of children.

Family 

Conclusions: The results suggest that modern scientifi c studies have the potential of discovering new antimicrobial compounds in the above-mentioned plants, which can be effective as remedy for microorganisms causing UTIs.

Outpatient Pelvic Floor Therapy (Biofeedback) in Girls with Repeated Urinary Tract Infection and Dysfunctional Voiding C.C. Mourani, B. Gerbaka, S. Merhej, N. Nehme. Pediatrics, Hotel Dieu de France, Beirut, Lebanon; Pediatrics, Hotel Dieu de France, Beirut, Lebanon; Urology, Hotel Dieu de France, Beirut, Lebanon. Objectives: We compare in a prospective study the treatment of girls with repeated UTI and dysfunctional voiding. First group (A) was treated by an outpatient pelvic fl oor therapy program. Second group (B) was treated by conventional treatment : associating anticholinergic drugs and preventive antibiotherapy. Methods: We analyzed the fi les of 62 girls (5 to 12 years) with at least one episode of UTI and high suspicion of overactive bladder (OBA Patients were already on antibiotic prophylaxis before 46% of the UTI episodes. Ultrasound, DMSA scan and VSUG were found abnormal in 39%, 42% and 32% of the patients, respectively. Overall, 64% of the patients had an underlying predisposing factor for UTI (voiding dysfunction in 38%, anomaly in 32%). When we divided the patients according to the age of the fi rst UTI episode due to ESBL producing bacteria; 34 (19 boys) were <12 (Group 1), 23 (7 boys) were in between 12-60 (Group 2) and 37 (2 boys) were >60 months of age (Group 3). Acute pyelonephritis occured in 68%, 52% and 27% of groups 1,2 and 3 respectively. 78% of the patients in group 3 had voiding dysfunction. Conclusions: Clinical pattern of UTIs due to ESBL producing bacteria does not seem to be different from the UTIs due to non-ESBL producing bacteria.

Nonkeratinizing Objectives: Acute infl ammation of renal tissue is considered to be one of the most common diseases in childhood, that leads to fi brosis. Recent studies show the important role of extracellular Zn-containing proteases in renal damage. In order to reveal the possible role of MMP-2,-9 and TIMP-1 in renal scarring formation 25 children (aged 1-12 years) with PN were examined. 8 of them with acute presentation of PN were examined twice (at onset and after 10-14 days of antibacterial treatment). Seven of 25 patients had a remission of PN. Methods: ELISA-measured MMP-2, MMP-9, TIMP-1 in urine standardized to urinary creatinine (uCr) concentrations were evaluated. Results: All children with acute PN demonstrated signifi cant increase of MMP-2, -9 and TIMP-1 in the urine. After treatment MMP-2 level decreased in 2 times, MMP-9 level-in 10 times. Only TIMP-1 was still elevated (p<0,05). Out of 7 children with remission of PN, four had higher urinary level of MMP-2, -9 and TIMP-1 than others (p<0,001), close to patients with acute infl ammation. Theremore TIMP-1 urinary level was higher than MMP-9. These children presented with severe onset and had VUR. Conclusions: High level of TIMP-1 in children with acute PN after treatment may be a result of progressing macrophages infi ltration of renal tissue, leading to fi brosis. Signifi cant disturbances in extracellular proteases system in patients with abnormal urodynamics may be considered as a risk factor of developing refl ux nephropathy.

Abstract# 304 Ultrasound and DMSA scintigraphy were performed as acute investigations and cystourethrography within 2 months. Important structural abnormality of the urinary tract detected both in as outside of the study cohort were recorded. Results: Ultrasound showed dilatation in 15% and increased kidney length in 28% of the patients. The sensitivity to detect scintigraphic abnormality was 48%.

Renal length was signifi cantly correlated to infl ammatory parameters including scintigraphic abnormality. Important structural abnormality was detected in 40 infants of whom ultrasound identifi ed 30, while 10 of 27 cases with dilating refl ux were missed, mostly grade III. Outside of the study there were a further 28 infants with structural abnormality of whom 15 were antenatally detected. Conclusions: Ultrasound detected most infants with structural abnormality with the exception of refl ux grade III. Being noninvasive, ultrasound has a place in the work-up of infants with urinary tract infection, especially when antenatal ultrasound during late pregnancy is lacking. Kidney length in infants with acute infection correlated with infl ammatory parameters and this fi nding needs to be studied further.

Prevalence Objectives: Investigate whether s-CRP and s-IL6 predict the risk of renal scarring after urinary tract infection in children (UTI).

We present an study carried out with 32 children (9 male) (range: 0.6-153 months); diagnosed with UTI. s-CRP and s-IL6 were measured at the time of UTI diagnosis and the patients forming renal scarring during follow up were identifi ed by means of DMSA renal scan.

The effectiveness of the two parameters for diagnosis of renal scarring was evaluated using the indices of diagnostic quality: sensitivity (S) and specifi city (Sp). Results: Eight children (25%) (range: 2-144 months) showed renal scarring after the follow up and 24 children (range: 0.6-153 months;) did not present post UTI damage. s-CRP was 110.23±59.69 and 52.46 ±63.13 mg/L respectively, in patients with, and without renal scarring. s-IL6 was 18.34±11.80 and 8.07 ± 9.51 pg/ml respectively, in patients with, and without renal scarring. The value of greatest S for CRP was >5mg/L (S:100%) and greatest Sp was >150mg/L (Sp: 95.83). The value of greatest S for s-IL6 was >4pg/ml (S: 100%) and maximum Sp was > 40pg/ml (Sp: 100%). The cut off points for optimum diagnostic result in diagnosing renal scarring were 115mg/L for s-CRP and 20pg/ml for s-IL6.

The results confi rm that children who will form renal scarring show higher levels of s-IL6 and s-CRP at the time of diagnosis of UTI and that very high values for these two parameters almost completely guarantee future formation of renal scarring. However, neither of the techniques provides suffi cient information for predicting renal damage in all patients. Objectives: The aim of this study was to evaluate the diagnostic accuracy of renal scintigraphy (CRE) and renal ultrasound scanning (US) in identifying high grade vesicoureteral refl ux (VUR) in children after the fi rst episode of urinary tract infection.

A total of 553 children after fi rst urinary tract infection which were assessed by three diagnostic imaging studies (renal US, DMSA scanning and voiding cystourethrography) were included in the analysis. The main event of interest was the occurrence of high grade VUR (III-V). Diagnostic odds ratio (DOR), sensitivity, specifi city, positive predictive value (PPV), negative predictive value (NPV), and likelihood ratio of renal scintigraphy and renal ultrasound scanning were determined for both methods separately and also in combination.

Results: From January 1985 until December 2007, 533 patients were included in the study. VUR was diagnosed in 246 (46.2%), 144 of them grade III -V. DOR values were lower for low grade VUR (I-II): 1.91 and 2.2, respectively by using the "OR rule" and "AND rule". On the other hand, higher DOR values were obtained in high grade VUR evaluation: 10.9 for DMSA (95% CI, 6.7 -17.7) and 7.9 for US (95% CI, 4.9 -12.78). The combination of US and DMSA features signifi cantly increased DOR to 25.3 and 16.9, respectively by using the "OR rule" and "AND rule".

Our fi ndings support that the combined use of US and DMSA scans are reliable predictors of clinical signifi cant VUR.

The Tc-Dimercaptosuccinic acid renal scintigraphy (DMSA) was performed within 3 days after admission and if abnormal, then follow-up DMSA was performed after 6-8 months. Cystourethrography was performed 1 month after UTI. Results: A total of 70 children were enrolled in the study. The main pathogen was E.coli. VUR was found in 21.5% of the children. 75% of the children had fi ndings of acute pyelonephritis (APN) in DMSA and there was a complete recovery in 68% of them. Scars were observed more frequently in older children, with VUR grade>III and not taking chemoprophylaxis. Conclusions: 1) fi rst episode of APN in children occurs in more than 50% during the fi rst year of life. Boys are mostly affected, 2) the 'classic' clinical and laboratory fi ndings of APN can serve as criteria for diagnosis of UTI level, 3) VUR does not appear to be associated with fi rst episode of APN, 4) children>1 year of age had a higher risk of renal scarring, 5) chemoprophylaxis may serve as a protective agent to renal scars formation.

were performed to exclude recurrent infections before renal scintigraphy. All patients underwent DMSA scintigraphy at least 6 months later to assess any renal scarring. Results: A total of 545 children (80 with nephromegaly and 465 without nephromegaly) were enrolled. UTI patients with nephromegaly have a more severe disease than patients without nephromegaly in terms of higher infl ammatory indices and C-reactive protein and longer fever durations before treatment and after treatment. The incidence of renal scarring is much higher in UTI patients with nephromegaly. When independent predictor variables and their interaction were evaluated to determine their infl uence on scarring, only nephromegaly was signifi cantly associated with a higher incidence of renal scarring.

Our results indicate that ultrasonographic nephromegaly at onset is associated with a very high incidence of renal scarring, and can be a way to pick out these UTI patients at increased risk of renal scarring. And we believe that renal ultrasonography should be done routinely in children with a fi rst febrile UTI. Strains found in patients with high grade refl ux are similar to those with cystitis, and often belong to phylogenetic group A, which is less virulent (23.6% vs 2.0% in patients with high grade VUR; p<0.001). Serum PCT is lower in patients with high grade (median=0.3 ng/ml) than low grade VUR (3.0 ng/ml) and without urinary tract anomalies (1.2 ng/ml). Absence of Pap GII is associated with major urological anomalies in 53% and has a negative predictive value of 94%. Conclusions: Analysis of E. coli virulence genes by multiplex PCR together with PCT serum levels may help to predict high grade refl ux in children with UTI.

The Postnatal Follow-Up of Infants with Antenatally Detected Hydronephrosis: The Importance of Early Circumcision O. Yavascan, N. Aksu, O. Turan, A. Bal, F. Kamit, P. Kuyum, C.N. Arslan, M. Anil, A.B. Anil. Pediatric Nephrology, Izmir Tepecik Teaching and Research Hospital, Izmir, Turkey. Objectives: The objective of this study was to determine the effect of circumcision on the frequencies UTIs and renal parenchymal damage as well as growth and nutrition of infants with antenatal hydronephrosis. Methods: Infants with a fetal pelvis diameter of ≥5mm identifi ed with antenatal US were followed-up. All patients were evaluated in terms of UTI frequency, pelvic diameter, scarring on DMSA and differential function on DTPA. Growth(heightSDS) and nutrition(relative weight) parameters were recorded as well. These parameters were assessed after circumcision. Statistical evaluation was made by using khi-square test. Results: The study included 178 patients. Of these, 29 were diagnosed by VUR, 87 by obstructive uropathy and 54 by non-obstructive uropathy. Of 133 males, 111 infants were circumcised. The pre-circumcision UTI frequency[(2.97±1.14/ year(y)] was signifi cantly higher than post-circumcision period(0.25±0.67/y). Also, pre-circumcision UTI frequency(2.97±1.14/y) was signifi cantly higher than the frequencies observed in female cases(0.85±0.91/y), in males who did not undergo circumcision(0.91±0.99/y) and in overall study group (0.73±0.79/y). In obstructive uropathy patients, the rate of renal damage was increased in uncircumcised patients(28.5%vs35.7%) whereas no change was observed in circumcised patients(11.2%vs12.5%). Overall, growth and nutrition parameters gradually improved after circumcision. Conclusions: The close follow-up and early circumcision of infants with AH will prevent UTIs and renal parenchymal damage, enabling normal growth and nutrition.

The Fibronectin, beta-2 Microglobulin, and High Sensitive CRP Levels in Urinary Tract Infections with Renal Damage and VUR H. Poyraz, N. Cetin, B. Yildiz, N. Kural. Pediatric Nephrology, Eskisehir Osmangazi University, Faculty of Medicine, Eskisehir, Turkey. Objectives: The aim of this study was to assess the urinary and serum fi bronectin and beta-2 microglobulin (B2MG) and high sensitive CRP (hsCRP) levels in urinary tract infections (UTI) with renal damage (RD) and vesico-ureteral refl ux (VUR). Methods: The patients were divided into fi ve groups: group I, 20 patients with fi rst UTI and VUR (-); group II, 20 patients with recurrent UTI and VUR (-); group III, 16 patients with recurrent UTI and VUR (+); group IV, 16 patients without UTI and VUR (+) and group V, 16 healthy children as the control group. RD was detected with DMSA in 17 patients. Results: The serum and urinary fi bronectin levels were similar in all study groups (p>0.05). The fi bronectin levels were not associated with presence of RD (p>0.05). B2MG levels were higher in patients with fi rst UTI than in those recurrent UTI. B2MG levels were higher in UTI(+)/VUR(+) patients than in controls. But B2MG levels were similar between in UTI(+)/VUR(+) patients and UTI(-)/VUR(+) patients (p>0.05). B2MG and hsCRP levels were higher in UTI with RD than in those UTI without RD. The hsCRP levels were higher in patients with RD than in without RD and controls (p<0.001). The hsCRP levels were higher in UTI(-)/VUR(+) patients than in controls. Conclusions: In conclusion, our results suggest that fi bronectin seems not to be useful for detection of levels of UTI and/or presence of VUR and RD. B2MG and hsCRP levels were related RD. The hsCRP may be useable for detection of ongoing infl ammation UTI (-)/VUR (+) patients with VUR that these patients may be candidate for scar nephropaty.

Producing Bacteria O. Kizilca, R. Siraneci, A. Yilmaz, N. Hatipoglu, E. Ozturk, A. Kiyak, D. Ozkok. Bakirkoy Maternity and Children Hospital, Istanbul, Turkey. Objectives: Our aim was to investigate the risk factors of antimicrobial resistance in children with urinary tract infection caused by extended spectrum beta lactamase (ESBL) producing bacteria. Methods: 344 patients, diagnosed as urinary tract infection (UTI) between January 2008 and December 2009, were enrolled in this retrospective study. Causative microorganisms were ESBL (+) in 148 patients and ESBL (-) in 196 patients.

The causative microorganism isolated most frequently was Escherichia coli and 41.4% was detected as ESBL (+). Among Klebsiella species, 53.2% was ESBL (+). The resistance rates were 83.1% for TMP-SMX, 18.2% for nitrofurantoin, 47.3% for quinolones, 39.9% for aminoglycosides in ESBL (+) group, whereas 62.2% for TMP-SMX, 4.6% for nitrofurantoin, 9.7% for quinolones, 9.7% for aminoglycosides in ESBL (-) group. Being younger than one year old, high annual reccurence rate of UTI, longer duration of antibiotic prophylaxis, using cephalosporins for prophylaxis, previous hospitalization in last 3 months, having vesicoureteral refl ux, using clean intermittent catheterization were found to be more frequent in ESBL (+) UTI group (p<0.05). According to logistic regression analysis age younger than one year old and high annual reccurence rate of UTI were identifi ed as independent risk factors; the risk increased by 1,74 fold and 2,25 fold respectively. Conclusions: Recognition of the risk factors for ESBL (+) bacteria may be helpful to determine new policies in management of UTI. Objectives: Clinically signifi cant markers of urinary tract infection (UTI) and vescioureteral refl ux (VUR) can improve their diagnosis and help determine the risk for renal damage. We hypothesized that hepatic and/or pulmonary nodules on the ultrasound and dimercaptosuccinic acid scintigraphy can predict the renal risks in patients with UTI. Methods: The clinical and radiological fi ndings of 387 children with a proven evidence of UTI were retrospectively reviewed. The study sample included 19 pediatric patients with UTI combined with hepatic and/or pulmonary nodular lesions diagnosed by the imaging tests performed for the UTI. Results: Hepatic nodules were detected in fi ve patients, pulmonary nodules in 12, and both hepatic and pulmonary nodules in two patients. The mean age of the children was 24.5 months. VUR was detected in nine out of 17 patients (52.9%), and high grade VUR in 55.6% children with VUR. Acute pyelonephritis was identifi ed in nine out of 18 patients and renal scarring in 57.1% patients with pyelonephritis. On follow-up, the hepatic and/or pulmonary nodules regressed in Tuesday, August 31 all but one case, where the nodule was reduced in size. About 85.7% of patients experienced a recurrence of UTI within 1 year. Conclusions: Our data shows that children with UTI and hepatic and/or pulmonary nodules had a high risk for renal damage, VUR, and recurrent infections. The hepatic and/or pulmonary nodules on the ultrasound and dimercaptosuccinic acid scintigraphy may offer a valuable sign for the proper management of the patients with UTI.

Abstract# 320 (O-41) Cystatin C Is Superior to Creatinine in Children with Active Malignancy H.N. Blufpand, J. Tromp, A. Bökenkamp. Pediatrics, VU University Medical Center, Amsterdam, Netherlands. Objectives: Monitoring of renal function is crucial in children treated for malignancy. Muscle wasting is common among these patients and hampers the use of serum creatinine. Cystatin C has emerged as an alternative because its metabolism is independent of muscle mass, but data on its use in pediatric oncology are sparse. Hypothesis: estimation of glomerular fi ltration rate using cystatin C is more accurate than creatinine in children treated for malignancy. Methods: Inulin clearance (Cin), estimated GFR using serum cystatin according to Filler (eGFRcys) and creatinine according to Schwartz (eGFRcrea) were measured in 68 patients with active malignancy and compared with 150 controls. Creatinine was measured using a kinetic Jaffé method, cystatin C by particleenhanced immunonephelometry. We analyzed the difference between measured and estimated GFRs and the performance of both eGFRs for the detection of mild renal insuffi ciency. Results: Multiple linear regression analysis showed an overestimation of GFR by eGFRcrea both in females (b=-8.65, p=0.001) and in patients with malignancy (b=-6.05, p=0.069). eGFRcys overestimated GFR in females (b=-7.33, p=0.016) but was independent of malignancy (b=3.90, p=0.335). Agreement between the eGFRs and Cin was comparable in the control group (k-value eGFRcrea 0.367 vs. eGFRcys 0.396), while eGFRcrea performed poorly (k -0.114) in the malignancy group. ROC analysis (cut-off of 90 ml/min/1.73m 2 ) showed that the diagnostic accuracy of eGFRcys was higher than eGFRcrea (AUC 0.823 vs. 0.599) in the malignancy group. Both performed comparably in the control group. Conclusions: Cystatin C is superior to creatinine as a marker of renal function in children with active malignancy. DISCLOSURE: Bökenkamp, A.: Other, The immunonephelometric assays were a kind gift from Siemens Healthcare, Marburg, Germany. Objectives: Progressive interstitial fi brosis is the most dramatic result of obstructive nephropathy (ON). The aim of this study is to show and compare the actions of simvastatin (Simv) and erythropoietin (Epo) in nuclear factor kappa B (NFkB), transforming growth factor-β (TGF β), basic fi broblast growth factor (bFGF), platelet-derived growth factor B (PDGF B), fi bronectin expression and development of interstitital fi brosis in rats with experimentally induced unilateral ureteral obstruction (UUO). Methods: Sprague Dawley rats, arranged in four groups as sham, Epo, Simv and control, were used. All rats except group sham underwent UUO. Epo, Simv and control groups were given Epo (1000IU/kg/every other day, intraperitoneal), Simv (2mg/kg/day) and vehicle by gavage respectively, for 14 days. For TGF-β, PDGF B, bFGF, NFkB and fi bronectin expressions, immunohistochemical methods were used. Results: TGF-β and fi bronectin expressions were higher in control group compared to Epo and Simv groups. In addition, fi bronectin expression in Simv group was higher than Epo group. Different than the Simv group, NfkB and bFGF expressions in control group were higher than Epo group. Conclusions: As a result it was seen that Epo and Simv prevented fi brosis in ON. Epo was superior to Simv in this effect by suppressing the expressions of profi brotic factors NFkB, bFGF and fi bronectin. Based on this fi ndings, it was thought that Epo might be a better agent than Simv in the prevention of fi brosis in ON. Objectives: The currently used chronic kidney disease (CKD) classifi cation system characterizes the severity of kidney disease by 5 stages, based on the calculation of an estimated glomerular fi ltration rate (eGFR). Recently, the Schwartz formula was found to routinely overestimate the eGFR, the difference based on a change in the methodology of serum creatinine determination from Jaffe to enzymatic. A new estimating equation has been generated by the Chronic Kidney Disease in Children (CKiD) study, which more accurately estimates GFR. We recently developed a program at our institution which automatically calculates the eGFR by the CKiD formula based on the patient's serum creatinine, blood urea nitrogen and height recorded after hospital admission. Methods: Collection of eGFR and demographic data from hospitalized children between 1-22 years over a 1 month period of observation. Results: eGFR values were recorded from 262 patients. Mean age: 8.63 years, mean serum creatinine: 0.33 mg/dl and mean eGFR: 102.27ml/min/1.73m2. Prevalence of low eGFR by CKD stage was: stage 4: 0.4%, stage 3:6.4% and stage 2:25.4%. The mean age of patients with low eGFR was 8.6 year. Greater than 50% of patients with low eGFR were male. The subspecialties with the highest prevalence of eGFR<90 were general pediatrics 41.3%, endocrinology 35%, cardiology 50%, critical care 56.2%, nephrology 80%, neurology 60%, surgery 33%, and ENT 50%. Conclusions: Based on this pilot study, the prevalence of impaired kidney function as determined by an accurate assessment of eGFR appears to effect more than one-quarter of hospitalized patients, potentially carrying the risk of previously undetected impaired kidney function in children.

Results: 92% of children with orthostatic proteinuria demonstrated renal vein entrapment on ultrasound examination as compared to 27% of children with glomerular proteinuria and 35% of healthy controls ( p= 0.0001 ). Cut off limits of D/N > 6.4 and VmaxN/VmaxD > 4.3 for upright ultrasound measurements showed a sensitivity of 92% and specifi city of 65% for diagnosis of NCS. ROC analysis demonstrated the highest predictive value for orthostatic proteinuria of both the above ratios when measured in the upright position (AUC=0.85). Conclusions: 1. Left renal vein entrapment can be demonstrated in the majority of Caucasian children with orthostatic proteinuria. 2. The best diagnostic performance of LRV sonographic indices are achieved for assessments in the standing position. 3. LRV sonography is a useful, noninvasive tool for confi rming NCS in the initial investigation of proteinuria in children.

(O-47) Melamine-Related Urolithiasis Children: Continuous Attention on the Long-Term Prognosis X.-Y. Kuang, J. Gao, H. Xu. Department of Nephrology and Rheumatology, Children's Hospital, Fudan University, Shanghai, China. Objectives: Melamine is the key factor of the outbreaks of urinary stones in China, 2008 . 105 children were diagnosed as urinary system stones of screening 8335 children in our hospital. Continuous attention on their long-term prognosis should be stressed. Methods: Urolithiasis children were followed up after six and eighteen months respectively. We rechecked the patients' urinalysis, urinary system ultrasonography, urinary Ca/Cr, ALB/Cr, IgG/Cr and NAG/Cr. Results: 91.4% and 76.2% children engaged in the fi rst (six months later) and second follow-up (eighteen months later), respectively. Ultrasonography showed 68.9% patients had disappeared stones in the fi rst follow-up, as well as 86.1% patients did in the second, difference was signifi cant (P=0.012). Similar results was found in 61 patients who participated in both follow-ups (54.1%, 86.9%; P<0.001). 74.3% and 76.2% patients were tested urinary ALB/Cr, IgG/Cr and NAG/Cr in both follow-ups. The glomerular injury detection rate in the second one (8.9%) was obviously less than that in the fi rst one (32.1%, P<0.001), but it was no signifi cant difference in the renal tubular injury rate (17.9%, 16.1%; P>0.05). Moreover, all quantitative analysis of IgG/Cr, ALB/Cr and NAG/Cr had statistically signifi cance between them (P IgG/Cr =0.038, P ALBU/Cr <0.001 and P NAG/ Cr =0.026). Similar results were obtained from 58 patients who participated in both follow-ups (P IgG/Cr =0.015, P ALBU/Cr <0.001 and P NAG/Cr =0.029). Conclusions: Most Melamine-related urolithiasis children can excrete calculus spontaneously. Renal injury resulted by urolithiasis is reversible in most of them. The renal tubular injury should be paid more attention at further follow-up.

The Long-Term Safety Profi le of Oral Desmopressin in Children with PNE C. Van Herzeele, 1 J. Evans, 2 P. Eggert, 3 H. Lottmann, 4 J. Vande Walle, 1 J.P. Norgaard. 5 1 Pediatric Nephrology, UGent, Gent, Belgium; 2 Pediatric Nephrology, Hospital, Nottingham, United Kingdom; 3 Pediatric Nephrology, Hospital, Kiel, Germany; 4 Pediatric Urology, Necker Enfants Malades, Paris, France; 5 Ferring International, Ferring, Copenhagen, Denmark. Objectives: The recent discussion on the safety-issue of desmopressin as a spray in, has led to the withdrawal of the spray for the indication of enure MNE. The aim of the study is to evaluate the long-term safety profi le of oral desmopressin in children with PNE.

Methods: prospective open-label, multinational, phase IV, observational study with up to 6 months follow up. 86 centres in 4 countries (UK, Canada, Germany, and France). Adverse events (AEs) were defi ned as any symptom during the study-period. Objectives: A total of 25 Chinese patients aged 6 to 36 months hospitalised at Beijing Children's Hospital due to melamine-induced kidney stones complicated by acute obstructive renal failure in 2008 were included in a study in order to diagnose and treat these special cases more effectively. Methods: Feeding history, clinical presentation, ultrasound fi ndings, treatments and effects were summarised. Twelve to seventeen months follow-up was reported also. Results: Ultrasound examination showed that calculi were located at the kidney and ureters. Stones were composed of both uric acid and melamine in a molar ratio of 1.2:1 to 2.1:1. Treatments providing liquid plus alkalisation of urine proved to be effective in helping the patients pass the stones. Surgical intervention was needed in severe cases. Renal function returned to normal in all 25 patients after various durations of therapy. Sixty-eight percent of the patients expelled all of the calculi within 3 months, 90% in 6 months and 95% in 9 months, without sequelae till the end of the follow-up. Conclusions: Melamine-contaminated milk formula can cause kidney stones in infants, which should be diagnosed by feeding history, clinical symptoms and ultrasound examination. Composition of the stones was not only of melamine but also uric acid. Providing liquid orally or intravenously plus alkalisation of urine proved to promote the removal of the stones. Follow-up of 12 to 17 months after discharge showed no sequelae. In adults with CKD, protein-energy wasting (PEW), characterized by a panoply of biochemical and nutritional indicators, has been implicated as a risk factor for death and cardiovascular disease. The clinical relevance of these markers in pediatric CKD populations is unknown. Methods: Using cross-sectional data from the Chronic Kidney Disease in Children study (CKiD), a defi nition of PEW was developed using markers identifi ed from adult CKD populations. We assessed the prevalence of 7 indicators of PEW (anemia, poor weight growth, poor height growth, acidosis, low LDL, low dietary intake and hypoalbuminemia) and investigated the association of GFR and PEW using ordinal logistic regression. The relationship between severe PEW (≥ 4 indicators) and blood pressure was also examined. Using data from 473 children we found 39 children with severe PEW (≥ 4 indicators) accounting for 8% of the cohort. The most common PEW indicator was anemia, occurring in 38% of the cohort and 92% of those with severe PEW. Acidosis and hypoalbuminemia were present in the cohort population at 18% and 6%, respectively, and they were 4 and 9 times as likely to be found in those with severe PEW (P<0.001). PEW and GFR were also associated (for every 10 ml/min decrease in GFR the odds ratio for being in a higher category of PEW was 1.43 [1.30, 1.57]). Severe PEW was associated with a 10 point higher systolic blood pressure percentile for age, sex and height (P<0.05). Indicators of PEW were highly prevalent in the CKiD cohort of children with CKD, and PEW severity was associated with lower kidney function and higher blood pressure.

Sickle Cell Nephropathy in Children Seen in an African Hospital O.T. Adedoyin, O.P. Fatoye, A.O. Bello, A. Adeniyi. Paediatrics and Child Health, University of Ilorin Teaching Hospital, Ilorin, Kwara, Nigeria; Paediatrics and Child Health, University of Ilorin Teaching Hospital, Ilorin, Kwara, Nigeria; Paediatrics and Child Health, University of Ilorin Teaching Hospital, Ilorin, Kwara, Nigeria; Paediatrics and Child Health, University of Ilorin Teaching Hospital, Ilorin, Kwara, Nigeria. Objectives: To review the cases of sickle cell nephropathy seen over the last 14 years (1995) (1996) (1997) (1998) (1999) (2000) (2001) (2002) (2003) (2004) (2005) (2006) (2007) (2008) (2009) at the Paediatric Nephrology clinic of University of Ilorin Teaching hospital, Ilorin, Nigeria. Methods: The fi ve cases of SCN seen during the period were analyzed for age, sex, and renal manifestations. Results: The age range of the children was 9-15years with a mean of 11years. Four of the fi ve patients were females, with one male. Three of the four females presented with features suggestive of nephrotic syndrome while the other one had gross haematuria which resolved within 24 hours. The only male had enuresis.

The nephrotic syndrome (NS) in one of the patients progressed to end stage renal disease requiring renal replacement therapy. Conclusions: Children with sickle cell disease should be screened for renal complications especially from the late fi rst decade of life. This will help in the early detection of renal disorder that could lead to chronic kidney disease. It is also suspected that the severe forms of Sickle cell nephropathy such as nephrotic syndrome may have a predilection for the female gender. A more extensive study is needed to test the veracity of this observation.

The Objectives: aHUS is an uncommon disease.Sporadic or familiar,affects children of all ages.Main features:severe hypertension,chronic renal failure (CRF):50%,mortality:10%.Defi ciency in complement regulatory proteins(factors H,I and B),MCP,ADMTS 13 or antibodies against factor H are main causes. Plasma therapy is an effective treatment, even though there is no agreement in their use regime. The risk of recurrence in kidney transplant is higher in factor H and I. To describe the clinical features and outcome of aHUS patients(p) in our Institution. Methods: Retrospective age, gender, familiar history, age at the onset, hypertension, low C3, treatment, follow-up and outcome were recordered. Continued variables expressed as median and categoric as percentages. Results: 11p.:7 male.Two pairs of sibblings and 2 cousins.Median age onset: 0.66 m(0.33-6.8).Hypertension: 9p(82%),low C3:3/9p. and relapse: 8p(73%). Treatment: 7p:plasma infusions.10 needed acute dialysis. Median time followup: 9.5y(4.1-15).Outcome: 4 p.normal renal function, 7 developed CRF: 2 in predialysis medical treatment and 5: ESRD. These 5 p. undergo cadaveric kidney transplants: 2 recurrenced with graft lost (one of them received 2nd successful one with plasma exchange prophylaxis), 1 had no recurrence (died due to no renal reason) and two (the cousins) received successful transplants with serial plasma exchange and infusions. Conclusions: aHUS was present in younger children, with more hypertension and higher requirement of dialysis. Outcome was unfavourable, with a high rate of recurrence and ESRD. Our results force us to enhance the efforts to identify the mutations in order to select the best treatment for each type of patient.

Signifi cance of Protein to Creatinine Ratio Determination in the Initial Work-Up for Pediatric Objectives: To estimate the frequency of urine protein to creatinine ratio testing in children found to have proteinuria on their routine dipstick urinalyses by primary care providers before these patients were referred to a pediatric nephrologist. To assess the impact of protein to creatinine ratio on further diagnostic work-up and outcomes of these patients.

We retrospectively reviewed medical records of patients referred to our pediatric nephrology clinic over the past 4 years. A total of 151 patients were referred with the diagnosis of proteinuria. The ultimate outcomes were analyzed separately for patients with and without urine protein to creatinine ratios ordered by their primary care providers. Results: Of all analysed patients, urine protein to creatinine ratio was tested by primary care providers in 31.2% of cases. Overall, glomerular disease was identifi ed in 8.5% of all referred patients and 4% went on to have a renal biopsy done. 31.3% of patients on their initial visit to nephrologist were found not to have any suffi cient reason to be followed in renal clinic based on clinical and laboratory investigation. Among patients with protein to creatinine ratio done by their primary care providers, only 10.0% were not recommended to have followup with nephrology, and for those who had not had this study done, 40.9% were cleared after their initial visit (p<0.05). Glomerular pathology was found more often in patients referred with a urine protein to creatinine ratio already having been performed (18.1% vs 4.5%). Conclusions: Determining urine protein to creatinine ratio in primary care settings can help practitioners to avoid unnecessary referrals to pediatric nephrologists and thus should be done more universally.

Ocular Abnormalities in Childhood Chronic Renal Failure A.J. Al Mosawi. Pediatrics, University Hospital in Al Kadhimiyia, Baghdad, Iraq. Objectives: Few literatures reported the incidence of ocular abnormalities in chronic renal failure (CRF).The aim of this paper is to determine the incidence of ocular abnormalities in childhood CRF. Methods: From January 1993 to July 2007, 80 patients with a diagnosis of chronic renal failure (CRF) were observed at the University Hospital in Al Kadhimiyia. They were examined to determine the presence of ocular abnormalities. Fifty one patients were males (63.75%) and 29 (36.25%) were females. The male-female ratio was 1.75, and the age at referral ranged from 2 months to 18 years (mean 9 years). Results: Corneal cystine crystals were the most common ocular abnormalities associated with childhood CRF occurring in 7.5% of the patients in association with Nephropathic cystinosis. Congenital cataract & glaucoma were observed in 3.75% of the patients in association with Oculo-cerebro-renal syndrome (OCRS). Objectives: The aim of this paper is to describe a new model for the management of end-stage renal disease (ESRD) in the developing world and its potential future role in the industrial countries when advancement in stem cell researches makes the need for long-term dialysis obsolete. Methods: During the period from January 1993 to July 2007, 80 patients with a diagnosis of chronic renal failure (CRF) were observed at the University Hospital in Al Kadhimiyia. Fifty one patients were males (63.75%) and 29 (36.25%) were females. The male-female ratio was 1.75, and the age at referral ranged from 2 months to 18 years (mean 9 years). Fourteen (16.5%) patients were treated a new therapeutic approach consisting of acacia gum supplementation plus the traditional conservative measures was used. Results: Amelioration of the uremic symptoms and lowering of blood urea levels delaying the need for dialysis was associated with this new therapy. In this sample of 80 patients the longest survival of 6 years was achieved in 2 patients, both treated initially with IPD. One of them was transplanted and the other was treated with the new therapeutic approach. Conclusions: It's a fact that with appropriate dietary and pharmacologic management, patients with non-terminal CRF can be maintained surprisingly well and the transition from non-terminal CRF to ESRD represents a small decrement of renal function resulting in a large physiologic hurdle for the patient. The addition to these effective traditional measures, an agent enhance fecal nitrogen excretion can possibly bridge this gap resulting from this small decrement of renal function obviating the need for dialysis for some period of time.

Serum Objectives: Intravenous maintenance fl uid is widely used in general pediatric practice and more children who come into the hospital receive intravenous fl uid than in the past. Recent studies show the potential harm in using hypotonic solutions in children especially in diseased states like pneumonia. The choice of parenteral fl uids is usually dictated by the serum sodium and urine sodium. The objective is to determine the random serum sodium and urine sodium levels among children with pneumonia. Methods: Serum sodium, BUN and creatinine concentrations were obtained upon the patients' admission and blood sampling took place before starting any intravenous fl uid. Urine samples were also sent within 24 hours upon admission to the Philippine General Hospital laboratory for random urine sodium and creatinine levels. Fractional excrtetion of sodium will be computed. Results: Infants were found to be more affected with pneumonia with males predominating(73%). 60% of the subjects presented with normal serum sodium. The mean serum sodium was 142 mmol/L (SD 5.29 mmol/L) For the urine sodium, 70% had normal values. Mean urine sodium was 90.77 mmol/L (SD 69.51 mmol/L). 57% had low FeNa. Using the Fisher's exact test, the risk for abnormal serum sodium and urine sodium was higher in children than infants. Conclusions: Children with pneumonia have normal serum and urine sodium. Near-isotonic or isotonic fl uid is a more physiologic parenteral fl uid to use in children with pneumonia.

Effect Objectives: The most widely used parenteral fl uid in patients admitted to the hospital is a dextrose-containing hypotonic maintenance fl uid. However, this practice has recently been called into question. The objective is to determine whether there will be lower mean serum sodium levels in patients who receive a hypotonic compared with isotonic parenteral fl uid.

In an open, randomized controlled trial, patients age 1-15 admitted to the emergency room were placed on nothing per orem and given intravenous fl uids for at least 24 hours. Subjects were to receive either D5NM or D5NR. The fl uid rate was determined by the attending physician. The serum sodium 24 hours after initiation of fl uids was measured. A p-value < 0.05 using the Student's t-test was considered statistically signifi cant. Results: Nineteen patients (11 in the isotonic group and 8 in the hypotonic group) were included. There was no statistical difference in baseline characteristics. Baseline serum sodium was 140.3±9.1 mmol/L in the hypotonic group and 139.9±4.3 in the isotonic group (p=0.43). After 24 hours of intravenous fl uid therapy, mean serum sodium was 138.7 ± 9.5 for the hypotonic group and 139.0 ± 3.0 for the isotonic group (p=0.44). One patient in the hypotonic group developed asymptomatic hyponatremia with serum sodium < 128.

Conclusions: This preliminary study shows no signifi cant difference in serum sodium 24 hours after initiation of either isotonic or hypotonic fl uids. A follow-up study with a larger sample size is recommended. 

Methods: A retrospective analysis of 54 consecutive patients with SSNS was performed. The incidence was 1.9/100.000 and follow-up 4.0 years. Two different steroid regimens were used and a subanalysis was performed in the group treated with the long steroid course (pred-long) (prednisone 60 mg/m 2 /24h for 6 weeks and 6 weeks prednisone 40 mg/m 2/ 48h). The short steroid treatment course (predshort) was 4 weeks with prednisone 60 mg/m 2 /24h and 4 weeks prednisone 40 mg/m 2 /48h. Results: In total, 30/54 of the patients were classifi ed with FR/SD. FR/SD patients were younger at debut compared to non-FR/SD patients (3.5 years vs. 8.5 years, p < 0.002). Males were overrepresented in the FR/SD group (69% vs. 38%, p = 0.03). No differences were found regarding hematuria, hypoalbuminemia, or days to achieve remission. 31 patients were treated with pred-long compared to pred-short in 23 patients reducing the overall number of FR/SD patients from 80% to 38% (p<0.006). In the pred-long group, 12 patients were FR/SD and these were younger vs. 19 non FR/SD patients (4.4±3.1 years vs 8.4±4.1 years, p<0.005). Conclusions: Low age at debut and male gender is associated with a high risk of FR/SD in this unselected series of patients. This tendency seems consistent despite the prolongation of the steroid course at the debut of SSNS.

Epidemiology Objectives: This study reports on clinical and biological prognostic factors of a good growth in children with hypophosphatemic rickets. Methods: It is based on a retrospective and multicenter collection of 38 children.

The mean follow-up was 6.9 years. The patients were treated with 1-α-cholecalciferol and phosphorus supplementation. Twelve patients had nephrocalcinosis with an average time to onset of 117 months with a lower growth than the patients without (∆Z= -0.75 vs 0.21). Seventeen patients had a poor growth (difference between fi nal and initial Z score: ∆Z <0) and 21 had a good growth (∆Z ≥ 0). There was no signifi cant improvement in size over the entire group between the beginning and end of the study (-1.18 SDS vs -1.28 SDS). But patients with a size at diagnosis <-2 SDS tended to have a better fi nal height. Sporadic cases had a signifi cantly better growth than familial cases. Patients with a size ≤ -2 SDS at 4 years (probably without the Hap1 variant of vitamine D receptor genotype) had a lower size at the end of study but a same growth. Factors predictive of a better ∆Z score are the 4 following: sporadic cases, initial Z score, female gender, and no nephrocalcinosis. Mean serum phosphate and treatment were not discriminating.

The prognostic factors of good growth seem more connected to the initial profi le of patients and the spontaneous evolution of the disease than the replacement therapy. 

Conclusions: The diversity of response to treatment and outcome observed in SRNS, recommends that multicentric studies should be undertaken in order to better identify the potential effi cacy, individual response, and side effects of long term therapy with the immunosuppressive drugs presently available. exposure to SHS routinely. While the majority were confi dent about being able to provide guidance to patients on the harmful effects of smoking, 75% were not confi dent about enrolling or referring patients to a quit line for smoking cessation. Over half (74%) were not confi dent in prescribing patients tobacco replacement products. Barriers identifi ed were time limitations (81%), lack of professional training (60%) and unfamiliarity with CPT reimbursement codes (57%). Conclusions: Tobacco counseling of children with CKD by pediatric nephrologists is suboptimal due to several barriers. Adequate training of the entire pediatric nephrology team about tobacco counseling is important in attepmts to reduce morbidity and mortality associated with cardiac disease in children with CKD.

Relation Objectives: Chronic diarrhea is the most reported side-effect of MMF. We investigated whether the intestinal permeability, as tested by the sugar absorption test (SAT), is different in children treated with MMF for NS, in comparison to ciclosporine A (CsA). Methods: In a multi-centre trial, children with frequently relapsing minimal change NS were randomized for treatment with MMF 1200 mg/m2/day or CsA 4-5 mg/kg/day. Before start and 3-6 months after start of the study medication, a SAT was performed. Results: Of 15/24 children, two samples were available for analysis (6 MMF, 9 CsA) . At start of the study, there was no difference in urinary lactulose-mannitol (LM) ratio between children being treated with CsA or without and all LM ratios at baseline were normal. At the second SAT, again all urinary LM ratios were within normal limits. The change in LM ratio compared to baseline was not different between the 2 groups (p=0.16).

Only one patient with MMF reported temporarily diarrhea. Mean weight gain during the study period of 12 months, -0.03 kg (-0 Objectives: Since the early 1990s cystinosis is treated with cysteamine to prevent accumulation of cystine. This therapy delays organ damage but may cause gastrointestinal side effects by hypersecretion of gastric acid. In laboratory rats it was found ulcerogenic. Methods: We report two severe gastrointestinal complications in a transplanted cystinosis patient treated with cysteamine. Results: A now 14-year-old girl with cystinosis received cysteamine since the age of 4 years. Doses ranged from 1.0 to 1.3 mg/m 2 /day; cystine concentrations in leucocytes were below 0.6 nmol/mg protein. Nevertheless she developed ESRD and was transplanted at the age of 9 years. Immunosuppression included prednisolone, tacrolimus, and 1 year MMF. At the age of 14, she was diagnosed with acute severe ulcerative colitis, necessitating subtotal colectomy despite intravenous steroids. Histopathology showed ulcerative lesions without cystine crystals. Cysteamine concentrations were normal. After an episode of acute tubular necrosis she developed duodenal ulcers with massive hematemesis. These were treated with blood transfusions and intervention duodenoscopy. Cysteamine therapy was then interrupted to promote healing of the duodenal ulcers. She was discharged after 2 months with maintenance immunosuppressive therapy for the renal graft consisting of low dose steroids and tacrolimus, and with a half dose of cysteamine and omeprazole. Conclusions: To our knowledge this is the fi rst report of duodenal ulcers in a cystinosis patient with cysteamine therapy (and the second on IBD). Although physical stress, steroids and uremia could all have contributed to the formation of duodenal ulcers, a possible role of cysteamine can-not be excluded.

The Objectives: Knowledge of number, location and size of renal stones is very important in predicting underlying causes and management of urolithiasis. The information about these issues in pediatric fi eld is scarce; therefore, we performed this study to evaluate these parameters in children with urolithiasis. Methods: 153 patients (82 boys and 71 girls) ranging in age from 2 months to 18 years (mean age =4.84 years ±4.81) with documented renal stones were enrolled in this prospective cross-sectional study. The number, location and size of the stones were determined by ultrasonography in at least 2 sessions. Results: 62 patients (40.5%) had one, 65 (42.5%) had two and 26 (17%) had three stones or more.The stones were bilateral in 56 patients (36.6%). Out of 244 stones, 127 were located on the right side and 117 on the left side. Of total stones, 241 (98.8%) were located in the kidneys; 103 (42.7%) of these stones were in the middle parts, 97 (40.2%) in lower poles and 41 (17%) in the upper poles of the kidneys. 3 ureteral stones were found while no pelvis or bladder stone was detected. Considering the greatest diameter of the stones, 107 patients (69.9%) had stones less than 5 mm, 7 (4.6%) had stones less than 5 mm and also stones measuring 5-10 mm, 35(22.9%) only had stones measuring 5-10 mm and 4 (2.6%) had stones greater than 10 mm. Conclusions: Compared with the previous reports, large stones are less frequent in this study and bilateral stones, the usual indicators of underlying metabolic disorders, are relatively more common; furthermore, the renal stones are less commonly located in upper poles of the kidneys and the ureters. evaluations of blood and urine were done for all of the patients. Results: 139 patients (90.8%) had at least one related metabolic abnormality. The frequencies of these abnormalities in decreasing order were as following: hypomagnesuria (n=93, 60.8%), hypocitraturia (n=81, 52.9%), hypercalciuria (n=72, 47.1%), hyperuricosuria( n=57, 37.3%), hypernatriuria ( n=52, 34.4%), hyperoxaluria( n=26, 17%), renal tubular acidosis( n=10, 6.5%), hyperuricemia(n=7, 4.6%) and cystinuria (n=3, 2 %). Anatomical abnormalities were detected in 6 patients; of these patients, 5 had associated metabolic abnormalities. Conclusions: In contrast to previous reports, higher proportion of our patients have underlying metabolic abnormalities, even in those with anatomical abnormalities. Furthermore, in other reports, hypercalciuria is the most frequent abnormality associated with urolithiasis; however, in our study, hypomagnesuria and hypocitraturia are more common.

Long Objectives: Although complete recovery occurs in 75% of D+HUS patients, renal dysfunction could reappear years later. The aim of the study was to describe renal outcome and to highlight long term prognostic factors for D+HUS patients and ≥10 years follow-up. Methods: Initial data as well as renal function tests at 6 months and at 1 year were collected for 219 patients admitted for D+HUS between 1972 and 1998. Renal function were assessed in these patients between 2007 and 2009 and they were classifi ed in 3 groups according to the level of GFR: CKD0 (no chronic kidney disease), CKD1 (proteinuria/ microalbuminuria/ hypertension, normal GFR); CKD2-5 (GFR 80-0 ml/min/1.73m²). Results: Data from 140 of 210 surviving patients were available with a median follow up of 18.7 years after HUS. Among them, 62 patients (45%) belonged to CKD0, 46 (33%) to CKD1, and 32 (22%) to CKD2-5. Median follow-up was respectively 17.3, 18.2, and 22.7 years*. Multivariate analysis showed that anuria ≥8 days*, need of dialysis*, serum creatinine >60µmol/L 1 month after HUS* were factors of poor long term renal outcome *(p<0,01). In contrast complete recovery of renal function by 1 year after D+HUS was not a reliable marker of favourable outcome. Conclusions: By 20 years of follow up, 55% of patients with a history of D+HUS present CKD. The signifi cant difference in the duration of follow-up between groups suggest that the rate of CKD worsen with time and that some patients currently in CKD1 are at risk to develop CKD2-5 in the next decade. We suggest that all D+HUS patients should benefi t of a life long follow-up even in case of complete recovery of renal function in the year following HUS.

Frequency . Sodium content in IV fl uids were higher in patients with hyponatremia, 78.5 ± 10 versus 68.5 ± 27 mmol/L (p<0.05). Vincristine and diuretics were prescribed in 85% and 43% of patients respectively, with hyponatremia (p<0.05). No signifi cant associations were found between hyponatremia and age, sex, weight and nutritional status. The initial serum sodium was 138 +/-7 mmol/L in hyponatremic patients and 141 +/-3 mmol/L in patients without hyponatremia (p<0.0002).

Conclusions: Hypotonic solutions are routinely used in chemotherapy hydration protocols. Hyponatremia is multifactorial and frequent in patients treated with large hypotonic IV fl uids. Acute leukemia diagnosis, vincristine and diuretic administration, and initial low serum sodium should be considered as risk factors. Objectives: A consensus has emerged that the renin-angiotensin system (RAS) blockers such as angiotensin converting enzyme inhibitors (ACEI) and angiotensin-II receptor blockers (ARB) exert renoprotective effects through anti-hypertensive and anti-proteinuric properties in patients with chronic kidney disease (CKD). It is not clear, however, whether CKD with a lower rate of urinary protein excretion like congenital kidney malformations benefi t from the RAS blockers. The aim of this study is to evaluate the renoprotective effects of the RAS blockers to slow renal deterioration in children with hypodysplastic nephropathies. Methods: We studied 8 patients with stage 2 to 4 CKD for bilateral hypodysplastic nephropathies, who ranged from 6.1 to 16.0 years old at the start of the treatment. Hypertension was present in 3 patients, 2 suffered moderate proteinuria and 5 had only mild proteinuria before the treatment. Four patients received ACEI and the rest were treated with ARB. Results: After the mean follow-up of 35 months, all the patients are on treatment. 3 patients with hypertension have shown enough decrements in blood pressure to be within normal range and mild proteinuria decreased in 3 of the 5 patients. The slope of the reciprocal serum creatinine (1/ Cr) curve fi t showed slowed deterioration in 3 patients. None developed adverse events such as hyperkalaemia.

Conclusions: Though the range of proteinuria in dysplastic kidney disease is usually mild, our results suggest the possible benefi ts of the RAS blockers to ameliorate the decline of renal function. Further large-scale clinical studies as well as a better understanding of the pathophysiological role of the RAS are expected.

Childhood Anti-Neutrophil Cytoplasmic Anti-Bodies (ANCA) Positive Vasculitis: A Case Series of Four Patients P. Gajjar, P. Nourse, P. Komala.

Objectives: To document the clinical, histological and serological characteristics of children with ANCA positive vasculitis presenting to our institution. Methods: A retrospective folder review of patients with clinical manifestations of small vessel infl ammation, necrotising glomerulonephritis and serological fi nding of ANCA positivity. Results: Four patients met the criteria. All were girls, ranging from 5 yrs to 12 yrs (mean age of 7.5 yrs). The main clinical fi ndings were: infl uenza-like symptoms (75%), haematuria/proteinuria (100%), pulmonary-renal syndrome with haemoptysis in 50%, and acute renal failure in 50 %. One patient presented with bilateral lacrimal gland swelling. All were hypertensive. One patient with rapidly progressive nephritis had a pulmonary haemorrhage six years later. In the rest, the diagnosis was confi rmed within two months. All had an elevated ESR, with normal complement and antinuclear antibody levels. Three of the four patients had an elevated pANCA; the patient with lacrimal gland swelling was cANCA positive. All underwent a renal biopsy, showing necrotising lesions with fi brous and fi brocellular crescents. Initial treatment included methylprednisone and 6 doses of monthly intravenous Cyclophosphamide followed by maintenance treatment. One patient progressed to end stage renal failure, and subsequently died. The rest are well, in remission.

Conclusions: Small vessel vasculitis should be considered early in the differential diagnosis of patients presenting with constitutional symptoms or unusual presentation involving the upper or lower respiratory tract, with abnormal

urinalysis. An autoimmune screen should include ANCA serology. 

Conclusions: HTN is present in 38% children with the fi rst episode of NS, but does not correlate with the volume status. Therefore, blood pressure is not a reliable marker of intravascular hypovolemia/hypervolemia in children with nephrotic syndrome.

Multicenter Objectives: By single center study on melamine associated urolithiasis we found potential risk factors. Large scale investigation is needed to further clarify the disease. Methods: Prospective case control research on children less than 36 months old screened for urolithiasis was performed in 6 centers in China. Children with urolithiasis, without congenital abnormality of urinary tract and history of renal diseases were included as cases. Controls were randomly sampled according to the case: control ratio of 1:1. The risk factors, stone status and manifestations were reassessed. Results: There were 1329 cases and 1317 controls with a mean age of 18.4 months. For melamine associated urolithiasis, we confi rmed that the factors of high melamine formula and preterm were risk factors, symptoms were lacking and most of urinalysis was normal. We also found children more than 1 year old were 0.538 times unlikely to have stones than younger; children fed together with breast milk have around one half chance to have stones than children fed only with formula. Children with symptom of vomiting or diarrhea were more likely to have stones. Most of stones were small and located at kidney. Children fed with high melamine formula were nearly twice more likely to have large stones. Conclusions: High melamine formula and preterm infant were confi rmed to be risk factors for stones. New identifi ed infl uencing factors were age, breast milk, and vomiting or diarrhea. Children less than 1 year old were at high risk to have stones and were recommended to be fed with breast milk. Objectives: Hemolytic and Uremic Syndrome related to Pneumococcal infection (HUS-P) is a rare condition. Pediatricians should be more familiar with this disease as early recognition could have important implications for treatment and prognosis. No consensus has been established for the diagnosis criteria of HUS-P and, in some cases, the diagnosis of certitude can be diffi cult.

We conducted a retrospective study evaluating the diagnosis strategy and criteria used for HUS-P in six nephrology pediatrics centers over the past 10 years. The criteria used for the diagnosis of pneumococcal infection and HUS were recorded as well as all the tests performed during the etiology diagnosis workup.

Results: A total of 18 children with HUS-P were studied. The bacteriological diagnosis of pneumococcal infection was confi rmed in only 72.2% of cases. Others etiologies of HUS were ruled out in 83.3 % whereas tests to directly confi rm HUS-P were performed in only a minority of cases. Indeed, Thomsen Friedenreich antigen testing -using lectin -was only performed in 11.1%. Conclusions: These results confi rmed the diffi culty to diagnose HUS-P supporting the absence of a consensual diagnosis strategy. An etiological diagnosis workup is proposed based on biological criteria (using Thomsen Friedenreich antigen testing). This should allow faster diagnosis confi rmation, avoid unnecessary tests, and lead clinicians more rapidly to the correct therapeutic strategy.

Lactate Objectives: To collate epidemiological information on pediatric chronic kidney disease (CKD) in India.

A summary of data on 435 children with an estimated GFR ≤ 59 ml/ min/1.73 m 2 (≥ CKD stage 3) from a pediatric CKD registry (13 centers) initiated in 2008. Participation in this is voluntary and mandates involvement of a pediatric nephrologist/ nephrologist in provision of care to patients. Results: Majority of entries are from Northern India (65%). There is a male preponderance (77%), mean age 7.20 ± 4.57 years and symptom onset for a mean period of 3.2 years. Children with CKD stages 3, 4, 5 were 147 (33.7%), 143 (32.8%) and 145 (33.3%) respectively. Commonest presentation was edema (33%); others seen frequently were failure to thrive (15%), acidosis (14 %), hypertension (9%). Common etiologies were obstructive uropathy (25%), vesicoureteric refl ux (14%) and renal hypoplasia/dysplasia (11%). Mean serum creatinine was 3.2+2.8 mg/dl, and mean hemoglobin 9 +2.3 g/dl. Majority of patients were on self fi nance; 20% receving eryhtropoietin and 0.45% receiving growth hormone. Sixty three patients were receiving renal replacement therapy as CAPD (6%), maintenance HD (5.5%) and 14 (3.2%) had received renal transplant.

Conclusions: As a pilot initiative, the data provides important insights: signifi cant delay from onset of symptoms to patient reaching center of care; obstructive uropathy, vesicoureteric refl ux, renal hypoplasia/dysplasia account for half the cases; very few receive ideal management viz erythropoietin, growth hormone. Acknowledgement: Indian Society of Nephrology; Janssen-Cilag India.

Risk Objectives: To assess the risk factors for developing HSN and the time period when nephritis is unlikely after the initial disease onset. Methods: A prospective study of 223 pediatric pts to examine renal manifestations of Henoch-Schönlein purpura (HSP). The pts' condition was monitored with 5 outpatient visits and urine dipstick tests at home. Of the pts 111 did not receive any medication, 89 were treated with prednisone prophylaxis and 24 either with Cyclosporine-A or MP-pulses due to severe HSN. Results: HSN occurred in 102/223(46%) of the pts consisting of isolated hematuria in 14%, proteinuria in 9%, both hematuria and proteinuria in 56%, nephrotic-range proteinuria in 20% and nephrotic-nephritic syndrome in one case. Nephritis occurred mean 14 days after HSP diagnosis and in 87% within one month. The incidence after 1 and 2 months was 14% and 2%, respectively. The occurrence of HSN increased with age, p<0.001 for linear trend. In multivariate analysis the risk factors for nephritis were age > 8yrs (OR 2.7, p=0.002, Cl 1.4-5.1), abdominal pain (OR 2.1, p=0.017, Cl 1.1-3.7) and HSP recurrences (OR 3.1, p=0.002, Cl 1.5-6.3). Pts with 2 or 3 risk factors developed nephritis in 63% and 87% of cases. Laboratory tests or blood pressure at onset did not predict HSN. Early prednisone treatment did not affect the frequency or timing of nephritis. Conclusions: Weekly urine dipstick tests are indicated for 2 months after the HSP diagnosis. Patients > 8yrs, with abdominal pain or HSP recurrences have higher risk for HSN. However, early renal dysfunction may be overlooked using that method.

The aim of the present study was to evaluate the level of neutrophil gelatinase-associated lipocalin (NGAL) in children with normal range albuminuria e.g. in those considered as not presenting diabetic nor hypertensive nephropathy. The study group consisted of 40 children with type 1 diabetes mellitus (T1DM) or primary hypertension (PHT). 15 healthy children served as a control group. [2] . Methods: Renal biopsies were performed by nephrologists themselves using a real-time ultrasound guidance and a 14-16 G automated needle. The target of the procedure was to obtain two macroscopically adequate specimens from the lower renal pole (usually the left). Only if one of the two specimens appeared too small, we try to obtain a third one. We obtained two specimens in 23 cases (60,3%), and three specimens in 35 cases (39,7%). Pathologic evaluation was based chiefl y on the combination of the light microscopic observation with immunofl uorescence microscopy data. In 35 cases electron microscopy was performed too. Results: Mean number of glomeruli per specimen was 25,3 ± 14,5 [6-65]. Pathological fi ndings included 5 minimal change diseases (8,6%), 7 FSGS (12,1%), 18 IgA-nephropathy (31,0%), 2 membranous glomerulonephritis (3.4%), 8 crescentic glomerulonephritis (13.8%), 4 Alport syndrome (6,9%), 6 thin basement membrane disease (10,4%), 2 trombotic microangiopathy (3,4%) and 6 other lesions (10.4%). Minor complications (gross hematuria and/or perinephric hematoma resolving without the need for transfusion or intervention) occurred in 16 patients (27.6%). No major complications were noted. Conclusions: A renal biopsy in pediatric patients is a safe, reliable and minimally invasive procedure. The results supplemented our knowledge of renal diseases in children in the Orenburg region.

Corticosteroid Decreases the Risk of Renal Involvement in Henoch-Schönlein Purpura but Not the Course Y. Kaku. Nephrology, Fukuoka Children's Hospital, Fukuoka, Japan.

Objectives: It is controversial yet whether steroid can prevent nephritis in Henoch-Schönlein purpura (HSP), although we reported that steroid decreased the risk of nephritis (Kidney Int, 1998) . We have treated the HSP patients with these risk factors for nephritis with steroid at the disease onset. The aim of this study is to verify the effect of preventing corticosteroid use against the development and the course of nephritis in HSP. Methods: Group A (historical control group, n=194): Before we were aware of the risk factors of renal involvement, steroids were administered in order to only control the pain. Group B (recent patients group, n=109): From 2001, we administer 1mg/kg/day of predonisolone for 1-2 weeks to the patients with the risk factors. We compared the rate of nephritis by using Kaplan-Meier method and log rank test. The course of nephritis in group B was analyzed similarly.

The severity of HSP in the two group was not differ. The rate of nephritis in group B was lower than group A (23.2% vs 38.6%, p <0.0057). 28 patients in group B developed nephritis. Steroid did not affect the course of nephritis. Urinary abnormalities persisted in the patients with persistent purpura.

Conclusions: These results elucidate that corticosteroid has the protective effect against nephritis in HSP. However, corticosteroid did not affect the course of nephritis. The therapy for nephritis maybe modifi ed the course. Objectives: Serious infection, such as spontaneous bacterial peritonitis (SBP), can complicate idiopathic nephrotic syndrome (INS). Though pathogenesis of susceptibility to bacterial infection remains unknown, multiple predisposing factors including defective opsonisation, altered T cell function, altered IgG concentrations have been proposed. Albumin is worthy of note because it plays an important role in the host immunity as one of the important antioxidants. This study was condcted to investigate whether hypoalbuminemia contributes to declined immunity in INS. Methods: Thirty fi ve heparinised blood samples were obtained from 4 patients with INS aged 10-15 years at the time of relapse or remission. Serum levels of reactive oxygen metabolites (ROM) and biological antioxidant potentials (BAP) were measured by spectrophotometry using a novel analyzer, FRAS4 (Diacron Int., Italy). Serum albumin level (HSA) was measured by colorimetric assay. Relationships between HSA and ROM, BAP, and ROM/BAP ratio were studied. Results: HSA varied from 0.8 to 4.4 g/dL, and correlated with ROM and BAP (p<0.01). Correlation coeffi cient between HSA and BAP (r s = 0.74) was higher than that between HSA and ROM (r s = 0.43). In addition, HSA correlated inversely with ROM/BAP ratio (r s = 0.31, p<0.05).

Our results suggest that low HSA contributes to a declined immunity, and agree with the fi nding that children with INS complicated by SBP had lower HSA. It is, therefore, worth studying the effi cacy of infusion of albumin in INS with hypoalbuminemia, not to increase osmotic pressure but to prevent SBP by supplying depleted BAP, because the benefi cial effect in liver cirrhosis has been confi rmed.

The Objectives: The paediatric population suffering from end-stage renal disease (ESRD) is growing, but current reliable epidemiological information on ESRD in children is missing from many countries. Aim of the study was to examine the occurence of ESRD in Slovak children; to compare it with earlier data on Slovakia and with data from neighbouring European countries; and fi nally to explore aetiology and treatment modes. Methods: Over the years 2005-2008 data on incident and prevalent cases of ESRD in children from all four tertiary paediatric centres in Slovakia were collected. Incidence and prevalence rates were calculated per million age related population (pmarp) and per million total population (pmtp 

Abstract# 381 Objectives: Recent studies suggest that extracellular matrix metalloproteinases (MMPs) and their tissue inhibitors are involved in many kinds of kidney diseases.

We assessed the role of MMPs and TIMPs in patients with minimal changes nephrotic syndrome and with moderate proteinuria due to other glomerulopathies.

The study group consisted of 60 patients subdivided into group IA-n=20 patients with nephrotic syndrome, group IB -patients with nephrotic syndrome after proteinuria subsided; group II (n=20) with moderate proteinuria. The control group (K) contained 20 healthy children in the same age and sex. Urinary levels of estimated MMP-2 and MMP-9 and their tissue inhibitor TIMP-1 and TIMP-2 were determined using immunoenzymatic method. Results: Our study revealed increased levels of MMP-2 in patients from I and II group compared with controls, and did not differ between group IA and II. Urinary concentration of TIMP-1 was higher in both groups (I and II) in comparison to healthy controls. TIMP-2 levels were higher only in group II. MMP-2/TIMP2 and MMP-9/TIMP-1 ratios among group IA, IB, II and group K were comparable. Increased MMP-2/TIMP2 ratio was positively correlated with protein level in the urine of children from group I. Positive correlation was found between MMP-2 and proteinuria in group II.

We demonstrated that the imbalance between MMP-2, MMP-9, TIMP-1, TIMP-2 may lead to fi brosis. Estimation of MMPs and TIMPs concentrations may be a predictive factor of kidney fi brosis and can be used for monitoring EMC renal accumulation. Objectives: Guidelines vary on LTB screening before pediatric immunosuppression (IS), particularly regarding reactivation (TBr) risk and new screening tests (interferon-gamma release assay, IGRA). Methods: CE analysis with Decision Maker® comparing universal (univ) skin testing (PPD), IGRA, and targeted (trgt) PPD with a risk questionnaire (Quest) over 1 year, using a 5 year-old with new-onset NS as base case, model inputs from the literature, Medicare, and published infl iximab data, and 2010 US$ and quality-adjusted life years (QALYs) as outcomes metrics (Table 1) . Results: Trgt PPD screening was the least costly and most effective option (Table 2 ) over a range of parameter values, including TBr rates. For IGRA to be superior, PPD and IGRA sens would have to be <20 and >85%, respectively, and LTB prevalence >60%. (n=101), mHU disappeared in 10 chidrden and proteinuria developed in 4, whose biopsy fi ndings were IgA nephropathy (IgAN) in 2, thin membranous disease (TMD) in 1 and normal in 1. No patient showed deteriorated renal function or hypertension during follow-up.

In PU group (n=42), half of them had orthostatic PU and 8 (19%) had elevated creatinine level at fi rst visit. The histological fi ndings of 11 cases with persistant PU were IgAN in 4, FSGS in 3, mesangial proliferative glomerulonephritis (mesPGN) in 1, Alport syndrome in 1, lupus nephritis (LN) in 1 and C1q nephropathy in 1.

In mHU+PU group (n=57), the histological fi ndings of 35 cases with persistent PU were IgAN in 18, FSGS in 3, Alport syndrome in 2, LN in 6, mesPGN in 2, MPGN in 3, and TMD in 1.

In conclusion, invasive studies including renal biopsy are considered for the patients with persistent proteinuria but not for the patient with mHU, since their clinical fi ndings after more than 3 years were favorable.

Association Objectives: To investigate the prevalence of symptoms of lower urinary tract in school children.

Methods: Cross-sectional study, including 739 children aged 6 to 12 years of age, enrolled in three public schools of Minas Gerais, with different socioeconomic levels. Symptoms were evaluated by the instrument "Voiding Dysfunction Scoring System" proposed by Farhat et al, which was adapted for children's language, in which the cutoff points by the score as an indicator of urinary tract dysfunction were greater than 6 for girls and greater than 9 for boys. Children with score indicating symptoms received an educational booklet on the function of the lower urinary tract and also a referral for medical evaluation.

The symptoms of dysfunction of the lower urinary tract were detected in 161 (21.8%) children. The urinary symptoms more common in children with increased score were holding maneuvers (37.2%), diurnal urinary incontinence (26.7%) and urgency (23.6%). The symptoms were more frequent in girls (p <0.001) in children between 6 and 8 years of age (p <0.028) and at school with the lowest social level (p <0.001). Constipation was the most prevalent symptom (30.7%), regardless of the value of the score. Emotional stress factors were present in 28.4% of children studied.

The study showed a high prevalence of lower urinary tract symptoms in children of school age children, higher in younger children and girls belonging to lower social class. These symptoms should be considered and investigated carefully in routine pediatric consultations. Objectives: Discussing and sharing the methodology of a successful Italian-Nicaraguan cooperative project,started in 2000,for the care of kidney and urinary tract diseases (KUTD).

The project,fi nanced by 2 Italian and Nicaraguan Foundations and 2 Italian Public Institutions and developed with the continuous involvement of local Hospital Authorities and Ministry of Health, included:1-training of medical and nurse team 2-provision to Managua Unit of devices for the diagnosis and treatment of all KUTD 3-implementation of a pediatric clinical-web network between Milan, Managua and 6 Nicaraguan dept hospitals; 4-collection of epidemiological data on KUTD over the territory. Results: The Managua Unit is now able to care for all kind of KUTD, both from the diagnostic(lab-instrumental exams,biopsy) and therapeutic point of view(medications,dialysis,LDR kidney transplant)and is the pediatric nephrology reference centre for all Nicaragua. The local staff progressively increased with three new positions:a pediatric urologist and 2 nephrologists. A network of dept hospitals (covering 61% of pediatric population) has been established for a prompt diagnosis and treatment of all basic KUTD. The KUTD registry contains now 5567 pts. Since 2007 annual costs for medications for pediatrc transplant and hemodialysis are progressively covered by Nicaraguan Health Authority.

The cooperation between public and private associations and a strong involvement of Health Institutions and local staff has been the key of success of the project.

Fractional Objectives: Recently a correlation between increased excretion of sodium and hypercalciuria in children have been revealed. The value of fractional excretion of sodium (FENa) have not been investigated in children with nephrolithiasis and in children with nephrocalcinosis. Methods: Three groups of children were studied: 40 children with nephrolithiasis (aged 6.6±4.8 years), 30 children without chronic and acute deseases (aged 7.0±5 years), and 6 children with nephrocalcinosis (aged 5.2±3.9 years). Spot urine sample and serum specimen were analyzed. Stone samples (30) were examined by X-ray diffraction method. Results: We found that FENa was signifi cantly increased in children with nephrolithiasis (0.99±1.1%) compared with control group (0.36±0.2%, p<0.05). Excretion of calcium was increased in the nephrocalcinosis group (1,5±1,9, p<0,05). Fractional excretion of potassium and other solutes didn't differ in all groups. Correlation (p<0.05) between FENa and excretion of oxalate, fractional excretion of calcium, and phosphate were revealed in children with nephrolithiasis only. That allowed to suggest tubular sodium transport disturbance in nephrolithiasis. FENa didn't depend from stone composition. There wasn't increased sodium excretion in children with nephrocalcinosis, that could be infl uenced by therapy.

The result indicates that increased FENa is associated with nephrolithiasis, suggesting that a reduction of salt intake may prevent stone recurrence in children.

The Local teams in the collaborating centres populate those datasets, including histology which will be analysed by a central pathology steering group. The information collected is owned by RaDaR and given to the DSWG in link-anonymised form. Additional data that is generated by the DSWG following analysis will be returned to RaDaR at the end of the study.

Conclusions: This project illustrates a comprehensive model for study, management and dissemination of information for rare diseases on a National basis, overcoming governance, data protection and IT hurdles in a coordinated strategy.

Diagnostics Objectives: Rituximab (RTX), an anti-CD20 monoclonal antibody, is a hopeful option for steroid-dependent (SDNS), frequent-relapsing (FRNS) and steroidresistant nephrotic syndrome (SRNS). We conducted a national survey of off-label RTX use for refractory NS. Methods: Questionnaires were sent to 130 hospitals in Japan. Sixty-seven hospitals responded and 10 hospitals reported its use. Objectives: Diabetic nephropathy (DN) is the leading cause of renal failure in the world and is becoming more prevalent in the young. It has previously been considered a hyperglycaemic complication directed against the renal microvasculature. DN has a natural history dominated by albuminuria and we have previously demonstrated that human podocytes are uniquely insulin sensitive. We therefore set out to examine the biological role of insulin signaling to the podocyte in the kidney.

To determine whether insulin signaling to podocytes affects glomerular function in vivo we generated transgenic mice with specifi c deletion of insulin receptors from their podocytes. Results: These animals develop signifi cant albuminuria with increased matrix production, mesangial expansion, loss of podocyte cytoskeletal architecture, thickening of the glomerular basement membrane and glomerulosclerosis. Thus, elimination of insulin signaling in podocytes alone is suffi cient to recapitulate the major features of DN despite normoglycaemia. Our studies suggest that the mechanism of this effect involves dynamic control of actin cytoskeleton remodeling in the podocyte by insulin receptor signaling. Conclusions: Collectively this shows the critical contribution of altered insulin signaling in the podocyte in the pathogenesis of DN. Thus, therapies that improve the insulin sensitivity of this cell could have great potential in treating this major global health problem. Objectives: CD2AP is a multifunctional adaptor molecule, and plays a crucial role in the kidney where it is essential for ultrafi ltration functions of the slit-diaphragm complex. Knockout of CD2AP in mice leads to nephrotic syndrome. The role of CD2AP in podocytes is not known. We established for the fi rst time CD2AP mutant podocytes from a child with diffuse mesangial sclerosis due to a missense mutation in exon 16 of CD2AP. Methods: Human conditionally immortalised meseangial cells were used as controls.

Results: CD2AP mutant podocytes displayed a fi broblast like morphology with spindle shape, WT podocytes grow as groups of cells that preserve cell-cell contacts. Nephrin, CD2AP, Podocin and WT1 were expressed at comparable level in both cell lines. Mesenchymal markers (fi bronectin and α-SMA) were signifi cantly overexpressed in mutant podocytes, while in WT they were expressed at low but detectable levels. PAX-2 was expressed in CD2AP mutant cells and not in WT podocytes. Mechanistically, WTIP was shown to translocate to the nucleus in mutant cells. Conclusions: Fibroblast like morphology of the mutant cells, disturbance of the cell sheets and overexpression of EMT markers and re-expression of PAX2 indicate that the CD2AP mutation may have led to podocyte dedifferentiation. We showed that normally CD2AP recruits WTIP to the cell membrane, whereas a mutant involved in human disease shuttles it to the nucleus where it suppresses WT1-dependent gene expression to permit simplifi cation of podocyte phenotype. This is a novel role for CD2AP, with implications for its role in development and progression of glomerular disease. Objectives: TGF-β plays a central role in renal fi brogenesis. We previously showed in vitro that PI3K and TGF-β stimulated Smad3 activity lead to collagen I expression, and now aimed to extend these fi ndings to an in-vivo model. Results: Balb/c mice treated with ADR (0.15 mg, iv) showed massive albuminuria and advanced global glomerulosclerosis, as described by others. We found increased renal mRNA for TGF-β1, type I collagen, fi bronectin and smooth muscle α-actin (αSMA). Immunoblotting showed phosphorylation of Akt, a downstream marker for PI3K activity. A PI3K inhibitor, LY294002 (25mg/kg, i.p, 2x/week), prevented ADR-induced histological changes, albuminuria, and upregulation of fi brosis markers. The γ isoform of the PI3K p110 catalytic unit was selectively increased in ADR kidneys and in cultured mouse podocytes treated

with TGF-β. 129sj mice treated with ADR showed similar nephrotic changes such as albuminuria and hypoalbuminemia, and azotemia, but histological changes were more consistent with human focal segmental glomerulosclerosis (FSGS) than those in Balb/c. Smad3 phosphorylation, and nuclear localization shown by immunostaining, implicated TGF-β. A TGF-β inhibitor, soluble human TGF-β type II receptor (sTβRII.Fc), protected 129/sj mice from ADR-induced histological changes and diminished collagen I and αSMA mRNA expression, but did not prevent proteinuria. Smad3 activation seen in ADR-treated 129/sj was ameliorated by sTbRII Fc. Conclusions: These results suggest that ADR-induced nephropathy in 129/sj mice shows features similar to human FSGS and suggest that TGF-β and PI3K are promising targets for intervention in kidney fi brosis. Objectives: We examined whether daily administration of maintenance prednisolone during intercurrent infections, reduces relapse rates in children with frequently relapsing nephrotic syndrome (FRNS). Methods: Patients with FRNS (>2 relapses in 6 mo) eligible for therapy with prolonged alternate day (ad) prednisolone (0.5-0.7 mg/kg ad) with/ without levamisole (2 mg/kg ad) were eligible.Randomization done to group I (Intervention) or C (Control). During presumed viral infections,patients in Gr.I were advised to take the same dose of prednisolone every day for 7 consecutive days; patients in Gr. C continued to receive ad prednisolone. Relapse (3+ proteinuria for 3 consecutive days) was considered infection-associated if it occurred within 2-wks of onset of viral illness. Patients were followed up for 12-mo. Results: For 100 patients included, Gr. I (n=50) showed signifi cantly lower relapse rates (mean difference 0.9 episodes/pt/yr; 95% CI 0.4-1.4) at 12-mo.The number of infection-associated relapses was signifi cantly lower in Gr. I (mean difference 0.7; 95% CI 0.3-1.1).The increase in relapses with the number of infections was more apparent in Gr.C. An adjustment for occurrence of infections, showed that daily administration of prednisolone during viral infections independently resulted in 59% reduction in rate of relapses (rate ratio 0.41; 95% CI 0.3-0.6). The intervention is likely to reduce the number of relapses to < 3 in 12 months, for one out of six children treated. Conclusions: Daily administration of maintenance doses of prednisolone, during viral infections, signifi cantly reduces relapse rates and the proportion of children with FRNS. Objectives: Elucidation of the role of the p38 MAPK/MK-2 signaling pathway in mediating nephrotic syndrome-related podocyte injury induced by puromycin aminonucleoside (PAN). Methods: PAN-induced injury of cultured podocytes was monitored by measuring cell viability and integrity of the actin cytoskeleton. Podocytes were treated with specifi c inhibitors, SB203580 and C23, to block the activity of p38 MAPK and MK-2, respectively. Electrophoretic analyses were used to verify the effects of these drugs in podocytes. Results: PAN-induced loss of podocyte viability was signifi cantly prevented by inhibition of p38 MAPK or MK-2. In addition, inhibition of p38 MAPK or MK-2 almost completely prevented PAN-induced disruption of podocyte actin fi laments. At the molecular level, both inhibitors markedly reduced basal phosphorylation of the MK-2 substrate Hsp25, suggesting that prevention of PAN-induced injury indeed was due to inhibition of this signaling cascade. In addition, we found that exposure of podocytes to serum albumin strongly activated p38 MAPK/ MK-2 signaling which suggests that this pathway may contribute to cell damage resulting from protein overload. Conclusions: Collectively, these data suggest a direct role of p38 MAPK/MK-2 signaling in mediating podocyte injury resulting from treatment with PAN or serum albumin. Consequently, specifi c pharmacological inhibition of p38 MAPK or MK-2 offers a potentially novel therapeutic strategy to prevent or treat podocyte injury in nephrotic syndrome. (25%)]. Ultrasonography showed increased echogencity in 9(20%) and increased kidney size in 9(23%). Histology showed the following: FSGS 32(64%) with 7(14%) having the collapsing FSGS; tubulointerstitial nephritis 8(16%); HIV immune complex disease 3(6%); membranous glomerulonephritis 3(6%); mesangial proliferative glomerulonephritis 1(2%) and others 4(8%). Objectives: Puromycin aminonucleoside (PAN)-induced nephrosis is a welldescribed model of human idiopathic nephrotic syndrome because PAN injection into rats results in increased glomerular permeability with the characteristic ultrastructural changes in glomerular epithelial cells (GEpC; podocytes) similar to human nephrosis. We investigated the role of zonula occludens (ZO)-1 and oxidative stress on PANinduced podocyte phenotypical changes and hyperpermeability in vitro. Methods: Rat GEpCs were incubated in media containing various concentrations of PAN. The phenotypical changes of ZO-1were analyzed by confocal imaging, Western blotting, and PCR. We also examined in vitro permeability and oxidative stress level. Results: Morphological assessment revealed that in vitro PAN not only induced the ultrastructural changes of GEpC, such as shortening and fusion of microvilli, but also separated the intercellular gaps and linear ZO-1 resulting in increased intercellular permeability. Oxidative stress level after PAN treatment was markedly higher than that of basal level. PAN induced the inner cytoplasmic translocation of ZO-1 protein and also reduced ZO-1 protein amount and mRNA expression in a dose-dependent manner. These phenotypical changes of podocyte caused by PAN were augmented by the antioxidative effect of vitamin C.

We suggested that the glomerular hyperpermeability caused by intercellular ZO-1 disturbances via oxidative stress would be the mechanism of proteinuria in experimental PAN-induced nephrosis. Objectives: In animal models of nephrotic syndrome (NS), glomerular fi ltration of plasminogen and activation to plasmin has been reported to activate the epithelial Na + channel (ENaC) and may cause sodium retention. Our aim was to investigate if plasminogen/plasmin (PLAS) is present in urine from patients with active NS and whether excretion is altered at remission. Methods: Patients with idiopathic NS were included. Urine samples were collected at the day of inclusion and again at remission. Total PLAS concentration was determined with ELISA. Urinary protease activity was examined in 17 of 20 patients by gelatin zymography and western blotting for PLAS in urine was performed in selected patients.

Results: A total of 20 patients (7 girls) with mean age 9.1±3.2 yrs were included. Median nephrotic urine PLAS was 1780 ng/ml (range 297-22700) versus 72 ng/ ml (range 25-143) in urine at remission (p<0.001). Zymograms showed protease activity at 70-80 kDa in urine in 10 of 17 patients compared to 3 of 17 patients at remission. Western blots showed a 75kDa protein compatible with plasmin in 5 of 10 tested patients and products at 90-100 kDa in 5 patients. At remission, all 10 urine samples were negative.

Our results demonstrate signifi cantly elevated excretion of PLAS and proteolytic activity in urine during active NS compared to remission. PLAS may be involved in sodium retention in NS and whether PLAS is indeed causing ENaC activation is currently tested in experiments using an in vitro patch clamp technique.

Predictors Objectives: We examined the involvement of glomerular RAS activation in the glomerular injury in anti-GBM GN of rats with AT1R antagonist Candesartan. Methods: Progressive anti-GBM GN was induced in rats. Nephritic rats were divided into two groups and given daily oral doses of vehicle or ARB in drinking water until day 28. Age-matched rats without nephritis were used as normal controls. Results: Treatment with ARB signifi cantly improved proteinuria. Vehicle-treated nephritic rats developed crescentic GN accompanied by marked macrophage infi ltration and the enhanced expression of glomerular angiotensinogen (AGT), Ang II and Nox2 (NADPH oxidase) on days 7 and 28 of GN. ARB improved glomerular alterations and had a signifi cant inhibitory effect on the levels of these parameters. Western blot using isolated glomeruli also showed that increased AGT and Nox2 expression was signifi cantly attenuated by ARB treatment. Enhanced O2production in nephritic glomeruli was also decreased by ARB treatment. Moreover, ELISA using the supernatant of glomerular culture revealed that the level of glomerular Ang II and TGF-β production was signifi cantly increased in vehicle-treated nephritic rats, while ARB treatment signifi cantly inhibited those production on day 28 (p<0.05).

Conclusions: This study indicates that increased glomerular Ang II production play an important role in the progressive glomerular injury through the induction of oxidative stress and TGF-β expression, and suggests that ARB treatment provides a novel therapeutic strategy for inhibiting the progression of glomerular injury in GN.

Neurological Objectives: Central nervous system involvement, the most frequent extrarenal complication in hemolytic uremic syndrome (HUS) includes reversible posterior leukoencephalopathy syndrome (RPLS) and thrombotic microangiopathic (TMA) lesions, diffi cult to differentiate since presentation is similar. Methods: We report the case of a 4 year old boy, diagnosed with atypical HUS due to an hybrid factor H. Results: He progressed to end-stage renal disease despite plasmatherapy and underwent bilateral nephrectomy because of uncontrolled hypertension. Three days after, he had complex seizures with normal blood pressure, normal blood count and normal magnetic resonance imaging (MRI) which recurred 1 month later. 8 months later, he had a third episode of seizures, with hemoglobin of 10g/ dl without schizocyte, low haptoglobin of 0.2g/l, and moderate thrombocytopenia (platelets 98.10 9 /L). He remained deeply confused during 2 days. The third MRI showed bilateral symmetrical hyperintensities of the cerebral pedonculae, caudate nuclei, putamens, thalami, hippocampi and insulae suggesting thrombotic microangiopathy secondary to a relapse of HUS rather than RPLS, usually occipital and asymmetrical. He was treated with daily plasma exchanges (PE) that led to a complete neurological recovery within 2 days although hypertension had remained uncontrolled. After 10 sessions, PE were replaced by weekly plasma infusions. The fourth MRI 10 weeks after was normal and clinical examination remained normal, except for high blood pressure. Objectives: Epidemiological data show a signifi cantly better renal prognosis for women with chronic glomerular diseases compared to men. Regarding the critical role of podocytes for pathogenesis, we studied the infl uences of estrogens on podocyte function. Methods: Estrogen receptor (ER) expression in cultured podocytes and renal biopsies of different diseases was examined on mRNA and protein level. Cultured murine podocytes were treated with Puromycin (PAN) to induce apoptosis, Estradiol (E2) alone or both combined. Apoptotic cells were quantifi ed by FACS analysis and Hoechst nuclear staining. Results: ERα mRNA and protein were detected in podocytes. In vitro, E2 reduced PAN-induced apoptosis by 27% (p=0.01, FACS). Hoechst staining showed similar results (57% reduction of apoptosis, p=0.01). In human biopsies, low ER expression in podocytes was found in 45-55% of controls and MCGN patients (gender-independent). In sclerosed glomeruli of FSGS patients, ER was detected in a signifi cantly higher percentage of women compared to men (89% vs. 33%, p=0.01).

The presented data show that podocytes contain specifi c target structures for estrogenic action. Experimentally induced apoptosis as in-vitro model of chronic glomerular diseases can be signifi cantly reduced by E2. In FSGS, ER positive glomeruli are found to be enriched in women, suggesting a selection of ER positive cells by E2 infl uence. These fi ndings represent a new model explaining gender differences in glomerular diseases. Objectives: This study aimed to determine glomerular disease frequencies in Macedonian children and it represents the basis for future studies. Methods: All native renal biopsies (January 1996 to December 2008) were reviewed, but only glomerular diseases were analyzed. The diagnosis of each case was based on histological, immunopathological and clinical features. As our Clinic is the only pediatric nephrology centre in the country, the results of this study relate to the whole country.

Results: A total of 82 patients < 15 years (mean age 8.59±3.9 %) were included in the study. Primary glomerular diseases were diagnosed in 65 biopsies (79%) and secondary in 17 (21%).The most common primary diseases were minimal change disease (35%), mesangiproliferative glomerulonephritis (17%), immunoglobulin A nephropathy (14%), focal and segmental glomerulosclerosis (12%), membranous glomerulonephritis (8%), crescentic glomerulonephritis (3%). Postinfectious glomerulonephritis (GN) represented 8% of the diagnoses if included as primary GN. In the group of secondary glomerulonephritis, Henoch Shonlein nephritis corresponded to 59% of the entire series and lupus nephritis to 29%. Alport syndrome was found in 12%.

The distribution of glomerular diseases in the pediatric age group at R.Macedonia is similar to that described in other countries with some differences. This study illustrates the importance of having a regional register for renal diseases in children. Results: NS was present in 15cases (16.5%) of renal patients. Five patients had presumed minimal change/steroid sensitive NS. Ten patients had either steroid resistant of frequently relapsing NS. Eight of these underwent renal biopsy. Only one specimen was analyzed locally and was reported as "showing features of membranous nephropathy and minimal change". The subsequent 7 specimens were analyzed at Red Cross Children's Hospital and showed: minimal change (2 cases), mesangioproliferative glomerulonephritis (2 cases), and focal segmental glomerulosclerosis (1 case). In 2 cases, specimen was not representative of anatomic site. Two patients could not afford the courier cost and we had to pay ourselves. Second line treatment included cyclosporine (1case) and cyclophosphamide (2cases). Drug level monitoring was not possible in the patient who underwent cyclosporine. Conclusions: Management of NS can be challenging in resources-limited countries but a collaborative approach involving hospitals from developed or emerging countries and developing ones can be benefi cial to the latter.

Ophtalmological (4,4%) . In pediatric ophthalmologists' evaluation just 10 patients (10,7%) have presented discrete posterior subcapsular cataract. None of them presented glaucoma or intraocular pressure alterations. Conclusions: Ophtalmological evaluation of children and adolescents with nephrotic syndrome should be performed by professionals specialized in pediatric ophthalmology. Nevertheless, in this group, the prevalence of cataract was lower than that reported in literature for chronic use of steroids(30%). Objectives: Focal and segmental glomerulosclerosis (FSGS) is one of the most common causes of chronic kidney disease. The objective of this study was to describe the response to treatment in children with FSGS and to correlate it with the clinical an laboratory parameters.

The present work is a retrospective study of 113 children and adolescents between 1 and 18 years with pathological diagnosis of primary FSGS, admitted to the Unit of Pediatric Nephrology at the Federal University of Minas Gerais between 1970 and 2007 Results: All patients were initially treated with oral prednisone: 37% achieved complete remission and 22% partial remission. Hematuria (p=0,001) and higher age at the presentation (p=0,034) were associated with poor response to prednisone. Eighty four patients were treated with oral cyclophosphamide (CPM): 50% achieved complete remission and 19% partial remission. Those with nonresponse to initial prednisone had 3.8 times more risk of nonresponse to CPM. Cyclosporin (CsA) was given to 27 patients: 81.5% achieved remission, including 71% of the steroid-resistant group. The level of initial proteinuria was a risk factor associated with nonresponse to CsA in multivariate analysis. Renal survival was better in the group of steroid-sensitive patients (p=0,0002). Response to the CPM or CsA were related to preservation of renal function.

Our study showed that initial hematuria is associated with poor response to steroids and CPM represents an alternative for steroid-resistant primary FSGS. Objectives: Disorders of complement are associated with atypical HUS and MPGN 2. Animal models, C3 deposition GN in humans and reports of complement activation independent of antibody in MPGN 1 led us to the hypothesis that complement dysregulation may be a pathogenic mechanism in MPGN 1.

We present a patient with MPGN 1 who did not respond to conventional therapy and had low C3. Based on our hypothesis we instituted a trial of plasma infusion.

A 7 year old male presented with an acute nephritis with low C3. A renal biopsy was consistent with MPGN 1. He was initially treated with steroids and enalapril and his creatinine, albumin and proteinuria normalised. Over the next 5 years he had multiple relapses and only achieved partial remission despite treatment with steroids, enalapril, losartan, mycophenolate mofetil and tacrolimus. Throughout this time his C3 remained low. In view of his persistent nephrotic state, his low C3 and his poor response to treatment we considered that he may benefi t from treatment targetting complement dysregulation. Genetic testing for MCP, Factor H, Factor I and Factor B showed a MCP variant of unknown signifi cance. Further genetic and functional testing is awaited. A trial of plasma infusions commenced and after one month his C3 normalised. By three months his albumin had also normalised and his proteinuria improved.

The clinical and pathological features of our patient and the response to plasma infusion would be consistent with a disorder of complement regulation. We recommend that genetic testing and trials of plasma infusion be considered in patients with MPGN 1 who have persistently low C3 and are unresponsive to conventional therapy. Results: A total of 9 patients (8M; 1F) were identifi ed. The median age at onset and presentation of nephrotic syndrome was 48 (16-108) months and 67.3 (17-110) months respectively. The structural abnormalities identifi ed in these patients were horse shoe kidney in one, unilateral PUJO in 2, bilateral grade V VUR in one, bilateral grade II VUR in 1 patient. One child had crossed fused ectopia of left kidney, another had unilateral dysplastic kidney and 2 patients had single kidney. The course of nephrotic syndrome was reviewed. Four children had a FRNS/SDNS course and received oral cyclophosphamide for 3 months. Two children had steroid resistant disease (1 MCD; 1 FSGS on biopsy) and received cyclosporine for attaining remission. Three subjects were infrequent relapsers. The presence of underlying structural abnormality in the above patients was associated with a diffi cult course of nephrotic syndrome in 67% (6/9) patients. Alternative drugs were required in all these children besides steroids. Age at presentation was younger in the Finnish group; proteinuria was higher in the Finnish and DMS groups (p 0.01 and 0.04 respectively). Extra-renal manifestations were common in the Finnish and DMS groups: 10 cases of major GI disorders, 8 major neurologic complications and 5 major congenital heart disease. The mortality rate was 26% (7) in the Finnish group at median follow-up of 49 months with 4 children going into remission at 2-28 months. These patients had normal renal function 1-15 years later. The mortality rate was 36% (4) and 9% (1) in the DMS and FSGS groups at median follow-up of 55 and 26 months respectively. One patient died from fatal haemorrhagic complications from the renal biopsy. Conclusions: NS during the fi rst two years of life is a rare but serious disease, with a higher mortality and complication rate in the Finnish and DMS groups.

Specifi Objectives: Antiphospholipid syndrome(APS) nephropathy was the result of vascular obstruction of renal vessles. There was not routinely accepted an immune glomerulonephritis as one of primary antiphospholipid syndrome nephropathy. We present the immune glomerulonephritis with nephrotic syndrome, of primary antiphospholipid syndrome.

A 11 year old female child was admitted with generalized edema and severe visual disturbances with nearly blindness for 1 month. BP was 120/80mmHg, On the physical examination, there was eyelid swelling and pitting edema. There's no cranial nerve abnormality. On the fundus examination, both optic disc severe swelling and blunted disc margin with star shaped hard exudate on macula were noted. Serum protein and albumin were 4.4/2.1 g/dL. Serum cholesterol and triglyceride were 342/224g/dL. CBC was as follows: WBC 8,360, Hb 11.7g/dL and platelet 130,000/mm 3 . PTT was 50.6sec (control 32.3sec).

Brain MRI was normal. A diagnosis of primary APS was made on the basis of antiphospholipid syndrome IgG/IgM (+/-), Cardiolipin IgG/IgM (+/+), ANA(-), anti-DNA Ab(-), anti-sm(-), anti-ENA(-), C3/C4 98.6/19.8mg/dL. Kidney tissue showed diffuse proliferative glomerulonephritis with subepithelial electron dense deposits. She has been treated with coumadin, steroid and cyclosphosphamide, but still remained proteinuria without edema. Her vision was much improved.

We suggest that there should be a need for concern about the possibility of the development of immune glomerulonephritis in primary antiphospholipid syndrome.

An Objectives: The management of Henoch-Schonlein purpura nephritis (HSPN) is still controversial. Some studies reported steroids or immunosuppressants were effective, but indication of these drugs remains uncertain. Here we retrospectively examine the effi cacy of treatment in HSPN. Methods: Renal biopsy was performed in patients with nephrotic syndrome or persistent proteinuria > 3 months. We divided patients into two groups and treated according to severity. GroupA (31 patients), or the mild case such as ≤ gradeIII(ISKDC) and serum albumin (ALB) > 2.5g/dl, was treated with ACE-I and/or ARB. GroupB (19 patients), or the severe case such as > gradeIII or ALB ≤ 2.5g/dl, received multiple combinated therapy(MCT) consisting of prednisolone, azathioprine/mizoribine, warfarin and dipyridamole. We assessed the effect of therapies by urinary protein/creatinine ratio and estimated GFR(EGFR). Results: After each treatment, all the patients disappeared proteinuria and kept EGFR normal. The mean period of proteinuria after therapeutic intervention was 7.4±1.9 and 6.4±1.2 months, respectively. There were no adverse events of therapy.

Conclusions: These fi ndings suggested that the MCT was used effectively in the treatment of severe case. It is necessary to emphasize that the MCT should be used for only severe cases because mild cases will improve enough with ACE-I/ ARB.

Renal And the staining of α-SMA and TGF-β1 were observed. Conclusions: RGC-32 protein located in cytoplasm of proximal tubular and distal renal tubular in renal tissues fi rstly. The expression of RGC-32 was positive correlation with the degree of renal lesions and tubulointerstitial injury in children with IgAN, and it is concordance to the expression of α-SMA and TGF-β1. Thus it was hinted that RGC-32 may participate in the course of TGF-induced EMT of renal tubular cells. Objectives: Cyclosporine A (CsA) is an effective agent for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can also be complicated by renal toxicity. Because no biochemical markers from urine or blood samples have yet been established for detecting CsA-induced renal injury to date, repeated renal biopsies are therefore required for all patients with long-term CsA treatment. The purpose of the present study was therefore to detect early change of CsA nephropathy (CsAN) using blood samples. Methods: Several biochemical markers were analyzed in an attempt to examine the renal function in 24 patients with FR-SDNS who had been treated with CsA. Those included serum cystatin C and indoxyl sulfate, as well as creatinine and b2-microglobulin. of MYH9 mutation and hematologic evaluation were performed. A renal biopsy was performed on the elder brother. Immunofl uorescence microscopy (IF) added anti-alpha1 to 6 antibody of collagen type 4, monoclonal antibody against nonmuscle myosin heavy chain IIA (NMMHC-IIA) and antibody of nehrin and podocin. Results: In blood, electron microscopy (EM) of leukocyte inclusion bodies revealed parallel fi laments. IF showed NMMHC-IIA antibodies in 1 leukocyte inclusion bodies. These fi ndings consistent with MHA and they were identifi ed to express the MYH9 mutation, D1424H. In the elder boy, the histology revealed mesangial proliferative glomerulonephritis with granular deposits of IgG and C1q. EM showed the dense deposits located in subendothelial cells, mesangial cells and Bowman's capsule. NMMHC-IIA antibodies were localized in podocyte and endothelial cells. The expression of nephrin and podocin was normal. His condition had a temporary effect for steroid therapy, but became refractory proteinuria later. Conclusions: About immune complex-related nephropathy, we concluded that an infl ammatory mechanism occur separately from MHA. (3 cm) in the left, and multiple metastases in the lungs. Ambiguous genitalia was absent. Prior to surgery, he was treated with a series of chemotherapy with vincristine, actinomycin D and adriamycin. Then, heavy proteinuria and hypertension developed. Serum Alb decreased to 2.1 g/dl as the proteinuria increased to 30 in Pr/Cr (mg/mg) ratio. He underwent right nephrectomy after two monthlong chemotherapy. The proteinuria gradually decreased, and the serum Alb increased to 3.6 g/dl by 2 months after surgery. His renal function was stable. His hypertension was controlled by ARB and Ca blocker, and nephrectomy seemed also effective. Genetic analysis of the tumor revealed a homizygous mutation of R390X in WT1 gene. Results: FGS was noted in 3 out of 50 glomeruli. Accumulation of podocytes around the collapsing tuft, and hyaline droplets in podocytes were observed,suggesting the podocyte injury. EM revealed foot process effacement in some capillary walls.

The proteinuria in our patient was presumably induced by the chemotherapy and hypertension, which damaged the podocytes, already predisposed to some fragility due to WT1 mutation.

Mycophenolate Methods: Children with IGAN proven by biopsy performed as soon as possible after the disease onset. CT protocol: Metilprednisolone 1g/1.73 m2 BA for 3 consecutive days, followed by oral prednisone (P) 0.5mg/Kg/day for a month and then on alternate days for the following 5 months. Results: Treated group (P): 15 children; Control group (C) 12 children age matched. Kidney biopsy was performed after 1.7 (group P) and 1.6 years (group C) from the presumed IGAN onset. Pathological classes (Lee) in group P and C, respectively: class I: 1 and 3 pts, class II: 9 and 8 pts and class III (3 and 3 pts). Mean follow up: 26.8 and 29.8 months in IgAN and C groups, respectively. GFR, A.P. and serum C3/IgA ratio were not different between the groups both at start and at the end of followup and inside each group at the start compared with the end of follow up. Mean basal proteinuria: 0.64 g/day in group P and 0.16 g/day in group C; at last followup mean proteinuria was signifi cantly decreased in group P (0.21 g/day) and unchanged in group C (0.26g/day). During followup 2 pts of group P and 5 pts of group C showed some episodes of gross hematuria. At last follow up, microscopic hematuria was absent in 5 pts of group P and in 1 pts of group C; in the remaining ones it, estimated as +1 to +5, was, as mean value, + 2.5 in group P and +3.75 in group C.

Our study indicates that CT administered as soon as possible with respect to the IgAN onset can infl uence positevely the course of the disease. (16), partial responders PR (19) and nonresponders NR (8) and also subdivided based on relapse/remission of NS. IL-10 and TNF-alfa was measured and IL-10/TNF-alfa ratio was determined after LPS stimulation and/or dexamethasone. Percentage of IL-10/TNF-alfa increase by dexamethasone was calculated. Results: Signifi cantly higher IL-10/TNF-alfa after steroids was observed in relapsing RE than in PR and/or NR. Dexamethasone concentration of 10 -8 M

signifi cantly increased IL-10/TNF-alfa ratio in relapsing RE in comparison to relapsing NR (p=0.049). Higher dexamethasone concentration of 10 -7 M increased this ratio in relapsing RE in comparison to both PR and NR (p<0.05). These changes were not observed in the remission. Objectives: To evaluate the factor which affect to the concentration of mizoribine (MZR) with idiopathic nephrotic syndrome patients. MZR is the inhibitor of inosine monophosphate dehydrogenase, which was made in Japan. The treatment of steroid-dependent nephrotic syndrome (SDNS) and frequently relapsing nephrotic syndrome (FRNS) has been considered. In Japan, we usually use cyclophosphamide, cyclosporin A and MZR with or without predonisolone. It was reported that the concentration of MZR was necessary to control proteinuria above 3.0 µg/ml. Methods: We retrospectively analyzed 17 cases of childhood-onset idiopathic nephrotic syndrome (9 males and 8 females, FRNS: 1, SDNS: 14, steroid resistant nephrotic syndrome: 1, FSGS: 1), and studied 60 samples. Objectives: Evaluation of clinical and laboratory response to cyclosporine in pediatric patients with SRNS.

The study evaluated retrospectively clinical and laboratory data of 36 pediatric patients with SRNS, previously treated with an 8-week course of prednisone 2 mg/kg/day with no response, followed by a 3-month course of cyclophosphamide 2 mg/kg/day. After this period these patients were treated with cyclosporine 5 mg/kg/day for 6 months, followed by 2,5 mg/kg/day for more 6 months, completing a one-year course of cyclosporine. 10 patients were excluded from this study (9 have had adverse effects and 1 was still under treatment).

Patients were submitted to clinical and laboratory evaluations once-a-month while they were using cyclosporine. different recipes in induction therapy, divided into CTX group, MMF group and other drugs group. According to whether receive renal biopsy and pathological classifi cation, divided into group A (type I, II), group B (type III, IV), group C (type V) and group D (no biopsy). According to different outcomes, patients were divided into remission group (complete and partial remission) and ineffective group (non-response). Results: Average follow up time is 19.98 months, maximum 11.5 years, 20 cases follow up more than 3 years. 2, 5 and 10 years renal survival rate is 88.9%, 84.0% and 60.0% respectively. Univariate analysis showed following 4 variables were correlated to prognosis: different pathological types (P=0.005), heavy proteinuria at onset (P=0.003), different recipes (P=0.001), and irregular treatment (P=0.001). No biopsy group and type V group have a relative worse prognosis. The prognosis of MMF group is better than the other groups. Heavy proteinuria at onset and irregular treatment are two infl uence prognosis factors. In multivariate analysis, it was confi rmed that only irregular treatment (OR=9.955) was signifi cantly associated with outcome. Conclusions: For SLE patients with evidence of nephritis, renal biopsy should be suggested, in order to guide treatment and predict prognosis. Regular treatment plays a more important role to prognosis than different therapies, especially in developing country.

Objectives: In a multicenter, randomised placebo controlled trial we compare the effi cacy of three months to six months corticosteroid therapy for a fi rst presentation of nephrotic syndrome (NS). Methods: Children 1-16 years old with a fi rst episode of NS are randomised into 2 treatment regimens with equal cumulative dosage of prednisolone. Regimen A: 6 weeks prednisolone 60 mg/m 2 daily, then 6 weeks prednisolone 40 mg/m 2 on alternate days, followed by 12 weeks placebo. Regimen B: 24 weeks prednisolone starting at 60 mg/m 2 daily in a gradually tapering scheme. Objectives: Studies have shown that microalbumin excretion is related to increased risk for progression of hypertension and cardiovascular diseases in adults. Furthermore, in healthy children, it has been previously demonstrated that albumin excretion rate (AER) is correlated with higher blood pressure and is higher in blacks than in whites. However, there are limited data available looking at the relationship between AER and urine endothelin-1 (ET-1). ET-1 is known for being a potent vasoconstrictor and plays a major role in blood pressure regulation. In the kidneys, we know that urine ET-1 acts on the microvessels, but it is still not clear as to its role in renal function.

We evaluated AER and urine ET-1 in healthy adolescents participating in a study of stress-induced change in urine ET-1. A total of 86 healthy, normotensive adolescents, 42 blacks and 44 whites (50% males, 50% females),

aged 15-18 years, participated in a 5-hour testing protocol. Subjects started with 2 hours of rest prior to a 1-hour period of mental stress and followed by a 2-hour rest period. Urine microalbumin and ET-1 levels were measured immediately before and after stress. Results: In blacks, the stress induced change in ET-1 correlates with lower AER (P = 0.008, R 2 = 0.16). This relationship is not as signifi cant in whites (P = 0.375, R 2 = 0.018). This means ET-1 regulation contributes to 16% of AER in blacks and only 1.8% of AER in whites.

These fi ndings suggest that renal ET-1 is a major player in renal function and may be used as marker for renal injury in blacks. Objectives: The purpose of our study was to work out a method of quantitative assessment of IHC staining in kidney glomeruli taking only cell volume into account. Methods: 31 needle renal biopsy specimens from children with nephrotic syndrome were studied. Histologically minimal change disease, mesangioproliferative nephropathy, and focal segmental glomerulosclerosis were diagnosed. IHC staining of specimens for podocalyxin was performed. Images of 5 glomeruli in the section were taken at a x400 magnifi cation and further analyzed using WCIF ImageJ 1.42q software. The glomerular area (GA) was calculated by drawing a region of interest around the glomerulus. Capillary lumen area (CLA) of glomerulus was calculated using color based thresholding. Cellular area (CA) was determined as a difference between GA and CLA. The number of positive pixels was calculated using color based thresholding with a spectrum specifi c for the diaminobenzidine staining. Staining density was stated as a number of positive pixels per square micrometer of CA. Results: Podocalyxin immunostaining was found in glomeruli along the membranes of podocytes and endotheliocytes. Staining density was calculated for each specimen and the mean was 29.68±17.78 pixels per square micrometer. Mean staining density calculated per GA was 20.09±12.74. This data shows that staining density per CA is more variable than per GA. This may have infl uence on the results of investigation of IHC markers expression in glomeruli.

The described method may be useful for quantitative assessment of IHC staining in glomeruli and for its comparison in different glomerular lesions. (94%) were steroid responsive and 7 (6%) were steroid resistance. Meantime of response was 11.5 days and meantime to relapse was 15.3 weeks. Among the initial steroid sensitive group 45% were infrequent relapser, 18% frequent relapser and 10% were steroid dependent. 22% were non relapser. Amongst 6 renal biopsy in steroid resistance cases, 3 had minimal changes, to had FSGS and 1 had DMPG on histology.

Conclusions: ISKDC recommendation is as effective in our study population as that of other.

Of the 48 who completed the course of IVCY therapy, 8 later died, and 26 of the 40 survivors were available for evaluation for this study. Of the 26, only 9 patients were free of medication; the other 17 patients still required medication to control their disease. Overall 23 patients died, and patient survival rates at 2, 5 and 10 years were 93%, 78% and 66%, respectively. Conclusions: Three years of IVCY treatment in severe lupus nephritis provides initial good remission, unfortunately, it is not permanent, since if long term outcomes are considered, some patients died and some patients required a return to medication.

Pharmacokinetics of Cyclosporin (Neoral) Objectives: To verify possible differences in the pharmacokinetic parameters of Cyclosporin (Neoral) between nephrotic children on remission and relapse.

Methods: This study evaluates the pharmacokinetic parameters of CSA during remission and relapse of the nephrotic syndrome with the same dose of CSA, with normal renal function and C0 between 50 and 150 ng/ml who achieved complete remission. We evaluated the 12-hour area under the curve (AUC 0-12 ). The patients were hospitalized to measure CSA trough level (C0) and after 1, 2, 4, 6, 8 and 12 h of the drug administration. We calculated the AUC 0-12 for each patient. Results: We studied 10 children (mean age at presentation, 3.0 ± 1.6 years). There was no signifi cant difference between creatinine clearance during remission and relapse (p=0.2). We detected no signifi cant differences at any points of the curve during remission vs. relapse; AUC 0-4 (on remission r= 0.95 and on relapse r= 0.93) and C2 (on remission r=0.86 and on relapse r=0,80) were the parameters that correlated best with AUC 0-12 . We were not able to detec a signifi cant correlation between cholesterol, albumin and 24 hour proteinuria with AUC 0-12 .

We found no signifi cant differences at any points of the curve during remission vs. relapse. Our study suggests C2 and AUC 0-4 could be the more adequate parameters to fi t CSA dosage in nephrotic children. We observed no signifi cant infl uence of the serum levels of cholesterol, albumin or hematocrit or proteinuria on the pharmacokinetics of CSA. More controlled studies should be done to verify the target value of C2 to maintain remission with minimal toxicity. Objectives: Glucocorticoid induced osteoporosis is caused by decreased bone formation and increased bone resorption. So, in our study we evaluated the effects of long and high dose steroid on bones in children with nephrotic syndrome. Methods: We performed dual-energy X-ray absorptiometry of the spine in 37 children with nephrotic syndrome and correlate the result of dexa with duration of steroid, age of onset, type of NS, laboratory data. Results: Bone mineral density(BMD) at lumbar spine (L2-L4) was measured by dual energy X-ray absorptiometry in 37 children with nephrotic syndrome with mean age (8.6 ± 3.3) years, according to their response they were classifi ed as steroid responsive wh (67.6%) and the steroid resistant (32.4%), the steroid responsive group is subdivided into frequent relapsers (FR) (21.6%) and steroid dependent (46%). The mean age of onset were (4.9 ± 2.8) years, mean duration of steroids (3.6 ± 2) years, (89.2%) were on steroid and (10.8%) were off steroid, as regards laboratory data all were within normal ranges. As regard BMD patients were divided into 3 groups: Mild osteopenic group (48.7%), severe osteopenic group (18.9%) and the remaining group are normal (32.4%). By comparison the severe osteopenic group have older age of onset (P = 0.003) and 85.7% of this group are steroid resistant., Z-score shows -ve correlation with age of the patients (r = -0.501) and +ve correlation with serum albumin and alkaline phosphatase, r = 0.408, r = 0.427 respectively. 

Results: Elevated urinary OPG clearly distinguished AR from all other analysis groups. Using an ROC curve, 236.8 pg/mL was chosen as an optimal cutoff, which resulted in perfect sensitivity (1.0) and high specifi city (0.77). The PPV using this cutoff was 0.7 and NPV was 1. While OPG identifi ed patients with AR, elevations in CXCL9 and CXCL10 identifi ed patients experiencing either AR or BKN.

Conclusions: These noninvasive assays identifi ed patients experiencing posttransplant graft infl ammation due to either AR or BKV. The ability to noninvasively screen for either AR or BK nephritis provides ease and fl exibility for ongoing pediatric transplant monitoring, identifying patients in need of attention and possible intervention by a transplant nephrologist. Objectives: Kidney transplant in patients with aHUS is associated with a poor outcome because of recurrent disease, especially in patients known to have a factor H mutation. Long-term prophylactic plasma exchange (PE) and combined liver-kidney transplant have prevented graft loss caused by recurrence. However, the mortality associated with liver transplant is not negligible, and prophylactic PE requires permanent vascular access and regular hospitalization and exposes the patient to allergic reactions to plasma. Eculizumab is a high-affi nity humanized monoclonal anti-C5 antibody that prevents C5a and MAC formation. We report the use of eculizumab instead of PE to prevent aHUS recurrence after transplantation in a patient with highly plasma-dependent aHUS related with a CFH mutation. Methods: A 17 years old girl with aHUS associated with CFH mutation and ESRF since the age of 3 y presenting with multiple cerebral vascular stenosis, PE dependency and severe allergic reactions to plasma after a third kidney transplantation received 900 mg of eculizumab intravenously 2 h after the last PE.The same dose of eculizumab was given weekly for 4 weeks. Thereafter, 1,200 mg was given every 2 weeks indefi nitely. Anti-meningococcus vaccination was performed prior to eculizumab. Results: Twelve months after the start of eculizumab and PE discontinuation, no relapse had occurred and plasma creatinine level is stable at 128 micromol/L. Methods: Early (G1,n=9) and late (G4,n=5) generation male Terc KO kidneys were transplanted into female WT recipients and sacrifi ced after 6 wks. Results: G4 transplants showed signifi cantly more interstitial fi brosis (IF) and a tendency to more glomerulosclerosis. Telomere-dependent senescence markers were signifi cantly higher in G4: more H2AX pos. tubular cells (fi g.1A), higher p21 expression (fi g.1B). p16 refl ecting telomere-independent senescence, even though higher in G4 prior to tx, was not different after tx (fi g.1C).

Conclusions: Mild transplantation stress induced telomere-dependent and -independent senescence. This was more pronounced leading to increased IF in G4 mice that refl ect the situation of an old donor. But p16 increases in G1 suggest that senescence can be triggered even in ideal donors. As this tx model enables us to mimic the human situation, it should be used to explore factors that reduce regeneration even in optimal transplants. Results: LVMi at HD onset was 101±5.7 g/m 2 . After one year on HD there was a signifi cant decrease (89 4.5 g/m 2 ) and stabilization until renal transplantation (86.3±4.7 g/m 2 ). Six months post transplant a dramatic re-increase was noticed (104±9.0 g/m 2 ) followed by a decrease until two year post RT (83.5 g/m 2 ). After the second year post transplant LVM developed very heterogeneously (99±10.0 g/ m 2 ), and a subset of patients had moderate to severe LVH. In the HD period LVM was positively correlated with mean, systolic and diastolic blood pressure (MBP, SBP, and DBP), but not with Hb levels. However, in the post RT period only correlation with SBP was identifi ed, but surprisingly not with DBP, MBP, or renal function. There was no difference in patients with CyA or FK based immunosuppression. The risk to have a LVH one year post RT was partially predictable by LVM at the beginning of the HD period (p<0.01). Conclusions: LVH decreases during HD and re-appears after the second year post transplant in about 50% of patients in particular those with severe LVH at the beginning of the HD period. ESA use was documented in 9 patients all of whom were treated with MMF (9/31 -29%). Conclusions: Early post transplant anaemia is common in paediatric renal allograft recipients. There appears to be an increased prevalence of anemia at Day 20 in those receiving MMF compared to AZA. The increased use of ESA's in this patient population may refl ect an alteration in prescribing practice over time.

Given the prevalence of anemia in both groups we advocate the continued use of ESA's in the peri and postoperative transplant period. Objectives: Focal segmental glomerulosclerosis(FSGS) is common but often recurs after renal transplant, so we designed this study to investigate the risk factors of recurrence in childhood FSGS and its optimal management. Methods: We reviewed the medical records of 50 renal transplants between Jan.1990 and Jan.2010 at out center in 46 children with primary FSGS. Results: The median age of onset was 5.8 years and the presentation was nephrotic syndrome(NS) (n=37) or asymptomatic urinary abnormality. They received kidney transplants from living donors (n=33) or cadaver donors (n=17).

In 18 allografts (38%), excepting 2 patients recurred after 3 years, FSGS recurred at a median of post-transplant 2 days. When compared the grafts with recurrence with those without recurrence, the recurrence group had more NS as the initial presentation (P=0.09). Recurrence did not signifi cantly affect the graft survival. The statistically signifi cant risk factors of recurrence were not found in our study, although recurrence group had more NS as initial presentation. Plasmapheresis, methylprednisolone pulse therapy and cyclophosphamide were tried, and the remission was achieved in 14 of 18 patients.

In Korean children with FSGS, recurrence occurred in 38% and the graft survival was poorer than total population of kidney transplant. Initial presentation of FSGS with NS was the important risk factor of recurrence, but the donor type or the duration of dialysis was not signifi cant in contrast to the previous studies.

Retrospective review was performed on data of patients who underwent kidney transplant between Jan 1999 to Jan 2010. Demographics, clinical data, estimated glomerular fi ltration rate (eGFR) and graft survival rates were compared between adolescents (12-22 years) and children ( <12 years Objectives: This is a report of graft outcomes with ATG induction compared with interleukin-2 receptor antagonists. Methods: 52 paediatric kidney cadaveric transplanted patients Group A, 26 patients received antithymocyte globulin ATG ( 3 mg/kg/dose) at days 0 and 1,3,5, (dose 12mg/kg). Group B: 26 patients received Basiliximab at days 0 and 4. Maintenance therapy tacrolimus, target 6-10 ng/ml, mycophenolate mofetil (MMF), target 2-4 ng/ml, and tappered steroids until 6-9 months. Delayed graft function (DGF), acute rejection, and renal function the 1, 3, 6 and 12 months were examined. Results: 24 over 26 patients of groupA presented immediate diuresis and 8 over 26 in Gorup B. CMV or EB disese were not registered in both groups. BK polyomavirus were positive in 3 patients in Group A and in 2 patient in Group B and were controlled reducing immunosuppression.

One year patient survival was 100% in both groups. One-year graft survival was 100% (group A) and 95 % (group B). Two patients in group B presented acute humoral rejection and 1 patient loosed the graft. At the 1,3, 6 and 12 months of follow-up no differences in serum creatinine or proteinuria were appreciated between both groups. Conclusions: ATG induction reduces delayed graft failure and acute rejection rate. DGF could be related to innate immunity activity, enhanced in brain-death donors and diminished under ATG treatment.

ATG treatment could also induce T regulatory cells and modulate the immune response related to the ischemic-reperfusion phenomena due to brain-death kidney donor procurement.

second living related renal transplantation (fi rst from father, second from mother) for end-stage renal failure (ESRF) on haemodialysis for steroid resistant nephrotic syndrome due to focal and segmental glomerulosclerosis (FSGS).

The recipient and donor (mother) blood groups were A and B respectively with anti-B titres of 1 in 8 pre-transplantation. His renal transplant was performed successfully without further antibody removal or surgical complications. Six months post-transplant his renal function has stabilised with eGFR of 62mls/min/1.73m 2 . He does not have clinical evidence of recurrent FSGS post-transplantation with negative anti-B titres and donor specifi c antibodies, low but detectable CD19 count and no evidence of viraemia (having required oral valganciclovir as CMV mismatch). He had a normal transplant renal biopsy at ten days post-transplant without evidence of acute rejection and one focal segmental sclerotic lesion seen on protocol biopsy at three months.

In view of the reported success of ABOiRT, clinicians must consider this option in ESRF patients whose parents have previously been excluded as living donors. Objectives: PRCA is an isolated arrest of erythropoiesis which has been reported to occur in patients with chronic kidney disease (CKD) secondary to the development of antibodies against erythropoiesis-stimulating agents, usually erythropoietin alpha. Methods: To report the successful outcome of pre-emptive living related renal transplantation in a 6.5 year old boy with CKD due to renal dysplasia who developed anti-erythropoietin antibodies (anti-epo Abs) resulting in transfusiondependent PRCA. Results: He was on subcutaneous erythropoietin beta for two years but two months prior to transplantation, his haemoglobin was 4.6g/dl with reticulocytes of 0.1% requiring his fi rst blood transfusion. His bone marrow aspirate and trephine biopsy one month before renal transplantation confi rmed PRCA (other causes excluded). His erythropoietin beta was discontinued and he had high titres of anti-epo Abs. He received his second blood transfusion before his renal transplant. He underwent a living related renal transplantation from his maternal uncle (mismatch 011) using triple immunosuppression. His anti-epo Abs reduced further two weeks post-transplantation but required his third blood transfusion. He is six months post-transplant with haemoglobin of 11.6g/dl, ferritin of 363mcg/l, negative anti-epo Abs and good renal allograft function (eGFR of 74mls/min/1.73m 2 ). Conclusions: This is the fi rst reported paediatric case where renal transplantation has successfully treated transfusion-dependent PRCA secondary to neutralising anti-epo Abs against erythropoietin beta.

Maintaining Objectives: Young children are at increased risk of graft loss due to thrombosis. A likely major contributing factor relates to peri-operative perfusion of the donor kidney. At our unit every effort is made to maintain peri-operative systolic blood pressure above 100mg Hg for all patients regardless of age and size of patients. Methods: An audit of peri-operative BP monitoring and fl uid management in children transplanted between 1/1/06 and 31/12/08 was undertaken. Results: 32 children received a transplant, 11 of whom were under 5 years of age. Graft survival at 1year was 94% in patients under fi ve and 95% in patients over fi ve. Patient survival at 1year was 100% in both groups. One graft was lost in each cohort due to early thrombosis. Patients under the age of 5 were more likely to require blood products and inotropes to maintain systolic BP above 100mg Hg compared with those older than 5 years.

Comparison by age of the support required to maintain a systolic blood pressure >100mmHg peri-operatively in renal transplantation Conclusions: Our data suggest that optimisation of systolic BP in younger children requires close attention to the need for blood products and inotropes compared with older children.

Timing Objectives: Basiliximab is a non-depleting recombinant chimeric murine/ human monoclonal anti-interleukin-2-receptor antibody used for prevention of acute organ rejection in renal transplant recipients. We analysed 2 groups of pediatric kidney recipients (R): one including patients treated with Basiliximab and corticosteroids, the other recipients receiving exclusively corticosteroid pulse induction. Immunosuppression in both groups was based on CsA or FK506 ± MMF ± corticosteroids. Methods: We retrospectively analysed 130 paediatric kidney recipients: 78 (60%) treated with Basiliximab (R/D age 12±7/15±8 yrs, HLA mismatches 3.6±0.9) and 52 (40%) with only corticosteroid pulses (R/D age 15±5/13±10 yrs, HLA mismatches 3.1±1.06). Results: Patients who received IL-2 receptor-antagonist induction had lower incidence of acute clinical rejections (p=0.0049). Steroid withdrawal at 12 and 24 months post-transplant was possible in a larger number of patients receiving Basiliximab then corticosteroid pulses (p=0.0015 and p=0.0001). No differences in terms of organ survival, creatinine clearance, acute/chronic subclinical lesions at protocol biopsies, mean corticosteroids dosage, CsA/FK506 blood through levels, and growth at 6, 12 and 24 months post-transplant. Conclusions: Our results highlight that the use of IL-2-receptor antagonists allows an early corticosteroid withdrawal and confi rm an higher effi cacy of Basiliximab in preventing clinical acute rejections. We found no differences regarding the prevalence of post-transplant short or long term acute and chronic histological lesions. Objectives: Focal segmental glomerulosclerosis (FSGS) is the most frequent acquired disease resulting in ESRD in children. The risk of recurrence in the allograft is reported to be ∼30%. Podocine gene mutations are well-known to be a protective factor for FSGS recurrence. The aim of the study was to compare the clinical features of FSGS in renal recipients with or without podocine gene mutations.

We sequenced all eight exons of NPHS2 in 18 Turkish pediatric renal recipients. All patients had biopsy-proven primary steroid-resistant FSGS. 3 of 18 children received living related donor. All patients were evaluated with clinical, laboratory and demographic features. Results: 11 of 18 patients had NPHS2 gene mutations while 7 had no mutation. The mean age at onset was signifi cantly younger in mutation positive group. The mean age at renal transplantation was 10.53±3,64 yr. Biopsy proven FSGS recurred only in 2 patients with homozygote mutation in the early and late period. Late FSGS recurrence resulted with graft failure. The patients without podocine gene mutation had no recurrence for 2,3-6,6 yr. The age onset of FSGS was the only difference between groups. Conclusions: In Conclusion, we detected FSGS recurrence in 2 children who had causative homozygote NPHS2 gene mutations.Podocine gene mutation positive group had early onset FSGS. Other clinical features are similar for patients with or without NPHS2 mutations. Contraversy to literature patients carrying homozygote mutation may display early or late onset FSGS recurrence. FSGS patients must be followed-up with urine protein in transplant clinics.

Systematic Anticoagulation Following Renal Transplantation: Benefi ts and Risks P. Niaudet, E. Balzamo, G. Guest, M. Charbit, O. Boyer, R. Salomon. Pediatric Nephrology, Hôpital Necker-Enfants Malades, Paris, France. Objectives: Vascular thrombosis is responsible for at least 10% of early graft failures. Factors that increase the risk include a thrombophylic state, a recipient less than 5 years or less than 15 kgBW, a donor aged less than 5 years, several arteries, history of prior transplantation or vascular rejection. The aim of this study was to retrospectively analyze the benefi ts and risks of systematic anticoagulation in renal transplant recipients. Methods: From March 2001 to January 2007, 116 renal transplants were performed in 112 patients aged 1.2 to 21.5 years (mean 10.3). Two patients with early graft loss were excluded as well as 4 pts who did not receive anticoagulants. Exaparine was started between 12 and 24 hours after surgery and continued during 3 weeks in 110 children. Results: One graft was lost from artery thrombosis in a 18 year old patient following a cadaveric transplantation (0.89%). The graft had two arteries. No other risk factor was present and antiX a activity was 0.41. Ten patients had bleeding complications (perirenal or intra renal hematoma, wound hematoma, digestive bleeding) necessitating blood transfusion ( Objectives: The number of acute rejections and infections after pediatric kidney transplantation could not be reduced in the last years. In order to improve these parameters, we investigated a new immunosuppressive protocol in a prospective, controlled trial. Methods: Twenty children were treated with Basilximab, CsA (trough-level=C0 200-250 ng/ml) and Prednisolone at the time of Tx. After 2 weeks, CsA dose was reduced to 50% (C0 50-100 ng/ml, after 6 months: 50-75 ng/ml) and Everolimus (1.6 mg/m²/d) was started (C0 3-6 ng/ml). Six months after Tx, Prednisolone was stopped. Results: All 20 protocol biopsies showed no acute rejection or borderline fi ndings. had ATN was found in 4 of 8 indication biopsies, two had borderline fi ndings, one 5% interstitial fi brosis, one was normal. Mean GFR 1 year after Tx was 71 ± 25 ml/min/1.73m². The number of serious adverse events, infections and gastrointestinal problems was signifi cantly lower as compared to a historic age matched cohort that was treated with normal-dose CsA, MMF and steroids. Without CMV-prophylaxis, only 2 primary CMV infections were seen despite a donor-CMV+, recipient-CMV-constellation in 10/20 children. Conclusions: In pediatric kidney transplantation primary immunosuppression with low dose CsA, Everolimus and steroid withdrawal after 6 months leads an absence of acute rejections and a reduction in infections, especially CMV.

Timing of Ureteric Stent Removal in Paediatric Renal Transplant Recipients (RTR) P. Patel, J. Olsburgh, S.D. Marks. Nephrology, Urology and Transplantation, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Nephrology, Urology and Transplantation, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom. Objectives: Transplant ureteric stents (TUS) reduce the incidence of urological complications post-operatively. However, there can be complications with TUS (UTI, migration, haematuria and pain) which may be related to how long the TUS remains in situ. We assessed two techniques for TUS removal in an aim to determine optimal timing for TUS removal. Objectives: BK Virus associated nephropathy (BKVAN) causes allograft dysfunction and graft loss.Treatment of BKVAN is not well defi ned.We report our experience with kidney transplant recipients, who developed BKVAN, the diagnosis, study and treatment. Methods: Retrospective case report. Results: Two female, aged 8, 11 and 9 yr respectively developed BKVAN at 24, 12 and 3 months after kidney transplant.All received, polyclonal antibody therapy, MMF, tacrolimus and prednisone. Both female have insidious rise of creatinine, at 12 and 24 months.The male had an abrupt rise in creatinine at the third month. All treated initially with intravenous methylprednisolone.BK viral DNA measure from plasma by quantitative PCR assay and allograft biopsy consistent with polyoma virus infection.Imunosuppression reduction and IVIg was administered. The fi rst patient also received ciprofl oxacin 4 weeks, but had progressive deterioration of renal function and BK PCR failed to decrease. Second patient received 6 course of cidofovir (0.25-0.50 mg/kg/dose), every 2 wk, and plasma viral load decreased to undetectable levels. Serum creatinine remained on average close to 2 mg/dl. Tthird patient BK viremia cleared with the immunnosuppression reduction and IVIg administration, but a month after had a rise in plasma BK viral DNA, initiating cidofovir.The plasma BK PCR began to decrease. Conclusions: BKVAN should be suspected in patients with graft dysfunction, especially those who receive aggressive induction. Cidofovir is effective in reducing BK viral load. Randomized, controlled trials regarding the most appropriate antiviral therapy are needed.

Effi cacy of Sirolimus in Children with Chronic Allograft Nephropathy T. Seeman, 1 L. Podracka. 2 1 Dept. Pediat., Medical Faculty, Charles Univ., Praha, Czech Republic; 2 Dept. Pediat., Medical Faculty, UPJS, Kosice, Slovakia (Slovak Republic) .

Objectives: Sirolimus (SRL) is a potent immunosuppressant acting on the mTOR receptor with no adverse effects on renal function. Methods: We retrospectively evaluated the effi cacy and safety of SRL in 8 pediatric renal transplant recipients (6 girls/ 2 boys),who were 10.90 ± 4.01 years at time of transplantation and received a CNI as the core immunosuppression. Results: The patients were converted to SRL at 3.8 ± 2.5 years posttransplantation. The indication for SRL therapy was chronic allograft nephropathy, in 3 of them due to calcineurin toxicity (37.5 %). Sirolimus was initiated at 1 to 2 mg/m 2 given orally once daily. Mean follow-up after the switch to SRL was 1 year. Patients and graft survival was 100%. No children experienced acute rejection episodes. The mean serum creatinin (Cr) at the time of switch to sirolimus was 179.6±63 umol/l and decreased slowly during receiving SRL to 147.6 ±53.3 umol/l after 12 months of treatment (n.s.). We observed slight improvement in glomerular fi ltration rate assessed using the Schwartz formula at 1 month (42.4 ±12.6 v.s. 54.9 ±23.8 ml/ml/1.73m2), which was sustained thereafter. No serious adverse events were observed. Proteinuria increased signifi cantly (p<0.05), however, no signifi cant changes in serum cholesterol and trombocytes have been found. Although the prevalence of hypertensive patients decreased during follow-up, it was not signifi cant. Conclusions: Sirolimus as a concomitant immunosuppressant is effective in chronic allograft nephropathy. Further data are needed in pediatric transplantation recipients to best assess the role of TOR inhibition in corticosteroid and/or calcineurin inhibitor-sparing regimens. Objectives: The Cylex Immunoknow® Assay (IK) measures cell mediated immunity in transplant recipients by determining ATP generation in PHA stimulated CD4 cells. We report a single center study of the utility of IK to predict rejection or viral infection and to identify predictors of IK levels. Methods: Retrospective analysis of 67 renal transplant patients (pt) ages 1-20 from 11/06 to 12/09. Results: 494 IK were performed on 67 pt ( average 5±3.6 tests/pt). The mean IK level was 362±151 ng/ml comparable to the adult normal range of 226-525 ng/ ml. In pt < 12 years IK was 350±149 and in pt >12 years was 369±152 (p>0.5). African American (AA) pt had lower IK compared to all other races (p=0.004). IK correlated with wbc (r=.38, p<0.0001), and with tacro dose (r= -0.186, p=0.0002), but did not correlate with MMF dose (r= -0.12, P=0.8), or CyA dose (r= -0.009, p=.9) Careful chart review identifi ed 14 rejection events (R) in 11 pts. 7 R had IK within 30 days prior to R. 1/7 had IK>nl, 1/7 had IK< nl. There were 12 EBV (IK 310±166), 8 BK(IK 328±115), 8 CMV (IK 298±123) infections in pts. Most were subclinical and diagnosed only by positive PCR. The UNC Hospital Lab cost to perform IK is $298 and the charge is $364 per test. The total 494 IK tests cost $147,212.

The Cylex Immunoknow® test in our hands is not useful to predict rejection or viral infection. It correlates best with the total wbc. In contrast to other studies, age was not signifi cant. The IK level was signifi cantly lower in AA pt. This fi nding needs further exploration.

Objectives: Chylous ascites (CA), an unusual complication of pediatric abdominal surgery has scarce data on management. We describe approach & outcome in 2 children with CA post renal transplant. Methods: We performed a retrospective chart review of 2 patients who developed CA after renal transplant to evaluate clinical course & management. Results: A 7 years old boy underwent a transperitoneal deceased donor renal transplant (DDRT) with venous anastomosis to portal vein. CA was diagnosed 2 months later on paracentesis. Feeds were changed to Medium Chain Triglyceride (MCT) formula. Ascites re-accumulated so feeds were held & total parenteral nutrition (TPN) was provided with some benefi t. CA recurred after introduction of low fat diet & paracentesis was done 4 times over 3 months. At 5 months post-DDRT a peritoneo-venous shunt (Denver) was placed. Shunt malfunction led to revision at 3 months & replacement after 4 months. To prevent malfunction, shunt was pumped frequently. A right sided chylothorax was managed symptomatically. At his last follow up at 16 months post-DDRT, CA was still present; otherwise he was doing well. Objectives: Renal transplantation in atypical hemolytic uremic syndrome (aHUS) associated with factor H mutation is characterized by a high rate of disease recurrence and subsequent graft loss. Here we present a case of prophylactic use of the humanized monoclonal anti C5 antibody Eculizumab in renal transplantation. Methods: Eculizumab monitoring was performed with the measurement of the terminal complement complex (TCC, C5b-9) in serum, plasma and after zymosan activation (ELISA). Results: In November 2008 a ten year old boy with aHUS associated with a heterozygous factor H mutation received a deceased donor kidney. Immunosuppression included prednisone, mycophenolate mofetil and tacrolimus. Before and after transplantation daily plasma exchange was performed until Eculizumab was available on day 10 after renal transplantation. 600mg i.v. was administered every second or every third week until present. Eculizumab managed to normalize platelets and complement immediately. For the treatment period of 16 months an excellent renal function, no recurrence and no need for plasma therapy can be reported. Objectives: MPA acts as a specifi c inhibitor of human lymphocyte proliferation by inhibiting IMPDH. Outcome after renal transplantation (RTx) has been shown to depend on pretransplant IMPDH activity in adults. We investigated a potential developmental regulation of IMPDH during childhood. Methods: We analyzed IMPDH activity in peripheral blood mononuclear cells (PBMC) by HPLC (normalized to the adenosine monophosphate (AMP) content of the cells) in 27 healthy infants (2-5.9 yrs), 31 school-age children (6-11.9 yrs), 22 adolescents (12-17.9 yrs) and 106 adults. Pretransplant IMPDH activity was obtained from 31 children and 81 adults with end-stage renal disease (ESRD). 12 hours PK/PD profi les of MPA and IMPDH after RTx were obtained in 17 children and 21 adults under MPA therapy. Results: IMPDH activity displayed high interindividual variability (coeffi cient of variation 40.5%). Median IMPDH did not differ signifi cantly in healthy infants (80.5 micro-mol/s/mol AMP), school-age children (60.6), adolescents (82.7) and adults (83.1). Pretransplant IMPDH was comparable in children (median 83.8) and adults with ESRD (92.0). IMPDH activity was inversely correlated with MPA plasma concentration in both children and adults. Conclusions: There is no pronounced developmental regulation of IMPDH activity in PBMCs in children. MPA inhibits IMPDH in children and adults to a comparable extent. The analysis of IMPDH activity prior to RTx may optimize MPA therapy.

Abstract# 512 Infl uence of Corticosteroid in Renal Transplantation G. Roussey, Y. Foucher, G. Guest, B. Ranchin, A. Maisin, R. Novo, J.L. André, S. Cloarec, C. Guyot. Pediatric Department, CHU Nantes, Nantes, France. Objectives: To evaluate the relationship between Corticosteroid (CS) and renal function and growth in pediatric renal transplant recipients. Methods: Data were extracted from the French pediatric database for renal transplantation (DIVAT). Children with a functional graft one year after a fi rst transplantation from Jan 1995 to Oct 2007 were included. CS was calculated as a Mean CS Dose per day (MCSD), and was divided into 3 classes: low (<0.1mg/ kg/d), intermediate ([0.1-0.2[mg/kg/d) and high (>0.2mg/kg/d). A decrease >30% of the initial eGFR was considered as a signifi cant degradation of renal function. A cox model with time-dependent variables was used to identify signifi cant factors associated with renal survival and dysfunction. Linear mixed model was used to study the growth evolution with time. Results: 535 children had a functional graft at 1 year. Only eGFR (>50ml/min) at 1 year (HR=3.7, p<0.05) and intermediate MCSD (HR= 2.6, p=0 .005) were signifi cantly associated with a better graft survival. A low MCSD was associated with a higher risk of renal function degradation. Growth was signifi cantly improved with a low MCSD and eGFR >50ml/min. Interestingly, growth was improved during the fi rst 3 years after renal transplantation for recipients younger than 6 years or older than 12 years, regardless of the MCSD. After this period, growth was improved only for low MCSD.

Conclusions: This observational retrospective study should be interpreted with caution. However, long-term administration of CS seemed to be positively associated with a better graft function, but with a negative association on growth. CS continuation or withdrawal has to be discussed for children after renal transplantation. 

Renal Transplantation in Immunologic High Risk Recipients P. Salas, V. Pinto, P. Zambrano, J. Grandy, B. Corta. Pediatrics Nephrology, Exequiel Gonzalez Cortes Hospital, Santiago, Chile. Objectives: Sensitized kidney recipients have increased significantly. New therapies modulating antiHLA antibody like intravenous immune globulin (IVIG), plasmapheresis (PP) and antiCD20 antibody (ab) have improved transplantation's chances. We report our experience with sensitized children. Pretransplant patients in waiting list for cadaveric transplantation received desensitization with IVIG and living donors received PP until negative crossmatch was achieved. After transplantation they received prophylactic PP and donor specifi c alloantibody (DSA) monitoring. Methods: Between May 1992 and February 2010, 126 patients underwent kidney transplantation. 9 had PRA> 40%. Median age 12.4 years, 3 females. 8 received graft from diceased donor and one from living donor; median cold ischemic time was16 hrs. Patients missmatch (MM) varied from 1 through 4MM. Patients pretransplant PRA ranges from 0-85%. Results: 2/9 had desensitization with IGEV; 5/9 treated with prophylactic PP after transplantation. All had induction therapy with antithymocite globulin and maintained on prednisone, MMF or azatioprine, and tacrolimus. In 3 an acute humoral rejection (AHR) was diagnosed. Two with graft dysfunction, 3 had DSA and 2 biopsyes showed positive C4D. They were treated with IVIG, PP and AntiCD20 ab. Patients have a mean follow up of 18 months. 100% of graft survival and mean creatinine of 0.94 mg/dl. Conclusions: New protocols with IVIG, PP and antiCD-20 antibodies have become sucessfull in lower anti HLA antibodies allowing renal transplantation. The same therapies can be used after transplantation for AHR. HLA sensitized patients should be monitored for increase risk of antibody mediated rejection.

Conversion from Twice-Daily Tacrolimus to Once-Daily Tacrolimus in Pediatric Renal Transplant P. Salas, V. Pinto, H. Corbalan. Nephrology, Exequiel Gonzalez Cortes Hospital, Santiago, Chile. Objectives: Non compliance induced from twice-daily tacrolimus (TAC) may adversely infl uence long term renal allograft outcome. We report 6 months follow-up of patients converted from TAC twice daily to once-daily (OD). Methods: In our department 129 patient have received a renal transplant. 70 are on prednisone, MMF and TAC. 9 patients receiving TAC twice-daily with stable graft function in the last 6 months were converted to OD TAC on a one to one (mg for mg) total daily-dose basis. Through levels of TAC were monitorized on day 7, 14, 28 after converted and then once a month. Target through level range between 4.5-6,5 ng/ml. Renal function and adverse events were analyzed. Results: Patients enrolled had a mean age of 14 years old, 7 female, non hypertension, mean creatinine 0.9 mg/dl, mean baseline TAC through level 5,7 ng/ml. Day 7 after conversion mean through level was 4.5ng/ml, 6 patients increased TAC daily dose. Day 14 after conversion mean trough level was 4.8 ng/ ml and 3 patients increased daily dose. One month after conversion mean through level was 4,8 ng/ml and daily dose remained stable in the 9 patients. Mean creatinine at month 2, 4 and 6 post conversion was 1.1 mg/dl. Mean through level at month 2, 4 and 6 were 6.36, 5.5, and 5.4 ng/ml respectively. During the follow up patients didn't showed hypertension, diabetes or hyperlipidemia. 2 patients had urinary infection and one patient had renal disfunction.

Conclusions: Children converted to OD TAC may improve compliance to medications. It seems that conversion should be done 1:1.1 (mg for mg) basis for the total daily dose. The OD TAC formulation did not suggest and increased risk of adverse events or greater incidence of acute rejection. Objectives: Infection with the varicella-zoster virus (VZV) is more dangerous in immunocompromised patients than it is in the general population. High doses of acyclovir and immediate reduction of immunosuppression, may improve the prognosis of severe forms, but multiorgan failure and death may occur despite early antiviral treatment. Methods: Here, we report four cases of VZV infection in children receiving steroid therapy and immunosuppressive drugs for renal allograft, idiopathic nephrotic syndrome, and systemic lupus. Results: The only clinical manifestation in three patients was general malaise, fever, and disseminated vesicular rash, whereas one patient also showed severe and diffuse visceral involvement with multiorgan failure. Adjuvant treatment with specifi c human varicella zoster polyclonal immunoglobulins (Varitect®) led to a dramatic improvement of VZV infection in all four patients.

In conclusion, Varitect® is probably a useful adjuvant therapy to acyclovir in declared and severe varicella in immunocompromised children. Objectives: Renal transplant is the treatment of choice in ESRD patients. Unfortunately complications are common due to previous metabolic disorders and side effects of immunosuppressive therapies.A great portion of these complications can be prevented if diagnosed at early stages.In the present study we try to evaluate the prevalence of hyperparathyroidism in kidney recipients. Methods: This study was conducted on 20 ESRD children receiving successful renal transplant.Serum Calcium, Phosphor,Alkaline phosphates,and Parathyroid hormone were assessed before and after transplantation,one and six months later. Results: Calcium levels were not signifi cantly different but PTH, Phosphor,Creatinine and ALP levels were signifi cantly different before and one month after transplantion :(p=0.005),(p=0.000),(p=0.000)and(p=0.001) respectively. Sera levels of Calcium, PTH, Phosphor,Creatinine and ALP were also signifi cantly different between the fi rst and sixth mo nth after operation with P values as followed: (p=0.014),(p=0.005), (p=0.000)and (p=0.005). Conclusions: These results showed screening sera of calcium, phosphor, PTH and ALP is helpful in hyperparathyroidism diagnosis. Data analysis showed the longerthe history of dialysis before transplant the higher prevalence of persistent hyperparathyroidism (p=0.0265). Renal transplant should be considered as soon as possible in ESRD patients to reduce the risk of persistent hyperthyroidism. Objectives: Decreased bone density is one of the most common complications in renal failure cases who underwent renal transplantation.Early diagnosis and treatment of osteoporosis and osteopenia may prevent these complication. Methods: Bone densitometry was done by DEXA method for 20 ESRD children and who had underwent renal transplantation. Bone densitometry was performed before, 6 and 12 month after renal transplantation. Z-score in children were used to compare the changes in bone density early after transplantation.

Results: All children were completed three stages, among them: 44.8% were female and 45.2% were male, mean age was 9.6 + 2.5 years. Decreased bone density (osteoporosis and osteopenia) in femur and spines was 42% and 29% respectively.Six months after renal transplantation, decreased bone density in femur and spines was 84% and 65% respectively that was signifi cantly different compare to pretransplantation time. 12 months after transplantation, decreased bone density in femur and spines, was seen in almost 68% for both that wasn't signifi cantly changed compare to the fi rst 6 month after transplantation. Conclusions: According to the prevalence of osteoporosis and osteopenia during the fi rst six months after renal transplantation we suggest prophylactic and curative interventions before and early transplantation. Objectives: To evaluate the outcome of early (<3months) and late (>3 months) episodes of corticosteroid resistant rejection (CRR) treated with anti-thymocyte globulin (ATG) in pediatric renal allograft recipients. Methods: Single centre retrospective study of 15 children (10 male), mean age 13.2 years (SD 5.16 y) who received 7-14 days of ATG for treatment of biopsy proven graft rejection which was unresponsive to methylprednisolone from 1994 to 2009. Results: Seven children received ATG for ER (median 26 days post transplantation, range 6-86 days) and 8 for LR (median 763, days, range 390-4223). All the children who developed ER were on a ciclosporin based immunosuppression (IS) at the time of the rejection episode. Following ATG, there was a signifi cant improvement in the 3 month (76umol/l, SD 21, p 0.03) and 6 month serum creatinine (74umol/l, SD 19, p 0.03) when compared with the value prior to ATG treatment (295umol/l, SD 224) in the ER group. In the LR group there was no signifi cant improvement in the creatinine at 3 (147umol/l, SD 45, p 0.17) or 6 months (158umol/l, SD 74, p 0.64) when compared to value prior to ATG (164umol/l, SD 59). At the latest review, the eGFR in the ER group was 72.3 ml/min/1.73m 2 (SD 33) (mean follow up 10.4 y, range 3-15y) compared to 37.7ml/min/1.73m 2 (SD 17.9) in the LR group (mean follow up of 1.2y, range 0.52-3.2y). During ATG therapy, three children developed septic shock like illness. There was one graft loss in the LR group. Conclusions: ATG therapy in CRR is associated with reversal of rejection and excellent graft outcome in children with ER while the benefi ts remain uncertain in those with LR. Objectives: The aim of this report was to show the development of Bartter-like syndrome in a renal transplanted boy treated with tacrolimus, mycophenolate and prednisone. Methods: CFC, male, 10 years old, previously diagnosed as endoextracapilar glomerulonephritis, was submitted to renal transplant in December 2009 from a deceased donor. Few days after transplant, serum creatinine normalized. Imunossupression included tacrolimus, mycophenolate and prednisone. At the second month after transplant, the patient presented vomits and dehydration accompanied by hypocalemia (1.3 mEq/L), hiponatremia (125 mEq/L), hypochloremia (84 mmol/L) and hypophosphatemia (1.8 mg/dL). He was hospitalized to investigate the etiology of these disturbances. Results: During hospitalization, the patient evolved with polyuria, polydipsia and hydroelectrolyte disorders that needed chronic replacement therapy with potassium salts (8mEq/Kg/day) and sodium chloride (3g/day). Laboratorial evaluation revealed elevation of urinary potassium excretion (excreted fraction, EF=36-86%), hyperphosphaturia (EF=23-36%), hypercalciuria (10-12mg/Kg/ day), metabolic alcalosis, hyperfi ltration (500ml/min/1.73m 2 ) and proteinuria (1.5g/day). Due to tubular function alterations, tacrolimus dose was reduced, determining a better metabolic profi le. However, the patient developed graft rejection (Banff IIb) that needed pulsotherapy and elevation of tacrolimus dose. Despite the recovery of renal function, metabolic disorders worsened. Conclusions: This is the fi rst report of Bartter-like syndrome due to tacrolimus.

Abstract# 521 Objectives: We report our experience of renal transplantation in children under 6 years of age over a 5 year period. Methods: Retrospective review of all children under the age of 6 years who had a renal transplant between 2004 and 2009. Results: Forty fi ve children (78%) male, aged 15-71 (median 37) months received a renal transplant with a median weight and height of 15 (9-25) kg and 87 (74.5-112) cm respectively. 53% had a kidney from a deceased donor. 92% were adult donors. 91% of the transplants were intra-peritoneal with 100% primary wound closure rate. Median hospital stay was 14.5 (7-60) days. 56% were admitted to PICU for a median duration of 24 (3-504) hours, for ventilation. 28% had a post-operative complication and 38% had an episode of acute rejection. Primary function was seen in 91% of the children, 7% had delayed graft function and 2% had primary non-function. The median creatinine at discharge and 5 years was 33 (12-126) and 94 (62-137) umoml/l respectively. The 2 year graft and patient survival was 98% and 100% respectively and the 5 year graft and patient survival were both 98%. Conclusions: Switch to CNI mono-therapy yielded stable eGFR and was successful in reducing EB viraemia. Larger prospective studies are needed to corroborate our fi ndings as well as determine the role of DSA as a monitoring tool.

Ten (10.4%) patients died in follow up time. One-year graft survival was 100% in the LRD group while 98% in cadaver group. Ten year graft survival was %76 in the LDR and %72 in the cadaver group. The graft survey was better in LRD than cadaveric group. Conclusions: The survival rate of LRDs was better than cadaveric grafts. We suggest that the best prognosis in graft survey will be obtaining by transplantation from young healthy donors.

The Objectives: Complement factor H autoantibodies (anti CFH) are described in 9% of aHUS patients, often concurrent with additional susceptibility factors or mutations in genes encoding complement regulator/activators. The natural history of anti CFH has not been well described as most reported cases had been pre-exposed to plasma. Methods: We report a girl presenting aged 9 in established renal failure with aHUS. She was not treated with plasma as biopsy showed end stage histology. No mutations were identifi ed in complement regulator genes. Stored serum samples from 4 years after presentation were assayed for anti CFH. Results: Anti CFH were elevated 4 years after presentation and declined to borderline at 6 years. Living related transplant was successful aged 18 after pre-transplant plasma exchange and.rituximab. Immunosuppression regime was basiliximab induction then tacrolimus, steroids and azathioprine. Post transplant plasma exchange was discontinued after 3 cycles as anti CFH became undetectable and remains so at follow up 10 months later. Conclusions: Anti CFH can naturally decline but may take many years. In this patient without other identifi ed complement regulator gene mutations, successful transplantation was possible. Results: In center A, 24 kidney biopsies were performed during the six months after transplantation. 16 protocol biopsies were performed at three months post Tx, 12 were normal and the other four showed 2 borderline rejection and 2 Banff II. 8 biopsies were motivated by increase of serum creatinine, four were normal and four reveled signs of acute rejection (2 borderline and 2 Banff II). 12 protocol biopsies were performed after 12 months; all were normal. In center B, 12 protocol transplant biopsies were performed at three months post Tx. None showed signs of rejection. All patients underwent a new protocol biopsy at 12 months post Tx and one borderline rejection was detected. No biopsy was performed for increase of serum creatinine in center B. There were no differences in patient age, number of HLA incompatibilities or other patient characteristics.

Conclusions: Patients on FK had less acute rejection episodes detected on protocol biopsies three months post transplant than those on CyA.

Abstract# 530 Objectives: End-stage renal disease (ESRD) in patients with aHUS due to factor H defi ciency is a challenge because of very high risk of disease relapse in an isolated kidney graft, requiring preventive high dose plasma therapy. Liver-kidney transplantation (LKT) is the only strategy to cure defi netely Factor H defi ciency.

Here, we report LKT in a patient with homozygous factor H defi ciency. Methods: Atypical HUS was diagnosed at the age of 5 months. C 3 was undetectable and factor H activity was <5%. A homozygous factor H (FH) mutation was detected (both parents are heterozygote and healthy). Despite plasma therapy he reached ESRD at the age of 10 years. As high dose plasma therapy with 2 weekly plasma exchanges (PE) was continued to avoid further disease manifestation, he was put on a liver-kidney transplant waiting list. Therefore, his FH serum levels were 25-55%. Immediately before LKT, the patient received a PE (30 ml/kg) and 30 ml/kg during surgery as a compensation of liquid losses. Results: Both grafts worked immediately, resulting in FH activity of 50%, 72%, and 100% on day 1, 2, and 3 respectively and normal C3 level. Three months post transplant, with a standard FK-MMF based immunosuppression, serum creatinine was 75 micromol/l, proteinuria negative and immunosuppression well tolerated. All hepatic markers are normal. Conclusions: LKT seems to be a possible strategy for patients with aHUS due to homozygous FH mutations. Individual clinical and biological evaluation is mandatory before LKT in patients with FH anomalies.

Switch Objectives: In adults, sirolimus has shown to prevent chronic allograft nephropathy. The aim of this study is to determine the evolution of renal function in pediatric kidney receptors after switching the immunosuppressive therapy to sirolimus. Methods: Retrospective analysis of 10 children with kidney allograft switched to sirolimus.

The cause of end stage renal disease was: renal dysplasia 2, unspecifi ed 2, ARPKD 1, HUS 1, refl ux nephropathy 1,obstructive nephropathy 1, cystinosis 1, FSGS 1. Mean age at transplantation was 8 years. All patients had induction therapy; maintenance treatment was prednisone, cyclosporin and mycophenolate mofetil in 9 and prednisone, tacrolimus and mycophenolate in 1. Patients were switched to sirolimus because of anticalcineurinic toxicity, CAN, hirsutism and PTLD. The switch was made in average 2 years after transplantation (range 3 months-9 years, 10 months). 6 patients were switched in the fi rst year of transplantation. Trough levels of sirolimus ranged between 4,6 and 11,8 ng/ml. The follow up is in average 2 years 6 months (range 2 months-5 years 8 months Results: Coincubation with IL-1ß (10 ng/ml), IL-6 (100 ng/ml) or TNF-α (50 ng/ml) inhibited IGF-I-stimulated cell proliferation by 50%, while baseline cell proliferation was not altered by these cytokines. IL-1ß, IL-6 or TNF-α inhibited the IGF-I-activated phosphatidylinositol-3 (PI-3) kinase pathway by 30-50% and the MAPK/ERK1/2 pathway by 50-75%. Also IGF-I-enhanced cell differentiation was reduced in the presence of IL-1ß, IL-6, or TNF-α by 50-80%. In parallel, we observed a 50-80% inhibition of the PI-3-kinase pathway. IL-1ß, IL-6 or TNF-α did not alter basal or IGF-I-modulated gene expression of the inhibitory IGFbinding proteins IGFBP-2, -4, or -6.

The resistance towards the anabolic action of IGF-I in the growth plate in conditions of chronic infl ammation such as uremia is partially due to the inhibition of IGF-I-specifi c signalling pathways by pro-infl ammatory cytokines.

Objectives: To measure growth and nitrogen balance in 14 prepubertal children (8 males) on chronic dialysis during treatment with rhGH. Methods: Growth was correlated to dialysis effi ciency (Kt/V) and bone disease as estimated by intact parathyroid hormone activity (iPTH). Anthropometric measurements were expressed as the standard deviation score (SDS). Dialysis effi ciency was calculated from the clearance per patient volume (Kt/V). Nitrogen Balance (NB) was calculated by urea kinetics. Growth was the change in HT-SDS after one year. Results: Baseline anthropometric measurements revealed markedly short stature with normal Wt/Ht ratios. A single NB correlated signifi cantly with Kt/V (r=0.74; p=0.005). Average dialysis effi ciency correlated signifi cantly with growth after one year(r=0.6; p=0.04). Moreover, growth response correlated positively and signifi cantly with bone disease activity as measured by iPTH (r=0.75 p=0.002).

In pediatric dialysis patients, positive nitrogen balance and increased dialysis effi ciency appear to improve growth response during treatment with rhGH.

Validation Objectives: Although risk factors are prevalent in dialysis subjects, studies with methodological fl aws often suggest absence of folate defi ciency (FD). A study of HD subjects, using therapeutic response to folic acid (FA) as diagnosis, found a high prevalence of FD. Using same criterion, study will examine i) prevalence of FD & ii) impact of FA intervention on EPO resistance in PD patients. Methods: 1-21 yr old PD cohort on EPO, stratifi ed by age, will be assigned to 1-2 mg daily-FA or placebo [PL] for 6 mos. Cross-over performed by 2nd 6 mos. Baseline & monthly lab for CBC, RBC FA, serum homocysteine & vit B 12 will be assessed. Quantitative score of i) FD (FDS) and ii) EPO requirement per Hb g/dl (ERI) will be obtained.

Indices for the composite score of folate defi ciency (FD) Folate indices FD Score MCV ≥ 1SD for age 2 RDW ≥ 1SD 1 Hb < 11 g/dl 1 MCH/ MCHC < 1SD 1 Serum folate < 2SD 2

Objectives: The growth hormone (GH)/insulin-like growth factor system plays important roles in the development and growth of kidney. Despite experimental studies on the role of growth GH, and IGF-1 on renal growth, no clinical data has been reported about the renal growth in children with GHD, that is the aim of this study.

Methods: A total of 21 children with IGHD and 15 with MPHD and 68 healthy controls were included. Kidney sizes were measured by ultrasonography and hormone levels were measured by standard methods. The GH treatment durations were 20 and 19.6 months in IGHD and MPHD groups, respectively. Results: Body height, weight and body surface area (BSA) and kidney sizes (kidney length, parenchymal width and kidney volume) were lower in GHdefi cient children compared with their controls (p<0.05). However, IGHD/ MPHD groups and their control groups had similar kidney volume/body weight and kidney volume/BSA ratios (p>0.05). Signifi cant positive correlations were found between kidney length, kidney volume and anthropometric measurements (p<0.05). Multiple regression analysis revealed that body height was the most signifi cant predictor of kidney volume in children with GHD (p<0.001).

Conclusions: Children with IGHD and MPHD had smaller kidneys compared with their healthy controls despite 20 months of rhGH treatment. However relative kidney volumes in respect of body weight and BSA were similar to controls.

Growth Objectives: To examine the effectiveness of intradialytic parenteral nutrition compared to oral supplementation to improve the nutritional state in hemodialysis children.

Methods: Prospective, crossover, randomized design in children (6-17 years) on hemodialysis program, with an ABN (anthropometry and bioelectric impedance analysis) score ≤ 10.33 at baseline. During dialysis procedure they received three months the intervention A (oral supplementation) or B (IDPN), designed to provide a third of the required daily caloric intake, protein RDA for age plus 0.5mg/kg/day. There is no washout period and patients switched to another three months of either Treatment A or B in order to complete the other arm of the crossover design. Nutritional status was evaluated monthly by anthropometry and the ABN score. Results: Nine patients completed six months of treatment. Five started with IDPN and four with oral supplementation. The ABN score improves after six months of nutritional intervention from 8.0 ± 1.5 to 9.5 ± 1.9 p= 0.02, there is also an increase in serum albumin from 3.2 ± 0.4 to 3.4 ± 0.30, p=0.07. Serum cholesterol decreased from 166 ± 42 to 132 ± 22, p= 0.02. There is no statistical difference between groups of nutritional intervention in the z scores of weight, height, body mass index, ABN score and serum albumin after six months of treatment.

Conclusions: Nutritional intervention (oral and IDPN) is safe and well tolerated in children with ESRD in hemodialysis. Objectives: The aim was to investigate the mechanisms of action of vitamin A defi ciency (VAD) on the lipid metabolism in rat kidney. Methods: Adult female rats and their offspring were randomized into three groups: Control: the mother and offspring received a normal diet (4000 retinol IU/kg diet) till 8 weeks; VAD: the mothers and offspring received a VAD diet (400 retinol IU/kg diet) till 8 weeks; Vitamin A-refed: one group of 8 weeks' VAD pups received a complete diet (6500 retinol IU/kg diet) 15 days. The lipid metabolism and immune status of offspring kidney and its relation to the expression of apolipoprotein B100 (Apo-B100), liver X receptor α (LXRα), retinoid X receptor-α/β (RXRα/β), peroxisome proliferator-activated receptorα(PPARα) mRNA expression and protein levels of transforming growth factor-β1 (TGF-β1), interleukin-1β(IL-1β) was analyzed. Results: VAD alters renal lipid metabolism and its immune environment due to expression of apo-B100. The nephrotic impairment of the patients are dicribed in table1. After diagnosis 7 patients were treated with high dose vitamin B12 and long term management was include vitamin B12 intramuscular injection and Calomide-Me oral administration. After 3-14 days MMA was decreased expressly, also the renal function of the patients' was improved except No.4 patient was dead of MODS. We followed up all the 6 patients with 1-6 months. The urine analyze, renal function test and mental development were all normal. Conclusions: Nephrotic impairment of the patients with methylmalonic aciduria is not common, most patients presented with tubulointerstitial injury. But in our patients, impairment of glomeruli was principally. Clinical presentations were include proteinuria, haematuria, edema, hypertension and renal failure. We consider we should screen metabolic disease and analyze urine organic acid as early as possible if the patient not only with proteinuria, haematuria and renal failure in nubibus but also with neurological impairment.

Plants Objectives: In Bangladesh, it is the custom to breast feed infants for prolonged periods ranging from 06 months up to even 03 years. According to ICMH, studies carried out on nursing mothers have revealed that when they were given extra amounts of body building foods, they produced a large amount of breast milk for their infants. At the same time their health also showed improvement. Inspiring from it, on the same line, a study was undertaken to record the nutritional plants intervention from Sundarbans given to lactating mother immediately after delivery for 42 days and why? Puerperium period is considered to be of 42 days. This paper is presented from a project which is on going to prepare a database on the diet of lactating mothers, nutritional intervention, and special care given to her among different communities of Barguna district, Bangladesh during puerperium. Methods: All the ladies who experienced at least one pregnancy were amongst the sample, or the elderly ladies of the family. The interview schedule was prepared, tested on a small sample, and then data collection was done. All plants were identifi ed and vouchers were stored at the Bangladesh National Herbarium. Objectives: Visceral adiposity has emerged as a better predictor of cardiovascular risk in obese patients. Accurate evaluation of childhood obesity must delineate visceral from subcutaneous adiposity in order to identify patients at high risk for future metabolic consequences. Waist circumference (WC) is a more accurate measure of truncal obesity than body mass index (BMI), but WC is a combined measure of subcutaneous and visceral fat masses. Abdominal volumetric estimate (AVE) may be a novel anthropometric marker of visceral adiposity. Methods: Given the cylindrical morphology of the abdominal cavity, abdominal volume (cm 3 )= (height)(length)(width)= (2)(abdominal length)(WC). Abdominal length will be measured as the distance from xiphoid process to pubic symphysis and WC will be measured at the superior aspect of the iliac crest. The AVE will be corrected for musculoskeletal mass and will be correlated with MRI-derived visceral fat measurements. Results: MRI-derived visceral fat measurements will be correlated with anthropometric indices (BMI, WC, waist-height ratio, skin fold thickness) and with each corrected AVE formula. The best predictive model will be selected using multiple regression analysis.

Conclusions: An anthropometric index that best correlates with visceral fat mass will be most predictive of cardiovascular morbidity. An improved method of estimating visceral fat mass will minimize the confounding effect of gender, ethnicity, and pubertal growth on adiposty. Abdominal volume estimate may be a superior marker of visceral fat mass and may be a better screening tool to target high-risk obese children for specifi c intervention.

Nutritional Objectives: Overnutrition during critical perinatal periods is associated with determining susceptibility to obesity and well known comorbidities. We aimed to investigate the effects of overnutrition during neonatal periods on the development of renal pathophysiological changes. Methods: Rat pups were adjusted to either 3 or 10 pups per mother (overnurition and control, respectively) from day 1 to days 21 of life. We measured the effects of early postnatal nutrition excess on potential renal changes related to obesity by 28 days. Results: Smaller litter male pups weighed heavier than controls on days 4 and between days 10 and 28 after birth (P < 0.05). By 28 days of age, overnutrition had no signifi cant effects on renal cell proliferation, apoptosis, numbers of macrophages and glomerulosclerosis. In immunoblots and immnnohistochemistry, renin and angiotensin II type (AT) 2 receptor protein expressions signifi cantly increased in overnutrition rats (P < 0.05). In contrast, plasminogen activator inhibitor (PAI)-1 and matrix metalloproteinase (MMP)-9 protein expressions decreased in the overnutrition group (P < 0.05). AT 1 receptor, monocyte chemoattractant protein-1, tissue inhibitor of MMP-1, tumor necrosis factor-α, osteopontin and adiponectin expressions were not different between the two groups. Conclusions: Our data demonstrate that postnatal overfeeding leads to unexpected and deleterious renal alterations. Increased renin and AT2 may decrease renal PAI-1 and MMP-9, suggesting an inhibitory effect on accumulation of extracellular matrix proteins or the impairment of matrix turnover in neonatally overfed male rats.

Platelet µg\l), p<0.05, also our study shows a lower level of serum arginine in group A1 (60.5±12.3 µg\l) than group A2 (83±27 µg\l) and a lower level in group A2 than group B (120.5±14.6 µg\l), and we found a negative correlation between serum arginine level and platelet aggregation. Conclusions: we concluded that patients with ESKD has a low level of L-arginine specially in those with malnutrition and that leads to increase in platelet aggregation and this may increase the possibility of thrombus formation, and we suggest the use of nutritional supplementation of L-arginine to suppress this process of atherothrombosis. Multivariate analysis in KDOQI 1 children showed a signifi cant association between 25OH and PTH / calcium (β parameter -0.36 and 0.21, respectively) whereas in KDOQI 2 children, 25 OH was signifi cantly associated with calcium and urine calcium/creatinine ratio (β parameter 0.56 and 0.24, respectively). Conclusions: Low vitamin D levels are highly prevalent in French children independently of their GFR. New roles of vitamin D in global health have recently been highlighted: vitamin D could be a protective factor against infections, cancers, auto-immune and cardiovascular diseases. All these data reinforce the need of regular vitamin D supplementation in children. Objectives: Kidney stones are one of the most painful conditions a child can have, and they are one of the most common disorders of the urinary tract. In this article we report our experience with the diagnostic screening and management of children with kidney stones. Objectives: Bone mineral metabolism disorders associated with chronic kidney disease persists following renal transplantation.

In 57 children demographic data, height, weight, serum calcium (Ca), phosphorus (P), alkaline phosphatase (Alk-P), parathyroid hormone (PTH), 25(OH)-Vitamin D 3, BUN and creatinine were obtained.Left hand-wrist radiography and "bone mineral densitometry"by DXA technique were carried out. Appropriate softwares were used for interpretation of Z-score of bone mineral density (BMD)and statistical analyses. Results: Fifty seven children all with well-functioning allograft,with a mean age of 18.7 ±4.25, age at transplantation 13.1±3.46 (4.5-20) years with a mean follow up of 67.1±33.8 months were studied. The patients' height-age and bone-age of 11.9±1.8 and15.6±3.3 years respectively, revealed a mean height-age and boneage retardation of 5.7±2.3 and 1.22±1.47 years; respectively. Hyperparathyroidism, hyperphosphatemia, hypercalcemia and hypophosphatemia was found in 27(47.3%),9 (15.8%), 9 (15.8%) and 5 (8.8%) of the patients respectively.

The mean BMD Z-score was -1.77±1.13 (-4.2 -1.1) for lumbar spine and -1.64±0.89 (-3.9-1.9) for femoral neck. The BMD Z-scores showed meaningful correlations with the serum Alk-P level independent of serum Ca, P and PTH (p=0.002). Inverse correlation was found between serum level of PTH and GFR (p = 0.011).

Relatively high prevalence of bone-mineral disorder in pediatric renal recipients warants a periodic bone densitometry.

Simple Objectives: Simple renal cysts are rare lesions in pediatric patients. It was considered that the association of simple renal cysts with certain symptoms is just coincidence.

There are no studies in children to establish a possible relationship between the presence of simple renal cysts and their association with metabolic abnormalities that cause kidney stones. Methods: We reviewed 22 patients (12M, 10F) diagnosed with simple renal cysts by renal ultrasound. We analyzed renal function, urinary excretion of calcium citrate and investigate about family history of kidney stones.

The mean age was 6.04 ± 2.9 years. The symptoms presented at the time of diagnosis were urinary tract infection (40.9%), abdominal pain (27.3%), hematuria (9.1%) and other (18.2%). One patient was asymptomatic (4.5%). The cyst presentation was more common on the left side (56.5%). Nine patients had hypercalciuria (40.9%), three hypocitraturia (13.6%) and two associated hypercalciuria hypocitraturia (9.1%). Thirteen families had history of renal stones. Overall metabolic abnormalities in patients and/or history of stones in their families were present in 19 of the 22 families (86.3%).

We found a high proportion of metabolic abnormalities and family history of urolithiasis in children with simple renal cysts. Our hypothesis is that both entities may be related. Further studies are needed to demonstrate the relationship described in this work and the pathological mechanisms involved. Objectives: Secondary hyperparathyroidism (sHPT) in patients with chronic kidney disease (CKD) is a strong contributor to the development of mineral and bone disorder (MBD). The aim of the research was to investigate the dynamics of the serum parathyroid hormone (PTH) level and growth inhibition in children with a transplanted kidney. Methods: We examined 12 children at the age of 9-17 (14.3 ± 2.9) who had gone through renal transplantation. The serum PTH level and the children's height were measured before the transplantation and a year after it. Results: The serum PTH level was increased in all children before the transplantation and varied: in 2 patients it was less than 300 pg/ml (178 ±5.1), in 4 patients -less than 1000 pg/ml (518.11±90.11) and in the others ranged from 1800 to 2500 pg/ml (2077.33 ± 329.4). The serum PTH level did not depend on the dialysis duration (R=0.5; p=0.39). 10 patients showed defi nite growth inhibition: 5 patients had height on the 3rd centile, 7 on the 10th centile. A year after the transplantation the serum PTH level remained increased 267.48±188.28

pg/ml in all patients. 3 patients showed considerable longitudinal growth, their height was on the 25th centile. The others remained on the 10th centile despite a certain increase in the height. The research did not reveal any correlations between the height increase and the serum PTH level before and after the transplantation (R=-0.6, p=0.28). Conclusions: Therefore, we concluded that kidney transplantation in patients with CKD leaded to decrease in serum PTH level and improvement of longitudinal growth but further sHPT treatment was still required. Objectives: Glucocorticoid therapy in children with nephrotic syndrome (NS) may lead to bone metabolism disorders. Vitamin D has a number of pleotropic effects in a variety of tissues. The aim of the study was to assess stores of vitamin D in children with nephrotic syndrome during one year observation. Methods: We retrospectively studied 25 children aged 5 to17, mean 10 years; 21 with idiopathic NS and 4 with IgA and Schönlein-Henoch nephropathy. At the start of the study 24 children were given 10-60 mg/48h of prednisone, 23 patients were supplemented with 400-800 IU of vitamin D. After 12 months 11 patients were given 2.5-47.5 mg/48h of prednison, vitamin D was supplemented in 14 children. In all children serum levels of 25-hydroxyvitaminD 3 (25OHD 3 ), 1,25(OH) 2 D 3 , parathormone (PTH), calcium, phosphorus, alkaline phosphatase (AP), osteocalcin (OC), bone AP were mesured.

The deffi ciency of 25OHD 3 (<20ng/mL) was found in 9 (36%) children before study period and in 10 (40%) after 12 months (5 of them without glucocorticoid therapy). Positive signifi cant correlation between 25OHD 3 and OC (R=0.47, p<0.05) and negative correlation between 25OHD 3 and PTH (R=-0.59, p<0.05) were found. There were no correlation between 25OHD 3 and prednisone dose and other tested parameters. Objectives: To evaluate the impact of potassium citrate and/or thiazides on bone mass density (BMD) and growth parameters of children and adolescents with primary hypercalciuria (PH). Methods: Historical cohort of 84 patients with PH. Median follow-up of 12years. All patients were submitted to bone mass densitometry with DEXA-Lunar DPX-IQ. Lumbar-spine BMD (L1-L4), Z-score before (BMD1 and ZS1) and after (BMD2 and ZS2) treatment with potassium citrate and/or thiazides (at least 24m) were performed. Patients were classifi ed in 2 groups: G1 those who followed the prescription and G2 who did not. Height, body weight and respective percentiles according to NCHS were obtained. Height percentiles were categorized (<P5 as 1, P5 as 2, P5-P10 as 3 and so on) and compared before and after treatment. Tests: T-test and Wilcoxon signed-rank test. Results: Forty-six (56%) boys and 38 (45%) girls were regularly followed for 154±40 months. Mean initial height percentile score (MIHPS) was 7.2±2.7 (P25-P50) compared to 7.8±2.6 (P25-P50)p<0.0001. Mean interval between BMD was 27.14±17m. Sixty-four (76%) patients were classifi ed as G1. MIHPS in G1 increased from 7.21±2.7(P25-50) to 8.12±2.69 (P50-75) (p<0.001). No difference was observed in G2. BMD changes signifi cantly from 0.745±0.175 to 0.858±0.184g/cm³(p<0.001).

Conclusions: Improvement of BMD with treatment was observed as well as of height percentile scores suggesting a benefi cial effect and potential need of treatment. Regular medication use seems to contribute effectively to this effect in contrast to irregular use. Objectives: Aim of this study was to assess bone parameters in children with advanced chronic kidney disease (CKD) and with renal transplantation by dual energy X-ray absorptiometry (DXA), quantitative ultrasonography (QUS) and biochemical markers. Methods: Twenty children with CKD stages III to V (mean age:9.47) and 11 transplanted patients (mean age:11.57) were included. Bone Mineral Density (BMD) at lumbar spine by QUS and Speed of Sound (SOS) at radius and at tibia by QUS were evaluated. Additionally, intact parathormone (iPTH), 25-hydroxycholecalciferol (25OHD 3 ), serum bone alkaline phosphatase (BAP) Ca, P and creatinine were measured. Results: Results showed that 30% of patients with CKD had SOS Z-score at tibia <-1 and 52.7% at radius <-1, whereas only 16.67% had BMD Z-score <-1. Regarding transplanted patients 27% had SOS Z-score at tibia <-1, 70% at radius <-1 and 81% BMD Z-score <-1. Serum iPTH and BAP were higher in patients with CKD compared to transplanted patients (p<0.001, p<0.001 respectively).

In patients with CKD multivariate regression analysis showed that SOS Z-score measured at tibia was the major predictor of increased levels of serum iPTH. No correlation between biochemical markers and DXA was found.

In patients with CKD, QUS is better than DXA correlated to biochemical indices of osteodystrophy such as PTH and seems to effectively detect bone disease. Transplaned patients presented high incidence of abnormal DXA, probably due to steroids induced osteoporosis.

Ultrasound Objectives: Secondary and tertiary hyperparathyroidism are commonly encountered in children with CKD. This may be due to hyperplastic (Hyp) or adenomatous (Ad) change in the parathyroid glands (PTG). We performed ultrasound (US) and scintigraphic (Tc99m-MIBI) imaging of the PTG at a specialist paediatric nephrology centre over a 5 year period, to evaluate the relationship between biochemical parameters and imaging fi ndings. Methods: Of 23 children, 6 were in CKD stage IV-V, 16 on dialysis and 1 posttransplant. The median age was 11.8 (range 1-18) years. All 23 underwent US and 9 had MIBI; results were categorized as normal, Hyp or Ad. Biochemical data and relevant medication history over the preceding 12 months was recorded. Objectives: SCFE is a common orthopedic disorder affecting otherwise healthy adolescents in which the femoral head is displaced from its normal position. The most cases are classifi ed as idiopathic but SCFE may be also secondary to different endocrinopathies. Exceptionally, SCFE was also described as a consequence of chronic renal failure (CRF). Methods: Therefore, we present a patient with FHHNC and bilateral SCFE as a consequence of untreated osteodystrophy in a course of CRF.

The 15-years old boy with diagnosis of FHHNC and CRF was admitted to our clinic due to severe knees pain. During last 2 years, he discontinued the adequate treatment. Initial laboratory tests revealed uremia, hypocalcemia, hyperphosphatemia, extreme hyperparathyroidism (PTH-3977 pg/ml), metabolic acidosis and anemia. Therefore hemodialysis accompanied by typical

conservative treatment was started. Radiographs of hips showed bilateral SCFE of severe grade. After 2 ½ months of treatment and lowering serum PTH level below 300 pg/ml, successful surgical stabilization in situ of both epiphyses was performed.

Conclusions: There is a fi rst reported case of FHHNC complicated with SCFE. Although this association is rather accidental, we believe that severe secondary hyperparathyroidism due to untreated renal osteodystrophy, additionally aggrevated by FHHNC per se, was a causative factor. Therefore proper management of these conditions is essential to prevent SCFE.

Parathyroid Objectives: This MC, intra-subject controlled, open-label, active-treatment study will assess in dialyzed children aged 2-18 yrs with SHPT not responsive to standard of care (SoC) therapy, the response after 6-mo cinacalcet (Ci) compared intra-subject to SoC alone at screening visit 6 mos before Ci start. Secondary objectives: effect on growth over 18 mos and PK profi le. Methods: At baseline children have PTH levels>300 pg/mL, plasma P<6 and Ca>9.4 mg/dL, or CaxP product>60. Initial Ci dosing will be 0.5-0.75 mg/Kg per os OD to be adjusted up to a max of 180mg for target PTH values<180 pg/mL. In 12 centres of a national pediatric dialysis registry, 30 children will be enrolled, corresponding to an α=0.05 and a power of 80% using McNemar test, with an expected % of responders to Ci or SoC of 40% or 5% respectively, and a 15% drop-out rate. Results: Primary endpoint (EP) will be % of children who will have a reduction from baseline >25% in mean PTH levels during the 6-mo effi cacy-assessment period. Secondary EPs over 18 mos will be: % of patients with mean PTH levels<300 pg/mL; % change in PTH, Ca, P, and CaxP product; PK profi le (or population profi le by age) and its correlation with PTH and testosterone levels; auxological indices and growth velocity; % of children with treatment-emergent AEs and lab abnormalities; retention on treatment and reasons of treatment withdrawal.

The study will evaluate whether Ci represents a safe and effective therapeutic option for SHPT children. Methods: 60 pts on CCPD (age 13.9 + 0.5 yrs) with BBx proven 2 o HPT received either sevelamer or CaCO 3 in conjunction with either calcitriol or doxercalciferol for 8 mos. S-Ca, P, Alk P'tase, 1 st PTH-IMA, and FGF-23 were obtained monthly. Vitamin D sterols and P binders were adjusted to maintain serum PTH levels between 300-500 pg/ml, Ca between 8.4 and 10.2 mg/dl, and P between 4-6 mg/dl. BBx was repeated after 8 mos of therapy. Predictors of response to therapy (fi nal PTH: 300-500 pg/ml) were assessed by ROC.

Results:

Pts treated with sevelamer received higher vitamin D sterol doses than those treated with CaCO 3 (p<0.05). FGF-23 levels increased and BFR/BS decreased similarly in all groups throughout the study. The AUC for baseline FGF-23 < 1000 RU/ml and baseline PTH were 0.76 and 0.58, respectively. FGF-23 levels increase similarly with different D analgues and P binders and baseline values did not predict response of BFR to therapy. Objectives: Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of pediatric ESRD. An innovative strategy to discover genes relies on genome-wide search for copy number variants (CNVs). Signifi cant evidence indicates that CAKUT can be caused by rare CNVs.

We performed a genome-wide search for CNVs using the ILMN 610-Quad chips in 165 CAKUT patients and 3,060 controls. We developed an analytic pipeline for identifi cation of rare pathogenic CNVs: absence in public databases and controls; disruption of genes; recurrence in multiple patients; prioritization based on linkage loci and gene expression.

The analysis yielded 2,532 CNVs. Comparison with 3,060 controls and with public databases resulted in 319 rare genic CNVs. 38 patients had highpriority CNVs: 9 carried CNVs diagnostic of known syndromes, such as 17q12 (TCF2: renal cyst-diabetes syndrome; 4 cases), indicating that our approach is robust for identifi cation of pathogenic CNVs; 29 carried novel high-priority CNVs. We validated 13 of these and 3 were de novo: chrs. 3q13-22 (novel), 13q12 (novel) and 17q12 (TCF2).

Our study suggests a major role of genic CNVs in CAKUT pathogenesis. Using a rigorous pipeline, we identifi ed high-priority CNVs that we are testing for replication in an independent cohort of patients to identify underlying genes. . Genotype and outcome were known in 408 of them (median followup of 17.7; range 0.3 -74.7 years). ESRD occurred in 36% at diagnosis. At last review 50.5% developed ESRD and 12% died. More than 80 AGXT mutations were identifi ed, of which the most common (allele frequency) were p.Gly170Arg (25%), c.33dupC (10%), p.Ile244Thr (7%), p.Phe152Ile (3%). In homozygous p.Gly170Arg patients, 50% developed ESRD occurring at a median age of 34 years, compared to 14.6 years in those with other mutations (p<0.001).

The heterogeneous clinical presentation in PH1 is related to a wide mutational spectrum. Whereas progression to ESRD occurs in all genotypes, renal function in PH1 is preserved longest in patients carrying the common p.Gly170Arg mutation. Ongoing data collection as part of an international registry will clarify disease outcomes further. Objectives: Focal and segmental glomerulosclerosis (FSGS) remains an important cause of nephrotic syndrome (NS) and chronic kidney disease (CKD). The objective of this study is to positionally cloned the gene mutated in a large kindred with familial CKD and proteinuria. Methods: We identifi ed a large family with eight affected individuals spanning three generations. We performed genome-wide linkage analysis (GWLA) using the Illumina Infi num II HumanLinkage-12 beadchip genotyping assay and fi ne mapping with informative microsatellites.

Results: Two of the affected individuals had 3g and 5g of proteinuria. Renal biopsy in four affected individuals showed foci of interstitial infi ltrates and focal global glomerulosclerosis. GWLA and fi ne mapping yielded a multipoint parametric LOD score of 2.9 on chromosome 16p. Positional cloning of the genes within the locus yielded a novel in-frame insertion deletion mutation 278_289delins TCTGCCCCGAAG>CCGCCTCCT in exon 3 of uromodulin. The in-frame change leads to loss of a highly conserved cysteine residue in position 94. The mutation segregates in affected individuals in the family. Conclusions: Phenotype previously associated with uromodulin defects include chronic interstitial nephritis, JFHS and glomerulocystic disease. This fi nding in combination with the recent GWAS reveal that uromodulin is a common disease locus in subjects with CKD and suggests that defective uromodulin may have wider deleterious effects in the kidney. In conclusion, a novel insertion-deletion mutation in uromodulin in a large family with CKD and proteinuria expands the clinical spectrum of disease associated with defective uromodulin. Objectives: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder. ADPKD is caused by mutations either in PKD1 or PKD2 genes. Recent studies have shown that some mutations classifi ed as missense and synonymous can induce pre-mRNA splicing defects. Our goal is to determine the functional consequences of this kind of PKD1 and PKD2 mutations in pre-RNA splicing. Methods: PKD1 and PKD2 minigenes were constructed with exons 2-3, 11, 23, 24-25-26 or 37-38-39, and with exons 4, 5 or 13, respectively. Mutations were introduced by site-directed mutagenesis. Minigenes were transfected into HEK293T cells and mRNA was analysed by RT-PCR. Results: Five missense and two synonymous PKD1 mutations and six PKD2 missense mutations were analysed. G109G creates a donor splice site, leading to an aberrant mRNA lacking the 3' end of exon 3. R3753R activates a cryptic donor splice site and generates an altered mRNA missing the 3´ end of exon 39. Analysis of PKD2 missense mutations R306Q, R322W, R325Q and A356P showed increased incorporation of exon 4 in the mRNA. The rest of mutations showed no effect in pre-mRNA splicing. Conclusions: PKD1 synonymous variants G109G and R3753R act as splicing mutations. These represent the fi rst PKD1 synonymous mutations that affect pre-mRNA splicing. PKD2 missense mutations seem to alter the regulation of the mRNA splicing process. Our results emphasize the importance of performing mRNA analysis when evaluating the effect of exonic mutations. Objectives: Primary vesicoureteral refl ux (pVUR) is a major cause of pediatric ESRD. We localized a gene for pVUR on chr. 12p11-q13 under a recessive model. Evidence for linkage was strongest among the Hasidic Jewish, which has a founder effect. To narrow down the linkage interval we performed a case control association study in our Hasidic Jewish cohort. Methods: Cases were ascertained with a positive VCUG. Controls were of the same ethnicity and did not have a family history of any urologic/kidney disease. We genotyped 18 familial Jewish cases and 437 controls with the Illumina 660W arrays. 3,489 SNPs in the LOD-1 interval (29.5 Mb) were used for the association study. Standard QC fi lters were performed. Results: Our analysis with PLINK revealed a genomic infl ation factor λ =1.03, indicating almost no population stratifi cation. Within the LOD-1 interval on chr. 12 we found 9 suggestive SNPs (p-value<10 -4 ). The most promising signal was an intronic SNP in a gene highly expressed in the metanephric mesenchyme and ureteric bud of the developing kidney (p-value=2.62x10 -5 , OR= 3.9). This association is signifi cant after Bonferroni correction. Conclusions: pVUR is a complex disorder with a recessive gene on chr. 12p11-q13. Initial association results in familial Jewish cases and controls indicate several potential candidate genes for pVUR in this interval. We are now performing a replication study in Jewish and non-Jewish cohorts to identify the gene underlying our locus. Objectives: Chronic kidney disease (CKD) is a serious illness that causes severe and irreversible reduction in kidney function. One consequence of CKD is an elevated cancer risk. However, the underlying mechanism is unclear. There is no genomic data which has been demonstrated by some cytogenetic markers in children with CKD. Methods: In this study, DNA/oxidative damage in lymphocytes from 17 children in pre-dialysis stage (PreD), 15 children on regular hemodialysis (HD), and 17 renal transplanted (TX) children has been compared to 20 healthy children by the strand breaks and endonuclease III (Endo III), formamidopyrimidine glycosylase (FPG) sensitive sites in Comet Assay. Blood chemistry parameters, TAC, CRP, IL-6 and homocysteine have also been measured. and aquaporin 2, respectively. Yet NDI can also occur as a secondary complication and we aimed to defi ne NDI in selected inherited renal diseases. Methods: Review of clinical features and analysis of AVPR2 and AQP2 genes of four patients with NDI and molecularly proven diagnosis of nephropathic cystinosis, Bartter syndrome, nephronophthisis and apparent mineralocorticoid excess (AME), respectively. Results: All patients excreted hypotonic urine during hypernatremic dehydration and had no further increase in urine osmolality after DDAVP, consistent with NDI, yet no mutation in AVPR2 and AQP2 genes could be identifi ed. In two patients the diagnosis of NDI was missed, leading to repeated hypernatremia. In the other 2 patients, NDI was mistakenly assumed to be the primary diagnosis, delaying identifi cation of the underlying disorder. In one patient, NDI was reversible with treatment of her AME.

The recognition of this potential complication is important as it has direct implications for the clinical management. The occurrence of NDI in these conditions provides clues for the etiology of aquaporin defi ciency. Objectives: Alport syndrome is a clinically and genetically heterogeneous nephropathy. The majority of cases are transmitted as an X-linked condition due to COL4A5 mutations. Methods: Case report: We report on an 11-year-old girl who has been followed since the age of 4-years for repeated urinary tract infections (cystitis), microscopic hematuria and later also proteinuria (< 0.5g/day) and hypertension. The ultrasound examination and the audiogram have been normal. She was treated with ramipril with a good effect on hypertension (ABPM). Renal biopsy revealed changes typical for Alport syndrome. Father of the girl has been suffering from perceptive hearing loss and glomerulonephritis since childhood. He underwent two renal transplantations. There are also 3 relatives with a similar symptomatology in the family. Molecular genetic analysis of the COL4A5 gene was performed and revealed a germline nonsense mutation c.372-373del.CA(p.C124X) in exon 6 of the gene.

Results: This mutation results in the synthesis of a shortened protein (123 aminoacid versus normal 1691 amino-acid chain). Based on the biopsy fi ndings and molecular genetic analysis the diagnosis of X-linked Alport syndrome has been proved.

The mutation found in the observed family is a new one and has not been previously described. The mutation has deleterious effect on the protein level. It may be considered to be the cause of Alport syndrome due to the segregation of the mutation with the disease in the presented family.

Genetic Objectives: Idiopathic nephrotic syndrome is one of the most commonly primary glomerular diseases in children, and glucocorticoid receptors may have an important role in responsiveness to steroid therapy. Methods: We investigated Bcl1 nucleotide polymorphism of the glucocorticoid receptor gene (NR3C1 gene) in 84 children with nephrotic syndrome and 66 healthy controls using polymerase chain reaction-restriction fragment length polymorphism, and analyzed the correlation between genotypes and clinicopathological features of the patients. Results: Forty nine children (58.3 %) were initial steroid responder and thirty fi ve (41.7 %) were steroid resistant. C/C and G/C genotype frequencies of Bcl1 were similar in all groups (steroid-responsive, steroid resistant and control group), while G/G genotype frequencies were similar in steroid-responsive and control groups. We could not fi nd G/G genotype in steroid-resistant group. Conclusions: These data suggested that C/C and G/C genotype of Bcl1 in the NR3C1 may not affect steroid-responsiveness in Turkish children with NS. On the other hand, absence of the GG genotype and G allele of Bcl1 may play a role in the steroid-resistance in these children. However, further studies in a larger population are needed to test this hypothesis.

Objectives: X-linked nephrogenic DI (X-NDI) is a rare disease caused by mutations in the AVPR2 gene. These patients are overwhelmingly males. Symptomatic presentation in females is rare and can occur due to skewed X chromosome inactivation. Methods: Three siblings (2 girls 3 yrs and 2 yrs, and a boy 4 mo) presented with severe polyuria and polydipsia. DDAVP challenge test confi rmed the diagnosis of NDI. Their mother was asymptomatic and all had different fathers (also asymptomatic) thus making the diagnosis of autosomal recessive or dominant form of NDI due to aquaporin 2 (AQP2) gene mutations unlikely. Results: Genetic testing was initially performed on the eldest sibling. There were no mutations in the AQP2 gene. However, she was heterozygous positive for a complex allele in the AVPR2 gene with three mutations (R64Q, A147V, and A163P). Of these mutations, A163P has been associated with X-NDI. Genetic testing of mother and other two siblings revealed the presence of identical mutations. The only other male family members (maternal grandfather and maternal cousin) were asymptomatic and genetic testing in both of them was negative for mutations in the AVPR2 gene. Over the last 2 years, the severity of DI has decreased in both sisters (urine osmolality improved from < 100 to slightly over 200 mOsm/kg).

Conclusions: Conclusion: Although rare in females, X-NDI should be included in the differential diagnosis of symptomatic females presenting with features of DI in early infancy. Severe phenotype in females can result from extremely skewed inactivation of X chromosome. X chromosome inactivation occurs early in embryogenesis and is irreversible in descendents of that cell. The reason for decrease in severity in the two sisters is thus unclear. Objectives: Neurotrophins (NTs) and their receptors (NTRs) are known to be important for pathogenesis of various infl ammatory diseases that occur in not only neuronal but also non-neuronal tissues, including kidney. Thus, we performed genetic association study between childhood IgA nephropathy (IgAN) and NTs and NTRs. Methods: We genotyped and analyzed single nucleotide polymorphisms (SNPs) of genes encoding NTs (nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF)) and NTRs (nerve growth factor receptor (NGFR) and neurotrophic tyrosine kinase receptor 1-3 (NTRK1-3)) in 197 IgAN patients and 289 healthy controls. Results: The genotyping data of IgAN patients and healthy control subjects revealed signifi cant association between NGF SNP rs11102930 and presence of IgAN. Patient subgroup analysis revealed that development of proteinuria (> 4 and ≤ 4 mg/m2/hr) was associated with rs1187321 and rs1187323 of NTRK2, and that presence of nephrotic range proteinuria (> 40 mg/m2/hr) was associated with rs6334 of NTRK1, and rs11030104, rs7103411, rs7103873, and rs6484320 of BDNF. Signifi cant differences were observed in podocyte foot process effacement for rs1187321 and rs1187323 of NTRK2. Furthermore, some SNP frequencies were signifi cantly different in patient subgroups with either pathologically mild or advanced disease, specifi cally in rs6334 of NTRK1.

Our results suggest that SNPs of NTs and NTRs are associated with susceptibility, pathological advancement, podocyte foot process effacement, and development of proteinuria in childhood IgAN. Objectives: This study evaluates the neuroimaging findings of a subset of ADPKD children presenting with headaches and/or family history of subarachnoid hemorrhage (SAH) in fi rst-degree relatives. Methods: We follow 63 children (age 1 -20 years; 25% < 1 year) with ADPKD. Twenty (31.7%), age 3-20 years, who presented with headaches or a family history of SAH had central nervous system (CNS) investigation. Results: Twenty ADPKD children who were imaged with CAT (n = 3) or MRI angiography (n = 17) had no cerebral vascular aneurysms. Five (25%) children (age 16-18 years) showed CNS abnormalities including areas of white matter demyelination (n = 2); cerebral/cerebellar atrophy and partial atrophy of posterior corpus callosum (n = 1); a 3 mm periventricular cyst (n = 1); and CNS lesions typical of known spina bifi da with VP shunt placement (n=1). Conclusions: This database documents neuroimaging anomalies before adulthood in 4 children (exclusive of expected fi ndings of VP shunted child), 2 of which had demyelinative changes, compared to an incidence of approximately 3% in non-ADPKD children with headaches. These fi ndings are likely developmental and are not located in a vascular pattern. There is a need for longer studies and an understanding of the relevance of these to the patients' complaints. ADPKD during early development has abnormal cilary function and primary cilia are critical to neural stem cell development as demonstrated by mice with Kif3A or Smo protein anomalies, which demonstrate failure of radial astrocytes responsible for adult neurogenesis. CNS neuroimaging fi ndings may predict a cohort of ADPKD children at higher risk of developing cerebral aneurysms.

Clinical Objectives: Hepatocyte nuclear factor-1 beta, coded by TCF2 gene plays an important role in the kidney, liver, gut and pancreatic development. Studies have shown that TCF2 abnormalities are associated with MODY 5 diabetes in adults and predominant kidney abnormalities in children. Studies have shown that these abnormalities could be secondary to point mutations or large deletions (1.5 Mb) of TCF2 gene. This latter abnormality is more frequent in children.These abnormalities are studied by molecular biology: QMPSF and direct sequencing. Our study aim was to demonstrate the presence of these deletions using FISH (Fluorescence in situ Hybridisation) technique. Results: Two clones RP11-115K3 and RP11-697E22 (BAC-PAC chori), located on TCF2 gene (17q12) -and validated by QMPSF®-, allowed to produce probes FISH in order to study deletion of this region. Results of FISH analysis of deleted patients (previous QMPSF® results) confi rmed the abnormality and allowed us to observe TCF2 deletion on metaphasic and interphasic cells.

These results confi rm that TCF2 deletion could be detected using FISH method on interphasic cells. This analysis can be performed on a buccal cells smear which is more easy to obtain than a blood sample. Moreover, FISH on interphasic cells is a rapid method allowing the results to be obtained within 24 hours. This approach could as well be proposed antenatally when implications of such an antenatal diagnosis are determined. Nevertheless, if FISH results are normal and the phenotype suggestive of TCF2 disorder, point mutations have to be searched for using direct sequencing.

Abstract# 603 Objectives: Denys-Drash syndrome is defi ned as early onset nephrotic syndrome with diffuse mesangial sclerosis (DMS), genital anomalities and bilateral Wilms´ tumor. It is characterized by mutations in the WT1-gene affecting the zinc fi nger regions of exon 8 and 9 which are responsible for DNA-binding. Methods: We describe an 11-months old girl presenting with microhematuria, proteinuria of nephrotic range, hypertension and abdominal tumor. Renal function was severely reduced. Genitalia were female, 46XX. Imaging revealed a huge tumor in the left kidney and a cystic structure in the right kidney. Results: Histologic analysis showed a stroma-rich nephroblastoma in the left and DMS with intralobular nephroblastomatosis in the right kidney.

Mutational analysis of the WT1 gene identifi ed a heterozygous germ line mutation c.1187A>G in exon 9 (p.D396G). In WT cells, DNA sequencing disclosed a homozygous/hemizygous state of the mutation, while the DMS cells were heterozygous as observed in blood cells. Array-CGH as well as quantitative copy number analyses revealed a disomic status of the WT1 locus in WT cells.

The genetic data in our patient indicate that the tumor cells harbored a homozygous WT1 mutation suggesting that the second hit was the same missense mutation as observed in germ line cells. Objectives: Autoimmune Polyendocrine Syndrome Type 1 (APS-1 or Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy Syndrome-APECED) is an autosomal recessive disease caused by mutations of the AutoImmune REgulator (AIRE) gene. We present a case of APS-1 in a 10 -year old girl caused by a rare mutation of the AIRE gene.

The child presented at the age of three years with recurrent episodes of mucocutaneous candidiasis and onychomycosis. Candidiasis subsided at the age of six, following intermittent treatment with itraconazole or fl uconazole. Hypoparathyroidism was diagnosed at the age of eight years. Results: Genetic analysis revealed the rare mutation T16M (exon 1) in homogeneity, which is different from the most common mutations R257X and 3-bp deletion. Autoantibodies to thyroid and adrenal gland were not detected and all other endocrine functions were normal. The hypoparathyroidism was treated with calcitriol and calcium carbonate, by which serum levels of plasma calcium and phosphate were normalized during the fi rst two years of treatment. However, a persistent increase of serum phosphate levels has been noted over the last six months for which therapy with the phosphate binder sevelamer has been instituted.

Conclusions: T16M mutation in this child caused APS-1 complicated by hypoparathyroidism at the age of eight; normal renal function is still maintained and therapy with sevelamer is required to control the abnormal serum phosphate levels.

Objectives: To investigate whether inflammatory stress exacerbates lipid accumulation and, thereby exacerbating the progression of renal injury by nonscavenger receptor-mediated pathways. Methods: Male CD36-/-SRA-/-ApoE-/-mice were fed a western diet and were randomly assigned to receive either subcutaneous injections of 10% casein (to induce infl ammation) or vehicle (control). Animals were sacrifi ced after 14 weeks, terminal blood samples were taken for plasma creatinine, BUN, serum cholesterol, triglycerides, LDL, HDL, amyloid A (SAA) and IL-6 assays. Renal sections were used for histological assessments. Objectives: Pseudohypoaldosteronism ( PHA) is a rare syndrome characterized by defective sodium transport in the distal nephron and renal salt wasting despite high serum aldosterone concentrations. Two clinically distinct forms of PHA have been described. The renal form is characterized by salt loss from the kidneys. It is inherited as an autosomal dominant trait and is caused by mutations in the mineralocorticoid-receptor gene.

We report a case of PHA type 1, renal form that presented as severe salt wasting on the 20th day of life, initially appearing like congenital adrenal hyperplasia (CAH).

The baby was with severe dehydration, hyponatremia (104-106 mmol/l), metabolic acidosis and normokalemia. Poor response of the dehydration and electrolyte abnormalities to steroid therapy should make one suspect PHA.

Diagnosis was established by demonstrating the greatly increased values of plasma renin activity >25 ng/ml/h and plasma aldosterone concentration 3518 pmol/l (N 471). The child was treated with sodium chloride and alkali therapy with sodium bicarbonate supplements. This patient presented as life threatening condition, required intensive care. After adequate supplementation the child was clinically stabilized. Conclusions: PHA might be life threatening condition required intensive care and adequate supplementation for clinical control. Objectives: Atypical hemolytic uremic syndrome (aHUS) is a complement dysregulation disorder with a major impact of genetic background. C3 gene mutations have been proposed as a novel susceptibility factor. We screened a large population of children with aHUS from Europe for mutations in the C3 gene. Methods: An unselected cohort of 67 children with aHUS was examined for C3 gene mutations. To investigate the pathogenic relevance of the identifi ed mutations, complement deposition on endothelial cell (EC) surfaces after incubation with sera of patient and their parents was studied. Results: Sequencing revealed 3 novel heterozygous mutations (4%) in 3 unrelated patients and one uncommon C3 polymorphism. The mutations were not found in 100 healthy donors. One mutation is located within the Factor H (SCR3) binding site of C3b. EC exposed to serum containing this mutant deposited increased amounts of C3 compared to normal, at a level similar to Factor H depleted serum. The second mutation did not caused increased C3 deposition in any of the tested conditions. The third one is located in proximity to the Factor B binding site. Carriers of this mutation present with low C3 levels, precluding the use of their sera in EC assays. Conclusions: We identifi ed 3 novel mutations in the C3 gene in our aHUS cohort. One mutation leads to markedly increased C3 deposition on EC surfaces. Another mutation possibly results in a hyperfunctional C3 convertase, leading to C3 consumption. Functional tests with recombinant C3 are in progress.

Nephrosis, Growth Hormone Defi ciency, and Mental Retardation in Three Brothers -Association or New X-Linked Syndrome? R. Mallmann. Dept. of Pediatrics and Adolescent Medicine, Elisabeth Hospital, Essen, Germany. Objectives: Three brothers and half-brothers respectively present with large proteinuria, intermittent microscopic hematuria, mental retardation and short stature. There is one completely healthy (half-) sister. Renal function as well as blood pressure have been normal over time. Renal biopsy, performed in the oldest boy, shows minimal changes with only a few glomeruli presenting thin membranes. No mutation is found in either NPHS2 or WT1. Magnetic resonance imaging of the brain shows so far non-diagnostic white matter lesions in the two older boys. Growth hormone defi ciency is diagnosed in all three. Growth hormone defi cieny is effectively treated by daily hormone injections, proteinuria well contolled by ACE inhibitor. In summary in this family an inherited X-linked disorder more than mere association is assumed. mutations we identifi ed, 736G>T(E246X) and nt2515del(C) were unique to Japanese and more frequently detected than other mutations. In that paper, we speculated as to whether the mutation is polymorphism or etiological cause using just the mode of inheritance, the preservation in species of amino acid, and a comparison with the mutations in healthy individuals. We examined functional analysis for the three NPHS1 mutations in vitro.

We generated the mutant nephrin expression vectors using a pcDNA3 vector containing human nephrin cDNA and a QuikChange II XL Site-Directed Mutagenesis Kit (Stratagene). Transfections of plasmids into HEK293 cells were performed using lipofectamine (Invitrogen). These cells were examined by an immunohitochemical study using an anti-nephrin antibody and a secondary antibody conjugated to FITC without a preincubation with triton-X.

The cells transfected plasmids carrying either 736G>T(E246X, nt2515del(C) were less stained by the anti nephrin antibody than the cells transfected with the wild type nephrin vector.

Conclusions: This functional analysis can help to judge whether the mutation is the etiological cause of CNS. We concluded both mutations are playing a crucial role in the etiology of CNS. Objectives: Studies on murine models demonstrated that Sprouty1 protein, encoded by Spry1 gene, modulates Gdnf/Ret signal, which activates a crucial gene network in urinary tract development. Spry1-knockout mice develop supernumerary ureteric buds, which result in multiple ureters and kidneys. In the literature, there is only one report about mutational analysis of human SPRY1 gene, the homologue of murine Spry1, even if the gene is known to be expressed in human fetal renal tissue. Methods: We carried out mutational analysis of SPRY1 gene in patients with duplex ureter: 23 isolated and 4 familial cases. On each DNA sample, the coding region and 5'UTR were analysed by SSCP and all PCR products were directly sequenced (ABI PRISM 3100 Applied Biosystem). DNA from 6 subjects without kidney and urinary tract anomalies was used as control. Results: We detected 5 polymorphisms (SNPs), previously reported in databases, and 1 nucleotide substitution, which has never been reported. The frequency of this substitution was estimated in 127 umbilical cord blood DNA samples and was 0.094.

In order to understand SPRY1 role in renal development, mutational analysis will be extended to a population of subjects with different malformative nephrouropathies and association studies with the detected polymorphisms will be performed. Objectives: We present two Croatian patients with Dent disease. Methods: First patient is a 12 years old boy diagnosed with persistent asymptomatic proteinuria and normal renal function at the age of 3 years. First renal ultrasonography (US) was normal. Kidney biopsy showed FSGS. 6 years later low-molecular-weight proteinuria, hypercalciuria and nephrocalcinosis grade II were found. Second patient is a 12 years old boy referred for nocturnal enuresis at the age of 6 years. Low-molecular-weight proteinuria and hypercalciuria with normal renal function were found. US showed nephrocalcinosis grade II. Kidney biopsy showed total sclerosis of 9% of investigated glomeruli and calcifi cations in interstitium and medular tubule. Results: DNA analysis of CLCN5 gene in fi rst patient revealed the 637delT mutation in exon 4 leading to a frame-shift mutation introducing a premature stop codon in position 137 of the protein chain (C116VfsX137). CLCN5 gene analysis in second patient showed a complex allele (IVS2-17T>G; H731P) constituted of nucleotide substitution in intron 2 and exon 12 leading to a missense mutation changing histidin with prolin in protein chain. This allele was inherited from the mother who is heterozygous for the complex mutation. Conclusions: These patients are the fi rst two patients with Dent disease confi rmed by DNA analysis in Croatian population. To our knowledge the mutation H731P is a novel mutation not reported before. previously appreciated. While most of these lesions have no morbidity before the third decade, many display steady growth and some warrant embolization and rarely even resection. Regular follow up of these lesions is required to identify those children for whom medical or mechanical intervention may eliminate renalassociated mortality and preserve renal longevity.

Prophylactic Objectives: Renal lesions are seen in a majority of patients with Tuberous Sclerosis Complex (TSC), most of whom have renal angiomyolipomas (AMLs). Since hemorrhage from these vascular hamartomas is a leading cause of mortality in TSC, prophylactic embolization is recommended for AMLs that exceed 4 cm in their longest diameter (LD). This project reports a single center experience with renal AML embolization in a series of 10 children with TSC. Methods: Retrospective chart review at the MGH Herscot Center for TSC identifi ed 10 patients assessed by angiography for prophylactic embolization of renal AMLs before age 21. Embolic methods, peri-procedure complications and long term changes are described.

Results: Thirteen of 16 renal AMLs (mean LD 6.7 cm; range 2.8-12.7) evaluated by renal angiography were selectively embolized in 10 children with TSC (mean age 12.6; range 5.8-19.5) in a total of 12 separate procedures. The only AML embolized to arrest bleeding was the smallest one in the series. Some children required antibiotics and glucocorticoids to treat post-embolization syndrome. None exhibited increases in baseline blood pressure or creatinine in a mean follow up of 26 months (range 6-60).

The AML size best suited for prophylactic embolization in children with TSC has yet to be determined. Most in our series were considerably larger than 4 cm and the only one to bleed was smaller. Nonetheless, the procedure is apparently safe in children with large renal AMLs, with no induction of hypertension or renal insuffi ciency and no post-procedure hemorrhage to date. Objectives: The objective of this study is to investigate the association between IL-8 gene 2767 G/A polymorphism and clinical features, kidney involvement and prognosis in childhood Henoch Schnölein purpura (HSP). Methods: A total of 115 patients with HSP (59 male, 56 female) were included in the study with age at diagnosis between 2 and 17 (8.0±3.0 years). Hundred and eight healthy adults were included in the study as controls. The patients had been followed up for kidney involvement for at least 6 months, and in average 8.2±7.5 months. Interleukin 8 (IL-8) gene 2767 G/A polymorphism was studied by PCR-RFLP method. Results: Frequency of the "A" allele was 0.37 in the patient group, whereas it was 0.36 in the control group. The difference was not statistically signifi cant (p=0.696). No association was detected between the IL-8 gene G/A polymorphism and the clinical, laboratory and demographic data related to the patients with HSP. Kidney involvement was more common in those with the G/A polymorphism of the IL-8 gene. While a 0.44 frequency of the "A" allele was detected in those with kidney involvement, this rate was 0.29 in those with no kidney involvement (p=0.046). Follow-up of those with the "A" allele revealed higher proteinuria (p=0.023, odds ratio 0.176, %95CI 0.034-0.917), and higher creatinine levels (p=0.049, odd's ratio 0.024, %95CI 0.036-0.094).

These results suggest that the kidney involvement to be more common in patients with the "A" allele, and higher proteinuria and creatinine levels in these patients at follow-up.

Abstract# 629 Objectives: 1) Determine the prevalence of mutations in TCF2 and PAX2 genes in the North American CKiD population with RHD.

2) In patients with no mutations in TCF2 or PAX2, explore the contribution of rare copy number variations using high-density SNP arrays. Methods: Genomic DNA was obtained from the NIH biorepository. We performed direct sequencing of TCF2 and PAX2 exons and intron boundaries. Sequence traces were analyzed using Sequencer 4.8 software. Pathogenic relevance was inferred by publicly available prediction programs. Results: We received 73 DNA samples of children with RHD. To date, we have confi rmed 5 likely pathogenic variants (7% of cases). Two are previously noted pathogenic mutations in TCF2: a premature termination signal (p.R181X) and a missense mutation (p.S148L). In PAX2, we found one missense mutation (p.G24E), one coding frame shift mutation (G24fsX28), and one splice site mutation (IVS4-1G>T) which are all likely pathogenic and have not been noted previously. These fi ve cases are Caucasian.

A proportion of patients with non-syndromic RHD carry mutations in either TCF2 or PAX2 genes. These patients should be evaluated for complications (e.g. diabetes for TCF2 mutations, colobomas for PAX2) and referred for genetic counseling.

A genome-wide screen for novel pathogenic structural variants in patients without mutations in these genes is ongoing.

Additional Objectives: Atypical hemolytic uremic syndrome (aHUS) is associated with predisposing mutations in genes encoding complement (regulating) proteins and with the presence of autoantibodies against complement factor H (αFH). About 50% of the patients progress to ESRD. In this study, we performed linkage analysis and mutational screening in a family with aHUS. Six family members have low C3 plasma values; three of them are diagnosed with aHUS with an age at onset of the disease of 33, 14, and 2, respectively. Methods: Linkage analysis was performed; in case of a positive LOD score, the function of the proteins encoded by genes located between these markers was checked in databases. In addition, mutational screening was performed for complement (regulating) genes: CFH, IF, MCP, C3, and FB. Two patients were tested for the presence of αFH. Results: Linkage was found on chr. 1 (LOD score: 1.73) and chr. 15 (LOD score: 1.46). No complement candidate genes were identifi ed within these regions. A heterozygous mutation (K323E) was found in FB, located on chr. 6, in the family members with low C3 values, including the aHUS patients. This change has been identifi ed as a gain of function mutation resulting in a C3bBb complex that is more resistant to decay by DAF and CFH. No genetic changes were found in the other screened genes and no αFH were identifi ed. Conclusions: A pathogenic mutation (K323E) was found in FB in three aHUS patients and three family members. An incomplete penetrance of the disease is found, indicating that, next to genetic predispositions, other factors are involved in the development of aHUS.

The Objectives: In infantile cystinosis document proteinuria and electrolyte supplementation following delayed initiation of cysteamine. Methods: Protein to creatinine ratio (PCR), albumin to creatinine ratio (ACR), alpha 1 microglobulin to creatinine ratio (α1MCR) and electrolyte supplementation was analysed in relation to the initiation of cysteamine and for the last 2 years. Results: At 7 months of age this infant was diagnosed with cystinosis with an elevated WBC cystine level of 0.7 nmol ½ cystine/mg protein (NR <0.1). Despite advice to start immediate treatment, cysteamine was only initiated at 17 months of age and reached a therapeutic dose (1.33 g/m 2 ) at 22 months of age with WBC cystine levels thereafter between <0.1 and 0.3 nmol ½ cystine/mg protein.

Proteinuria improved after the initiation of cysteamine with a log plot of α1MCR vs ACR showing a linear progression towards the normal range suggesting an improvement in tubular proteinuria. The table shows a 75% reduction in PCR, a 79% in ACR, and a 64% decrease in α1MCR when comparing the pre-treatment with the late time period. There was also a reduction in the requirement for electrolyte supplementation with this effect being greatest for bicarbonate. Conclusions: These data suggest that there is an improvement in tubular function as measured by proteinuria and electrolyte supplementation after the start of cysteamine in cystinosis.

Study Methods: Blood samples were collected for genetic analysis from 3 probands from Chinese families with autosomal recessive SRNS, and their siblings and parents, and 50 adults with normal urinalysis. Genomic DNA was isolated from blood leucocytes. Ten exons, exon-intron boundaries of WT1 and 31 exons, exon-intron boundaries of PLCE1 were amplifi ed by polymerase chain reaction. Mutational analysis was performed by DNA sequencing directly. Results: No mutation of WT1 and PLCE1 in all exons and exon-intron boundaries was found in the 3 probands. However, three variants of WT1 and 13 variants of PLCE1 were identifi ed in all 3 probands and some controls, indicating that these variants are polymorphisms. One novel variant of WT1, IVS5-64A>G, and one novel variant of PLCE1, IVS22-26T>A, were also identifi ed in two probands as well as some controls. There was no signifi cant difference in the allelic frequencies of the two polymorphisms of WT1 and PLCE1 between the patients and controls.

The results suggest that WT1 and PLCE1 mutations are not major causes of familial SRNS in Chinese in the study. Methods: Peripheral blood samples were collected for genetic analysis from 6 children with CRF in Chinese Han ethnic group, and 50 unrelated adult volunteers in Chinese Han ethnic group whose urinalysis was normal studied as controls. Genomic DNA was isolated from peripheral blood leucocytes. Eight exons and exon-intron boundaries of NPHS2, and 10 exons and exonintron boundaries of WT1 were amplifi ed by polymerase chain reaction (PCR). Mutational analysis was performed by DNA sequencing directly and RFLP/PCR (RFLP, restriction fragment length polymorphism).

No mutation in both NPHS2 and WT1 was detected in the 6 patients with CRF, whereas two already reported polymorphisms of NPHS2, 102G>A and 954T>C, and 4 variants of WT1, 5'-UTR-7G>T, 126C>T, IVS5-9T>C and 903A>G, were identifi ed in some of the patients. Three already reported variants of WT1 (5'-UTR-7G>T, 126C>T and 903A>G) were also identifi ed in some controls, indicating that these variants are polymorphisms. There was no signifi cant difference in the genotypic and allelic frequencies of 102G>A of NPHS2, and 5'-UTR-7G>T, 126C>T and 903A>G of WT1 between the 6 patients and 50 controls, respectively. In addition, a novel variant, IVS5-9T>C of WT1, was identifi ed in one patient and his father whose urinalysis was normal, whereas it was not found in 50 controls. Conclusions: Mutations in both the NPHS2 gene and the WT1 gene are not major causes of the six children with CRF in the study. 

Results: A heterozygous missense mutation in exon 5 of NPHS2, 596A>T, which leads to an asparagine to isoleucine substitution (N199I) and is novel, was detected in the child, whereas it was not found in 100 chromosomes from 50 controls. A heterozygous silent mutation of 954T>C in exon 8 of NPHS2 was also detected in the case. Objectives: Children with nephrotic syndrome the relapses are frequently triggered by viral upper respiratory tract infections, possibly mediated by cytokine release. Montelukast sodium has been successfully used to reduce the exacerbations of bronchial asthma due to viral infections in children. The hypothesis that montelukast sodium would reduce the incidence of viral infections thereby reduce the risk of relapse was tested by this study. Methods: Sequential patients receiving low dose (<0.6mg/kg) prednisolone on alternate day with or without adjuvant therapy as maintenance were recruited. Montelukast sodium was prescribed for a period of one year at a dose of 4mg for children below 5 years and 8mg for above 5 years. The number of relapse episodes was compared for the twelve months before and after commencing on montelukast therapy. A freshly voided urine sample was tested each morning and the presence of 3 + proteinuria 3+ proteinuria for three consecutive days was diagnostic of relapse. Results: 118 patients were recruited and 112 completed the study. 79 male ; 33 female. Age at entry ranged from 2.5 to 13.2 (median 6.2) years. The rates of relapse episodes before and after montelukast therapy were analysed by using a Poisson regression model. There was a signifi cant reduction in the rate of relapse during the 12 months of montelukast therapy when compared to the 12 months before (p<0.01). The average yearly relapse episode rate was 1.42 during montelukast therapy compared with 2.51 for the 12 months before.

The results suggest that prescribing montelukast sodium can reduce the risk of relapse in frequently relapsing nephrotic syndrome. The results however need to be confi rmed by randomised controlled studies. Objectives: The inulin clearance is regarded as the gold standard measurement for the calculation of the kidney function in children, but it is time consuming and expensive. Simpler methods like the "old" serum creatinine based Schwartz-formula (OS) are available. GFR can also be calculated by cystatin-C based formulae e.g. the "new" Schwartz-(NS), LeBricon-(LB), Larsson-(L), and Rule-formula (R). The goal of this study was to compare the GFR calculated by using these four formulae with the OS-formula.

The GFR was calculated in 281 children with renal diseases using the above mentioned formulae (1003 measurements). Correlation analyses and analyses by the Bland-Altman-plot were performed. Results: Although, there was a signifi cant correlation between the GFR calculation according to NS, LB, L, R and OS, the Bland-Altman-plot showed a mean negative difference of GFR between these formulae and the OS-formula. Objectives: Autosomal-recessive polycystic kidney disease (ARPKD) is an inherited progressive disease leading to ESRD. Almost all patients develop liver fi brosis that may require liver-kidney-transplantation. Early detection and quantifi cation of fi brosis can provide useful information for management of patients with ARPKD. Methods: We report the use of a new sonography based method (transient elastography, Fibroscan®) for early and noninvasive detection of liver fi brosis in patients suffering from ARPKD. This method is already well established in adult patients with hepatic disease, measuring ultrasound wave spreading as an indicator for tissue stiffness. Results: ARPKD patients (n=7, mean age 9.5 years) showed a signifi cantly increased liver stiffness compared to matched healthy controls (11.2 vs. 4.1 kPa; p=0.018). All patients had results above the upper reference value (7.5 kPa), while healthy controls and three patients with renal cysts of other reasons and no liver fi brosis were within normal reference values. Conventional sonography in ARPKD patients gave evidence for hepatic fi brosis in 4 patients, while 3 had still normal liver ultrasound.

The presented method provides an early sensitive non-invasive detection of liver fi brosis in children with ARPKD. This method could provide diagnostic information for unclear cystic renal diseases by identifi cation of liver involvement. Objectives: To estimate glomerular fi ltration rate using cystatin C based prediction equations and compare them with serum creatinine derived GFR for detection of early chronic kidney disease (CKD). Methods: 111 children (age 2-17 yr) with GFR between 60-90 ml/min/1.73m 2 were studied. Serum cystatin C (CysC) and creatinine (Cr) were determined by immunonephelometry using DAKO PET kit and kinetic Jaffe method respectively. Creatinine derived GFR was estimated using 2 equations: (1) Schwartz (0.55 x height/Cr), (2) our creatinine formula (0.43 x height/Cr). GFR was derived from cystatin using 3 equations: (1) Larsson (99.43 x CysC -1.5837 ), Objectives: Effacement of podocyte foot processes occurs in many proteinuric nephropathies and is accompanied with rearrangement of the actin cytoskeleton. In this study the intracellular pathways of EGF in podocytes, which induced abnormality of cytoskeletal architecture and dysfunction of podocytes, was investigated. Methods: EGF expression was detected both in nephrotic rat model and podocyte injury induced by puromycin. F-actin distribution, nephrin expression, and RhoA activation as well, were detected in mouse podocytes after EGF treatment. Results: Both in nephrotic rat model and podocyte injury induced by puromycin, an increased EGF expression was revealed. In addition, redistribution of F-actin fi bers and down-regulation of nephrin expression in mouse podocytes were also detected with the treatment of EGF. Meanwhile the increased RhoA activity was revealed in podocytes exposed to EGF. The rearrangement of F-actin as well as the decreased nephrin expression could be rescued by the addition of Y27632, a reagent to inhibit Rho kinase-dependent stress fi ber formation.

The results from this study suggested a role of EGF in the regulation of the cytoskeleton arrangement, nephrin expression and podocyte dysfunction, which might involve the activation of RhoA.

Acute Objectives: The cause of postural proteinuria is not clear but may often relate to obstruction of the left renal vein in the fork between the aorta and the superior mesenteric artery (= renal nutcracker). However, reports dealing with proteinuria only marginally refer to this cause of postural proteinuria. We performed a formal analysis of the literature. Methods: All reports published in peer-reviewed journals through 1992 were considered. For the fi nal analysis, we retained 13 reports providing details on renal nutcracker in postural proteinuria. Results: Five reports addressed the frequency of renal nutcracker in a total of 229 subjects with postural proteinuria. Their aged ranged between 5 and 17 years (female-to-male ratio: 1.05:1.00. Imaging studies demonstrated renal nutcracker in 156 (68 percent) subjects. Renal nutcracker was also demonstrated in 10 anecdotal reports for a total of 51 subjects with postural proteinuria. Very recently, 13 Italian subjects with postural proteinuria associated with renal nutcracker were reassessed ≥6 years after the initial diagnosis: in 9 subjects both postural proteinuria and renal nutcracker had disappeared, in 3 subjects both postural proteinuria and renal nutcracker had persisted, and in one case postural proteinuria had persisted unassociated with renal nutcracker.

Conclusions: This review of the literature provides substantial support for renal nutcracker as the most common and benign cause of postural proteinuria. Objectives: HUS is a common cause of acute kidney injury in children. The aim of the study was to analyze the clinical course and prognosis in children with HUS.

The study group consisted of 23 children with HUS treated 1996-2009, 11 girls and 12 boys aged 3 months -9 yrs ( 3 yrs 3 m). 14 pts had typical D+HUS (preceded by diarrhea), in 9 atypical D-HUS was diagnosed. The clinical course was analyzed after mean 4,2±4 yrs of follow-up (3 m -13 yrs).

The general condition of patient at admission was severe in 17/23 (74%) cases (7 neurological signs, 2 pancreatitis). In 5/14 D+ pts enterohemorrhagic E.coli was diagnosed. In D-HUS pts 1 tonsillitis, 1 streptococcal sepsis, 1 varicella, 4 respiratory tract infection were diagnosed. The renal replacement therapy (RRT) was introduced 1,8±1,5 days after admission in 16/23 pts (4 HD a.

12 PD). Anuria occured in 12/23 patients for 5-49 days (mean 16±12). 3/14 D+ and 4/9 D-HUS patients improved without RRT. Plasmapheresis was performed in 3/14 D+ and in 6/9 D-pts (with dialysis 2 and 4 respectively). another 10 pts received fresh frozen plasma (8D+, 2D- Objectives: Parvovirus B19 (ParvoB19) infection has been reported in some types of vasculitis such as Henoch-Schonlein purpura (HSP). Renal involvement may be induced by both an indirect (immunocomplex) and direct (TNF-alfa-mediated) mechanism. Methods: In a 6-months period in 2009, 5 children with HSP and persistent proteinuria or nephritic/nephrotic syndrome underwent renal biopsy in our Unit.

We screened blood and renal biopsy samples of the patients for ParvoB19 and other viruses (EBV, CMV, Adenovirus, HHV6 and 8).

Results: ParvoB19-DNA was positive in both blood and renal tissue in 2 children. In both boys, an upper respiratory tract infection had preceded the onset of symptoms. The fi rst boy (4 years) had a histological picture of mesangioproliferative glomerulonephritis. After two i.v. Ig courses, viraemia disappeared in 2 weeks and proteinuria in 2 months. The second boy (5 years), had a histological picture of mesangial glomerulonephritis with crescents in 80% of glomeruli. Viraemia and proteinuria failed to decrease after i.v. Ig injection, prednisone, plasmapheresis with oral cyclophosphamide, and mofetil-micofenolate therapy. Anti-TNF-alfa-antibody (Infl iximab) was then administered. Proteinuria desappeared in 6 months and viraemia after 9 months. No crescents were detected in the control biopsy.

We suggest a screen for viral infections in patients with acute vasculitis-associated renal involvement. In ParvoB19 associated forms, Infl iximab may be used as rescue therapy in patients with glomerulonephritis who do not respond to conventional therapy. Objectives: To document the prevalence of proteinuria and microalbuminuria in HIV infected children. Methods: Consecutive HIV-infected children aged zero to 16 years admitted to our institution whose parents/guardian gave written informed consent were studied. From each child demographics and HIV clinical and immunological parameters were documented. Urine was tested with dipstick for protein and if present was sent for protein-creatinine ratio; if negative it was tested with dipstick for microalbuminuria. Urine samples testing positive for microalbuminuria were sent for microalbumin-creatinine ratio. Immunological staging of HIV disease was done using the CDC criteria. Continuous and dichotonuous data were summarized as mean ±SD and proportions respectively. Univariate analysis was performed were appropriate. Results: Thirty-six children aged 0.2 to 10.9 years (2.8 ±3.1) with 58.3% males were studied. Fourteen (38.9%) had advanced immunosuppression and 17 (47.2%) were on HAART. The frequency of proteinuria, nephrotic range proteinuria and microalbuminuria were 38.9%, 11.1% and 11.1% respectively. The frequency of proteinuria was not statistically different across gender, immunological stage of HIV disease, use of HAART or otherwise and age ≤2 years or older.

Hospital. Microalbuminuria is also more common than the general population and may be an early marker of subclinical renal disease.

Abstract# 660 Objectives: To evaluate functional response of kidney to oral protein load (OPL) in children with single functioning kidney (SFK).

Methods: RFR was measured during 45 studies in 37 children (25 boys) median age of 10 years (range 2,5-16) as the difference between the baseline clearance of creatinine (CsCr) and CsCr following a meat-free protein meal (1 g/kg body weight). Most patients had congenital SFK due to renal agenesis (54%) or multicystic dysplastic kidney (22%), while remaining patients had nephrectomy. Cimetidine was given 48h prior to the study at daily dose of 12 ±0.75 mg/kg, while they were on a diet free of meat, fi sh and fowl. Ambulatory blood pressure (ABP) monitoring was performed with Spacelab 90207. Serum Cystatin C was examined before and 2 hours after OPL and urinary protein/creatinine (mg/mg) was determined from accurately timed urine collection of approximately 2 h duration pre and post OPL. Data were expressed as mean±SEM. Results: After OPL CsCr (92.81± 4.95 vs 81.04±4.72) and proteinuria/creatinuria (0.32±0.05 vs 0.23± 0.02) signifi cantly increased while serum cystatin C remained unchanged. There was no difference in RFR between patients with congenital and acquired SFK(4.27±7.55 vs 6.72±11.96). RFR was inversely correlated with stimulated proteinuria, but not with ABP.

The defective renal response to OPL manifested by reduced RFR and increased proteinuria may be markers of glomerular hyperfi ltration in children with single functioning kidney. No change in serum cystatin C after protein load was observed. The lack of correlation between RFR and ABP suggest that RFR is not directly modifi ed by blood pressure.

Renal Involvement in Obese Children and Adolescents A. Savino, P. Pelliccia, C. Cecamore, C. Giannini, T. de Giorgis, A. Mohn, F. Chiarelli.

Objectives: The present study aimed to investigate whether renal function indexes (GFR estimated by the Schwartz formula -eGFR-, cystatin C -CysC-, albumin excretion rate -AER-), nitric oxide (-NO-an important modulator of renal function and morphology), urinary isoprostanes (a marker of oxidative stress) and blood pressure (BP) modifi cations can be detected in obese children and adolescents when compared to normal weight controls. Methods: 107 obese children and adolescents and 50 controls were evaluated. Blood and urinary samples were collected to evaluate markers of renal function, serum and urinary NO and urinary isoprostanes. Ambulatory BP monitoring was performed in all. Obesity and insulin resistance (IR) degree were expressed by SDS-BMI and HOMA-IR. Results: CysC and e-GFR did not signifi cant differ between the 2 groups. AER was increased in obese children. CysC and GFR were related to HOMA-IR, AER was related to SDS-BMI and HOMA-IR. Signifi cantly reduced NO levels and increased urinary isoprostanes and BP measurement were observed in obese children. All were signifi cantly related to adiposity and IR indexes. Conclusions: Difference seen in AER supported a role of obesity and IR in the genesis of glomerular leaking of albumin; we also observed differences in NO and urinary isoprostanes, but they were probably related to adiposity and IR rather than represent a clear risk factor for renal disease. Renal involvement is not an early manifestation of obesity in childhood, since no relevant differences can be detected when comparing renal function indexes of obese and non-obese children. A longer exposure to obesity and IR is probably needed. Objectives: We aimed to asses effects of prematurity and on blood pressure and renal functions in medium-and long-term by evaluating the children older than 5 years who were previously monitered for prematurity. Methods: One hundred and four children who were born as premature between 2000 and 2004 were enrolled in this study. All children were examined, blood pressures were measured and renal function tests were calculated by analysing urine and blood chemistry.

The mean age of 104 children (56 boys, 48 girls) included the study was 8.1±0.9 years. Growth retardation was found in three and obesity in fi ve of the children. Blood urine nitrogen (BUN) and serum creatinine levels were in normal ranges in all children. Six children had mild reduction in GFR. Increased phosphorus excreation in urine was found in two children, natriuria in four, kaliuria in eight, mild proteinuria in 11. In the ultrasound evaluation of 208 kidneys, renal heights in 59 kidneys were found smaller than expected values in same age group. At causal blood pressure measurement, all children were normotensive. However, at ambulatory blood pressure monitoring, mean systolic blood pressure was higher than 95 percentile in 10 children, mean diastolic blood pressure in 14 children and mean arterial pressure in nine children.

Conclusions: Children who were born as premature may have increased blood pressure and impaired renal functions in advancing years. Periodic monitoring of blood pressure and renal functions may be helpful to early detection of possible hypertension and renal dysfunction.

Compliance with H1N1 Immunization in Children with Chronic Renal Disease N. Printza, E. Farmaki, J. Bosdou, C. Goga, F. Koukourgianni, F. Papachristou. 1st Pediatric Department, Aristotle University, Thessaloniki, Greece. Objectives: WHO recommended the immunization of children with chronic renal disease (CKD) and immunosuppressed patients as the most effective way to reduce H1N1 morbidity and mortality. Aim of our study was to evaluate the compliance of our patients with H1N1 vaccination. Methods: A total of 64 eligible children/parents were approached to fi ll in a standardised questionnaire on infl uenza immunization profi le and kind of infl uence on being or not vaccinated with H1N1 vaccine. Objectives: Data on health-related issues in children and adolescents with a migration background are yet scarce but needed for future public health planning. The present study was conducted to obtain data on migration background, family social and fi nancial status, education, and patient satisfaction of children and their families treated by our institution. Methods: In a cross sectional survey of patients seen during 2008 in the Pediatric Nephrology Outpatient Department at the Charité University Children's Hospital in Berlin, a total of 348 families answered a prepared questionaire. The fi nal data set contained basic information, a standardized patient satisfaction score, a subjective categorical rating of disease severity and satisfaction with treatment, and a subjective categorical external evaluation (by doctors and nurses) of patient compliance. Results: A migration background was present in 131 patients (38%). Patient satisfaction (on a scale from 8-40) was signifi cantly higher in families without (32.9 + 4.6) than in those with a migration backgroud (30.8 + 4.7; p<0.0001). In contrast, patient satisfaction was not signifi cantly associated with income, education (school level achieved by parents), and religious background. Patient satisfaction was not signifi cantly associated with subjective ratings of disease severity or friendliness of nurses or doctors, but showed a signifi cant correlation with trust in doctors (p<0.0001). There was no apparent correlation of patient satisfaction with compliance. Conclusions: Measurement of patient satisfaction by the ZUF-8 score is a promising tool for evaluating quality of care in pediatric populations. Objectives: Background: Age related functional decline has been associated with macro-autophagy and chaperone mediated autophagy. This association has been characterized in many organs. Since the kidney suffers a functional deterioration with age and the molecular mechanisms responsible are not fully identifi ed, we have studied the expression of autophagy related molecules in kidneys of aging mice. Methods: Methods: Immunoblot of mouse renal tissue from three different age groups (young, middle aged and old) was performed for essential proteins of the two major autophagic pathways in mammalian cells: macro-autophagy and chaperone mediated autophagy. Functional studies were carried out using lysosomes isolated from kidneys of the same mice groups. Results: Results: 1) Levels of both effector and regulator proteins of macroautophagy and chaperone mediated autophagy increase considerably with age in mice.

2) The ability of lysosomes from old mice kidney to take up cytosolic proteins for degradation is impaired. Conclusions: Conclusion: Expression of proteins related to macro-autophagy and chaperone mediated autophagy is decreased in a time dependent fashion in kidney and this may contribute to the observed functional decline in autophagic activity in this organ with age. Objectives: The study attempts to determine the effect that the condition has on enuretic children and their parents as well as their opinion of it. Methods: 104 enuretic children (7 -14 years) and their parents were surveyed on the effects of enuresis on their lives. Both groups were asked the following questions: Do you consider enuresis a normal condition? Do you consider enuresis to be a disease? Do you feel indifferent, angry, or embarrassed about enuresis? Does enuresis limit your ability to socialize?

Parents were asked if their children felt guilty about their enuresis Children were asked if their parents worried about their problem. Results: 77% of parents, but only 18% of children consider enuresis a normal and frequent occurrence 22% of parents and children consider enuresis a disease Regarding their children's enuresis, parents feel: indifferent (77%), angry (8%) and embarrassed (4%). Enuretic children feel: indifferent (33%), angry (13%) and embarrassed (53%) 25% of the parents and 17% of the children believe that enuresis affects the children's ability to socialize 28% of the parents believe that enuresis elicits feelings of guilt in their children and 21% of the children believe that their parents are not worried about their enuresis. Conclusions: Enuresis is not seen as a normal condition and it causes embarrassment for the children who suffer it. However, most parents consider enuresis a normal condition and do not experience any negative effects of their children's circumstances. 22% of the parents and enuretic children still consider enuresis a disease.

Urinary Objectives: to evaluate the urine levels of TGF-β1 as a noninvasive marker in the pathogenesis of tubulointerstitial damage in children with different grade of vesicoureteral refl ux (VUR). Methods: 33 patients aged between 1 and 14 years (78,8% female) with VUR and 10 normal children (control group) were enrolled in the study. Recently, a more accurate test for renal parenchymal damage such as the DMSA scan has been used. Children were divided into 2 groups according of renal parenchymal scars: gr. I -24 patients (1-2 scars) and gr. II -9 patients (more than 3-4 scars). Urinary excretion of TGF-β1/ creatinine was measured by ELISA method.

Results: all the patients demonstrated signifi cantly elevated urine level of TGF-β1 in comparison with controls. Children with high degree of nerosclerosis showed higher urine concentration of TGF-β1/creatinine than patients with RN gr.1-2 (p<0.05). Conclusions: high urine level of TGF-β1/creatinine is closely associated with renal parenchimal scars in children with different degree of VUR. The results of this study have showed the important role of TGF-β1 in progression of interstitial fi brosis and maybe used as diagnostic marker for renal parenchymal scarring.

Abstract# 671 Objectives: β -Thalassemia minor is a common heterozygous hemoglobinopathy that is characterized by both microcytosis and hypochromia. It has been postulated that low grade hemolysis, tubular iron deposition and toxins derived from erythrocytes might cause renal tubular damage in adult patients with β -thalassemia minor. The aim of this study is to investigate the renal tulbar function in children with βthalassemia minor and to determine its possible harmful effects.

The study was conducted on 50 children (22 male and 28 female) at the age of years (range 4-19 years) with β -Thalassemia minor. A control group was formed with 50 healthy children whose ages and sexes match those in the fi rst group. Blood and 24-hour urine samples were obtained for hematologic and biochemical analysis.

Results: There was statistically signifi cant difference among the two groups in terms of the results of FE UA (%), TPR (%), FE k (%), GFR, Urine uric Acid, serum Mg (P < 0/05). But other signifi cant signs of renal tubulopathy such as hypercalciuria, and tubular proteinuria (β 2 -microglobolinuria) were not seen. Conclusions: On the contrary of children with β -Thalassemia major, renal tubular dysfunction is not common in children with β -Thalassemia minor. For this cause following up the patients with β -Thalassemia minor after 20 years is reasonable. Alsow it suggested that in future studies to confi rm renal tubular disfunction should be considered specifi c test such as NAG measurement.

Determining There was no signifi cant difference in urinary protein/kreat ratio and mean blood cystine-levels of the last 5 years. Conclusions: Renal failure and not fanconi syndrome seems to be an indicating factor for thyroid deterioration. A renal transplant does not protect. Indeed, early starting with an adequate dose of cysteamine prevents or delays thyroid failure. Objectives: The fractional excretion of sodium (FeNa) may be helpful in establishing the cause of acute renal failure. This study was performed to determine the infl uence of the glomerular fi ltration rate (GFR), sodium intake and tubular function on FeNa in children without renal failure. Methods: Reliable 24h-urine collections from patients (4-18years of age, GFR >60 ml/min/1.73m 2 ) were used to determine sodium excretion, GFR and FeNa. The infl uence of tubular function was studied in 5 patients with generalized tubular dysfunction. Results: Based on data from 761 patients, a multiple regression formula was designed based on GFR and sodium excretion that predicted over 80% of the variation in FeNa (R square=0.824, p<0.001). Using this formula, the predicted FeNa was signifi cantly lower than the measured FeNa in the children with tubular dysfunction. Conclusions: FeNa depends on GFR, sodium intake, and tubular function, and interpretation is impossible without knowledge on all factors.

Therefore, no normal range for FeNa can be given. However, a large difference between measured and predicted FeNa may indicate tubular dysfunction. The clinical usefulness of our formula should be tested in other cohorts of children both with normal and abnormal tubular function. entrapment syndrome, 6 cases with idiopathic hypercalciuria and urinary tract

Objectives: The WAGR contiguous gene deletion syndrome is a combination of Wilms tumor, aniridia, genito-urinary abnormalities, and mental retardation.

Herein we report a 1.5 year old female infant with WAGR syndrome and bilateral cystic kidneys.

The index patient underwent detailed clinical examination, ultrasound of the kidneys and FISH study. Results: A 1.5 year old female infant was investigated for congenital bilateral aniridia, cataracts, glaucoma and epicantus. The ultrasound (US) scan showed several cysts in both kidneys with the overall appearance mimicking polycystic kidney disease. A small atrial septal defect and a dysplastic pulmonary valve without haemodynamic effect were also diagnosed. FISH study revealed a deletion of the WT-1 and PAX6 gene in the 11p13 WAGR region. Forty days after the fi rst kidney US, the second US revealed a 3 cm tumor in the right kidney. After nephrectomy histological analysis revealed Wilms tumour, treated successfully with the Wilm's tumor protocol.

Conclusions: Identifi cation of deletion in the WAGR region in patients with aniridia should be mandatory. In addition, Wilms tumor can have a very rapid growth, which, per se requires frequent and careful ultrasound kidney controls. Cystic kidneys may be part of the WAGR presentation and precede development of Wilms tumor.

Long Objectives: Charts of twenty-four cystinosis patients were evaluated. Methods: Nineteen of these patients were investigated for extrarenal complications. Swallowing studies, electromyography, abdominal ultrasonograpy, respiratory function test and ophthalmic examination were performed. Results: Mean age of diagnosis was 36.7 months (range 1month-14 years). Most of the patients were suffering from polyuria, polydipsia and growth retardation at the time of diagnosis. At mean age 10.6 year, 40% of patients progressed to renal failure. Five patients (20%) had renal transplantation. 84% of patients had cystine crystals in cornea and 42% had photophobia at time of diagnosis. One patient had elevated creatin kinase levels and electromyography revealed myopathy. 31% of patients were found to have gastroesophageal refl ux, and 21 % of them had remnants in esophagus while swallowing. Two out of 4 patients had restrictive pattern of respiratory functions. While 72% of patients had hepatomegaly, 38% of them had fi ne granular echo pattern and two had minimal heterogeneity of liver at ultrasonography, 55% also had increased minimum and maximum velocities of blood fl ow in portal vein. Conclusions: Renal complications are early manifestations of cystinosis, some of patients need renal replacement at fi rst decade even under cysteamine therapy, but many could survive until adulthood without great loss of renal functions. Thus extra renal complications are more common in adults, cystinotic children also have the risk of developing many extrarenal complications and deserve close follow-up and management.

Long Objectives: We aimed to evaluate endocrine involvement in 19 patients with cystinosis. Methods: Anthropometric measurements, pubertal stage were noted. IGF1, IGFBP3, thyroid functions, gonadotropins, fasting and postprandial glucose, insulin, c-peptid levels, bone mineral density were measured and oral glucose tolerance test was performed. Results: While mean age of patients (12 male, 7 female) was 11.8 years, mean age at diagnosis was 35.2 months. Mean duration of follow up was 9.7 years. 57% of patients had low body weight (≤3p), 84% had short stature (≤3p). 29% of patients have decreased bone mineral density (z score <-2), one of these patients had renal transplantation and 2 of them have end stage renal disease. 42% of the patients were found to have primary hypothyroidism, one of these Wednesday, September 1 patients had renal transplantation and other 3 patients had end stage renal failure. 10% had central hypothyroidism, one of them was on CAPD. Out of 9 patients who reached pubertal age, 33% of them had pubertal delay and one had hypogonadotrophic hypogonadism, all of these 4 were boys. OGTT was performed in 13 patients, 10% were found to have diabetes, both of them are in end stage renal failure and 26% had glucose intolerance 2 of them had renal transplantation and 2 of them were at end stage renal failure. Conclusions: Endocrinologic complications are seen in patients with poor renal functions as well as in patients with no deterioration of renal functions and under cysteamin therapy. Therefore endocrinologic functions should be meticulously evaluated to prevent future problems.

Screening for Renal Diseases in Asymptommatic Children V.H. Tru, T.T. Phong. Pediatric Nephrology, University of Medicine, Hochiminh City, Viet Nam; Pediatrics, Children Hospital N 2, Hochiminh City, Viet Nam. Objectives: Screening for renal diseases in asymptommatic children. Methods: Using dipstick to detect blood, protein, le, and nitrit First urine test for : ph, density, protein, blood, LE, nitrit The positive children receive the second urinary dipstick 7 days later. After that they were tested for 24 h proteinuria, urine culture, ultrasound. They were divided into 4 groups : Group 1: Microscopic hematuria without proteinuria. Group 2: Proteinuria without hematuria Group 3: Microscopic hematuria with proteinuria Group 4 : LE (+) and nitrit (+). Results: 762 children aged 1 to 15 years old were examined. The prevalences of grade 1,2,3,4 for the fi rst screening were 4,6 %; 0,9 %; 0,2%; 4,6 %. The prevalences of grade 1,2,3,4 for the second screening were 1,4 % 0,4 % ; 0,1% ; 1 %. In the group 4 there are 53,3 % abnormalities in ultrasound : cystitis, hydronephosis.

Conclusions: Urine screening tests should be mandatoty to detect asymatomatic renal diseases in children. Objectives: Proteinuria is an independent predictor of renal function decline. Proximal tubules overloaded with protein stimulates the production of chemokines, especially monocyte chemotactic protein 1 (MCP-1). The aim of the study was to examine the urinary levels of monocyte chemoattractant protein -1 (uMCP-1) in children according to histological diagnosis and degree of proteinuria.

The study group consisted of 49 children with proteinuria: I -20 children with idiopathic nephrotic syndrome (INS), examined twice: A -during INS relapse; B -after proteinuria subsided, group II -17 children, with persistent proteinuria due to focal segmental glomerulosclerosis (FSGS) and 12 children with IgA nephropathy (IgAN). The control group (C) contained 22 healthy children. UMCP-1 was determined using ELISA method by Quantikine kit.

The median uMCP-1/ cr. in group IA was signifi cantly higher compared to C (p< 0.05). Children from groups II and III revealed higher uMCP-1/ cr. levels when compared to MCD children and healthy controls (p< 0.01).

We found a strong positive correlation between the uMCP-1/ cr. and serum total cholesterol and LDL. Assessment of the uMCP-1/ cr. according to the histological diagnosis revealed that patients with higher uMCP- Objectives: The management of chronic kidney disease requires clinical and psychosocial knowledge to be integrated. These domains can be rather disparate and require KB across professional boundaries. We examined patterns of KB amongst the MPT to identify the barriers and facilitators to brokering clinical and psychosocial knowledge. Methods: A mixed methods research design was undertaken over 3 yrs with 2 rounds of social network analysis and interviews with all involved complimented by quarterly observations at team meetings and ward rounds. Results: KB is facilitated through development of social capital (SC). Organisational structures and human resources practices link knowledge domains and members laterally; i.e. structural dimension to SC. Co-location of team members and frequent interaction builds high trust relationships and are a prerequisite for effective KB at a healthcare system level; i.e. relational dimension of SC. Effective knowledge brokers in healthcare possess expertise related to the knowledge domains between which they are brokering; i.e. cognitive dimension to SC. Conclusions: Clinical managers can help build necessary social capital for effective knowledge exchange through the ways in which staff are socialised into a new way of working that integrates clinical and psychosocial knowledge for patient benefi t; eg ensuring the provision of psychosocial as well as clinical expertise; recruiting staff already exposed to the new way of working (eg underwent their training in the department); ensuring that situated learning across clinical and psychosocial domains ensues in meetings or ward rounds.

The Objectives: To examine Split Renal Function (SRF) over time in children with vesicoureteric refl ux (VUR) and/or urinary tract infection (UTI) where one kidney was found to contribute ≥30% to overall function on initial DMSA scan and the contralateral kidney was unscarred. Methods: We identifi ed 30 children (27 boys, 3 girls) who met the inclusion criteria and who had multiple renal radionuclide studies between January 1997 and September 2008. Information on the pathology, interventions, UTIs and scarring was recorded and SRF at 1 st and last scan was compared. Results: Twenty-seven boys and 3 girls were fi rst scanned at a median of 0.8 years (0.08-13.05). Eight patients had unilateral refl ux, 21 patients had bilateral refl ux, and 1 patient had UTIs without refl ux. High grade VUR (IV-V) was present in 70% of poorly functioning kidneys and in 38% of contralateral Objectives: Microscopic Polyangiitis (MPA) is an autoimmune disease characterized by pauci-immune, necrotizing and small-vessel vasculitis without necrotizinggranulomatous infl ammation.

We describe a patient with ANCA (-) MPA with bowel perforation and multiple vasculitic skin lesions that resulted in fi ngers amputation. Results: A four months old girl with no signifi cant medical history was admitted to the hospital because of purpuric lesions of the left fi fth fi nger and fever. Her blood pressure was 130/90 mmHg. Laboratory examinations were revealed coombs positive anemia, proteinuria, narrowing ulnar artery with blood fl ow deceleration in doppler ultrasound, a neutrophilic vasculitis involving capillaries, venules, and arterioles in skin biopsy. The complements, protein S and C and anticardiolipin antibodies levels were normal. Factor 5 Leiden and prothrombin gene mutation were negative. The patient was treated with ilioprost, pulse methyl prednizolone and cyclosporin. Despite this treatment, gangrene occurred on the second, third, fourth and fi fty left fi ngers. New purpuric lesions occurred on the third and fi fty right fi ngers, also. Gangrene was treated with fi nger amputation and purpuric lesions on right fi ngers resolved with this treatment. Later diagnosis of our patient was bowel perforation with abdominal distension and free air on x-ray. Therefore, patient was operated but bowel biopsy was non-specifi c for MPA.

In conclusion, ANCA negative MPA is rare in children but should be promptly diagnosed because it may cause mortality and morbidity if left untreated.

Hospital, Sapporo, Hokkaido, Japan.

Objectives: A long-range follow up study was performed to clarify the clinicopathological changes in long-term and low dose ciclosporin monotherapy in four patients with steroid resistant nephrotic syndrome (SRNS). Methods: Histological diagnosis showed minimal change (MC) in two patients, another had IgA nephropathy (IgAN) and the fourth patient had membranoproliferative glomerulonephritis (MPGN). Patients' age at initial onset ranged from 7 to 16 years old. Ciclosporin monotherapy for at least three months was started at 3-7mg/kg/day at relapses of nephrotic syndrome that occurred after using large amounts of steroids. When proteinuria decreased from 4.2-15.4g/ day to below 0.3g/day, ciclosporin dosage was reduced to below 1.0mg/kg/day. Renal biopsies were performed before steroid therapy and at 3 to 17 years after initiation of the therapy. Results: 1) A marked reduction of proteinuria (0.1-0.3g/day) was observed in all patients. 2) Post-therapy biopsies after 3-17 years of ciclosporin treatment showed mild tubular atrophy accompanying interstitial fi brosis in only one case, the patient with IgAN. In the two cases with MC, no nephrotoxicity was detected.

In the patient with MPGN, gradual decreases in endocapillary proliferation and in double contours were observed.

3) The complete remission could be obtained despite ciclosporin monotherapy being terminated 5 to 13 years after the onset of nephrotic syndrome in all cases. Conclusions: Long-term and low dose ciclosporin monotherapy proved to be quite effective for patients with SRNS because of the low incidence of adverse effects such as nephrotoxicity and a better quality of adult life could be achieved.

Value 

we concluded that RI is increased in TIDM as early as it can be a predictor marker for DN and that it may refl ect increased GFR, it is increased with poor glycemic control, and it is independent to microalbuminuria.

How Should GFR Really Be Estimated in Children? K. Zachwieja, 1 P. Korohoda, 2 J. Kwinta-Rybicka, 1 M. Miklaszewska, 1 J. Berska, 1 J. Bugajska, 1 J.A. Pietrzyk. 1 1 JUMC, Cracow, Poland; 2 AGH, Cracow, Poland.

Objectives: The aim of the study was to assess the various methods of estimating GFR in children.

The study group consisted of 123 patients aged 2-19 (mean 12.5±4.2). GFR was calculated according to: 1. the classical Schwartz method (S1); 2. the Schwartz equation with a new coeffi cient (S2); 3. the new Schwartz equation based on three markers (S3); 4. creatinine clearance based on daily urine collection (DUC); 5. the Filler equation (F). The results were compared to the reference method of serum iohexol elimination technique (I).

Results: The best agreement in GFR was achieved between S3 and I (r=0.84, p<0.0001), as corroborated by Bland-Altman analysis. The lowest accordance was observed for DUC and I (r= 0.68, p<0.00001).The differences between the methods of Schwartz, new Schwartz, Filler, DUC and the reference iohexol method were greater for values of GFR > 60 than for GFR < 60 ml/min/1.73m 2 . It was shown that Filler and and the classical Schwartz equation overestimated GFR by a mean of 21 and 15.5 ml/min/1.73m 2 , respectively, in comparison to the iohexol method, and the S2 and the S3 formula underestimated GFR by 15.1 and 17.6 ml/min/1.73m 2 , respectively. GFR according to DUC had the lowest precision and an unpredictable error. The same tendency was noted in children with BSA < 1m 2 and BSA> 1 m 2 . Conclusions: Estimating GFR by means of a single formula might be imprecise, especially at high GFR. The three markers Schwartz formula yields the lowest error in comparison with the reference iohexol method. Objectives: It is known that excessive intake of sodium leading to hypernatriuria (> 3 mEq/kg/day) is associated with hypercalciuria (> 4 mg/kg/day) and/or hyperuricosuria (10 -15 mg/kg/day). The objective was to evaluate this association in children and adolescents referred to an university clinic because of hematuria. Methods: Observational and retrospective study of medical records of 177 children and adolescents presenting complaint and/or laboratory evidence of macro (76,3%) or microscopic (23,7%) hematuria, treated at the Pediatric Nefrology Clinic from May 1999 to January 2009. Of the patients studied, 117 (66.1%) were male and the mean age at initial consultation was 8.0 years (9 months to 15 years). Thrity patients were excluded from the analysis for having hematuria of glomerular origin, and 31 who did not complete the metabolic investigation. The investigation was carried out from the interview data and laboratory tests. Results: Of 109 patients studied, the etiology was not detected in 19 (17%); 27 had nephrolithiasis and 63 had metabolic disorders. Of the patients with nephrolithiasis, in 7 was the cause not found and among the remaining 20 with metabolic disorders, 17 (85%) had hypernatriurua and in 6 of them (30%) it was the only fi nding. Among patients with metabolic disorders, 39 (62%) had hypernatriuria, which was isolated in 8 (13%). Conclusions: Among the causes of non-glomerular hematuria in childhood there was a predominance of metabolic disorders in agreement with the description in literature, and the presence of hypernatriuria, probably due to high sodium ingest, was quite high. Therefore the prescription of low sodium diet for these patients should always be recommended.

Effectiveness Objectives: Raise awareness and warn children about the importance of kidney care and evaluate the level of knowledge about the prevention of kidney disease identifying the risk factors as an important strategy for health promotion.

The campaign theme was about diabetes and kidney in a private school in Brazil. All the kids were informed about diabetes risks and complications and kidney disease, as well as general guidance on changes in lifestyle. At the approach, 240 male and female individuals aged between 9 and 13 years of age answered a two-choice questionnaire with the options YES (quantifi ed) or NO.

Results: In our sample, 97% have the habit of eating fast food or snacks at least once a week, 26% intake more than 2 liters of water per day and 90% pratice physical exercise at least twice a week. None of the children knew what creatinine was, 44% had already had urinalysis previously, 88% have a history of high blood pressure or diabetes in the family and 34% have a history of renal Wednesday, September 1 disease in one of the family members. Most children evaluated the approach as very important for the prevention of diseases that affect the kidney (98%), and the initiative of the researchers (94%). Conclusions: Health education and prevention for kidney disease in children is feasible and have great epidemiological signifi cance. The children are overweight, with a high sodium intake, low fl uid intake, and most are unaware of behaviors that harm kidney function. There is a defi ciency in advising children in establishing rules and habits, as well as warning about the importance the family history has for risk factors. Objectives: Radionuclide has offered an alternative method of estimating GFR that avoids some of the practical disadvantages of inulin clearance. But radioisotopic method has the disadvantage of precautions being required in handling and disposal of radioactive materials. To avoid the practical diffi culties of formal measurement of clearance, several prediction formulas have been published. The most commonly used in children is the Schwartz formula. The aim of this study is to identify an adequate measurement of GFR in pediatric clinical practice. Methods: 99m Tc-DTPA clearance (mGFR) was measured as GFR marker in 170 patients with different chronic kidney diseases. Estimated GFR (eGFR) was calculated via Schwartz formula. The agreement between eGFRs and mGFRs was evaluated by Kappa statistics. The precision was evaluated by R 2 from linear regression and the accuracy was measured by the percentage of GFR estimates within 30% of the mGFR (P 30 ).

The mean mGFR was (73.26±22.58) ml/min/1.73m 2 and the eGFR was (80.35±18.79) ml/min/1.73m 2 . The median bias was 5.28 ml/min/1.73m 2 (P< 0.01). Pearson correlation analysis showed good correlation between mGFR and eGFR. Both of them correlated well with age and serum creatinine (P< 0.05). For Schwartz formula, the Kappa value, R 2 and P 30 was 0.362, 0.573 and 69.4% respectively. The overestimation of the Schwartz formula increased while GFR decreased. Conclusions: Schwartz equation shows a certain degree of accuracy and it is suitable in estimating GFR in children without radioisotopic equipment. The equation remains useful for follow-up of renal function in patients.

Time Objectives: To study the clinical characteristics of ceftriaxone-associated biliary pseudolithiasis in children with renal diseases. Methods: We retrospectively reviewed three children with renal diseases who developed biliary pseudolithiasis when treated with ceftriaxone. Results: Case one was an 11-year-old boy. The initial diagnosis was primary nephrotic syndrome. Ceftriaxone was administered intravenously at a dose of 50 mg/kg/d for gastroenteritis. After that he complained of nausea and loss of appetite. Abdominal sonogram obtained on day 3 of ceftriaxone therapy revealed gallbladder sludge. Case two was a 10-year-old boy. The primary diagnosis was post-streptococcal glomerulonephritis with acute renal failure. He was treated with 30 mg/kg/d intravenous ceftriaxone for gastroenteritis. Then he complained of abdominal pain with positive Murphy's sign. Gallstone was detected on day 6 of ceftriaxone therapy. Case three was an 12-year-old boy. The primary diagnosis was nephrotic syndrome. He was treated with 40 mg/kg·d ceftriaxone for gastroenteritis. Gallbladder lithiasis was detected on day 17 of ceftriaxone therapy. After cessation of ceftriaxone treatment, the symptoms gradually disappeared, with complete sonographic resolution in all of the three children.

None of them showed abnormal gallbladder ultrasonography before ceftriaxone was administered.

The risk of developing ceftriaxone-associated biliary pseudolithiasis might increase in patients with renal diseases who are treated with ceftriaxone.

Continuous Hemofi ltration in the Treatment of Acute Renal Failure in Children F. Zhong, Y. Gao. Nephrology, Guangzhou Women and Children's Medical Center, Guangzhou, Guangdong, China; Nephrology, Guangzhou Women and Children's Medical Center, Guangzhou, Guangdong, China. Objectives: Observation on the effect and characteristic for the treatment of children with acute renal failure by continuous hemofi ltration. Methods: Clinical data of 42 children patients with acute renal failure treated by continuous venovenous hemofi ltration(CVVH)and 51 children patients treated by HD(hemodialysis) in Guangzhou Children's Hospital were analyzed retrospectively.

Results: Original disease of the HD group mainly made by glomerulonephritis, while the CVVH group appeared serious infection. After blood purifi cation, the Bun, Scr and the serum levels of Na + in CVVH group and HD group were signifi cantly improved than before it(P<0.05). After blood purifi cation, the comparison on Bun, Scr, the serum levels of Na + between CVVH group and HD group shows no difference(P>0.05). In CVVH group, metabolic acidosis, hyperkalemia and hypokalemia all be corrected, however 9 patients with metabolic acidosis and 1 patient with hyperkalemia in group HD are not be corrected. Capacity of ultrafi ltration in CVVH group is more than HD group(P<0.05), but less in appearing these complication of hypotension and imbalance syndrome. Conclusions: Continuous hemofi ltration is an effective and safe renal replacement therapy to children with acute renal failure.

The Objectives: To study the epidemiological characteristics of the screening urinalysis among children younger than 3 years old from urban and rural districts in Beijing. Methods: The children were selected by stratifi ed, cluster and random sampling. If the fi rst urinalysis was abnormal, a second screening would be performed to confi rm the result. The guardians of the children were asked to fi ll out the uniform questionnaire. Results: Approximate 12,105 children (from 4 urban districts and 4 rural districts) have been studied. The analysis of the questionnaire showed that 27.1% of the guardians knew nothing about the impairment caused by kidney diseases (urban, 20.4% vs rural, 33.3%; P<0.05). In the fi rst screening, the prevalence of abnormal urinalysis was 12.2% (urban, 11.1% vs rural, 13.2%; P<0.05). During the second screening, abnormal urinalysis was identifi ed in 10.0% of the subjects (urban, 14.2% vs rural, 7.0%; P<0.05). The overall prevalence of confi rmed Renal Replacement Therapy Objectives: Left ventricular hypertrophy (LVH) is a risk factor and intermediate endpoint of cardiovascular (CV) morbidity. We aimed to assess the prevalence, incidence and predictors of LVH in pediatric PD patients. Methods: LVH was defi ned by the 95 th percentile of the Khoury LVMI percentiles, using height age to account for growth retardation. Results: 507 children followed in 25 countries were analyzed. 39% of the patients had offi ce hypertension. The overall LVH prevalence was 48.1% (30.8 concentric and 17.4% eccentric); 21.3% had concentric remodeling. Longitudinal analysis of 128 pts revealed 33 % incidence of de novo LVH and a 45 % incidence of LVH regression per year on dialysis. Transformation to and reversion from concentric geometry were equally common (37%). By logistic regression analysis, hypertension, obesity, the use of the CAPD, renal disease other than hypodysplasia, and hyperparathyroidism were risk factors for the presence of LVH. The use of RAS antagonists and a high total fl uid output were protective from concentricity. The risk of LVH (persistent/de novo) at one year was increased by systolic BP SDS and reduced in children with renal hypodysplasia. Conclusions: LVH was highly prevalent among pediatric PD patients. Dynamic changes were observed during follow up in favour of LVH regression with better BP control. Hypodysplasia appears to be protective against LVH, likely due to lower BP and polyuria even on dialysis. Obesity, hyperparathyroidism and fl uid overload are additional, potentially modifi able determinants of LVH. The use of APD modalities and RAS antagonists may be associated with better CV outcomes. Objectives: Maintaining RRF is known to reduce the cardiovascular complications and mortality in patients on dialysis. However, only a few studies on RRF have been performed in children. Thus we investigated the risk factors of deterioration of RRF in children on PD. Methods: Fifty-fi ve children (M:F 36:19, median age 13 years) with RRF and on chronic PD were retrospectively studied. RRF deterioration rates in 136 interval periods were measured by dividing ∆ renal Kt/V, ∆ renal Ccr and ∆ urine volume per body surface area (UV/BSA) by the length of the period. Clinical and biochemical parameters of the periods were analyzed to determine the risk factors of losing RRF. Results: Presence of hypertension by the defi nition of NHBPEP correlated with more rapid decline of renal Kt/V (P=0.017), renal Ccr (P=0.010) and UV/ BSA (P=0.007); high systolic BP correlated with all the three indices while high diastolic BP correlated only with UV/BSA. Low hemoglobin level was associated with loss of renal Kt/V (P=0.023) and Ccr (P=0.002), but not with UV/BSA. However, gender, age, underlying disease, baseline RRF, BMI standard deviation score, PD modalities and duration, PD solutions, peritoneal permeability, and echocardiographic indices were not associated with the decline rate of RRF. Conclusions: Hypertension and anemia are associated with more rapid loss of RRF in children on PD. Prospective studies are necessary to prove the causal relationship between these factors and RRF loss. Objectives: To evaluate the advantages and disadvantages of a nocturnal hemodialysis regimen in children and adolescents. Methods: A hospital-based NHD program was started as a regular treatment option for maintenance HD patients in 2006. Over a period of 48 months, 15 patients (12 to 17 years old) were prospectively enrolled in this program. NHD was performed for 8 hours, 3 nights per week and treatment was supervised at all times by a pediatric nephrologist and dialysis nurses. Central venous lines or fi stulae were used for blood access. Uremia-associated parameters, medication dosage as well as dietary parameters were regularly monitored. The SF36 test was used to evaluate the quality of life. Results were compared to data from agematched patients treated with conventional HD (3x4 hours/week). Results: Compared with controls, NHD patients had a signifi cant decrease in predialytic mean arterial blood pressure, as well as in phosphate levels, calciumphosphate product and parathyroid hormone levels. Objectives: The pathophysiologic mechanism of nephrotic syndrome is not yet known clearly. At least in some cases, certain 'circulating factors' are thought to increase the glomerular protein permeability, which may have systemic effect on peritoneal membrane. We evaluated the loss of protein through peritoneal membrane in patients on peritoneal dialysis due to the end stage renal disease (ESRD) caused by steroid resistant nephrotic syndrome (SRNS).

We reviewed retrospectively the medical records of 26 pediatric patients on peritoneal dialysis ensued during the period from 2001 to 2007 at our clinic. Twelve patients had SRNS, while 14 patients had ESRD caused by the congenital anomalies of urinary system. Results: While the other parameters including nPNA indicating the adequacy of protein intake were similar between the two groups, serum albumin was lower in SRNS patients than the non-SRNS patients (3.7 ± 0.3 g/dL vs. 4.0 ± 0.4 g/dL, P= 0.021) and peritoneal protein loss was higher (3044.4 ± 837.6 mg/m 2 /day vs. Results: For 31 days, urine output remained < 0.15ml/kg/hr. The PD functioned effectively with signifi cant improvement in the biochemical parameters. Objectives: Infectious complications are a major problem in hemodialyzed (HD) children. Differentiation between bacterial and viral infections is often diffi cult. In order to treat patients rapidly a sensitive and specifi c biological marker would be of interest. Procalcitonin (PCT) has been recognized as an early, sensitive and specifi c marker for bacterial infections in children with normal renal function.

We have retrospectively analyzed PCT serum levels in end-stage renal disease (ESRD) children on hemodialysis who presented with febrile episodes attributed to bacterial infections or presumed viral infection. Results: Twenty-three patients (age 12.3±6.2 years) were included and 35 febrile episodes were identifi ed in 16 out of 23 patients between 2006 and 2009. The mean interval of a febrile episode after the onset of HD was 3.9±4.2 months. Mean interval between HD onsetand a fi rst bacterial infection was 1.9 months (8 days -2 months) PCT serum levels during bacterial infections were higher (101.5 ng/ml; 1.18-976.4) than those detected during seven non-bacterial (presumably viral) infections with negative blood culture and absence of infectious focus (35; 5.27-510 ng/ml; p<0.0002). Serum PCT and CRP levels were highly correlated (p<0.001).

PCT has a small molecular weight and is eliminated partially by glomerular fi ltration. Conclusions: Reduction of renal PCT clearance in children on HD is probably the reason for increased PCT levels even in presumably viral infections. A smaller stimulation of PCT synthesis during viral infections produces a dramatic increase of PCT serum levels. Therefore, PCT levels have to be interpreted with caution in ESRD children. Results: Mean age of these 11 children commencing Haemodialysis (HD) was 23.9 months (range 5-64months). Mean duration on HD was 23.7months (range 5-50months). Mean weight was 9.8 kg at the start of HD (range 7.5-14.2 kg). 7 patients had renal dysplasia, 2 bilateral wilm's tumor, 1 congenital anapheric, 1 infantile oxalosis. 8 patients were switched from peritoneal dialysis (PD) to HD. Central Venous Catheters (CVL) used were10 Kimal and1Quinton permcath, placed in right internal jugular in all. 6 CVLs were changed, 3 due to breakages, 1 pulled by patient, 2 revised due to malposition. 3 CVLs had confi rmed thrombus and were salvaged with Tinzaparin subcutaneous therapy daily for 4 weeks. One episode of CVL infection due to strep was successfully treated. Poor growth remains a major challenge for this particular cohort, 10 patients are/were on Nasogastric or Peg feeds. 1 had renal osteodystrophy and required osteotomies. 2 patients had hypertension and required oral medications. 7 patients have been transplanted, 1 switched backed to PD after 50 months on HD. Conclusions: Haemodialysis is an erroneous but suitable option for children less than 15 kg. CVL breakages were the main reason for changing lines in our patients. Very low CVL infection in our unit is a result of strict adherence to our CVL access protocol. CVL thrombus can be safely and adequately resolved with Tinzaparin in renal failure children receiving HD.

Renal Objectives: The objectives of this study were to evaluate the incidence, renal replacement therapy (RRT), mortality of hemolytic uremic syndrome (HUS) in children in Belarus from Jan 2005 to Jan 2010. The medical records of 103 children (48 m, 55 f) at average age of 2,08±2,47 years at the time of admission to hospital were analyzed. Methods: Retrospective, folder review. Results: The incidence of HUS in Belarus was 4,24 cases/100 000 in children < 5 y and 1,37 cases/100 000 when all children < 15 y were taken into account. 100 typical and 3 atypical HUS was diagnosed. 74 children (71,8%) required dialysis (59 PD and 15 HD). The duration of PD was 12,09±5,13d and HD 10,08±4,25d. The mean time of anuria duration was 9,66±4,92 d. Two children (1,94%) died at the acute phase of disease. Using RIFLE criteria for ARF allow us decreased the time from developing oligoanuria to admission to dialysis center from 25,12±5,34h to 14,28±4,64h (p<0,05). The status of 66 children was analyzed at the end of treatment and outpatient's observation. The mean time of ambulatory observation was 3,2±2,3 y. End-stage renal failure was established in 1 child (1,54%), chronic renal disease at grades 2-4 was revealed in 2 (3,08%) patients, 77,5% of children had the arterial hypertension by discharge from the hospital, in 3 cases (4,6%) severe proteinuria was present.

The main method of RRT in children with HUS in Belarus is peritoneal dialysis. Using acute peritoneal dialysis and RIFLE criteria for ARF let us decrease considerably mortality in children with HUS for the last 5 years (from 22,1% in 2004 to 1,94 % in 2005-2010, p<0,001).

Interactive Music Therapy with Children during the Hemodialysis Sessions L. Barcellos, M. Barcellos, F. Bandeira. Fundação do Rim, RJ, Brazil; CDR Botafogo, RJ, Brazil. Objectives: Interactive music therapy (IMT) with children on HD sessions is a pioneering intervention with such patients (P) interacting with music therapists (MT) by means of voice, music instruments and their own bodies. Assessing the effects of IMT upon P on HD to favor the expression of inner contents; enhancing non traumatic, pleasant treatment aspects, provoking and activating the unhazardous creative skill by means of the music experiment unpredictability of improvisation and composition as opposed to the predictability of a chronic disease while favoring empowerment toward stress confrontation. Methods: 7 P (3-20 y) undergo IMT 1 h/week in which MT and P interact on music experiences with employed music instruments built by the P according to their abilities with the MT collaboration. The work was designed at fi rst as a group activity, but is also individually performed as well as in pairs depending on the willingness and conditions presented by P. MT creativity and fl exibility are fundamental to understand P as the therapy core and to accept different age brackets lead into diverse musical preferences and choices. During music improvisations P, parents, nurses and MT interact. Results: 4 P have individually composed lyrics and tunes and 2 more did the same collaboratively. The 3y P continuously improvises on the contents of a video referring to the existence of miracles. Through tune and lyrics content analysis the importance of expressing feelings turned toward future, strength, adversity facing, and transplant is observed. Music has favored interaction among staff, parents and P. Conclusions: Duly-adapted, fl exible music therapy can be employed during HD and can be another supporting aid to dialysis children.

Register of Children and Adolescents in Dialysis Treatment in Rio de Janeiro E. Klen, N. Santos, F. Bandeira. Fundação do Rim, Rio de Janeiro, RJ, Brazil.

Objectives: There are few data on the incidence and prevalence of Chronic Renal Disease (CRD) under dialysis treatment (DT) in children and adolescents in Brazil. One non-profi t organization which renders assistance to chronic renal children started a Register to know the epidemiology of CRD in DT, to analyse the profi le of this population and the reasons for drop-out. . The incidence was of 11 new cases/million pop/ age and the prevalence is of 24 cases/million pop/age. Distribution per gender: 61% for the males. In the distribution by DT, HD is more utilized 72% and PD -28%. Distribution by age: 0-5yo -6,5%, 5-10yo -9,4%, 10-15yo -26% and 58% above 15 yo. The survival of all those under DT, in the end of 36 m is of 80,8% (> 15yo -83,1%, 10-15 yo -81,8 % and 67,4% for those under 10 yo). The survival by dialytic modality was 77,6% for HD and 91% for DP in 36 m. Kidney transplantantion constitutes the most frequent reason for drop-out (36%) and death 24%. Conclusions: Epidemiologic information of this register demonstrates the reality in our state and assists all those involved in the treatment of these P. The knowledge of these informations is important to guarantee the effi ciency of assistance, to apply the best resources and to provide data for further research.

Indications, Risks, and Outcome of CRRT in Pediatric Intensive Care Unit over 5 Years N. Stajic, R. Bogdanovic, J. Putnik, A. Paripovic.

Objectives: During the recent years CRRT became the therapy of choice in ARF and various metabolic disturbances in the ICU. We present our 5-years experience in this fi eld. Methods: 52 children who received CRRT over the last 5 yrs were retrospectively analyzed using: sex, age, body weight, primary diagnosis, indication for CRRT, number of failed organs.Severity scores (PRISM III and PELOD) were calculated at admission, on the day CRRT was started and 24h later. Outcome of CRRT and signifi cance of severity scores were analyzed. Results: 52 pts (29 m, 23 f) were treated (17 neonates,10 infants). Mean age was 48 mos (0-216) and weight 17.9 kg (1.7-85).Primary diagnoses were: sepsis-16 children (31%), renal disease-11 (21%), and metabolic disturbances or intoxications-11 (21%), whilst 14 (27%) had other diseases. The most common indicationwas sepsis with MOD, with or without fl uid overload in 13 (25%) and 10 (19%) pts, respectively. Mean number of failed organs was 3.2 (1-7). Mean PRISM III scores at the beginning of CRRT and after 24h of treatment was 8.8 and 7.7, respectively (p<0,01). Mean PELOD score at the beginning of the CRRT was 15.8, with consecutive mean mortality risk of 33.1%. The overall survival was 35/52 (67%). 21/22 pts with < 3 organs failed survived (95%), contrary to 14/30 (46%) pts with 3 and more organs affected. There is signifi cant difference between PRISM III score at the beginning of the CRRT among survivors and non survivors (p<0,01). Objectives: Our study was conducted to evaluate longitudinal changes in dialysate cancer antigen 125 (dCA125) levels over time and to analyze relationship between dCA125 and peritoneal glucose exposure (PGE) in children treated with long-term peritoneal dialysis (PD). Methods: Peritoneal equilibration test (PET) was performed in 11 patients (7 boys) with a mean age of 13±5.1 years and a mean PD duration of 84.0±1.1 months. Peritoneal transport parameters were calculated, and dCA125 levels were measured after 4-hour dwells of PETs. Peritoneal appearance rates (AR) of dCA125, the velocity of the decrease in ARCA125 values and annual PGE levels were calculated. Results: Final investigations were performed in 11 children at a mean of 63.3±3.5 (range 59.0-66.8) months after the initial ones. Both dCA125 and ARCA125 levels had shown statistically signifi cant decreaments during the follow up period (p=0.003) and the velocity of decrease in ARCA125 was found to be 52.6±19.4%. No signifi cant difference was found regarding peritoneal transport parameters. PGE values were signifi cantly higher in the last year as compared to the fi rst year (p=0,014). The velocity of the decrease in ARCA125 levels were not related to total PGE values.

We can speculate that after 5 years of PD treatment, mesothelial cell mass reduction is likely to occur. However, mesothelial cells are not likely to play a direct role in peritoneal transport during PD treatment. Furthermore, PGE may not have any infl uence on peritoneal transport parameters of patients with longer PD duration. Objectives: Hemodialysis (HD) in children and infants with ESRD is performed mainly via central venous catheters (CVC). CVC-related infections are the leading cause of morbidity and mortality in this group of patients. We aimed to determine HD-CVC infection rates and survival times in a our Pediatric Dialysis Unit. Methods: We analyzed the data of all children with ESRD who received HD therapy in our Unit between 2001 and 2009. Our strict care of HD-CVC makes no use of prophylactic (topical or systemic) antibiotic therapy. Results: Twenty nine children with ESRD (mean age 8.3 yrs) received HD via a CVC for a total of 22892 days during the study period. Eleven (38%) were infants (<1yr of age) who received HD for cumulative 3779 days (16% of total). Fifty nine CVCs were inserted, of which 13 (22%) were in infants. There were 12 episodes of CVC infections-a ratio of 0.52/1000 CVC days. Four (33%) episodes occurred in infants-a ratio of 1.06/1000 CVC days. Only 3 (5%) CVCs were removed due to infection and additional 28 (47.5%) were removed due to obstruction/clot/tear. The remaining 28 (47.5%) CVCs were either removed while properly functioning (17) or still in place (11). Mean CVC survival time for all children was 388 days and for infants-291 days. Conclusions: Very low CVC infection rates-one infection per 5 catheter years -and prolonged CVC survival times-more than one year -, are achievable in infants and children with ESRD receiving HD therapy by strictly adhering to practices designed to prevent infections. Objectives: The aim of the study was to establish importance of extracorporeal blood purifi cation (EBP) techniques in treatment and rate of renal complications after common intoxications.

The study group consisted of 555 children (259 male, 296 female; aged 12.4+5.2yr) who were admitted to our department between June 2007 and March 2010 were evaluated. Results: Two main causes of poisoning were: medications (216 cases, 104 suicide attempts among them) and alcohol (206 cases). Less often were: chemical substances (44), carbon oxide (33), narcotics (20) and toxic plants (10). In 26 cases no toxic factor was found. Etiology of intoxication depended on sex and age. Only 7 patients required EBP: 3 girls aged 15-16yr and 3 boys aged 12-17yr. All of them were intoxicated with medicines (6 suicide attempts) and had clinical symptoms of poisoning and very high drug level in blood. Four of them used carbamazepine (blood concentration 33-50µg/ml), 2 -acetylsalicylic acid (ASA; 72-149mg/dl) and 1 -acetaminophen (346 µg/ml). Children poisoned with ASA were treated with hemodialysis and the other with hemoperfusion through a cartridge packed with activated charcoal (Adsorba, Gambro). Improvement was achieved after one session and all patients were cured after short term sequels. Only one patient, a 15-yr-old girl poisoned with ASA, had renal complication. She presented acute interstitial nephritis 3 days after drug ingestion. Symptoms disappeared without treatment in 5 days.

The indications to EBP in pediatric poisoning are rare. Renal complications of common intoxications appear incidentally (in less than 1% of cases Objectives: We report the case of a previously healthy 8-day-old newborn, who needed neonatal intensive care treatment after the inadvertent administration of an osmotically active hypertonic phosphate enema that was successfully managed with a modifi cation of the continuous peritoneal dialysis technique. Considering that phosphate removal by peritoneal dialysis strongly depends on total dialysate turnover, continuous fl ow peritoneal dialysis (CFPD) was indicated. Methods: A closed peritoneal dialysis system was created using two urethral catheters. Initially the peritoneal cavity was fi lled with 10ml/kg of peritoneal dialysis fl uide (PDF) via the infl ow catheter while the outfl ow catheter was kept closed. After completion of the infusion, the outfl ow catheter was opened and continuous fl ow of PDF was maintained at a rate of 5ml/min. The dialysate fl ow rate was adjusted for maximum effi ciency by an infusion pump set to maintain fl ow at 300ml/hour. Serum electrolytes were collected every 3 hours.

The dialysis could be discontinued after 6 hours, with a total ultrafi ltration of 253 ml, with signifi cant laboratory and clinical improvement and restoration of spontaneous diuresis. This clinical experience is limited to a few case reports in adult patients in which the procedure was used for 4-8 hours. To our knowledge this is the fi rst description of CFPD in a pediatric patient.

The modality of PD herewith described could be an alternative to current management options, as it is highly effi cient, methodologically simple and low cost method without need for any sophisticated equipment. Results: Comparing the results with European adult and UK NICE guidelines 65% of dialysis and 54% of transplant patients were anaemic. In dialysis patients predictors of anaemia were younger age, female gender, haemodialysis (HD), and a primary diagnosis (PRD) of pyelonephritis or Haemolytic Uraemic Syndrome. Factors signifi cantly associated with being over the target range were older age and peritoneal dialysis (PD). In transplant patients predictors of anaemia were younger age, female gender and a PRD of glomerulonephritis, while being older, male and having a PRD of pyelonephritis were associated with a Hb level over the target. In both dialysis and transplantation groups, patients from Eastern Europe had the highest, and Western and Southern Europe had the lowest Hb levels, with a difference between these regions after adjustment of 1.5 mg/dl. Conclusions: Anaemia is a common and signifi cant problem in European children with ERF. Children on HD are more prone to anaemia than those on PD and those who have been transplanted. Younger children are most at risk. European guidelines are needed to help optimise the management of anaemia in children with ERF. Objectives: Use of low molecular weight heparin for prevention of extracorporeal circuit (ECC) clotting during hemodialysis (HD) is recommended in adults. Our goal was to explore the safety, effi cacy and convenience of a single bolus dose of tinzaparin in our pediatric HD unit.

We performed a prospective study where all stable HD patients were converted from unfractionated heparin (UFH) to tinzaparin. Initial dose of tinzaparin was 40% of the patient's total UFH dose. Patients were monitored for a minimum of 10 weeks after conversion. Hemorrhagic event, visual aspect of the Wednesday, September 1 ECC, nurses satisfaction and anti-Xa activity (U/mL) were recorded. Results: 172 HD sessions were evaluated in 7 patients aged from 2,3 to 17,4 (median: 13,1). 2 patients did not require any adjustment in tinzaparin dose while 5 needed increments of 20 to 62 % from the initial dose. Effective median tinzaparin dose was 73 UI/kg (range: 57-110). Clotting was observed in the air chamber traps in 46/172 sessions but only one was interrupted for massive clotting of ECC. Visual aspect scale of ECC showed signifi cant clotting and coagulated fi lter in 16 and 2 /172 sessions. No hemorrhagic event was reported. Nurses preferred tinzaparin over UFH for ease of administration and lack of monitoring. AntiXa activity with effective dose of tinzaparin ranged between 0.26 to 0.68 at mid-time, <0.1 to 0.24 at the end of the session. No accumulation of tinzaparin activity was noted between HD sessions. Conclusions: Our experience with tinzaparin was positive: it represents a safe, effective and convenient method of anticoagulation for ECC in a pediatric HD unit. Objectives: The primary objectives of this study were to defi ne the incidence and demographic features of paediatric ESRD in Malaysia and to determine the morbidity and mortality of these patients one year after diagnosis. Methods: A prospective cohort study of children < 15 years old presenting with ESRD (eGFR <10ml/min/1.73m2) between January 2007 till December 2008 in Malaysia. One-year outcome in terms of death or renal replacement therapy (RRT) were collected for all patients. Clinical encounters and hospitalization data were collected for those who were started on RRT.

Results: 91 children were diagnosed with ESRD giving an incidence rate of 6.6 per million age related population. There were more boys (54%) and higher incidence among the older age groups. The commonest cause of ESRD was glomerulonephritis (29%), renal dysplasia (15%) and neurogenic bladder (11%). 78% of patients were started on renal replacement therapy; 22% of patients was treated conservatively. The commonest cause for conservative treatment (18 children) was because of young age < 5 years old (10 children) and severe co-morbid conditions (5 children). PD was the fi rst treatment modality 86% compared to HD 14%. The commonest reasons for clinical encounters were fl uid overload (19% )and hypertension (19% ). At 1 year follow-up; 12% (8 children) on dialysis had died. The main causes of death were fl uid overload (3) and lack of dialysis access (2) . Conclusions: Paediatric renal replacement therapy in Malaysia is quite established by now but more need to be done for children < 5 years old. Fluid overload was the main cause of morbitiy and mortality among the cohort on renal replacement therapy. Results: Over a 20 year period 177 patients <19 years received PD in our unit. 23 are still on PD therapy. The median age of commencement was 9.25 years (interquartile range of 2.3 to 13.2 years). 23% were below the age of 2 years at PD start. The median duration of PD was 12.6 months in those who had ceased therapy (range 0.07 to 92.7 months) compared to a median of 11.6 months in those still on PD (range 2.8 to 35.5 months). 4 patients developed EPS. 1 was diagnosed at transplantation but was asymptomatic. She had been on PD for 19.9 months. The other 3 were male and were all symptomatic. Their durations of PD were 57.5, 70.5 and 92.7 months. 2 have died from the EPS. 1 has required peritonectomy. All 4 had lost peritoneal membrane function. 2 only recieved non biocompatible fl uids, 2 were treated with both. Whilst 3 had a number of episodes of peritonitis the child who required peritonectomy had none. Conclusions: In this cohort 2% of patients were known to have developed EPS but 27% of those treated >48 months developed it. All showed signs of membrane failure, 3 had recurrent peritonitis. PD is the mainstay of paediatric dialysis therapy. Patients who lose allografts may well return to PD. Carers need to know the risk of EPS increases with duration of PD. The length of PD in childhood needs to be recorded and passed onto adult clinics at transfer. Objectives: To study the type and incidence of complications of vascular access in children and adolescent who started hemodialysis (HD) treatment over 11 years period. Methods: A retrospective cohort study tha evaluate the type of initial vascular access and the incidence of complications of vascular access in children and adolescents aged 0 to younger than 18 years who started HD in the city of Belo Horizonte, Brazil from 1997 to 2007. Results: A total of 249 vascular accesses in 61 pts (33M, 28 F, median age 13.5 range 2.9-17.8 years), 97 permanent (94 arteriovenous fi stula -AVF and 3 grafts) and 152 temporary accesses (uncuffed CVC) were studied. CVC was the fi rst access in 51% of patients in the study. Patient weight did not infl uence the choice of CVC over AVF: 7 pts under 20 kg started HD using CVC and 7 AVF. However, for pts under 15 kg we detected a ratio of 3 CVC for 1 AVF. The main cause of CVC removal was infection in 35%, followed by the use of AVF in 37.8%. The median survival of the uncuffed CVC was 40 days. Primary failure of AVF was detected in 37.8% fi stulas. The median survival time of AVF was 54 months; main cause of AVF failure was thrombosis (84%). No AVF was lost due to infection. Conclusions: Infection was the major cause of uncuffed CVC removal and the risk of infection was 34 times higher in patients using uncuffed CVC vs AVF. Our results suggest that uncuffed CVC must be avoided for ESRD children on HD and replaced by AVF or cuffed CVC.

Predictive Results: At initiation of HD 30 out of 61 (49%) pts studied were using a patent AVF and 31 (51%) were using an uncuffed central venous catheter (CVC). The failure rate of the fi rst AVF among patients who started HD with AVF or CVC was similar: 37% and 32%, respectively. The risk of AVF loss was 3.8 times higher in pts who started HD using a CVC compared to pts who have a patent AVF since the beginning of HD (p = 0.025), whose AVF survival rate at 1, 3 and 5 years was 73%, 46% and 27% respectively; pts who started HD using a CVC had a AVF survival rate at 1, 3 and 5 years of 40%, 20% and 10 % respectively. Pts with uropathy had 3.2 greater risk of AVF loss compared with other pathologies (p = 0.012). The maturation time less than 90 days was also predictive of loss of AVF: the risk was 4.4 (p = 0.011). Conclusions: Our study identifi ed three independent predictive factors for AVF loss. Pts with uropathy have a higher risk of AVF loss and therefore, placement and use of vascular access in this group of patients should receive special care. Our results also suggest avoid AVF cannulation before 90 days after placement and avoiding the use of CVC as a vascular access for chronic pts. Objectives: The aim our study is to clarify the low density lipoprotein apheresis procedure for Lebanese pediatric patients with homozygous familial hypercholesterolemia (HFH) in terms of effi cacy, adverse effects and diffi culties.

Methods: This study has been realized in a governmental centre. LDLcholesterol apheresis was performed by direct adsorption of LDL by Dali fi ltration from whole blood and performed every other week. The follow-up was carried out using an open, prospective uncontrolled clinical design. Data were collected from 18 patients (age 12 +/-6 years). Venous puncture was used for vascular access without any need of AV-fi stula operation. Priming the extracorporeal volume was done by normal saline at the beginning of the the fi rst 5 sessions in all patients.

The follow-up covered fi ve years, and more than 971 sessions were evaluated. The mean low density lipoprotein cholesterol (LDL-C) pre-treatment value was 680 mg/dL, and the post-treatment value was 270 mg/dL. The most frequently occurring technical problems were related to blood lines: puncture diffi culties, insuffi cient blood fl ow and coagulation. The main clinical adverse effects were hypotension, chills, nausea and vomiting. Conclusions: LDL apheresis, is a well tolerated, safe, effective and a simple way of treating Homozygous FH pediatric patients. Combined with lipid-lowering drugs, LDL apheresis provides a safe and effective lowering of the mean LDL-C levels in pediatric homozygous FH. This technique is more simple than hemodialysis and pediatric nephrologists should be more involved in realizing this procedure in the future. Results: 4 different diameters of catheters were used: 11F (20 CVCs), 8F (53 CVCs), 7 F (5 CVCs) and 6 F (4 CVCs). The most common site of cannulation was right or left subclavian vein (66 CVCs, 80.5%). There were no complications seen in 57 CVCs (69.5%). At least one CVC-related complication was noted in 25 CVCs (30.5%). The lower was the diameter of CVC the higher was the risk of complications (r= -0,50, p<0.01) with 75.0% of all 6 F but only 20.0% of all 11 F CVCs experiencing at least one complication during the treatment. The most commonly seen (60.0%) complications were mechanical. 15 CVCs (18.3%) required recannulation due to kinking (6 CVCs), clotting (4 CVCs), malfunction (4 CVCs) or rupture (1 CVC). 2 CVCs (2.4%) were removed due to MRSE blood stream infection. 8 CVCs (9.8%) insertions were complicated with local bleeding (6 CVCs) or haematoma (2 CVCs) . Patients with CVC-related complications were cannulated for a longer time comparing to those experiencing no catheter diffi culties (11.5 and 6.8 days, respectively, p<0.01) and required more CVCs per treatment (2.15 and 1.24 CVCs, respectively, p<0.05).

The most commonly seen CVC-related complications in children on acute hemodialysis were mechanical with catheter kinking leading the way. From those complications not requiring CVC replacement local bleeding was the most frequently observed. Objectives: EBV and BKV PCR monitoring experience in kidney transplantation in Pediatrics Hospital in Toulouse France. Methods: We report 12 children, out of 70 successive kidney transplantation, who experienced prolonged positive blood PCR test for 7 EBV (4 reactivation, 3 primary infection) and 5 BK virus (3 proven BKV nephropathy) undergoing classical triple IS therapy (steroid/AZA or MMF/ CsA or FK). In these patients we prospectively decided to decrease the drug dosage, fi rst anti metabolite and second anticalcineurin, until a negative PCR test was obtained.

Results: The lowering of the IS drugs allowed to negative clinical virus symptoms and PCR test in all of the patients without any rejection (follow up from 1 to 5 years) and lymphoma. The drug dosage was continuously adapted to the level of PCR test and today 4 patients remain free of anticalcineurin, and 1 of them had only low dose of steroid, 8 continue triple therapy with half dosage of AZA or MMF and low level of anticalcineurin trough level in sera ( CsA 60 to 70 ng/ml, FK 3 to 5 mg/l). Conclusion: Routinely virus detection is mandatory in tranplantaion. An EBV or BK virus blood PCR positive test is a sign of over IS and allows to decrease the IS regimen with no risk of rejection and to cure the viral disease and avoid EBV B lymphoma that we had previously experienced. Objectives: To evaluate the peritonitis epidemiology in children in South Africa and to identify risk factors for peritonitis. Methods: A retrospective cohort study was performed. All children enrolled in the peritoneal dialysis program between 2000 and 2008 were analyzed. Baseline characteristics and potential risk factors were recorded, including: housing, socioeconomic circumstances, distance to PD centre, type of PD, mode of catheter placement, weight and height. Outcome indices for peritonitis were peritonitis rate, time to fi rst peritonitis and peritonitis free patients. All peritonitis episodes were analyzed concerning causative organism, severity of peritonitis (fungal or gram negative), catheter policy, occurrence of technique failure and survival. Results: 67 patients on PD for a total of 544 months were included. Total peritonitis episodes was 129. Median peritonitis rate was 0.23/patient month (range 0-1.76). Median time to fi rst infection was 2.03 months (range 0-21.5 months). 28.4% of patients remained peritonitis free. Patients with good housing and good socio-economic circumstances had a signifi cantly lower peritonitis rate and a longer time to fi rst peritonitis episode. No other risk factors were associated with peritonitis outcome. Conclusions: Peritonitis rate is high in this cohort compared tothe developed world. The characteristics of causative organisms are comparable. Most important risk factors for development of peritonitis are poor housing and poor socioeconomic circumstances. Objectives: To assess the feasibility and effi cacy of CAPD in children from families living under socioeconomic constraints. Methods: Retrospective study of children started on CAPD in the last 5 years at our hospital. Data was collected for the clinical profi le, details of CAPD, fi nancial status and living conditions of the families, complications and outcome. Results: 13 children mean age 6.9 years (range 2-11) were on CAPD. Straight 2 cuffed Tenckhoff catheter was surgically placed in all. Mean break in period was 8.7 days. Manual CPD with 4 cycles/day and fi ll volume of 1200ml/m2 was given. Due to fi nancial constraints and non availability of 1 litre bags all used 2 litre bags with 2 cycles from each bag making the CPD ambulatory for 2 cycles only. Mean duration of CPD was 18.6months.All patients showed improvement in height and biochemistry, reduction in need for transfusions and antihypertensives on CAPD.7 were regularly going to school. Mother was the caregiver in 12, father in 1.6 patients had an isolated room for dialysis, the rest used a common living room. Mean monthly family income was Conclusions: Time of previous intermittent peritoneal dialysis, previous peritonitis and parental education were risk factors for peritonitis and can represent socioeconomic diffi culties. Albumin, residual renal function and Kt/v also were risk factors for peritonitis and agree with the literature.

When the patients were consulted with the pediatric nephrologists, most of them were in failure class. We suggest that if they were detected AKI earlier, the prognosis would be better. Therefore early diagnosis of AKI with p RIFLE criteria and early initiation of PD, will improve prognosis. Objectives: Due to numerous risks, neonatal CRRT is the most challenging aspect of this procedure. We present our 5-years experience with CRRT in neonates. Methods: A retrospective study was performed in 17 neonates treated with CRRT over the last 5 yaars, including their sex, age, body weight, primary diagnosis, indications for the CRRT, number of failed organs and severity scores. Results: 11 male and 6 female neonates were treated, with mean age of 7.9 days (0-28) and weight 3,1kg (1.8-4.6). Mean PRISM III scores were 6.5, 7.2 and 5.5 on admission, at the beginning of CRRT, and after 24h of treatment, respectively. The difference of later two was signifi cant (p<0.05). The mean number of failed organs was 2.5 (1-6). Primary diagnosis was congenital metabolic disturbances in 5 (hyperammoniemia 3, MSUD 2), isolated renal failure in 4, MOD with renal failure in 8.Ten pts received CVVHDF, 6 CVVHD, and 1 CVVH. The mean duration of CRRT was 5.7 days, mean circuit life span 2.6 days and ultrafi ltrate rate 32.9 ml/kg/h. Overall survival rate was 70.5%; 100% in patient with 1-2 failed organs and 44.4% in patients with MOD (3 and more failed organs). Six patients had fl uid overload (>10% BW), 4 of them having ARF without MOD survived, and both patients with fl uid overload and MOD died. Conclusions: CRRT is a safe and effi cient procedure in the treatment of congenital metabolic disturbances and isolated renal failure in neonates. This procedure was less successful in the treatment of MOD with ARF. Our results are among the best of those published so far.

Ultrasound Evaluation of Peritoneal Thickness in Children and Young Patients on Peritoneal Dialysis (PD) S. Testa, I. Borzani, F. Paglialonga, U. Matta, A. Castelli, M. Pavesi, G. Ardissino, A. Edefonti. Pediatric Nephrology and Radiology, Fondazione IRCCS Osp. Maggiore Policlinico, Milano, Italy. Objectives: Sclerosing peritonitis is a rare but life-threatening complication of PD, characterized by peritoneal membrane (PM) proliferation, thickening and calcifi cation. The role of ultrasound (US) evaluation in this condition is not well established: we propose US screening to assess PM thickening in pts on PD. Methods: Between April and September 2009 we prospectively performed US peritoneal evaluation in 21 pts (16M) on PD, median age 6.4yrs (0.6-27); median PD duration was 27.6mo. (0.1-108). We studied the PM with high frequency probe at 3 different ventral windows, at a distance of at least 10cm from the peritoneal catheter insertion. Results: 12 pts (57%) showed a PM thickness ≤1mm (reference normal upper limit), 5 pts (24%) between 1 and 1.5mm and 4 pts (19%) >1.5mm. 2 out of 4 pts in the last group also showed small bowel loops fi xed and tethered posteriorly as well as symptoms of subocclusion. The regression lines between PM thickness and PD duration and the number of peritonitis, showed a statistically signifi cant correlation (r:0,85, p<0,0001 and r:0,56, p<0,001, respectively). Conclusions: CT is considered the gold standard in the diagnosis of sclerosing peritonitis, however it encompasses high radiation dose and is not suitable for repeated examinations. Our data suggest that US examination represents a safe tool to monitor PM changes during PD, allowing good peritoneal visualisation and dynamic evidence of small bowel distribution that can correlate with clinical symptoms even when they are still mild. We suggest to address all PD patients to US peritoneal screening once every 6 mo. . Both CVC types were tunnelled through subcutaneous tissue, the tip was positioned at the atrial-cava junction or just inside the right atrium. Indications for CVC placement were cardiac congenital malformations, low body weight (BW) or inadequacy of venous outfl ow, which contraindicated AV fi stula creation. Results: Survival of Perm-cath and Ash-split CVC at 100 days was 18% and 40% respectively. The access was lost because of bacteremia (1 case in each CVC group), malfunction (5 and 2) and dislodgement (4 and 2) . Complications of CVC insertion were bleeding from the jugular vein which required surgical correction in 1 pts after Perm-cath placement and hemothorax due to subclavian artery puncture during Ash-split placement in 1 pts. Mean dialytic URR in pts with the Ash-split CVC and BW <20 kg was 77.1±8.5%, higher than that of pts with Ash-split CVC and BW >20 kg (53.1±7.0%, p<0.005) and not different from that achieved with Perm-cath (79.2±7.3%).

In conclusion, owing to the lack of venotomy (thus the possibility of preserving the access site), the better duration and the good dialytic performance, Ash-split CVC can be a good access option for children undergoing chronic HD.

The Objectives: The RICH-Q study (Renal Insuffi ciency therapy in CHildren -Quality assessment and improvement) is a collaborative project in which all centers in the Netherlands and Belgium that provide pediatric chronic Renal Replacement Therapy (cRRT) collaborate to improve the quality of care of pediatric cRRT. At the start of the project a survey was performed to investigate the variation in management policies between the centers. Methods: Twice a year representatives from all 9 centres convene to discuss patient data from all centres. Data on treatment and outcomes are registered centrally and monitored according to GCP standards. A questionnaire about ESRD patient management policies was sent to all centers at baseline. We compared reported policies with actual management in 2008. Results: Questionnaires were fi lled in by 8 of the 9 centers. Indications to start RRT included clinical condition in all centers; additionally, some centers mentioned low GFR, uremia or hyperphosphatemia. The preferred treatment modality in all centers was renal transplantation, below the age of 3 years peritoneal dialysis. Contrary to the offi cial local policy, in children older than 3 years with no residual diuresis hemodialysis was applied most frequently. Policies concerning PD care and transplantation policies regarding the offer of pre-emptive transplantation, the acceptance of non-HLA-identical donors, nonheart-beating donors and non-related living donors varied considerably, even within one country. and start of RRT, were associated with lower eGFR at start of RRT. Gender differences were only present during adolescent age, and disappeared when using the same K value for both genders. The various PRDs showed large differences in the rate of decline in eGFR between the fi rst visit to a PN and start of RRT, however this did not result in differences in eGFR adj at start of RRT.

The main determinants of eGFR at start of RRT were age, gender, treatment modality at start, and the time between the fi rst visit to a PN and start of RRT. Research is needed to determine the consequences of these differences. Objectives: We compared proteome profi le of peritoneal effl uent (PE) obtained with icodextrin (Ico) or glucose (Glu) peritoneal dialysis solution in paediatric patients and defi ned protein oxido-redox status (ORS). Methods: Two 14-hour daytime dwells were performed on 2 subsequent days with 7.5% Ico and 3.86% Glu solutions in 16 patients. Protein composition was analysed by 2D-PAGE and mass spectrometry; oxidised products were evaluated from the amount of free SH labelled with new cyanines. Results: Peritoneal transport kinetics of β2-microglobulin and cystatin C was linear for both solutions, but slightly higher with Ico than Glu suggesting a better effi ciency for low-molecular weight molecules. There was a linear correlation between total protein removal during Ico and Glu dialysis in the same patient, suggesting that removal is a function of peritoneal membrane characteristics. The ratio between proteins removed by Ico and by Glu solutions was higher at low removal rate. Image gel analysis revealed a complex protein composition of PE (1064 and 774 spots respectively in Ico and Glu solutions, 524 of which were common to both conditions); 314 spots were higher in Ico compared to Glu PE. Analysis of protein ORS showed a greater amount of oxidised albumin in Ico PE that was correlated with lower serum levels.

Conclusions: Our results indicate better effi ciency of Ico in removing small proteins. Removal of big proteins and of their oxidized isoforms refl ects potentially opposite effects of the treatment that may lead to loss of substances with functional role and debris deriving from their conversion.

Morphological Study of Peritoneum in Children on Peritoneal Dialysis (PD) E. Vidal, E. Benetti, M. Della Vella, L. Murer. Pediatric Nephrology and Transplantation Unit, University of Padua, Padua, Italy. Objectives: The aim of this study is to examine the relationship between clinical, dialytic parameters and peritoneum histological characteristics in PD patients.

We retrospectively evaluated 17 children in CPD. Biopsies were performed after 6 mths from CPD initiation, during catheter removal due to malfunction or after treatment drop-out. Parietal peritoneal biopsies were taken surgically. All samples were examined by light microscopy and sections stained by H&E and Masson's trichrome methods. Children were divided into 2 groups according to the peritoneal biopsy presence (Group P) or absence (Group A) of infl ammatory infi ltrate, vasculopathy and submesothelial sclerosis (>100 um). Results: Median age at the start of CPD was 6.2 years (range 0-17) and median CPD duration was 18.7 mths (6-55). Peritonitis incidence was 1:17 episode:CPDmths. Mean glucose concentration was 1.36% in 10 patients, while in the others mixed glucose concentrations were used. We found that Group P of children had signifi cant greater: -median duration of CPD: 22 (5-56) vs 5.5 mths (4-15); p<0.05; -median number of peritonitis episodes: 2 (0-4) vs 0 episodes (0-1); p<0.05; -peritonitis incidence: 1:13 vs 1:46 episode:CPD-mths; p<0.05. Median age at the start of CPD was 12.9 years (0.8-17.8) in Group A children, signifi cantly greater than that of Group P (4.2 years; 0-10.9). Moreover, the use of hyperosmolar glucose solutions was larger in Group P patients (45% vs 33%; n.s.).

Our study suggests that PD length and glucose load are mainly involved in mesothelial impairment. Peritoneal infectious episodes play a major role in determining chronic alterations. Objectives: CRRT was as new treatment in our hospital for preterm infants admitted to the NICU in 2006. Whether CRRT in preterm infants effetive and safe? Methods: CVVH were used in tow preterm infants with renal failure.5 F double lumaen venous catheter have been inserted into the umbilical cord vein.The machine (Prisma/JH2000) was placed in the CVVH mode.The circuit should be primed with blood.Heparin was used for anticoagulation ( loading dose of 25 units /kg, followed by a maintenance dose of 10 uints/kg). Blood fl ow rate were 5-10l/kg/min, replacement fl uid rate was 30% of BFR. Results: case 1, male 34 weeks gestation,body weight 1.6 kg. 3 days after birth because of shock, RDS and acute renal failure, artifi cial ventilation and life support management had been used, but the baby was still anuria.and creatinine increased to 426 umol/L. So began to CVVH. After 13.5 hours CVVH the patient's condition improves signifi cantly.Oxygen was given only by a headbox, urine output increased to 160ml/24h., creatinine was reduced to152umol/L. During the time of CVVH, there was a little bleeding from the umbilical. at the end of CVVH the bleeding stopped. One month later the baby was dischaged from the hospital. Case 2.,male, 35 weeks gestation. body weight 2.5kg. Suffer from haemolytic uraemic syndrome, serum creatinine increased to 221umol/L. 3 days after birth began to CVVH. 10 days after CVVH, ( total 54 hours ). the patient 's urine output still less.After CVVH the serum creatinine of the patient can be reduced signifi cantly,but creatinine increased again the next day. So the patient change to peritoneal dialysis. Conclusions: Application of CRRT in critically ill preterm infants is effective and safe.

The Objectives: Loss of peritoneal function due to peritoneal fi brosis is a major factor leading to treatment failure in chronic peritoneal dialysis(CPD) patients. The aim of the present study is to uncover the relationship between functional parameters of peritoneum and peritoneal thickness(PT) measured by US in children on CPD.

We recruited two groups of patients: 23(13 females,10 males) on CPD (patient group) and 26 (7 females, 19 males) on predialysis follow-up(CrCl: 20-60 ml/dak/1.73 m 2 ,control group). Age, sex, weight, height, body mass index(BMI), CPD duration, episodes of peritonitis and peritoneal equilibration test results were recorded. Hemoglobine(Hb), blood pressure(BP), left ventricular mass index(LVMI) and renal osteodystrophy(ROD) parameters were also obtained. Statistical analyses were performed by using student-t and Pearson correlation tests. 2 Pediatric Nephrology & Transplantation, CZD, Warsaw, Poland; 3 Pediatric Nephrology, UJ, Cracow, Poland. Objectives: To assess the state of renal replacement therapy for children in Poland, where unrestricted treatment became available following political transformation in 1989. Methods: A National RRT Registry set up in 2000 accumulated individual patient data of all children receiving dialysis or Tx at 13 pediatric dialysis centers. Data of 779 children aged 0-18 years was analyzed. Incidence rates, causes of CKD and choice of initial therapy were calculated for the different 5 year age groups. Results: The standardized annual incidence of CKD5 was 6.4 per million age related population (pmarp) and was highest for the 0-4 age group (7.1 pmarp). CAKUT and genetic diseases accounted for 58% of all causes of CKD5. CAKUT was the predominant diagnostic category in all 5 year age groups (1.6-3.4 pmarp), followed by genetic diseases (2.0 and 1.2 pmarp) in 0-4 and 5-9 year olds and glomerular diseases (1.4 and 1.8 pmarp) in 10-14 and 15-18 year age groups. The preferred initial mode of therapy was PD (62%), followed by HD (32%) and PET (6%). HD was the most frequent choice (49%) only in the 14-18 age groups. Conclusions: 1. There is unrestricted access to RRT for all pediatric age groups in Poland with a similar incidence rate to that of West European countries. 2. There is a free choice of both PD and HD modes of treatment with a disproportionately low choice of PET. 3. Slowing progression is the major procedure for decreasing the incidence of CKD5 as nearly 60% of its causes in east european children are congenital and/or genetic. Objectives: Hepcidin, a key regulator of iron homeostasis, may be the molecular link between infl ammation and anemia in chronic kidney disease. Given that hepcidin may be signifi cantly cleared by hemodialysis (HD) we sought to compare serum hepcidin levels between pediatric peritoneal (PD) and HD patients (pts). Methods: Serum hepcidin levels were measured in 26 PD and 30 HD pts by competitive ELISA and compared to indicators of anemia, infl ammation and iron status. In addition, hepcidin clearance was determined by measuring serum hepcidin before, during and after HD in 8 pts.

Results: In HD pts, hepcidin levels were higher than in normals but were lower than in PD pts despite similar rhEPO dose and higher markers of infl ammation, iron stores, and anemia.

In HD pts, multivariate regression revealed that hepcidin was independently predicted by ferritin ( Objectives: PD(peritoneal dialysis) is the most effective method for ESRD. The infection is dwindling because of the improvements of dialysis apparatus and operative technique. However, the nutrition problems of PD become more and more prominent. We conducted this study to evaluate children's nutrition status before and after PD with the aim to guide the treatment. Methods: Included were 10 children receiving PD. For evaluating their nutrition status, the levels of blood BUN, Scr, Alb, prealbumin (PA), transferrin (TF), hemoglobin (Hb) and hematocrit (Hct) were all detected before and after PD. Also recorded were their heights and weights before and after PD. We elucidate the nutrition requirement of PD children to their parents, making them clear with the calculation of composition of food. Children's diets were formulated by discussing with their parents, and were urged to follow as often as possible.

The average time receiving PD treatment was(12.5±9.28)months, ranging from 4 to 36months. Average level of BUN decreased signifi cantly from (31.4±7.4)mmol/L before PD to (19.6±3.4)moml/L after PD(p<0.01). Hb and HCT levels increased respectively from (77.1±19.9)g/L, (22±6)% to (98.4±40.2) g/ L, (34±4)%. The average blood pressure was 14.6/9.6kPa after PD, comparing with 18.9/11.7kPa before PD (P<0.01). The average blood ALB after PD was (41.0±3.2)g/L. The average increase of heights was (0.67±0.59)cm/month. Conclusions: Chronic PD can improve children's nurtrition status and growth.

In the process of chronic PD, the nutrition guidance and health education play a key role. Objectives: Advanced glycation endproducts (AGEs) are thought to contribute to infl ammatory processes and atherosclerosis in CKD population. We determine if skin AF, the measure of tissue AGEs, is increased and related to vascular changes. Methods: 36 patients with CKD (stage 2-4) and 20 hemodialysed subjects were included in the study. We evaluated: AF using AGE-Reader, carotide intima-media thickness (IMT), aortic pulse wave velocity (PWV) using analyzer (SphygmoCor, Australia). Serum ADMA, MMP-9 and sE-selectin were measured by ELISA method. Study groups were compared to 26 healthy age-matched subjects.

Results: AF, IMT, PWV, serum ADMA, sE-selectin, MMP-9, SBP and DBP were signifi cantly higher in all patients vs. controls. All parameters in HDs were signifi cantly higher than in CKD group. Multiple regression analysis showed a signifi cantly positive correlation between AF and IMT (p<0,004), PWV (p<0,03), ADMA (p<0,0001), sE-selectin (p<0,0001) and MMP-9 (p<0,008) in the group of all subjects. Conclusions: Elevated AF in CKD indicate for the accumulation of tissue AGEs. A signifi cant association between AF and investigated markers supports a role for AGEs as a contributor to vascular damage and shows that AF may be a good, easily applicable tool for assessing the risk of atherosclerosis development and cardiovascular events in CKD children. Objectives: Aortic pulse wave velocity (aPWV), an indicator of arterial stiffness predicts cardiovascular mortality risk in adults. Arterial stiffening advances with age and is accelerated in specifi c disease conditions. In childhood aPWV has not been investigated in larger cohorts. The aim of this study is to provide normal values and to prove the suspected increase with age. Methods: Pulse waves were captured simultaneously by oscillometry on the right carotid and femoral artery (Vicorder) in 405 healthy school children aged 6 to 18 years. We also measured intima-media thickness (IMT) and elasticity on both carotid arteries by B-and M-Mode ultrasound. 

Conclusions: This study defi nes aPWV normal values in children and young adults using a new non-invasive oscillometric method. Even in healthy young individuals correlations to cardiovascular risk factors can be seen. Interestingly, a connection of aPWV to functional parameters of arterial elasticity was observed. Objectives: To determine the response of cerebral blood fl ow (CBF) velocity to hypercapnia in children with hypertension (HT). Methods: We evaluated middle cerebral artery blood fl ow velocity in 5 groups of patients using transcranial doppler (TCD): 1: controls; 2: white-coat (Wc) HT, 3: preHT, 4: untreated HT, 5: treated HT. TCD was performed before and after rebreathing from a bag to increase carbon dioxide (CO 2 ) levels. Outcome variables were mean and peak blood velocity, end tidal CO 2 and mean reactivity. Reactivity slopes were generated using linear regression. ANOVA and unpaired t tests were used to compare among groups. We considered a p<0.05 as signifi cant.

Results: There were no differences in baseline TCD velocities, baseline end tidal CO 2 and CO 2 change among groups. In regard to reactivity slopes, there were no signifi cant differences between genders or age. All HT patients were treated with enalapril. Children with untreated HT had signifi cantly lower CBF reactivity slopes (p<0.05), and reached control values with antihypertensive treatment. Conclusions: This abnormal CBF reactivity to hypercapnia in children with HT may be a marker for potential cerebrovascular disease, which may be treated (or prevented) with antihypertensive medication.

Ambulatory Arterial Stiffness Index (AASI) and Pulse Pressure in Children with Diabetes Mellitus Type 1 T. Šuláková, 1 J. Janda, 2 J. Cerná, 1 V. Janštová, 1 J. Feber. 3 1 Pediatrics, University Hospital Ostrava and Faculty of Health Studies, Ostrava, Czech Republic; 2 Pediatrics, University Hospital Motol, Prague, Czech Republic; 3 Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. Objectives: The aim of the study was to assess pulse pressure (PP) and ambulatory arterial stiffness index (AASI) in children with diabetes mellitus type 1 (DMT1) compared to non-diabetic normotensive children. Methods: We retrospectively evaluated ambulatory blood pressure monitoring (ABPM) records in 84 children (43 boys) with DMT1. Based on offi ce BP and ABPM, patients with DMT1 were divided into 3 groups [true hypertension (DM HTN) 24/84, normotension (DM NT) 33/84, white coat hypertension (DM WCH) 27/84] and compared with 27 normotensive controls (NT). PP and AASI (regression analysis of diastolic on systolic BP values corrected by the regression coeffi cient) were obtained from ABPM. Results: DM NT, DM WCH and DM HTN did not differ in metabolic control, but DM HTN had signifi cantly longer duration of DMT1 and signifi cantly higher 24h, day and night BP on ABPM than DM NT, DM WCH and NT controls (p< 0.0001). DM WCH and DM HTN had signifi cantly higher PP compared to DM NT and NT (47.6±27.3 and 47.4±38.7 vs. 41.4±54.4 and 41.9±25.9, respectively; p=0.0045) . AASI was signifi cantly elevated in both DM WCH (0.35±0.14) and DM HTN (0.36±0.15) compared to NT (0.22±0.16); p=0.0021. Conclusions: Children with DMT1 and white coat or true hypertension had signifi cantly higher PP and AASI compared to normotensive controls and normotensive diabetics. This suggests that these children may be at increased risk for developing cardiovascular complications later on their life.

Ambulatory Blood Pressure Monitoring after Renal Pediatric Transplantation M. Aglony, M. Peredo, L. Bolte, A. Vogel. Pontifi cia Universidad Catolica de Chile, Santiago, Chile. Objectives: Arterial hypertension (AH) is frequent after renal transplantation and risk factor for end-organ damage. We looked for blood pressure (BP) in renal transplant recipients with casual BP (CBP) measurement and ambulatory BP monitoring(ABPM).

Methods: Cross-sectional study of 10 children of our renal clinic who had ABPM (Spacelabs 90217). Results: All had stable renal function and 4 mild proteinuria. Eutrophic 9, overweight 1. CBP was altered in 70%, abnormal ABPM 90% (all with AH and 2 nondippers also). Previous AH with treatment (1-3 drugs):5 with regular control(2 normotensive). All were persistent AH by ABPM. 3/5 patients without treatment had altered CBP at their last evaluation. The ABPM was abnormal in 4.

The prevalence of AH in our renal transplant recipients was high, being ABPM more sensitive than CBP. These data represent a pioneer experience in our country.

Aldosterone, Plasma Renin Activity and Aldosterone-Renin Ratio in Normotensive Pediatric Population as Screening for Primary Aldosteronism M. Aglony, A. Martinez, C. Campino, H. Garcia, R. Bancalari, L. Bolte, C. Avalos, C. Loureiro, C. Carvajal, C. Fardella. Pontifi cia Universidad Catolica de Chile, Santiago, Chile. Objectives: Primary aldosteronism(PA) is a frequent cause of secondary hypertension in adults and is suspected with an aldosterone-renin ratio(ARR)>25. In children its prevalence and normal ARR are unknown.We characterised serum aldosterone(SA), Plasma Renin Activity(PRA) and ARR in pediatric normotensive healthy population. Methods: Prospective study, 211 healthy normotensive subjects (4-16 years) . SA, PRA, ARR and DNA were obtained. They were divided in 2 groups: normotensive children with hipertensive parents(NH) and normotensive children with normotensive parents(NN). ARR cut off was established using the 97 th percentile(P 97 ) and mean plus two standard deviation(X+2SD). Results: In total group, SA was 7.5±5.9 ng/dL, PRA 2.7±1.8 ng/mL/h and ARR 3.8± 6.2, subgroups values were similar. Systolic blood pressure was higher in NH than NN group.1 subject in the NH group had a chimeric CYP11B1/ CYP11B2 gene. The P 97 and X+2SD values for ARR in NH group was 15 and 20.1 and in the NN group was 13.5 and 9.8 respectively. SA and PRA were negatively correlated with age and body fat percentage. ARR was not correlated with age or body fat in the 3 groups. Conclusions: We demonstrated that the normal ARR in pediatric healthy normotensive population without hypertensive parents is surprisingly lower than those communicated for adult population. We propose a new cut off point for ARR that should be >10 to be used as screening in the diagnosis of PA in pediatric population Supported by Chilean Grant Fondecyt 1100356 2 Pediatric Nephrology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. Objectives: The aim of the study was to evaluate biomarkers of infl ammation and oxidative stress, and carotid intima-media thickness (CIMT) in childhood hypertension (HTN) and to assess the impact of obesity on these parameters. Methods: Plasma levels of uric acid, IL-18, IL-1β, ICAM-1, MCP-1, adiponectin, TBARS and 8-Isoprostanes and CIMT were measured in newly diagnosed, untreated obese (BMI>95th perc.) and nonobese children with primary HTN and compared with normotensive obese and nonobese children. Results: 29 children (age14±2 yrs) were enrolled: 10 hypertensive (7 obese; 3 nonobese), 19 controls (9 obese, 10 nonobese). Plasma infl ammatory and oxidative stress markers did not differ signifi cantly between children with or without HTN; CIMT tended to be increased in hypertensive children compared to controls (p=0.26). Obese children with or without HTN had higher uric acid and increased CIMT compared to nonobese children. Objectives: Obesity is likely one of the major causes of the increased prevalence of hypertension in children.The aim of this study was to investigate the prevalence of obesity and its association with hypertension in Iranian children.

Methods: A total of 2000 healthy students aged 11-17 years were screened. Data on weight, height, systolic and diastolic BP, parental history of hypertension and maternal educational level were obtained. Hypertension was defi ned as the average of three systolic and diastolic BP recordings ≥ 95th age-, sex-and heightmatched percentile of the reference standard. BMI ≥ 95% percentile was defi ned as obesity and between 85% and 95% percentile for age and sex considered overweight. Statistical analysis was done with Chi-square and multivariate multiple regression tests.

Results: Overall 7% and 11.8% of students were obese and hypertensive, respectively. 30.7% of obese compared with 8.4% of normal weight children were hypertensive. There was a strong association between obesity and hypertension ( p= 0.0001).

In multivariate analysis both BMI Z-score and age were associated with systolic and diastolic blood pressure, respectively ( p= 0.016, p< 0.001 for systolic BP, p=0.01, p< 0.001 for diastolic BP). Parental history of hypertension and maternal educational level were not associated with hypertension. Conclusions: These results confi rm that obesity is an important risk factor for hypertension in children. The high prevalence of hypertension in obese children emphasizes the need for prevention of childhood obesity.

Atherosclerosis (Fig 1) may imply a role for erythropoietin in the mechanism that defi nes these differences. Whether protective in females or pathologic in males, the mechanism remains unclear. Objectives: Familial hyperaldosteronism type I(FH-1) or glucocorticoid-remediable aldosteronism is an autosomal dominant disorder caused by a chimeric gene(CG) CYP11B1/CYP11B2. Its prevalence and clinical presentation in pediatric population is not well defi ned. Our aim is to report the prevalence of FH-1 in hypertensive children and to describe their clinical and biochemical characteristics. Methods: We studied 120 untreated hypertensive children aged 4 to 15 years. Plasma potassium(K), Plasma Renin activity(PRA) and serum aldosterone(SA) were measured. If aldosterone-renin ratio(ARR)>25,detection of CG was performed using long-extension PCR. Results: In 4/120(3.3%) children ARR was>25. Genetic study confi rmed a CG in all of them. They belonged to unrelated families, 21 fi rst relatives were studied(9 affected), making a total of 13 patients. Severe hypertension 9/13, pre-hypertension 3/13 and normotension 1/13. PRA was suppressed 6/13, hypokalemia in 3/13, high SA level 10/13 (range 8. Objectives: In the last years the detection of pediatric hypertension (HYT) has increased considerably. Blood pressure (BP) is not easy to measure in children.

Auscultatory method of measurement of BP is considered the "gold standard". New methods need to be validated and their reliability in diagnosing hypertension is unknown. We investigated the variability of BP measurements in a school population with three different devices. Unit, JUMC, Krakow, Poland; 2 Dpt. of Pediatrics, JUMC, Krakow, Poland; 3 Dpt. of Ped. Cardiology, JUMC, Krakow, Poland; 4 Dpt. of Clin. Biochemistry, JUMC, Krakow, Poland; 5 JUMC, Krakow, Poland. Objectives: Cardiovascular morbidity and mortaliy are most serious sequelae either in adults or in children with CKD but in the later group the risk factors may vary. compared to THTN (81%). There were no differences between patients with THTN and WCH in amplitudes/acrophases, LVM SDS and AASI. No signifi cant correlation was found between BPR and TOD (LVM SDS, albuminuria); however, 24h and 12h amplitudes inversely correlated with the AASI (r²=-0.39, p<0.01 and r²=-0.29, p=0.01, respectively). Conclusions: Children with primary and white coat hypertension had similar blood pressure rhythmicity, which was not correlated to target organ damage. However, decreased blood pressure amplitude was associated with increased arterial stiffness. This fi nding suggests that abnormal blood pressure rhythmicity may lead to early vascular dysfunction in children with hypertension.

Not Ready for Prime Time: Aliskiren for Treatment of Hypertension or Proteinuria in Children J.T. Flynn. Nephrology, Seattle Children's Hospital, Seattle, WA, United States. Objectives: Aliskiren, the fi rst orally active direct renin inhibitor, has been shown to be effective in treatment of hypertension (HTN) in adults and may also reduce proteinuria in patients with chronic kidney disease (CKD). To date, no controlled studies of aliskiren have been conducted in children, but off-label use may be considered by some practitioners. Methods: An e-mail survey of the PEDHTN and PEDNEPH listservs was conducted to solicit information on off-label use of aliskiren in children. Results: Information on 9 patients was supplied from 5 centers. Mean age was 11.6y (range, 4-17y) . Underlying diagnoses included renal disease (7), primary HTN (1)and unspecifi ed (1). Indications for use of aliskiren included HTN (5) and proteinuria (6) (2 patients were treated for both HTN and proteinuria). Doses used ranged from 37.5-300 mg/day; no information was provided on dosing by body weight or surface area. All children were receiving at least 1 other antihypertensive medication at the time aliskiren was started; most were receiving multiple medications (range, [2] [3] [4] [5] . Aliskiren treatment was considered successful in 3 patients, partially successful in 2 patients, unsuccessful in one patient and not specifi ed in 3 patients. Adverse effects reported included hypotension, angioedema and hyperkalemia (1 patient with each). Aliskiren was either withdrawn or the dose was reduced in 4 patients because of adverse effects or lack of clinical benefi t. Results: Fourteen children were diagnosed with renovascular hypertension. Four (28%) were found to have HHS, all presented with polyuria and polydipsia, electrolyte disturbances, metabolic alkalosis, variable tubular dysfunction and nephrotic range proteinuria along with hypertension. In one patient, glomerular and tubular abnormalities preceded the development of hypertension. Symptoms resolved after resolution of ischemia following percutaneous angioplasty. Conclusions: HHS is probably more common than previously thought and is reversible after correction of underlying renal artery stenosis. Objectives: In contrast to the general population, total adiponectin is increased in patients with chronic kidney disease (CKD) despite increased cardiovascular disease (CVD). Adiponectin circulates as trimer, low-molecular-weight (LMW) and highmolecular-weight (HMW) complexes. The distribution and role of each subfraction in CKD is unknown. We hypothesized that imbalances in the subfractions rather then total adiponectin is associated with increased CV risk in children with CKD. Methods: Total adiponectin and subfractions were measured cross-sectionally in a sub-cohort (n=105) of patients from the CKiD study. Uni-and multi-variate log-linear regression was used to quantify the relationships between adiponectin fractions and CV risk factors. Objectives: Pediatric obesity is reaching epidemic fi gures. It has been argued that insulin resistance (IR) is the fi siopathological link between obesity and hypertension. Several molecules produced by the adipose tissue (adipokines) have been implicated in the pathology of both processes, indicating the existence of a low grade infl ammatory state in the base of atherosclerosis.We aimed to analyse the association between blood pressure (BP), insulin resistance and adipokines in obese children and adolescents. 

There was a signifi cant correlation between the higher PVW and AIx values and obesity(OR 2.2, p =0.01andOR 3.1, p =0.00, respectively). Also cIMT was higher in obese patients than others(p< 0.05). Conclusions: We found that; in primary hypertensive child, the cardiovasculary risk markers are increased. Also additional obesity may aggregate this damage.

In the light of these data, we suggest that simultaneously being of hypertension and obesity; increases vascular risk factors in children. Early determination of these parameters will lead us to know this risk group earlier and to prevent cardiovascular damage in adulthood.

Arterial Stiffness in Normotensive and Hypertensive Children A. Stelcar, N. Marcun Varda, M. Miksic, A. Gregoric. Pediatrics, University Medical Centre, Maribor, Slovenia. Objectives: The important part of investigations in hypertensive children is to establish the effects of elevated blood pressure on the hypertensive target organs. One potential early marker of this damage is the increase of arterial stiffness now recognized as a major driver of cardiovascular disease. The aim of our study was to investigate if there are any differences between normotensive and hypertensive children according to the measures of arterial stiffness.

Methods: Twenty normotensive and 20 hypertensive children of both sexes, matched by age and sex, were included in the study. Carotid-radial pulse wave velocity (PWV) was measured in hypertensive children as an index of arterial stiffness using the applanation tonometry-SphygmoCor and compared to the values in normotensive children. Central aortic systolic pressure (AoSP), aortic pulse pressure (AoPP), augmentation pressure (AP) and the augmentation index (AIx@HR75) were also compared between the two groups. Objectives: The aim of the study was to assess whether urine level of Matrix metalloproteinase 9 (uMMP9) and Tissue inhibitor of metalloproteinase 1 (uTIMP1) could represent novel markers of vesicoureteral refl ux (VUR) and to determine the optimal cut-off level for these biomarkers to predict VUR in children.

Methods: Sixty seven patients with VUR and 20 healthy controls were enrolled the study. Urine MMP9 and TIMP1 were measured by enzyme-linked immunosorbent assay. Results: Mean uMMP9 level was signifi cantly higher in the VUR group than in the controls (1539,8pg/ml vs 256,4pg/ml, p= 0,0001) and using a cut-off 1054pg/ ml for uMMP9 for diagnosis of VUR, sensitivity and specifi city were 67.2% and 85%, respectively (AUC: 0.77). Mean uTIMP1 level was signifi cantly higher in the VUR group than in the controls (182pg/ml vs 32.6pg/ml, p= 0,0001) and using a cut-off 18.7pg/ml for uTIMP1 for diagnosis of VUR, sensitivity and specifi city were 74.6% and 65%, respectively (AUC: 0.73). Mean uTIMP1 level was also signifi cantly higher in patients with high grade VUR than in those low grade VUR (927.4pg/ml vs 132.2pg/ml, p=0.012). Mean uMMP9/creatinine and mean uTIMP1/creatinine ratios were also signifi cantly higher in the VUR group. Conclusions: Both uMMP9 and uTIMP1can be useful markers for identifi ng patients who have high risk for VUR to avoid unnecessary cystourethrografi es. Objectives: Clean intermittent catheterization (CIC) treatment is a classical and successful method in resolving urinary retention and intravesical high pressure. In some cases, on daytime CIC protocol alone, recurring urinary infections, advancing incontinence, renal scarring and advancing hydronephrosis may be seen due to over distended bladder during night sleep.

In the present study, in 12 cases on CIC, with myelodisplasia and neurogenic bladder dysfunction, urinary osmolarity and nocturnal bladder capacity were measured. The urine volume acquired by CIC was measured in the evaluation of the bladder capacity. Results: In 5 cases, nocturnal accidental voiding was seen between the last and the fi rst ICI. These patients present an interval of 10-12 hours between the last CIC performed before bedtime and the fi rst CIC in the morning. After adjusting the CIC and after an 8-hour sleep if the case awakes dry and the urine volume at fi rst ICI is considered the nocturnal bladder capacity. After such an evaluation in 6 cases, a midnight CIC is suggested. Generally, CIC only during daytime is not found adequate. Objectives: Meningomyelocele (MMC) is a common neural development disorder. The objective of this study was to evaluate the infl uence of ambulatory status of children with MMC on clinical fi ndings and renal outcome. Methods: The records of 95 children with MMC followed up at least a year from 2005 to 2010 were reviewed retrospectively. Ambulatory status was classifi ed as independent walkers (walks without assistive appliances), assisted walkers (requires walking aid), and non-ambulatory (wheelchair bound). Demographic, clinical and radiological characteristics of the patients were evaluated according to the ambulatory status. Results: Ninety-fi ve patients (53 boys and 42 girls) were enrolled to the study. The median follow-up of was 55 months ( 12 to199 months). Forty-six patients (49.5 %) were operated during the fi rst three days of life. Ninety-two (97.9%) had lomber, 3 had (3.3%) thoracal defects. The technetium dimercaptosuccinic acid (DMSA) scan showed unilateral renal parenchymal scarring (RPS) in fourteen (21.5%) patients and bilateral RPS in 6 (9.2%) patients. Eighteen patients (26.9%) had vesicoureteral refl ux (VUR). Seventy-three children (76.8%) were non-ambulatory, 14 of the children (14.7%) were assisted walkers and 8 of them (8.4%) were independent walkers. Patients whose follow-ups started at an early age showed less hydronephrosis at USG and deformed bladder at VCUG. Ambulatory status was not found to have infl uence on operation time, frequent UTI, hydronephrosis, VUR, renal parenchimal scarring of the patients. Conclusions: Our data shows that ambulatory status of children with MMC does not affect the clinical fi ndings and renal outcome. (3), enuresis (2) . Surgical procedures employed: valve ablation alone 29 (60.4%), vesicostomy alone 12 (25.0%), cutaneous ureterostomies in combination with other surgeries 7 (14.5%) patients. Median time from confi rmation to primary surgery was 8.5 days (IQR 6-15days). At end of follow-up, GFR could be estimated for 37 patients, 20 (54.1%) of which were in stage 1 CKD. Only 1 (2.7%) had reached ESRF over 5.25 years; he has undergone successful renal transplant with functioning graft after 4 years 2 months (serum creatinine 48 µmol/l). No death recorded but signifi cant numbers (29%) lost to follow up. Prognostic factors identifi ed were serum creatinine at presentation and at 1 year of age, nadir serum creatinine 1-year post surgery. Conclusions: PUV could be successfully managed in Africa to decrease disease progression to ESRF. Objectives: The objective of the study was to perform a comparative analysis of the incidence and structure of congenital malformations of the urinary system in fetuses and deceased newborns in Almaty. Methods: It was analysed 1014 protocols of autopsies on aborted fetuses and 1868 deceased newborns that died for three years in Almaty.

The quantity in fetuses with congenital malformations of the urinary system from 1014 has made systems 17 (1,67%). In all 17 cases the basis for pregnancy interruption were prenatal the revealed anomalies of development. From 1868 died of children the quantity of newborns with developmental anomalies has made 46 (2,46 %). It was determined that the number of the newborns with congenital malformations of the urinary system signifi cantly exceeds the number of the induced aborted fetuses. Among the most frequently diagnosed intrauterine malformations are hydronephrosis(50 %), aplasia and renal agenesis (16 %). At the died newborns numerous congenital malformations (40 %) and the combined congenital developmental anomalies urinary systems (27 %) more often were registered. Numerous congenital malformations represented a combination of congenital defects urinal system and congenital defects of gastroenteric system, cardiovascular system. In 2 cases congenital defects urinary systems have been diagnosed for children with defects of nervous system. Conclusions: Among the deceased newborns it has been revealed frequently registered numerous congenital malformations and combined congenital breaches of the development of the urinary system that suggests an insuffi cient prenatal diagnostics of congenital malformations of the urinary system. Objectives: The term inherited hypokalemic disorder involves a set of renal-tubular diseases that present with normal blood pressure, hyperreninemia and hypokalemia. The management has improved over the last years and the issue of pregnancy has become important for the patients. Therefore, we extensively review reported information on pregnancies and neonatal-postneonatal outcome in children born to affected females. Furthermore we report our experience. Methods: There were 93 pregnancies (literature, N=88; our experience, N=5) in 45 women with Gitelman syndrome (N=25), antenatal Bartter syndrome (N=1) or an unclassifi ed hypokalemic disorder (N=19). Results: There were 12 spontaneous abortions and 6 terminations. Pregnancy was associated with a decline in potassium level. Drug management included supplementation with potassium in all, with magnesium in at least 19, and with potassium-sparing diuretics in 11 pregnancies. A term or near term birth was noted in the 75 pregnancies. Babies exposed in utero to potassium sparing diuretics were also found to be normal. Somatic growth and neuropsychological development are currently normal in 5 subjects aged between 1 and 18, median 10 years born to 4 patients with the biochemical and molecular diagnosis of Gitelman syndrome, who are on follow up at our institutions. Conclusions: Females affected by inherited hypokalemic disorders can become pregnant and the disorder may be managed without outward effect on the fetus and with an excellent long-term outcome. Objectives: CNS is classifi ed in two types: Finnish type and other,including diffuse mesangial sclerosis (DMS).WT1 gene mutations have been demonstrating in some pts with DMS.Monozygous twins with CNS and early onset of ESRD and their genetic analysis are presented. Methods: Female twins from fi rst pregnancy without polyhydramnion and normal placenta were born at 32 g.w. with 46 XX karyotype, normal female phenotype. On admission at the age of 2 months edema, ascites, oliguria/ anuria, hypoproteinaemia, hypoalbuminaemia, low IgG, low ATIII, proteinuria (12g/L in a girl with oliguria) were presented. USG showed a small kidney enlargement,parenchymal hyperechogenicity and poor corticomedullary differentiation. Girls reached ESRD within a few weeks. Peritoneal dialysis (PD) was started with a shift to CVVH/CVVHD in one of them. This one died at the age of 23 weeks.Preliminary light microscopy showed DMS. Results: After excluding the most common CNS mutations (NPHS1,2), direct sequencing of a WT1 gene (exon 8 and 9) revealed the presence of an identical heterozygous mutation R417R in boths girls. Objectives: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most -common inherited cystic kidney diseases and can lead to chronic renal failure. In this study, we utilized siRNA technology to knock down the polycystin 1 protein expression in immortalized renal tubular cell line to investigate the future study. Methods: Stable knockdown of PC1 in M-1 mouse CCD cells were achieved by lipo-polymeric formulation with a specifi c small interfering RNA for Pkd1. After transfection, stable clone of miRNA PKD1 cell lines were sorted by FACS and selected by blasticidin. For immunodetection, cells were lysed and detected by rabbit anti-PC1 polyclonal antibody. Proliferation assay was performed and tubulogenesis and cysts formation were investigated in an ice-cold matrigel solution. Results: Polycystin 1 knock-down cell miRNA 6820 exhibited signifi cant effects on cell proliferation at 24h later to 120h cell cultures. According to the GFP fl uorescence intensity, miRNA 6820 cells could be sorted by high and low fl uorescence. Down regulation of polycystin 1 protein in transcriptional level were confi rmed by RT-PCR and real time PCR and these were parallel to the intensity of GFP. Cysts formation presented in 3-D matrigel system and low expression of polycystin 1 cell line would produce larger cysts. Conclusions: Polycystin 1 knock-down cell lines were set up successful and their cellular biologic behaviors would present as human ADPKD. These cell lines will provide as a good in vitro cellular model to investigate further cellular mechanical signal pathway of cyst formation in future.

The Importance of Duplex Collecting System Diagnosed by Ultrasonographic Screening in Infants K. Drnasin, 1 M. Saraga, 2 M. Saraga-Babic. 3 1 Pediatric Outpatient Clinic, Solin, Croatia; 2 Pediatric, University Hospital, Split, Croatia; 3 School of Medicine, University of Split, Split, Croatia.

Objectives: To perform ultrasonographic screening (USc) of clinically asymptomatic infants in order to determine the frequency of duplex collecting system (DCS) and follow up the urinary tract infections (UTI) and/or other coexisting urinary tract anomalies (CUTA). Methods: USc of urinary tract (UT) was performed on an unselected population of 2471 healthy infants (1276 boys and 1195 girls). All of them had normal result of prenatal USc of UT. Out of a total number of postnataly screened infants, 1749 of them were followed up for 5-60 months regarding the presence of UTI or urinary tract anomalies (UTA). Results: In the examined group, 58 infants (2.3%) had UTA, while 33 (1.3%) infants with UTA had DCS. UTI was found in 16 (48.5%) infants with DCS, and in 146 (8.5%) out of 1716 followed infants without DCS. Other CUTA were found in 6 (18.1%) infants with DCS. Pyelocaliceal dilation (PCD) was found in 13 infants with DCS. In that group UTI was found in 10 (77%) infants, while in the rest of 20 infants with DCS, UTI was found in 5 (25%) of them. Conclusions: Postnatal USc of UT in children is an useful and valuable method for detecting DCS. Our results indicate that DCS in infants increases the risk for UTI approximately 5.7 times and for other CUTA 7.9 times, compared to an unselected population. Moreover, children with DCS associated with PCD have a triple greater risk for UTI than those with DCS only. Early diagnosis of DCS warns about the increase of UTI and CUTA possibility, so we recommend the postnatal USc of UT in all children during the fi rst months of life in order to prevent kidney damage.

Objectives: We carried a prospective hospital based study to assess the out come of mild to sever antenatal hydronephrosis in the fi rst 8 weeks of life in our population, to highlight the diagnostic and therapeutic dilemma encountered and determine the future mode and frequency of necessary radiological investigations. Methods: Babies born at SQUH with the diagnosis of prenatal hydronephrosis between 1998-2007 were included in a prospective study. Infants were accessed with combination of renal ultrasonography, micturating voiding cystogram (MCUG) and a well tempered diuretic renogram. Hydronephrosis was classifi es following Gringnon et al classifi cation. Results: 17 babies had vesicoureteric refl ex with total of 22 refl uxing unit. 4 babies had sever pelviureteric junction obstruction (PUJ). 7 babies had unequivocal fi ndings of mild PUJ on ultrasonography and renogram. 16 patients (10.4%) had IVU for persistent hydronephrosis and confi rmed 11 units of PUJ. 20 babies had equivocal fi nding of mild PUJ/idiopathic HN. 20 babies had unequivocal fi nding suggesting idiopathic hydronephrosis. Other pathological ginding will be discussed. Conclusions: With wide use of antenatal ultrasonography, more case of congenital renal formation are detected. Babies with prenatal hydronephrosis should be promptly investigated to determine the aetiology, counsel parents and prevent future refl ex nephropathy.

Liver Involvement in Autosomal Rezessive Polycystic Kidney Disease (ARPKD) M. Feldkötter, 1 T. Ronda, 1 S. Habbig, 1 B. Beck, 1 F. Körber, 2 B. Hoppe. 1 1 Pediatric Nephrology, University Hospital, Cologne, Germany; 2 Pediatric Radiology, University Hospital, Cologne, Germany. Objectives: Autosomal recessive polycystic kidney disease is always accompanied by congenital hepatic fi brosis (CHF), characterized by hyperplastic bile ducts and portal fi brosis. We here describe diagnostic criteria and the clinical course of liver involvement in 18 patients now aged 0.3-21 years. Methods: Duration of clinical observation varied between 0.3 to 17.9 years. Type and degree of liver involvement was determined by laboratory tests and (Duplex) sonography, in some by MR-cholangiography, oesophago-gastroscopy or liver biopsy. Results: In all patients with liver biopsy (9/9), CHF was histologically confi rmed, however, clinical follow up was heterogenous. Caroli syndrome was identifi ed with ultrasound and confi rmed by MR-cholangiography in 5 patients. A portal hypertensive form was found in 4 children. Patients with Caroli syndrome demonstrated at younger age a hepatic manifestation with portal hypertension in two patients expressed as gastro-esophageal varicosis. Also, prominent accompanying complications were found in the other patients with portal hypertension. Duplex sonography showed re-canalized umbilical as well as periportal veins in 2 patients. Endoscopically, esophageal varices were found in 6 of 11 patients. Reasons for liver or liver-kidney transplantation were either severe esophageal hemorrhage requiring sclerotherapy, or recurrent cholangitis and esophageal hemorrhage, or cholangitis and renal failure. Conclusions: Hence, specifi c attention has to be paid to liver involvement in patients with ARPKD, as it adds signifi cant long term problems and morbidity. Objectives: Reduction of parenchymal thickness (PT) detected by ultrasound examination is one of the criteria for surgery in obstructive uropathy. In a solitary kidney (SK) with obstruction, determination of severe reduction of the parenchyma is diffi cult. This is due to the fact that as the SK develops, hypertrophy of the parenchyma occurs. As a result, the reduction may be misrepresented. Moreover, there is no comparison with a healthy kidney. Therefore, a study was performed to determine normal PT in children with a healthy functionally SK. Methods: The prospective multicenter study comprised 163 children, aged 11 days to 18.96 years (mean age 6.46 years; 64% males). The function of the SK was confi rmed by a DMSA scan. All the children had normal glomerular fi ltration rates. In addition to other parameters, the thickness of the renal parenchyma was measured during an ultrasound examination of the SK. The study assessed the dependence of PT on the children's age, height and weight (non-parametric Spearman's correlation analysis).

The correlation analysis showed a positive correlation of PT with all the studied parameters. Linear dependence was only revealed between the thickness of the renal parenchyma and body height. The correlation coeffi cient for height was 0.837. The linear regression equation was y=0.087x+5.902. The coeffi cient of determination was R 2 =0.665. Conclusions: Parenchymal thickness in a functionally SK is well correlated with the body height. The 5th and 95th percentiles were estimated for PT in a SK depending on the body height. Objectives: We report a 3 years old female who was born from unconsanguineous Turkish parents. Results: On the physical examination; head circumference, weight and length were under the 3 rd percentiles and high blood presure. Her psychomotor and mental development was severely delayed. She's malformations include long face, frontal bossing, bitemporal depression,asymmetric ears, radial deviation of both wrists, clinodactyly, rudimentation and ulnar deviation of fi rst fi nger of left hand, aplasia of fi rst fi nger of right hand, bilateral radial hypoplasia, sacral agenesis, developmental hip dsyplasia and bilateral equinovarus. On the laboratory fi ndings echocardiography demonstrated a secundum ASD, abdominal US showed bilateral pelvicaliectasy, MCUG showed bilateral grade 5 VUR. Abdominal CT demonstrated the absence of inferior vena cava. Conclusions: In our knowledge vena cava inferior agenesis association with skeletal anomalies and mental motor retardation has not been reported in lecture so far. We offer stillborn or infants with limb anomalies would better evaluated for especially urinary tract anomalies. Because early diagnosis is crucial in such cases as potential targeted therapy (either surgical or conservative) might prevent irreversible damage of renal parenchyma. Objectives: The simple renal pyelectasis (SRP) is defi ned as a moderate dilatation of the renal pelvis in the absence of vesicoureteral refl ux or urinary tract obstruction. Recently, we described that might indicate a genetic predisposition to suffer from urolithiasis in adulthood. We have studied the longitudinal renal function in a group of children with SRP. Methods: We included 44 children (34M, 10F). The age at initial study (S1) was 104.8 ± 93.1 days (range: 4-330 days). The age at the fi nal study (S2) was 3.6 ± 2.5 years (range: 1.02-10.5 years). We determined the maximum urine osmolality (Uosm) after stimulation with desmopressin and urinary albumin/creatinine (Alb/ cr) and N-acetilglucosaminidasa/creatinine (NAG/cr). Results: In S2, Uosm values were signifi cantly higher than in S1 (938.3 ± 138.4 vs. 574.3 ± 135.3, p<0.001). Similarly, in S2 values of MAU/cr (1.4 ± 1.7 vs. 5.6 ± 4.5 µg/µmol, p<0.001) and NAG/cr (4.2 ± 3.6 vs. 18.9 ± 19.6 U/g, p = 0.001) were signifi cantly lower than in S1. In S1, 18/44 infants (40.9%) showed renal concentrating defect and S2 only 8/44 children (18.2%). In S1, 11/35 (31.4%) had the ratio Alb/cr high and S2 only 4/36 (10%). In 26/44 (59.1%) of families, family history of urolithiasis were in fi rst and/or second generations. Conclusions: The SRP is a benign process. It is unknown why some patients have impared renal function initially although a progressive improvement of the same one exists.

Wednesday, September 1 restriction, hypocalvaria, and death when used in the second and third trimesters of pregnancy. The target is to show a case born in our service with all his clinical characteristics and his renal biopsy. Methods: we check his clinical chart and his renal biopsy. Results: This was a 35-week, 1,995-g infant of a 35-year-old pregnant woman who had been treated with lisinopril throughout her pregnancy for hypertension. Spontaneous rupture of membranes and fetal distress led to the premature delivery of the infant who was noted to have severe oligohydramnios, intrauterine growth retardation, hypocalvaria, profound hypotension, and renal failure with a renal ultrasound that showed big-sized kidneys.[/bold] Renal biopsy at 8 weeks, which showed tubular focal dilation and absent differentiation of proximal convoluted tubules. These fi nding are known like disgenesia tubular renal typical from the defi cit of angiotensina during the fetal period. Conclusions: We conclude that ACE inhibitor induced renal tubular dysgenesis is an unusual but important cause of acute renal failure neonatal. ACE inhibitors have been shown to cause fetal toxicity, including profound fetal hypotension, anuria, oliguria, and growth restriction. The best strategy to prevent ACE fetopathy is to aggressively educate physicians and the public that these drugs should not be used during the second and third trimester of pregnancy. Objectives: Congenital nephritic syndrome (CNS) is often complicated with thrombosis. We had a patient with CNS complicated with brain infarction developed during fetal period. Since complication of congenital brain infarction with CNS has not been reported before, the possibility of prenatal onset and the time point of brain infarction is discussed. Methods: A 10-month-old boy without family history was born at 38 weeks of gestation and weighed 2510g. Giant placenta was noted at birth. No abnormalities were noted in his growth and development. He was transferred at 2 months of age to our hospital suspected of nephritic syndrome. A high level of proteinuria was noted, and extensive brain infarction was identifi ed by diagnostic imaging. Genetic analysis revealed no mutations. Renal biopsy established the diagnosis of CNS of the Finnish type. Results: Although MRI/MRA identifi ed old brain infarction in the left middle cerebral artery region, no neurological abnormalities were observed before hospitalization. Since formation of a secondary cerebral sulcus was observed at the infarction site, onset of infarction was estimated to be 32 weeks of gestation or later. The fractional excreation of total protein (FETP) was 0.5, and the prenatal FETP was estimated to be 0.05, assuming a prenatal GFR of 10 ml/min. This suggested a theoretical possibility that hyperproteinuria and hypoproteinemia might have developed during fetal life to trigger prenatal brain infarction. Conclusions: We had a patient with CNS of the Finnish type complicated with brain infarction occurred during fetal period.

The Of the 23 kidney units which were found to be normal in the fi rst postnatal USG, only 11 were found out to be normal in the second USG. There was no signifi cant relation in between the degree of the hydronephrosis of the kidneys with or without VUR. Conclusions: Predicting postnatal genitourinary pathologies in patients with antenatal hydronephrosis by means of renal USG is very important for the clinician. Even if the fi rst postnatal USG is normal, USG should be repeated in 4 weeks. Mild and modarate hydronephrosis should be followed with USG. For mild, moderate an even severe hydronephrosis, we recommend to delay performing a VCU as much as possible, considering the electromagnetic radiation exposure. Objectives: Primary vesicoureteral refl ux (VUR), especially low grade, resolves in time spontaneously in most cases. We aimed to evaluate if the rate of anthropometric development of children affects the closure of VUR. Methods: Children with primary low grade (I to III) VUR were grouped as those with spontaneous closure (Group 1) and those without spontaneous closure (Group 2). All children were evaluated retrospectively for height SDS, weight SDS and BMI at the time of diagnosis and yearly thereafter until the last voiding cystography. In addition, the rate of increase in height and weight for each year was evaluated for all children. Both groups were compared with respect to these parameters. Results: Group 1 (n=32) and Group 2 (n=10) were similar for sex, age at diagnosis and follow up period. With regard to anthropometric development, only height SDS was different between the Group 1 and Group 2 at the time of diagnosis (-0.10±1.18 vs 0.93±1.20, p=0,022). At the end of follow up period, none of the parameters were different between these groups. In addition, no difference was determined for the evaluated parameters within each group at the time of diagnosis and at the last cystography. Conclusions: Although VUR resolves in time, we could not demonstrate that the rate of anthropometric development affect the closure of low grade VUR.

testing of her brother (6 years old) did not show a PAX 2 mutation. Treatment strategies: routine treatment of arterial hypertension and syndroms of chronic renal failure, follow-up by ophthalmologist to monitor vision. Conclusions: The association of unilateral multicystic dysplastic kidney, optic disk coloboma and gene PAX2 mutation is consistent with the diagnosis of congenital Renal-Coloboma syndrome in girl.

Asymptomatic Children in China Q. Li, H.P. Yang. Children's Hospital of Chongqing Medical Universtiy, Chongqing, China; Centre for Lipid Research, Chongqing Medical University, Chongqing, China. Objectives: The aim of this study is to assess the characteristics of urinary system diseases and the role of the ultrasound screening and urinalysis screening for chronic kidney disease (CKD) in asymptomatic children in China. Methods: Between September 2008 and November 2008, 14 256 children excluding those with obvious symptoms and signs were enrolled in our study. All the subjects accepted ultrasound and urinary screening. A case-control study was performed to evaluate the relative risk of having stones in those children exposed to melamine formula. Results: Of the enrolled children, 6.10% (869 of 14 256) showed abnormalities, of which 409 (2.87%) were established by ultrasound, 572 (4.01%) by urinalysis and 112(0.79%) by both ultrasound screening and urinalysis. The abnormalities included congenital anomalies of kidney and urinary tract, urinary stones and/ or hydronephrosis, leucocyturia and haematuria and/or proteinuria. Children exposed to melamine formula were 5.17 times as likely to have kidney stones as children exposed to no-melamine formula (95% confi dence interval, 3.28-8.14; P < 0.001); the probability of kidney stones in melamine-fed infants were 6.28 times as likely as those no melamine-fed (95% confi dence interval, 3.71-10.65; P < 0.001). Conclusions: Ultrasonography and urinalysis could complement each other and play important roles in the early diagnosis of anomalies of the urinary system, but urinalysis is a more cost-effective screening tool for CKD in children in China. Exposure to melamine-contaminated formula associated with urinary stones, especially in infants, was signifi cantly higher than the control group. Objectives: Angiotensin-receptor blockers (ARBs) and angiotensin converting enzyme inhibitors (ACEIs) are widely used drugs for hypertension and heart failure. Recent reports show that the maternal use of them may induce ACEI/ARB fetopathy, characterized by the symptoms such as oligohydramios, neonatal pulmonary hypoplasia, renal insuffi ciency, limb contracture, fetal hypotension, and so on. To date, there are few reports on ARB fetopathy. We report a case with NDI followed by neonatal renal failure due to fetal ARB exposure. Results: Case: a 2-year-old boy His 28-year-old mother had treated hypertension with valsartan throughout pregnancy. He was born at a gestational age of 34 weeks with 2308g body weight, 47cm length, APGAR score 7/8. He was transferred to our NICU, where transient tachypnea due to mild pulmonary hypoplasia, renal dysfunction and hypocalvaria was managed with oxygen supply and intravenous fl uid support for several days. These outcomes were favorable and he was discharged on day 35. At the age of 2 years, he developed polydipsia and polyuria and admitted to our hospital to evaluate his renal function. His plasma / urinary osmolality were 281 / 189 mOsm respectively, which suggested diabetes insipidus. Water deprivation test revealed maximum urine osmolality of 167mOsm/kg with no response to the injection of arginin vasopressin. As a result, we could diagnose him as nephrogenic diabetes insipidus. Conclusions: Among a few reports of ARB fetopathy, cases with NDI are rare. More attentions should be paied for the occurrence of late-onset complications on the cases with ACEI/ARB fetopathy. Results: 100 children with PUJo were reviewed. This constituted 133 renal units with PUJo. The male: female ratio was 4:1; the left kidney was most commonly affected in 40%. 70% of those with hydronephrosis confi rmed to be secondary to PUJo regressed spontaneously at 6 months of follow up while the rest progressed at variable interval over the 12 months of review of each case. Mostly all the children (99%) with the disease were antenatally diagnosed. 19 (14.3%) had surgery in the form of pyeloplasty, 31% of this number had surgery in the fi rst 6 months of life. 11 children had confi rmed UTI and the commonest organism isolated was Klebsiella pneumoniae followed by E coli. Outcome of surgical management showed that there was overall improvement in function (MAG3) renogram and decrease of AP pelvis diameter at 12 months of follow up. Conclusions: Pelviureteric junction obstruction is an important and common cause of prenatal hydronephrosis. Signifi cant proportion of children had spontaneous regression of hydronephrosis, however a few had surgical intervention with appreciable improvement of renal function.

The (2) days and thirteen (13) years, with mean age of 3 years. The duration of symptoms before presentation is a mean of 2.6years. 2(9.52%) of cases presented at the neonatal age, 10(47.62%) were aged 1month to 1 year, 5(23.81%) were aged between 1 -5 years and 4(19.05%) were aged 5-13 years. 19(91%) of cases presented with urosepsis while 8(38%) presented with signifi cant renal insuffi ciency. Laboratory fi ndings varied from slight raise to marked elevation in serum creatinine level. Conclusions: Early presentation with prompt intervention will improve the renal function and prevent the renal complication especially in the neonatal period, in a setting where renal replacement therapy is not readily available.

Cystatin C at Birth in Neonates with Congenital Kidney Malformation Diagnosed on Prenatal Ultrasound Compared to a Normal Population P. Parvex, 1 M.H. Billieux, 2 C. Combescure, 3 R. Robyr, 4 E. Girardin. 1 1 Pediatric Nephrology, HUG, Geneva, Switzerland; 2 Obstetrics, HUG, Geneva, Switzerland; 3 Clinical Research, HUG, Geneva, Switzerland; 4 Obtstetrics, La Tour Hospital, Geneva, Switzerland. Objectives: Congenital abnormality of kidney and urinary tract (CAKUT) accounts for 20% of all anomalies diagnosed on prenatal ultrasound(US). As Cystatin C (Cys C) is a sensible marker for renal function in neonates, we compared Cys C at birth in neonates with prenatal diagnosed of CAKUT with Cys C in normal neonates. Methods: In the control group, 100 neonates with normal prenatal US were recruited and Cys C was measured at birth to obtain reference interval. In the same period, Cys C was measured in 19 neonates, with CAKUT, diagnosed on prenatal US (13 dilated kidneys(K) >10mm ±dilated ureters; 3 dysplastic K, 2 multicystic K disease, 1 megabladder). Among these, 11 had one kidney anomaly the 8 others have two kidneys involved. Results: The 19 patients(pts) with CAKUT were comparable in gestational age, weight and size to the control group. In the control group, Cys C reference interval was [1.55-2.66 mg/l] with a mean (M) value of Cys C of 2.04 ± 0.27SD. In the 19 pts with CAKUT, M Cys C was 2.18 ± 0.44SD and the difference between the 2 groups was not signifi cant (P=0.069). However the M Cys C (2.43 ± 0.38SD) was signifi cantly higher in pts with bilateral kidney anomalies compared to pts with only one kidney involved (M Cys C of 1.9 ± 0.4SD) (p=0.03), and to the pts from the control group (P=0.00037). Conclusions: Cys C is a sensible marker of renal function in neonates with CAKUT. Preliminary results showed signifi cant increased of Cys C in neonates with both kidney involved compared to one or to the control group.

Correlation of Hypertension with Renal Function in Children with Obstructive Uropathy H. Rahman, A. Begum, M. Hossain, G. Muinuddin, A. Rahman. Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. Objectives: The aim and objective of the study was to see the correlation of hypertension with renal function and renal function with obstructive uropathy. Methods: In this study 41 different types of obstructive uropathy patients with age range from 4 months to 14 years (Mean age: 5.7 ± 2.3 years) were enrolled. Patients were diagnosed by detailed history, physical examination and relevant imaging studies like ultrasound of the kidney, ureter, bladder region with post voidal residue, micturating cystourethrogram, DMSA and DTPA renogram with total and split renal function. Chronic kidney disease (CKD) Stagings were done by Schwarts formula. Results: Important observation of this study were out of 41 patients 11 (26.88%) had hypertension 30(73.17%) were normotensive. Among the hypertensive patients 5 (45.45%) were in stage V (mean Ccr 10.7 ± 2.57 ml/1.73 m 2 /min) and 2 (18.18%) were in stage IV (mean Ccr 22.4 ± 7.58 ml/1.73 m 2 /min) CKD, which is statistically signifi cant (P<0.001). When normotensive patients were considered 13(40.62%), 5(15.62%) and 4(13.33%) were in stage III, IV and V CKD respectively. On the other hand, when CKD status were correlated with types of obstructive uropathy posterior urethral valve 25 (61%) were present in the majority cases and all (100%) of the patients had different grades of CKD. Conclusions: It was observed from this study, patients with CKD due to obstructive uropathy may be normotensive ever in stage V CKD, and posterior urethral valve was the commonest cases of obstructive uropathy in children. Objectives: Antenatal echogenic kidneys suggest differential diagnoses of polycystic (PKD), cystic dysplastic (CDK) or multicystic dysplastic (MCDK) kidneys. Postnatal prognosis is guided by the liquor volume, any other system anomalies and presence of parental renal cysts. We aim to study the postnatal outcome in children with antenatally diagnosed echogenic kidneys. Methods: A retrospective case note review was undertaken. The spectrum of postnatal investigations (renal function, ultrasonography of kidneys) and clinical course (blood pressure, need for medication, or renal replacement) over a fi ve year follow up period is presented here. Results: In the 4 year recruitment phase, 47 foetuses with echogenic kidneys were seen in the Foetal Medicine Unit (19 with macrocysts). Twenty (10F, current age 5-9 years) of the 29 live births (7 with macrocysts) were included in this study (data unavailable in 9). Six died in infancy (3 PKD, 2 bilateral MCDK, 1 congenital nephrotic syndrome). Survivors were followed up for at least 5 years. Eight remained normotensive with normal renal function, 5 had renal impairment (3 ARPKD, 2 CDK) -two were also hypertensive. Three of the 4 with ADPKD had family history of PKD. Conclusions: The postnatal outcome of antenatally diagnosed echogenic kidneys is variable. The morbidity in survivors beyond the neonatal period is signifi cant, with high incidence of hypertension and/ or severe renal impairment on 5 years' follow up. Long term studies with larger numbers are required to further delineate the prognosis.

Prune Belly Syndrome: Burden and Outcome H.H. Twahir. Department of Paediatrics, Coast Provincial General Hospital, Mombasa, Coast Province, Kenya. Objectives: In the last 48 months, the Coast Provincial General Hospital (CPGH) in Mombasa admitted about 18,000 patients in the department of paediatrics. Some of these admissions were congenital disorders. Prune Belly syndrome is a congenital disorder defi ned by a characteristic clinical triad; urinary tract abnormalities, abdominal muscle deficiency and bilateral cryptorchidism. Prognosis is quite favourable for the less severe types, best management is to do nothing surgically. Prophylactic antibiotics may be indicated for the urinary stasis. Methods: Retrospective audit of the last two years (48 months) looking for patients that fi t the clinical criteria of prune belly syndrome and describe their clinical characteristics. Results: Five patients fi tting the criteria of prune belly syndrome were identifi ed. Age at presentation ranged from one day to 60 months. Three of the patients were identifi ed at the neonatal unit having been admitted after birth for abdominal wall defects. The remaining two were admitted to the general Paediatric ward, one with sepsis and the other severe malnutrition. One of the patients had other anomalies; a patent urachus and skeletal anomalies. Echocardiography was not done in any of the patients, as all patients were haemodynamically stable and also because of fi nancial constraints Average creatinine at presentation was 139ummol/l (range 40µmol/l -420µmol/l) Two of the patients are lost to follow up. Two have been referred to Kenyatta National Hospital (KNH) for surgically intervention because of gradually deteriorating renal function and one to the surgical team locally. Conclusions: Prune belly syndrome appears to be a fairy common abnormality locally compared to international statistics of 1 in 35,000 to 1 in 50,000.

Phenotypic and Genetic Characterization of 13 Colombian Families with Bartter and Gitelman's Syndrome J.J. Vanegas, 1,2 L.M. Serna-Higuita, 1,2 L. Urbano, 2 L.M. Betancur, 1 C.M. Medina, 1 A.M. 3 Objectives: To determine the phenotypic and genetic characteristics of 13 colombian families with Bartter and Gitelman's syndrome. Methods: 13 colombian families were phenotypically characterized. 2 SNPs were typed in 6 genes (KCNJ1, SLC12A1, SLC12A3, BSDN, CASR, CLCNKB) previously associated with the syndrome. Association, linkage and haplotype analyses were applied. Results: 17 patients were analyzed. 47% with Gitelman's, 47% with neonatal Bartter's and 6% with classical Bartter. 38% of the children were consanguineous. Average age at diagnosis was: Neonatal Bartter 18.4 mo, classical Bartter 9.6 y and Gitelman 15.5 y. Main clinical symptoms were: polyhydramnios, preterm delivery, polyurea and hydroelectrolytic disturbances. All of the patients presented with hypokalemia and required treatment. Nephrocalcinosis was found in 33% of the patients with classical Bartter, 66% with neonatal Bartter. It was found association to two SNPs in KCNJ1 (p=0.0186). Haplotype analysis did increase the signifi cance of association (p=0.007). This gene has been previously reported as associated with type II Bartter. In addition two sibs with Gitelman's presented clear inheritance implicating the SLC12A3. Conclusions: This is the fi rst study in Colombia looking for a genetic component of Bartter and Gitelman's syndrome. It is remarkable that only association to KCNJ1 was found. It may correspond to phenotypic variability. Sequence analysis of both KCNJ1 and SLC12A3 genes is underway.

Urinary Levels of Transforming Growth Factor β1 and of Infl ammatory Cytokines in Patients with Fetal Hydronephrosis M.A. Vasconcelos, 1 M.C.F. Bouzada, 1 K.D. Silveira, 2 L.R. Moura, 1 F.F. Santos, 1 J.M. Oliveira, 1 F.F. Carvalho, 1 M.M. Teixeira, 2 A.C. Simoes e Silva, 1 E.A. Oliveira. 1 1 Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil; 2 Biochemistry and Immunology, Federal University of Minas, Belo Horizonte, Minas Gerais, Brazil. Objectives: The aim of this cross-sectional study was to identify noninvasive biomarkers of clinically significant uropathies in patients with antenatal hydronephrosis. Methods: We have evaluated spot-urine levels of interleukin-6(IL-6), transforming growth factor-β1(TGF-β1) and tumoral necrosis factor-α(TNF-α) of 100 patients with intra-uterine hydronephrosis. Patients were divided into three groups: idiopathic renal pelvic dilatation (n=47), uropathies (n=35), and dysplastic kidneys (n=18). Results: The most frequent uropathies were ureteropelvic junction obstruction (n=13) and vesicoureteral refl ux (n=11). Dysplastic kidneys included multicystic dysplasia (n=14) and hypodysplasia (n=4). No signifi cant differences of urinary TGF-β1, IL-6 and TNF-α levels were found in the comparison between the groups. Of 100 patients, 29 presented a reduction in DMSA uptake at the fi rst renal scintigraphy. Both absolute urinary concentration of TGF-β1 and TGF-β1 levels standardized to urinary creatinine presented signifi cant elevation in patients with reduced DMSA uptake in comparison to those with normal DMSA (p<0.05). Urinary concentrations of IL6 and TNF-α did not differ in the comparison between these groups. Conclusions: Although urinary cytokine measurements seemed not to be useful as screening test for clinically signifi cant uropathies, increased concentrations of TGF-β1 pointed out to renal damage. Objectives: Current consensus is that unilateral MCDK is a benign condition with few patients suffering complications of urinary tract infection, proteinuria, hypertension or malignant transformation. Long-term follow-up has shown the persistence of the MCDK in some patients. We studied whether this was related to initial size of the MCDK. We also examined whether patients could be discharged if involution had occurred by 5 yrs. Methods: Data were retrieved from a prospective regional registry of patients with MCDK between 1985 and 2009. Children were followed using a common protocol of investigation with follow up clinical assessments and ultrasound scans (USS) at 2, 5 and 10 years. Results: Of 323 initial patients 249 had USS at 2 years, 180 at 5, 94 at 10 and 17 at 15 years follow up. Survival analysis of MCDK kidneys >5cm at birth showed persistence on USS at 10 years in 85% compared to 40% in those where the initial kidney size was <5cm (p < 0.0001).Of 180 patients followed to 5 yrs, 126 demonstrated compensatory hypertrophy of the contralateral kidney and 59 of the 126 were associated with complete involution of the MCDK. 20 of the 59 had been followed for 10 yrs with a mean eGFR of 88ml/min/1.73m 2 (range 46-108). None of the patients have had hypertension, signifi cant proteinuria or developed malignancy. Conclusions: MCDK kidneys are less likely to involute if they are large at birth. However the clinical progress appears to be benign and conservative management is justifi ed. The data would suggest that up to 1/3 of patients could be discharged at 5 yrs with compensatory hypertrophy of the contralateral kidney in association with involution of the MCDK and benign clinical status. Objectives: To describe prevalence, prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD) in Europe. Methods: Data from a large European database for surveillance of congenital malformations (EUROCAT). The registries are based on multiple sources of information including live births, fetal deaths, terminations of pregnancy, prenatal diagnosis and gestational age at diagnosis. Included were all cases form 20 registries with MCKD born in 1997-2004. Results: There were 1206 cases with MCKD, giving an overall prevalence of 3,78 per 10.000 births, with an increasing trend over the period. Among all cases there were 698 male (58 %) and 471 female (39%). Similar sex distribution were seen when cases were divided into uni-and bilateral MCKD. The majority of cases were unilateral (76%) and of these most were live born (84 %) and had isolated renal malformation (79%). Of the bilateral MCKD the majority of the pregnancies were terminated (61%) and only half had isolated renal malformation (52%). Time of diagnosis were prenatal for 1087 cases (87%) and the prenatal detection rate for all registries together were high (81-93%) with no trend seen over the years. There were a large regional difference in both prevalence (0,2 -9,5 per 10.000) and in prenatal diagnosis. Conclusions: Cases with unilateral MCKD were mainly live births, while pregnancies with bilateral cases often were terminated. There were large regional differences in prevalence in Europe. There seems to be an increasing prevalence of MCKD in Europe that cannot be explained by increasing prenatal detection rates.

Prospective Follow up of the Patients with Antenatal Hydronephrosis F. Duzenli, 1 F. Yalcinkaya, 1 S. Fitoz, 2 Z.B. Ozcakar, 1 M. Ekim. 1 1 Pediatric Nephrology, Ankara University Medical School, Ankara, Turkey; 2 Radiodiagnostic, Ankara University Medical School, Ankara, Turkey.

Objectives: The aim of this study was to defi ne clinical features and follow up of the patients with antenatal hydronephrosis. Methods: The study consisted of prospective data of 136 infants (27F, 109M) with antenatal hydronephrosis. Hydronephrosis was graded in accordance with the Society of Fetal Urology (SFU). Results: The mean follow up period was 18 months (min 6 month, max 3 years). Antenatal hydronephrosis was detected with the ultrasonographies performed in the second and third trimester in 80% and 20% of the patients, respectively. Only 10% of the patients had a family history of urinary tract disease. Grade 2 and 3 hydronephrosis were seen in 56% and 43% of the patients, respectively. Half of the patients (50%) had unilateral hydronephrosis. Vesicoureteral refl ux was detected in 14% of the patients. During the follow up antimicrobial prophylaxis was given to 36% of the study group. Urinary tract infection occured in 22% of the patients and renal scarring was observed in 8%. Surgical correction was performed in 10% of the patients. Conclusions: Antenatal hydronephrosis, which was more frequently seen in boys, has a low incidence of urinary tract infection. However, surgical correction although rarely, might be indicated. Non-invasive longterm postnatal follow up should be justifi ed in the majority of the patients.

Severe Form of Schimke Immuno-Osseous Dysplasia with Unilateral Kidney Agenesis J. Zieg, 1 M. Balascakova, 2 J. Dusek, 1 N. Simankova, 1 E. Seemanová, 2 J. Lebl, 1 F. Fencl, 1 K. Blahova, 1 A. Krepelova. 2 1 Dpt. of Paediatrics, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic; 2 Dpt. of Biology and Medical Genetics, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic. Objectives: Schimke immuno-osseous dysplasia (SIOD) is a disease affecting many organ systems. Severe form of genetically confi rmed SIOD with unique features is presented. Methods: 3 year old girl with severe form of SIOD presented initially with growth failure, solitary kidney, nephrotic syndrome, T-cell immunodeffi ciency and characteristic physical features. Subsequently she developed hematologic abnormalities-rituximab resistant idiopatic trombocytopenic purpura (ITP) and central nervous system symptoms-reccurent transient ischemic attacks. To our current knowledge it is the fi rst described association of SIOD, unilateral renal agensis and rituximab resistant ITP. Results: Finally we analysed DNA and confi rmed mutations in SMARCAL 1 gene. The patient is compound heterozygous for mutations c.2542G>T (p. Glu848X) in exon 17 and c,1439>T (p.Pro480Leu) in exon 8. According to mutation type-nonsense, missense, the patient was expected to have milder form of SIOD. Genotype-fonotype variability in SIOD is apparent. Conclusions: SIOD affects many organ systems. Severe forms lead to death of patient in early childhood. Therapy of SIOD is only symptomatic. Our efforts to infl uence the course of SIOD associated ITP by rituximab and neurologic manifestations of this disease was unsuccessful. Molecular genetic testing can be done. Mutations our found in 50-60% of cases. Prenatal diagnosis is possible in these families. Objectives: Intrauterine growth restriction (IUGR) is associated with hypertension, diabetes and chronic kidney disease in adulthood. Postnatal overnutrition following IUGR may be of pathogenic importance for the development of diabetes and cardiovascular disease. The aim of this study was to identify the possible pathogenesis of kidney disease in IUGR and the effect of postnatal overnutrition by comparative proteomic approach. Methods: IUGR was induced in rats by isocaloric protein restriction in pregnant dams. IUGR pups were divided into two groups, fed either standard-protein diet (IUGR group) or high-protein diet (HP group). At the age of 12 weeks, kidney proteins were obtained from each group. 2-DE, staining, mass spectrometry and database searching were used. Results: The differential proteomic expression analysis between IUGR and control group found 12 proteins had signifi cantly differential expressions, which were transcription regulators including prohibitin and ribonuclease UK114, structural molecules including capping protein, enzymes and so on. Subsequently, the differential proteomic expression analysis between IUGR and HP group found 16 proteins had signifi cantly differential expressions, which were transcription regulators including ribonuclease UK114 and NADH-ubiquinone oxidoreductase, structural molecules including ezrin and gamma-actin, enzymes and so on. Conclusions: Data from this study may provide, at least partly, valuable experimental evidence of proteins involved in the pathogenesis of kidney disease in IUGR and the effect of postnatal overnutrition. Pediatrics, U of Cincinatti, Cincinatti, OH, United States; 3 Medicine, Yale, New Haven, CT, United States; 4 Pediatrics, Baylor, Houston, TX, United States. Objectives: AKI is a strong predictor of mortality in VLBW infants. Novel urine biomarkers of AKI predict mortality in other populations. We evaluate the utility of 6 biomakers to predict mortality in VLBW infants. Methods: Urine samples were collected on postnatal days 1 through 6 for 105 VLBW infants in whom 13 died at (age 26 +/-6 days). The maximum concentration for each biomarker was compared between survivors and nonsurvivors. Results: Neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule 1 (KIM-1), interleukin 18 (IL-18) and osteopontin (OPN) levels were higher in non-survivors vs. survivors, while cystatin C and B2 Microglobulin (B2 Mgb) were not statistically different. Objectives: AKI is a strong predictor of mortality in VLBW infants (birthweight 500-1500g) Novel urine biomarkers predict AKI in others. We evaluate the utility of 6 urine biomarkers to predict AKI in VLBW infants. Methods: Urine samples were collected on postnatal days 1 through 6 in 78 VLBW infants. Maximum concentration for each biomarker was determined. AKI is defi ned as a rise in serum creatinine (SCr) by 0.3 mg/dl within 48 hours or SCr rise by > 50% at any time.

Results: Urinary concentrations of neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), Interleukin 18 (IL-18), and osteopontin (OPN) were increased in infants with AKI. No difference was seen in B2 Microglobulin or Cystatin C. Objectives: To establish clinical outcomes of antenatally detected isolated, fetal echogenic kidneys to aid prenatal counselling. Methods: The database of a UK tertiary fetal medicine unit was used to identify all cases of fetal echogenic kidneys from 1998-2008. Cases with renal macrocysts, hydronephrosis and other fetal anomalies were excluded, and antenatal renal size was recorded. Paediatric notes were reviewed to identify clinical diagnosis and renal function.

Results: 17 cases of isolated renal echogenicity were identifi ed. 2 were lost to follow up and 1 resulted in stillbirth. Postnatal renal investigations found 4 patients with no abnormality, 1 with autosomal dominant polycystic kidney disease (with a family history), 2 with autosomal recessive polycystic kidney disease (in the same family), 2 with HNF1Beta mutation, 1 with Bardet-Biedel syndrome (with polydactyly), 2 with unilateral multicystic dysplastic kidney, 1 with a dysplastic kidney which involuted in pregnancy and 1 with a unilateral dysplastic kidney. There was 1 neonatal death, 2 cases with chronic kidney disease and 2 patients with hypertension but normal renal function.

In the 10 cases with postnatally confi rmed disease 8 had kidneys >97 th centile prenatally. Only 1 of the 4 normal patients had kidneys >97 th centile prenatally. Conclusions: Isolated echogenic kidneys are an uncommon prenatal fi nding and overall prognosis appears to be good. Family history and detailed ultrasound for extra-renal anomalies are important and kidney size >97th centile appears to predict worse outcome.

The Conclusions: Third trimester fetal renal pelvis measurement is useful in predicting postnatal outcome. Patients with sever and moderate RPD had a high prevalence of uropathy, although they rarely needed surgical intervention and the renal pelvic dilatation tended to resolve spontaneously. Objectives: Acute kidney injury (AKI) is an established contributing factor of morbidity and mortality in preterm infants but the infl uence of perinatal factors on AKI is unclear. This study aimed to evaluate the perinatal risk factors in infants born before 32 weeks gestation with AKI. Methods: Data was collected retrospectively from a level 2 neonatal unit at a District General Hospital in the UK identifying all preterm infants born between January 2007 and January 2010. Infants with AKI were identifi ed using the Acute Kidney Injury Network criteria. Each case was matched by birth weight (BW) (±100gms) and gestational age (GA) (± 1week) with up to 2 controls. Data on perinatal factors and serum creatinine was collected from the case notes for the fi rst week of life.

Results: 55 cases were identifi ed and matched to 65 controls. Antenatal steroids were found to make signifi cant difference with OR of 0.23 (95%0.07-0.78,p 0.01). Infants needing ventilatory support were more likely to have AKI (p 0.001). Conclusions: Antenatal steroids are likely to have a protective effect against AKI in preterm infants. Large prospective randomised studies are needed to confi rm the relation between antenatal steroids and AKI. 4 Pediatric Nephrology, University of Alexandria, Alexandria, Egypt.

Objectives: We examined effect of IN,IB and/or FU on AQP gene family in neonatal rat kidney. Methods: At birth P0, neonatal rats(12/group) were randomly assigned to receive IP injections:1) 0.2 mg/kg IN on P0 and 0.1 mg/kg on P1,2; 2) 10 mg/kg IB on P0 and 5 mg/kg on P1,2; 3) 5 mg/kg FU on P0,1,2; 4) IN+FU on P0,1,2; 5) IB+FU on P0,1,2; or 6) same volume saline (S) on P0,1,2. PCR examined expression of AQP gene family in kidneys at P3. 4 Pediatric Nephrology, University of Alexandria, Alexandria, Egypt.

Objectives: Aquaporins (AQPs) belong to superfamily of membrane proteins that control transfer of water and small solutes across cellular membranes.We examined the ontogenic profi les of the AQP gene family in kidneys of newborn, suckling and weanling rats. Methods: Rat kidneys were collected at birthP0,4,7,14, 21 for mRNA expression of AQP1-9,11,and12 using PCR. Gender differences were determined at P14,P21. Results: Compared to P0, AQP1, 4, and 5 were robustly expressed at birth and increased with advancing age from 24fold, 52fold, and 19fold at P4, respectively, to 300fold at P21 for AQP1 and 5. AQP4 peaked at P14, declined at P21. At P14, AQP1 expression was 30% less in males, but by P21, no differences were seen. AQP2 was expressed at birth but progressively increased until P14, then declined at P21. AQP3 was consistently elevated from birth to P21, but highest expression was P7. AQP6 was expressed at birth and P4, but was not seen at P7 to 21. AQP7, 9, and 12 mRNA expression was present at birth, did not change with age. AQP8 was not present at birth and P4, mildly expressed at P7 to 21. AQP11 was not detected. Conclusions: AQP1,2,4, and 5 mRNA are expressed at birth but gene expression dramatically increases during suckling in rats, crucial for water homeostasis during kidney development. Data emphasize importance of lactation for kidney development and function. Objectives: To analyze the frequency and association of AKI risk factors with pediatric RIFLE (pRIFLE) criteria in neonates. Methods: Retrospective, observational study. From 340 admissions to NICU, 140 fi les were randomized, 100 fulfi lled inclusion criteria. AKI cases were identifi ed as having high serum creatinine and/or pRIFLE criteria. Multivariate logistic regression and polythetic analyses were performed with R 2.10.1 software. Results: AKI was identifi ed in 47% of the patients, 39% with pRIFLE criteria. In the polythetic analysis sepsis (p=0.005), gestational age (p=0.02) (fi gure1) asfi xia (p=0.01), oliguria (p<0.001), Apgar at 1 (p=0.007) (fi gure 2) and 5 minute (p=0.007) were signifi cant.

Conclusions: pRIFLE criteria is useful in evaluating AKI in newborn. The effects of the risk factors are different for each criteria.

Objectives: Major predisposing risk factors of acute renal failure (ARF) in neonates are congenital urinary tract (CUT) or cardiovascular system (CVS) abnormalities and acquired conditions such as perinatal asphyxia (PA), septicemia (S) and respiratory distress syndrome (RDS).Aim of the study: To retrospectively determine the incidence, major causes, treatment and outcome of ARF in one of our neonatal intensive care units (ICU) over a period of eight years. Methods: Medical records of neonates in ARF were reviewed according to the following criteria:1) Increased for age blood urea nitrogen (BUN) and creatinine (>50% above baseline level), 2) Concomitant reduction in urine output,< 0.5-1 ml/Kgr/hour. Analyzed data included gestational age (GE),birth weight (BW),causes,treatment,and outcome.

Of 3199 on ICU hospitalised neonates, 39 ARF cases were identifi ed (1.2%). According to their GE, neonates were grouped into three categories: Group I, GE :< 32 w, n =15, Group II, GE: 32-35 w, n=7, GE III: >37 w, n=17. Major causes was RDS (100%) and S 73.3% in group I, CUT42.8%, CVS 28.5%, PA 42.8%, S 28.5% in group II, CUT 17.6%, CVS 35.2%, PA 35.2%, S 35.2% in group III. Treatment by Peritoneal Dialysis (PD) required 3/15 (20%) in group I and 5/17(29.4%) in group III. Outcome: 11/15 (73.3%) deaths in group I, 4/7 (57.14%) group II and 8/17(47%) group III. Conclusions: Among a high risk group of neonates the incidence of ARF, by using certain criteria, was only 1.2%. Although no statistically signifi cant it seems that prematurity remains of the most important predisposing factors affecting mortality.

Renal Impairment in ELBW Infants Can Be Defi ned on Day 3 I. George, E. Levtchenko, M. Rayyan, K. Allegaert, D. Mekahli. Pediatrics, University Hospital Gasthuisberg, Leuven, Belgium. Objectives: Due to successful neonatal care, survival of extremely low birth weight (ELBW) neonates continues to increase. Recent data showed that very low birth weight (VLBW) neonates are at high risk of acute renal failure, however data on renal function of ELBWgroup is scare. Furthermore, serum creatinine (Scr) remains a widely used clinical tool to estimate GFR, however normal values in this population are not yet defi ned. We aim to assess Scr of this group and defi ne predictors of renal impairment. Methods: Retrospective chart review of ELBWneonates assessed in a single centre between 2000-2005. Neonates with congenital renal abnormalities and/ or death ≤7 days were excluded. Neonatal Scr (mg/dl) was analysed according to prenatal, peripartal and postnatal variables. Results are described as median (range). Results: In 151 ELBW (66 females), gestational age(GA) at birth was 27 (23-33)w, birthweight(BW) was 810 (330-1000)g with 34% SGA. Peak Scr level was reached on day 3:1.3 (0. 4-2.5) . We defi ned severe renal impairment as peak Scr on day 3>75 th percentile. Thirty seven (24.5%) neonates had severe renal impairment. Their Scr remains higher on day 14 and 28 (p<0.001).They had lower BW, lower GA, more ibuprofen and steroid exposure and longer ventilation period. Multivariate analysis confi rmed that GA and ventilation period (p<0.0001) were signifi cant risks factors, while SGA was not (p=0.17). Conclusions: ELBW neonates are at risk for severe renal impairment in the neonatal period with similar risks factors as the VLBW peers. Our data showed that peak Scr on day 3 was a good predictor for renal impairment in this weight category. Prospective studies for long term renal function of this group are needed.

Has Maternal Preeclampsia an Infl uence on Neonatal Blood Pressure Level? M. Reveret, 1 B. Marin, 2 A. Bedu, 1 V. Guigonis. 1 1 Pediatrics, CHU, Limoges, France; 2 Statistics and Epidemiology, CHU, Limoges, France.

Objectives: Recent data give evidence that preeclampsia could be an antibody mediated disease. Therefore, pathogenic antibodies (i.e. directed against angiotensine II receptor type 1) (AT1R-AA) could have an impact in neonates. It has been reported that blood in neonates less than 1350g of birth weight and 29 SG and born from preeclamptic mothers, blood pressure was signifi cantly higher than in controls (neonates born from normotensive mothers). We conducted a retrospective study in premature newborn -older than 29SG and with a weight higher than 1350g -in order to compare blood pressure levels whether their mother presented with preeclampsia or not. Methods: Blood pressure levels of 24 premature newborns from preeclamptic mothers and their matched controls were retrospectively studied and compared during their fi rst three days of life. Results: Mean gestational age (31.1 vs. 31.2 SG) and birth weight (1774 vs. 1865g) were not different between the two groups. The only difference was the delivery mode with more caesarian section in the preeclampsia group. Diastolic blood pressure was signifi cantly higher in the preeclampsia group of newborn than in the control group (mean difference: + 3.13 mmHg, p<0.05). No difference was present between the two groups considering systolic or mean blood pressure. Conclusions: Even if not clinically relevant, these data seem to confi rm that newborns from preeclamptic mothers are susceptible to have higher blood pressures than newborns from normotensive mothers. This difference could be explained by the transplacental transfer of AT1R-AA to fetuses. Objectives: There is limited data on anatomic & functional renal development in growth restricted neonates.We compared glomerular function on day 3 in appropriate for date (AFD) & small for date (SFD) neonates.Glomerular,tubular functions & renal volumes were also assessed in fi rst 6-wks. Methods: After consents, 103 neonates (32-41 wk gestation) delivered at this center included. Serum creatinine, cystatin, Schwartz GFR, urine osmolality & fractional excretion of sodium (FENa), potassium (FEK) measured starting from 12-24 hr of life until 6 wk. Renal volume assessed by ultrasonography at 2-4 wk after birth. Results: No signifi cant difference in serum creatinine (mg/dl) observed in preterm AFD, term SFD& term AFD at day 3 (0.85±0.3, 0.80± 0.3 & 0.82±0.2 respectively, P=0.9). In each group, GFR correlated positively to postnatal age (P<0.01).Levels of cystatin did not change (P=0.8) while urine osmolality rose with postnatal age (P<0.001).On multivariate analysis, smaller kidney volume correlated signifi cantly with prematurity and low weight for gestational age. Conclusions: Renal function and volume in preterm AFD and term SFD babies is compromised. Follow up studies are required to determine implications on long term renal outcome.

The Roles of Antenatal and Postnatal 2 nd Week USG on Prediction of Severe Hydronephrosis M. Bayram, A. Soylu, D. Alaygut, B. Kasap, M. Turkmen, S. Kavukcu. Pediatrics, Dokuz Eylul University Medical Faculty, Izmir, Turkey. Objectives: To compare the predictive powers of antenatal USG and postnatal 2 nd week USG for obstructive uropathy and/or VUR. Methods: Patients with antenatal hydronephrosis were evaluated by serial postnatal USG (2 nd week, 3-6-12-24 months) and, if needed, dynamic renal scintigraphy and/or voiding cystourethrography. Each renal unit (RU) was classifi ed as normal, non-obstructive dilatation, obstructive uropathy or VUR. The predictive powers of antenatal and postnatal 2nd week USG for obstructive uropathy ± VUR were determined according to these data. Results: Evaluations were performed on 92 patients (184 RU) having both antenatal USG (hydronephrosis in 120 RU) and postnatal USG (hydronephrosis in 113 RU). The diagnoses of RU with vs without hydronephrosis in antenatal USG were normal (1 vs 48), non-obstructive dilatation (70 vs 12), obstructive uropathy (33 vs 2) and VUR (16 vs 2), respectively (p<0.001) (OR 10, sensitivity 93%, specifi city 46%, PPV 41%, NPV 94% for obstructive uropathy ± VUR). The diagnoses of RU with vs without hydronephrosis in postnatal USG were normal (8 vs 41), non-obstructive dilatation (59 vs 23), obstructive uropathy (33 vs 2) and VUR (13 vs 5), respectively (p<0.001) (OR 6, sensitivity 87%, specifi city 49%, PPV 41%, NPV 90% for obstructive uropathy ± VUR). Conclusions: Antenatal and postnatal 2 nd week USG were sensitive but not specifi c for the prediction of signifi cant hydronephrosis. Furthermore, presence of hydronephrosis in antenatal or postnatal USG did not differ with regard to the prediction of fi nal diagnosis.

Renal Tubular Dysgenesis: Clinical Case I. Kazyra, J. Grygorenko, A. Sukalo, T. Letkovskaja, S. Bayko, V. Savosh, N. Tur. Pediatrics, Belarus State Meducal University, Minsk, Belarus; Chair of Pathology, Belarus State Medical University, Minsk, Belarus. Objectives: We report the fi rst case of intravital diagnosis of renal tubular dysgenesis in neonate in our Republic of Belarus probably associated with Nimesulide using in the second and third trimester of pregnancy. Methods: A 2880g male neonate of 36-37 weeks gestation was born to a 28 year-old prima gravida mother (non-consanguineous couple). The pregnancy was complicated by the intrauterine fetal hypoxia and oligohydramnios. There was a history of acute respiratory illness and urogenitaly tract infection in the fi rst trimester of pregnancy. The baby was delivered by cesarean section because of mother's symphysitis. Symphysitis was diagnosed on 29 weeks gestation and she was treated by Nimesulide. Third trimester sonographic (US) demonstrated of oligohydramnios with structurally normal kidneys.

At delivery Apgar scores were 6 and 7 at 1 and 5 min respectively. He was intubated after 3 hours after birth because of poor oxygenation and transferred to our hospital for evaluation of anuria at 3 days-old age. Results: Our examination revealed nornal face, peripheral edema, high blood urea and creatinin, acidosis, anemia. His kidneys were normal on size and shape on US, there was no evidence of thrombosis and urological problems. The peritoneal dialysis was started. At the age of 11 days-old kidney's biopsy was undertaken. No markers of proximal tubular function were detected immunohistochemically (AB to EMA). To date, our patient 1 year and 10 months, he is on peritoneal dialysis, and continues to be in anuria. Conclusions: We should be extremely careful in the prescription of drugs during pregnancy.

Long-Term Follow-Up of Antenatal Oligohydramnios of Renal Origin (ROH) I. Klaassen, 1 S. Schmidtke, 1 G.F. Laube, 2 D.E. Mueller-Wiefel, 1 M.J. Kemper. 1,2 1 Pediatric Nephrology, Universitiy Children's Hospital, Hamburg, Germany; 2 Pediatric Nephrology, University Children's Hospital, Zuerich, Switzerland. Objectives: Prognosis of ROH has been regarded as unfavorable, although clinical data are scarce. Due to progress in neonatal intensive care and the treatment of infants with chronic kidney disease, the overall prognosis of these children has improved considerably. The aim of this study was the evaluation of complications and longterm follow-up in patients with ROH. Methods: Data on 39 fetuses (26 male, 13 female) with ROH from 1990 to 2010 were evaluated. Results: Primary diseases included urinary tract malformations (n=26), ARPKD and ADPKD (n=9) and other (n=4). Of 9 non-survivors (23%), 6 died within the neonatal period. 27 patients required mechanical ventilation (16 with associated pneumothorax). The surviving 30 children have a current median age of 5.6 (0,1-16) years. All developed CKD, which could be managed conservatively in 14 patients (median GFR 50.1 (range 19.9-130) ml/min/1.73m²). 16 patients reached ESRD at a median age of 3 months (range 2 days to 8.2 years); two received a preemptive kidney transplantation (KT), and 14 started peritoneal dialysis. 11 of these patients underwent successful KT at a median age of 3.1 (range 1.1-12) years. Two of them had a combined hepatorenal transplantation due to ARPKD. Cognitive and motor development was normal in 25 of 30 patients (83%) and showed a delay in 5 children. Conclusions: In ROH long-term prognosis is encouraging and range from CKD stage 1 to combined hepatorenal transplantation. The rate of perinatal problems and complications is high and may increase, since more severely affected fetuses are actively treated.

One Year Follow-Up Results of Patients Prenatally Diagnosed Hydronephrosis S. Yel, H.M. Poyrazoglu, Z. Gündüz, S. Tülpar, R. Düsünsel. Pediatric Nephrology, Erciyes University Medical Faculty, Kayseri, Turkey. Objectives: We aimed to determine the etiological reasons and frequency of prenatal determined hydronephrosis, and to evaluate the association between some parameters and renal functions during study. Methods: Forty-eigth patients born between April 2006-October 2007 with prenatal hydronephrosis were followed prospectively. The urinary ultrasound scan and renal functioning tests were performed on day 3-7 of life and repeated on week 4-6, months 3, 6 and 12. Results: In 29.3% of all 76 renal units with prenatal hydronephrosis, transient hydronephrosis was diagnosed. Ureteropelvic junction obstruction was the most common cause of prenatal hydronephrosis. Thirty-two patients (66.6%) had urinary tract infection during study. Eshericia coli was the most common cause of urinary tract infection. There were negative correlations between GFR of at the end of study and fractione K + excretion at the 3 th month and 1 st year. There were negative correlations between GFR of at the end of study and fractione Mg ++ excretion at the 3 th and 6 th months. There were positive correlation between GFR of at the end of study and tubular reabsorbtion of phosphate 1 st year and negative correlation between GFR of at the end of study and serum BUN and creatinine levels 1 st year. Only one patient had stage 2 chronic renal disease at the end of study. Conclusions: Transient hydronephrosis is one of the most important reasons of prenatal hydronephrosis. Tubular functioning tests may be impaired early stages. The patients with prenatal hydronephrosis must be followed-up closely and the episodic evaluation of tubular functions may predict renal damage before renal failure development. Objectives: Various nanoparticles are produced with development of nanotechnology. The safety is not confi rmed precisely at present. We have already reported that some nanoparticles exposure to pregnancy animal exert severe damages on nervous and respiratory systems of offspring. In this study, the infl uence of exposure to ultrafi ne carbon black (UfCB) in fetal period on renal system of offspring was examined morphologically. Methods: Pregnant ICR mice were exposed to a total of 100 µg of UfCB (particle size; 14nm) by intranasal instillation on gestational days 5 and 9 for exposure group (CB group); comparative control animals were treated with vehicle. After delivered their pups, kidney tissues were collected from 12-week-old male pups, and examined with light and electron microscope. All experimental animals were handled in accordance with institutional and national guidelines for the care and use of laboratory animals. Results: In renal cortex of CB group, collagen fi bers were diffusely observed surroundings of renal corpuscle and tubule, and signifi cantly larger than in control group. In addition, specifi c and interesting renal failure, that is similar to characteristic fi ndings in a part of genetics diseases, were found in tubular epithelial cell of renal cortex of CB group. Conclusions: These observations indicate that perinatally-exposed ultrafi ne carbon black caused renal failure in offspring. Our fi ndings indicate existence of a severe health hazard to infants borne from mothers exposed nanomaterials during pregnancy. It is possible that these inborn damages carry a severe disorder, which is recognized as a genetic disorder. Objectives: The study was done to fi nd out any relationship between maternal BMI with kidney length and volume of term neonate. Methods: Fifty eight (58) healthy, term, appropriate for gestational age (37 to 41 wks) newborn were examined prospectively by sonography within 72 hours of birth by a single sonologist. In 30 boys and 28 girls, body weight(BW), supine length(SL) and occipitofrontal circumference(OFC) were collected from delivery room records. Maternal BMI was calculated by the formula BMI=wt in kg.ht 2 in meter.Scanning was performed with 6.5 MHZ transducer. Maximum length of each kidney was determined. Volume of the kidneys were determined by in built formula of software. Kidney length and kidney volume were then correlated with maternal BMI. This two were also correlated with different parameters of newborn. Results: There were no signifi cant differences in mean kidney length and volume between right (38.41mm, 9.67cc) and left (38.43mm, 9.79cc) kidney. The length of kidneys between boys and girls showed no difference, but volume of right kidney between boys and girls showed signifi cant difference (<0.01). Kidney length (KL) was correlated better with infant BMI (<0.01), infant body weight (<0.01) than infant body surface area (BSA) (<0.05).Kidney volume was also correlated with BMI (<0.05).body weight (<0.05) and BSA (<0.05). But kidney length and volume showed no signifi cant correlation with maternal BMI. Conclusions: Despite expectation that maternal BMI might have some correlation with neonatal kidney size but our study showed no signifi cant correlation with maternal BMI.

Ultrasound Determination of Kidney Length and Volume in Premature Newborn S. Sultana, S. Rahman, B.K. Basak, S. Ferdaus, N. Hossain. Paediatrics, Uttara Adhunik Medical College Hospital, Dhaka, Bangladesh. Objectives: To determine the normal range of kidney length and volume in premature Bangladeshi newborn. Methods: Forty one inborn, appropriate for gestational ages, preterm infant (31 to 36 weeks) were prospectively examined by sonography within 72 hours of birth by a single sonologist, who were unaware about the gestational age of the baby. In 23 boys and 18 girls, gestational age was estimated by Ballard score. Body weight (BW), supine length (SL),occipito frontal circumference (OFC) were collected from delivery room records. Body surface area (BSA) and BMI were calculated using the formula. Scanning was performed with 6.5 MHZ transducer. Maximum length of each kidney was determined. Kidney length and volume were then correlated with different parameter of infant.

Results: There were no signifi cant differences in mean kidney length and volume between right (35.45 mm, 7.55cc) and left (34.30mm, 7.23cc) and kidneys in boys and girls. Kidney length correlated more with body wt (<0.001), BMI (<0.001) and BSA (<0.01) than gestational age (<0.05) and OFC (<0.05). Here no correlation found with length. Kidney volume also correlated better with body wt (<0.001), BSA (<0.001) and BMI (<0.01) than height (<0.05) and OFC (<0.05). Here no correlation found with gestational age. Conclusions: The present ongoing study provides an important baseline data in preterm babies for kidney dimension in Bangladeshi neonate as well as neonate of Indian subcontinent. BW, BMI, BSA all are correlated with kidney length and volume. So other two along with BW can also be selected as the independent variable for preparation of normogram for kidney length and Volume in premature infant. Objectives: The aim of the study was to evaluate the effect of total parenteral nutrition on renal functions of prematrue infants by comparing the serum cystatin C (cysC) levels and urinary N-acetyl-β-D glucosaminidase (NAG), β 2 microglobulin (β2M), gluthatione S transferase (GST) π levels in premature infants receiving total parenteral nutrition or enteral feding. Methods: Premature infants hospitalized in neonatal intensive care unit of Inonu University between January 2007 and July 2008, with a gestational age of 28 and 34 weeks were included in the study. Exclusion criteria consisted of presence of congenital malformations, parental refusal, onset of TPN or death prior to 72 hours. Prenatal, natal and postnatal characteristics of infants including the presence of respiratory distress syndrome, early neonatal sepsis, late-onset neonatal sepsis and the use of aminoglycosides were recorded. on the 3 rd and 30 th day of life, blood samples of all patients were obtained for evaluating biochemical parameters and cysC, and urine samples for the evaluation of NAG, GST π, β2M, sodium, creatinin levels, density and pH of the urine. Results: Serum cysS, urinary β2M, NAG and GST π excretion were signifi cantly higher in samples of patients receiving TPN both on 3 rd and 30 th days (p<0.05 for each parameter on each day). Conclusions: This study have shown for the 1 st time in premature infants that, TPN together with the presence of sepsis and use of aminoglycosides can have adverse effects on glomerular and tubular functions of the kidney which can be manifested with cysC, β2M, NAG and GST π.

Early Appearance of Hypokalemia in Gitelman Syndrome F. Tammaro, 1 A. Bettinelli, 1 D. Cattarelli, 2 C. Colombo, 3 S. Tedeschi, 4 M.L. Syrén, 5 M.G. Bianchetti. 6 1 Pediatrics, Mandic Hospital, Merate, Italy; 2 Pediatrics, Gavardo Hospital, Brescia, Italy; 3 Intensive Care, S.Gerardo Hospital, Monza, Italy; 4 Laboratory of Medical Genetics, Fondazione IRCCS Ca'Granda Policlinico, Milano, Italy; Università degli Studi, Milano, Italy; 6 Pediatrics, S.Giovanni Hospital, Bellinzona, Switzerland. Objectives: Inactivating mutations in the SLC12A3 gene that encodes the thiazidesensitive co-transporter cause Gitelman syndrome whose features include normal-low blood pressure, hypokalemia, metabolic alkalosis, hypomagnesemia, hypocalciuria, and hyperreninemia. These patients are at low risk for preterm birth and the condition is usually diagnosed in childhood or in adult life. We report on 4 patients, 2 pairs of prematurely born twins, in whom hypokalemia was demonstrated early in life. Methods: We evaluated 2 pairs of twins born after 28 and 34 weeks of gestation. In the 4 children, hypokalemia was noted during the third week of life, associated with normal blood pressure, normal total plasma magnesium level, hypochloremia and hyperreninemia. A marginal tendency towards hypomagnesemia (0.70 mmol/L) was observed after 2 years of life. Results: In both pairs of twins we demonstrated compound heterozygous mutations in the SLC12A3 gene: a frame shift mutation in exon 10 (c.1196_1202dup7bp), leading to the truncated protein p.Ser402X, and a missense mutation in exon 11, p.Ser475Cys (c.1424C>G) were disclosed in the fi rst pair and two missense mutations, p.Thr392Ile (c.1175C>T) in exon 9 and p.Ser615Leu in exon 15 (c.1844C>T), in the second pair. Conclusions: Gitelman syndrome may be considered as an additional cause of hypokalemia in the neonatal period. Objectives: We examined ontogeny of renal arachidonic acid(AA) metabolites and gene expression of CYPs during renal maturation in newborn,suckling and weanling periods in neonatal rats. We determined gender differences in CYP enzymes. Methods: Kidneys from rat pups were examined at birth P0,P4,P7,P14,P21 for levels of PGE2,PGF2,6-ketoPGF1,thromboxane(Tx) B2,8-isoPGF2,leukotriene (LKT) B4, and (15-HETE) using ELISA, and the gene expression of CYPs using PCR. Results: All AA metabolites were high at birth(PGE2:412611;PGF2:44298;6-ketoPGF1: 47196;TxB2:350548;8-isoPGF2:1181725;LKTB4:59983114;and 15-HETE: 41026109. During low Na suckling, metabolites exhibited different maturation. All decreased by(%)90,40,95,100,98,100; p<0.01,respectively,except TxB2 3390609, which declined at P7(46145) and P14(12248) compared to P0. At high Na weaning, metabolites increased with vasoconstrictors,TxB2(1364.09),8-isoPGF2(126545859), and 15-HETE(3063165) being highest. Ontogenic patterns of CYP enzymes paralleled metabolites. The main renal isoform of CYP3A regulates BP and R-A system, downregulated during suckling, and upregulated during weaning, 5-fold higher in females than males. Conclusions: Renal AA metabolites favor vasoconstrictors at weaning and may be important for drug disposition/Na excretion. Higher expression of CYP3A5 in females has early gender-specifi c difference.

Angiotensin AT1 Receptor Inhibition-Induced Apoptosis by Rho A Activation Associated with Na+/H+ Exchanger 1 NHE1 Downregulation in Neonatal Unilateral Ureteral Obstruction (UUO) P.G. Vallés, V. Bocanegra, W. Manucha, A. Gil Lorenzo, M. Rinaldi. Facultad de Medicina.Universidad Nacional de Cuyo, Mendoza, Argentina; CONICET, Mendoza, Argentina. Objectives: We examined the involvement of NHE1 associated with RhoA in the regulation of epithelial cell apoptotic response after AT1 receptor inhibition in obstruction. NHE1 modulation of the ERK1/2 kinase signal pathway was also evaluated. Methods: Neonatal rats subjected to complete UUO within the fi rst 48 hours of life and sham received saline, Losartan, or PD-123319 AT2 inhibitor for 14 days.For apoptosis study TUNEL assay confi rmed by electron microscopy, Bax/ BcL2 expression and caspase 3 expression and activity, were performed. Western blotting and immunofl uorecence were conducted for proteins expression.

Results: Tubular cell apoptotic response associated with mitochondrial signaling pathway through the increased proapoptotic ratio Bax/BcL-2 and consequently increased caspase3 expression and activity was demonstrated in Losartan obstructed kidney. Stimulation of RhoA activity that in turn reduced NHE1 expression was demonstrated in 14 day Losartan UUO kidney.Interaction between NHE1 and pERK was determined by coimmunoprecipitation showing that membrane downregulation of NHE1 was associated with pERK overexpression. Absence of increased AT2 protein expression after Losartan on day 14 of obstruction was shown. PD-123319 had no protective effect on the renal response to complete 14 day UUO. Conclusions: Our fi ndings suggest a role of RhoA on the negative regulation of NHE1 inducing phosphorylation of ERK 1/2 as events involved in tubular cell apoptosis regulation after AT1 receptor inhibition in neonatal UUO. Objectives: Hypertension and chronic kidney disease in the adulthood are related to prenatal insults that usually result in low birth weight and reduced nephron number. In animals, maternal nutrient restriction produces offspring with fewer nephrons. We studied whether the reduced nephron number is due to the inhibition of ureteric branching and developmentally regulated signaling pathways. Methods: The offspring of dams given food ad libitum (CON) and those subjected to 50% food restriction throughout pregnancy (NR) were examined. Embryonic day 13 (E13) to day 15 (E15) metanephroi were stained with Dolichos bifl ous lectin. Lysates of E15 metanephroi were analyzed by immunoblot. Results: At E13, there was no difference between NR and CON in either body weight or kidney surface area. Ureteric buds branched once in both NR and CON. At E14 and E15, body weight and kidney surface area were signifi cantly reduced in NR compared with CON. Ureteric bud tip numbers per kidney were signifi cantly reduced in NR (11.2±1.3 vs 21.3±1.0 at E14 and 31.0±1.7 vs 59.6±3.5 at E15). Activated forms of ERK, p38, PI3-kinase, and Akt and protein expression of β catenin were decreased 0.6-, 0.8-, 0.7-, 0.5-, and 0.7-fold, respectively, in NR. The expression of PCNA, a marker of proliferation, was not different between NR and CON, whereas that of cleaved caspase 3, a marker of apoptosis, was markedly decreased (0.4-fold of CON). Conclusions: Ureteric branching and metanephros growth were inhibited by maternal nutrient restriction after E13. Downregulation of developmentally regulated signaling pathways may play a role in abnormal kidney development in this pathophysiological setting.

HNF1B and FXYD2 Co-Expression Helps Explain Renal Magnesium Wasting in the Renal Cysts and Diabetes Syndrome S. Adalat, 1 J. Papakrivopoulou, 1 A.S. Woolf, 2 D. Bockenhauer. 1 1 Institute of Child Health, London, United Kingdom; 2 University of Manchester, Manchester, United Kingdom.

Objectives: Hepatocyte nuclear factor 1B (HNF1B) mutations cause kidney malformations in the renal cysts and diabetes (RCAD) syndrome. We reported hypomagnesaemia in children with HNF1B mutations and found that, in vitro, HNF1B transactivated FXYD2 encoding a distal convoluted tubule (DCT) Na + K + ATPase subunit itself mutated in another inherited hypomagnesaemia. However, whether HNF1B protein exists in DCT is unknown and we accordingly investigated expression of this and fxyd2 in mouse kidneys. Methods: Immunohistochemistry and western blotting in adult mouse kidneys and an established DCT cell line. Results: In tissue sections, hnf1b transcription factor was immunodetected in nuclei of kidney epithelia, being most prominent in collecting ducts and distal tubules, the latter characterised by expression of the transporter slc12a3. Hnf1b was weakly expressed in proximal tubules but glomerular epithelia, interstitial and vascular cells were negative. Fxyd2 itself had an overlapping distribution with hnf1b, being expressed in the distal tubule and collecting ducts. The DCT cell line expressed hnf1b as assessed by western blotting. Conclusions: Hnf1b and fxyd2 co-expression in distal tubules support the hypothesis that HNF1B mutations cause magnesium wasting by altering DCT physiology. Interestingly, expression of hnf1b in proximal tubules is consistent with a role in uric acid handling, as hyperuricaemia can be part of the syndrome.

Objectives: FAs play a critical role as the central to transduce signals by cell-matrix interactions and regulate cell bahaviors. FAK, ILK, paxillin and Hic-5 are major proteins to contribute these signaling events. We investigated the expression of these proteins in developing kidneys. Methods: We performed western blotting and immunohistochemistry in rat developing kidneys which removed from embryos on day 14 to 18 of gestation, and on 1 to 42 days after birth. Results: Western blotting revealed that the expression of FAK (p-FAK 397 ), paxillin (p-paxillin 118 ) and Hic-5 were high in embryonic kidneys. Meanwhile, ILK expression retain from embryonic to matured kidney. Immunohistochemistry revealed that FAK and paxillin were strongly expressed in mesenchymal cells and ureteric bud, following detected in elongating tubular epithelial cells, collecting duct, immature glomerular endothelial and mesangial cells. Compared with distribution of PCNA positive cells, FAK and paxillin might be involved in renal growth and morphogenesis. Hic-5 was dominantly expressed in mesenchymal and immature glomerular endothelial and mesangial cells, similar to α-SMA positive cells, suggesting that Hic-5 might be related to mesenchymal cell behavior. In mature kidneys, FAK and paxillin were limited in tubules and Hic-5 was limited in vascular smooth muscle cells. Although ILK expression similar to FAK in developing stages, it strongly expressed at matured podocytes and tubules. It suggests that ILK plays a role in epithelial cell differentiation as well as kidney growth and morphogenesis. Conclusions: Temporo-spatially regulated expression of FAs might play a role in kidney morphogenesis and differentiation.

In Objectives: We'll characterize vasculogenesis and angiogenesis in vivo in kidney development. We'll analyse in vitro vascular development limitations. We'll propose a novel kidney culture method, to improve vascular development. Methods: We used B6 mice. IF was done on Zeiss LSM 510 Meta. qRT-PCR conducted on LightCycler 1.5 (Roche). P values of less than 0,05 considered signifi cant. Results: In vivo, endothelial cells (PECAM1+) bud from the aorta at 10.5 dpc and migrate towards the metanephric mesenchyme (WT1+) to form the primitive renal artery at 11.5 dpc. Angioblasts (FLT1+ and KDR+) gather among the metanephric mesenchyme at 10.5 dpc and participate in renal vascular network development. Vascular tuft formation occurs between 12.5 and 14.5 dpc.

In vitro vascular development is impaired. It requires the PGC-1α pathway, HIF-independent, activated by hypoxia. In vitro kidney cultures with vascular microperfusion in 18.5 dpc embryos show signifi cant renal vasculature, and fl uid excretion. Conclusions: We showed that angiogenesis and vasculogenesis occur early in vivo in kidney development. In vitro vascular development requires PGC-1α pathway in addition to HIF. We proposed a novel kidney culture method, to optimize vasculature, with encouraging preliminary results.

Early vs Late Antenatal Betamethasone Effects on Renal Eicosanoids in the Newborn and Suckling Neonatal Rats J. Sharma, 1 A. Ahmad, 1 A. D'Souza, 1 C. Charles, 1 D. Kumar, 1 L. Forjour, 1 G. Valencia, 1 N. Anwar, 1 J.V. Aranda, 1 M. Schoeneman, 1 K. Objectives: Exposure to AB (170µg/kg IM) infl uences renal eicosanoids in the newborn and suckling neonatal rat and determine the effects of hyperoxia with brief hypoxia on renal prostanoids. Methods: Pregnant rats received a single 2-dose course of: 1) AB on E17 and E18 (early); 2) AB on E19 and E20 (late); 3) equivalent volumes of early and late saline (Sal). Pups were randomly kept on room air or placed in hyperoxia (50% O2) with random, brief hypoxia (O210%) until P4. Kidneys were analyzed for PGE2, PGF2α, 6-keto-PGF1α, TXB2, and 12(S)-HETE. Hypoxia-hyperoxia appears to have a more deleterious effect on renal eicosanoids.

Objectives: It is known that transient receptor potential cation channel 6 (TRPC6) is one of the key molecules for maintenance the structure and function of fi ltration barrier in podocytes. It is important for the study on pathogenesis of podocyte injury as well as mechanism of proteinuria. This study aimed to establish a stable technique to test TRPC6 not only the expression level but also the electrophysiology in podocytes. Methods: The protein expression of over-expression TRPC6 was evaluated with western blot. The intracellular Ca 2+ in podocyte was measured by laser scanning confocal microscope. The current recording was utilized in the whole-cell mode to detect the channel function of over-expression TRPC6. Results: Exposure of the podocyte to carbachol (CCh) and/or 1-oleoyl-acetyl-snglycerol (OAG) after initiating whole-cell dialysis via the patch pipette, caused a time-dependent activation of membrane current. The current density increased dramatically in over-expression group than in control by using CCh and OAG (P<0.05). After inhibiting the TRPC6 ion channel by treated with U73122, the current density was decreased in both over-expression TRPC6 group and control group (P>0.05). Conclusions: Functional changes of TRPC6 were detected for the fi rst time in podocytes of MPC5. It was found that TRPC6 ion channel could be activated excessively with increasing of Ca 2+ concentration via over-expression of TRPC6 in cultured podocytes, which might provide a cell model for further study on TRPC6 ion channel in podocytes.

Chronic Vasodilation Results in Plasma Volume Expansion (PVE) -Support for the Underfi ll Theory A. Fekete, 1,2 J.M. Sasser, 2 C. Baylis. 2 1 1st Department of Pediatrics, Semmelweis University, Budapest, Hungary; 2 Department of Physiology and Functional Genomics, University of Florida, Gainesville, FL, United States. Objectives: In pregnancy, systemic vasodilation results in renal Na and water retention and consequent PVE. Previously we showed that increased renal phosphodiesterase 5 (PDE5) activity inhibits the natriuretic response, contributing to the PVE in pregnancy. Methods: To test the "underfi ll hypothesis" of pregnancy, we produced chronic vasodilation by 14 days po. nifedipine (NIF, 10 mg/kg/day) or sodium nitrite (NaNO2, 70 mg/kg/day) to female Sprague Dawley rats vs those on control diet (CON). Mean arterial blood pressure (MAP) was monitored by telemetry, PV was determined by the Evans-blue method, hematocrit (Hct), osmolarity (Posm), Na (PNa), and total protein (CA) concentrations were also measured. mRNA expression and protein abundance of PDE5A and its isoforms were determined in kidney and aorta. Results: MAP was reduced in NIF by 6.7%, in NaNO2 rats by 7.6% vs. baseline and CON (p<0.05). NIF and NaNO2 lowered Hct and increased PV. Posm and PNa both fell, as did CA (*p<0.05 vs. CON).Renal medullary PDE5A and PDE5A1 mRNA expression increased both in NIF and NaNO2 group vs CON (p<0.05), while PDE5A2 isoform was the same in all groups. PDE5A protein level increased both in NIF and NaNO2 vs CON (p<0.05), however, neither renal cortical nor aortic PDE5 level was changed by vasodilator treatment. Conclusions: A primary vasodilation produced by NIF or NaNO2 drives increased medullary PDE5 expression, renal Na retention causing volume expansion and partial "refi lling" of the vasculature. These responses closely resemble the changes in normal pregnancy with hemodilution due to marked PVE. DISCLOSURE: Baylis, C.: Other, Forrest Pharma. Objectives: We investigated whether glomerular can express ANGPTL3 and whether ANGPTL3 can directly or indirectly bind to integrin αVβ3 in podocytes. Methods: We studied normal and MCD kidneys from humans and rats to evaluate the location and sub cellular features of ANGPTL3 in the podocytes. Using ELISA and quantitative real-time PCR, we evaluated ANGPTL3 produced by cultured podocytes. And we evaluated the bind model of ANGPTL3 and integrin αVβ3 in podocytes by confocal microscopy and immuno-precipitation. Results: Our results showed that ANGPTL3 were specially localized to the podocyte foot processes in glomerular. Quantitative analysis revealed a signifi cantly higher concentration of colloid-gold particles in the nephrotic podocytes compared to normal podocytes (P<0.05). Results of ELISA and real-time PCR suggested that the ability of cultured podocytes to synthesize ANGPTL3, which was greatly enhanced after ADR treatment. ANGPTL3 could directly bind to integrin β3. The active integrin β3 expression was signifi cantly up-regulated in podocytes over-expressing ANGPLT3 compared with control cells (P<0.01). Compared with an un-knockdown ANGPTL3 podocyte, ANGPTL3 knockdown cell did not show signifi cant the active integrin β3 upregulation after ADR-stimulation, suggesting that ANGPTL3 could regulate integrin β3 expression under certain pathological conditions. Conclusions: It is fi rst report to demonstrate that ANGPTL3 is found mainly within the foot processes of podocyte. And ANGPTL3 is a new key ligand that induces integrin β3 activation in podocytes.

Chinese Medicine Extractum Trametes Robiniophila Murr Improves Actin Rearrangement of Podocytes X. Gao, H. Xu. Department of Nephrology and Rheumatology, Children's Hospital of Fudan University, Shanghai, China. Objectives: Chinese medicine Extractum trametes robiniophila murr is a kind of fungus. Recent study has suggested that it could be capable to effi ciently improve the effacement of foot process in Adriamycin (ADR) induced rats. The aim of this study was to identify whether Extractum trametes robiniophila murr modulates actin rearrangement of podocytes and signaling molecules such as ACTN4, nephrin and podocin. Methods: The podocytes were divided into four groups as follows: Extractum trametes robiniophila murr group (Extractum trametes robiniophila murr 10mg/ ml + ADR 0.5umol/L); DXM group (DXM 0.1umol/L + ADR 0.5umol/L); ADR group (ADR 0.5umol/L) and control group. F-actin of podocytes in each group were stained by fl uorescent phallotoxins of invitrogen and observed by confocal microscope. The expression levels of ACTN4, nephrin and podocin in each group were tested by real-time PCR and western blot. Results: ADR group had signifi cant actin rearrangement. However, in Extractum trametes robiniophila murr group, podocytes showed a more powerful resistance to actin rearrangement of ADR induction than DXM group. In ADR group, the expression levels of ACTN4, nephrin and podocin were signifi cantly higher than those in control group. The expression level of ACTN4 in Extractum trametes robiniophila murr group was almost same as that in control group, while in DXM group, the expression level of ACTN4 was higher than that in control group. Extractum trametes robiniophila murr could not regulate the altering expression of nephrin and podocin after ADR treatment. Conclusions: Extractum trametes robiniophila murr is a powerfully potential modulator to actin rearrangement of podocytes. Objectives: To investigate effects of PHB on cell growth and ECM expression induced by TGF-β1 in renal interstitial fi broblasts, and ascertain whether PHB can suppress TGF-β1-mediated Smad signaling passway. Methods: Cell growth curves were detected. PHB, ECM proteins fi bronectin (Fn) and collagen type III (Col III), and matrix metalloproteinase-1(MMP-1) protein and mRNA expression were detected. Phosphorylation and activation of Smad3 by TGF-β1 and Smad7 expression were detected, and phosphorylation of Smad3 and its association with Smad4 in cells after TGF-β1 stimulation were further investigated by co-immunoprecipitation. Results: Stimulation of cells with 1ng/ml TGF-β1 induced a remarkable cells growth. Transfected with PHB plasmid suppressed cells growth, and the cell growth was 27%, 48% and 35% inhibited after transfection for 24, 48 and 72 h, respectively. TGF-β1 induced Col III and Fn proteins and mRNA expression and the effects were dose-dependent. Over-expression of PHB substantially attenuated the up-regulation of Col III and Fn, although it had almost no effect on the up-regulation of MMP1 induced by TGF-β1. Smad3 was phosphorylated after TGF-β1 treatment. Co-immunoprecipitation revealed that p-Smad3 was physically associated with Smad4. However, Over-expression of PHB did not signifi cantly affect Smad3 phosphorylation and its association with Smad4. Conclusions: Extraneous PHB suppresses renal interstitial fi broblasts proliferation and ECM expression induced by TGF-β1, and suppresses profi brotic cytokine TGF-β1 by intercepting Smad3 nuclear translocation. Objectives: This study was to investigate molecular mechanism of RGC-32 gene regulation in TGF-beta-induced EMT. Methods: (1) Plasmid Construction;(2) RNA interference;(3) Transfection and luciferase activity assay;(4) EMSAs. Results: (1) TGF-beta1 can active RGC-32 gene expression driven by pLuc-1500, pLuc-2107, or pLuc+844, but not by pLuc-85, pLuc-228, pLuc-600, or pLuc-1021 (P<0.001) after treated by TGF-beta1. It is indicated binding site which involved TGF-beta1 has located at RGC-32 promoter region from -1021 to -1500. SBE (GTCTGGAC) is located at the site from -1344 to -1337 of RGC-32 promoter, as well as a PEA3 binding site (AGGAAG) align at 35bp upstream of SBE from -1379 to -1374. Mutation of Smad site signifi cantly inhibited RGC-32 promoter activity (P<0.01), double mutations of Smad and PEA3 sites completely abolished promoter activity. (2) Smad2, Smad4 and PEA3 proteins are contribute to regulation of TGF-beta-induced RGC-32 expression: Smad2 and Smad4 antibody shown the same degree of supershift, Smad2, Smad4 and PEA3 proteins are contribute to regulation of TGF-beta-induced RGC-32 promoter activity. Smad2 was essential for TGF-beta induction of RGC-32 promoter, knockdown of PEA3 alone did not signifi cantly inhibit RGC-32 promoter, but knockdown of both Smad2 and PEA3 completely blocked TGF-beta function. Conclusions: SBE and PEA3 binding site were found at the upstream of RGC-32 promoter. Smad2 and PEA3 synergistically regulate TGF-beta induction of RGC-32 transcription during EMT.

Hyperfi ltration Affects Accuracy of Creatinine but Not Cystatin C or Beta Trace Protein eGFR Measurement S.-h.S. Huang, 1 A.P. Sharma, 2 G. Filler. 2 1 Medicine, University of Western Ontario, London, ON, Canada; 2 Pediatrics, University of Western Ontario, London, ON, Canada.

Objectives: Surrogate markers, such as creatinine, cystatin C, and beta trace protein have been used to estimate Glomerular Filtration Rate (eGFR). The accuracy of eGFR for these markers may be altered with hyperfi ltration and differences in fi ltration fraction. We hypothesize that the accuracy of creatinine for eGFR may be affected by hyperfi ltration and estimated renal plasma fl ow (ERPF). Methods: A total of 127 pediatric patients with various renal diseases underwent simultaneous measurements of both GFR using 51 Cr-ethylenediamine tetraacetic acid (EDTA) renal scan and ERPF ( 131 I Hippurate clearance) to calculate the fi ltration fraction (FF=GFR/ERPF). The eGFRs were calculated using the commonly used Schwartz's (creatinine), Filler's (cystatin C) and Benlamri's (beta trace protein) formulae. Agreement of the various eGFRs with the measured isotope GFRs was assessed by Bland&Altman plots. Correlation analysis was performed using non-parametric tests to compare FF with eGFR-GFR.

The 127 children at a median age (with 25 th percentile, 75 th percentile) of 11.9 (8.5, 14.9) years had a mean 51 Cr EDTA GFR of 100.6 ± 32.1 ml/min and a median 131 I hippurate clearance (ERPF) of 588 (398, 739) mL/min/1.73 m 2 . Mean fi ltration fraction was 17.7± 4.5% with no correlation between the FF and the error for Cystatin C eGFR and BTP eGFR, whereas there was a signifi cant negative correlation between the error for the Schwartz eGFR and the FF. Conclusions: There is a signifi cant negative correlation between the error for the Schwartz eGFR and the FF. Cystatin C and BTP are not affected by differences in fi ltration fraction.

Abstract# 937 mTORC1 Activation Induces Unfolded Protein Response of Podocytes in Nephrotic Syndrome N. Ito, K. Yan. Pediatrics, Kyorin University School of Medicine, Tokyo, Japan.

Objectives: The pathomechanism of minimal change nephropathy (MCN) has remained unclear. We previously showed that unfolded protein response (UPR) caused by lack of ATP in the endoplasmic reticulum might underlie the pathomechanism of MCN. The purpose of this study was to further investigate whether ATP consuming signaling cascade, mammalian target of rapamycin complex 1 (mTORC1), precedes UPR in MCN. Methods: Rat MCN was induced by puromycin aminonucleoside (PAN). Isolated glomeruli and kidney tissue from rats treated with or without mTOR inhibitor everolimus were subjected to immunohistochemistry, dual-immunofl uorescence and confocal microscopy, and Western blot analysis for mTORC1 and UPR phosphorylation. Immortalized mouse podocytes were treated with PAN in a time dependent manner, and subjected to Western blot analysis for mTORC1 and UPR phosphorylation. Results: Rats developed signifi cant proteinuria from day 2 after injection of PAN. Activation of both UPR and mTORC1 in glomerular podocytes was reveled at day 1 whereas expression of nephrin and podocin was reduced from day 2. Interestingly, mTORC1 activation was revealed to precede UPR when cultured podocytes were treated with PAN. Pre-treatment of everolimus at day 1 before PAN injection interfered with proteinuria compared with placebo-treated rats. The activation of both mTORC1 and UPR in the glomerular podocytes of rats treated with everolimus was decreased compared with placebo-rats. Conclusions: mTORC1 may be a potential signaling cascade that initiates UPR through dysregulation of energy system. Investigation of precise regulatory mechanism of ATP in podocytes may contribute to develop new therapeutic reagents for MCN. Objectives: Patients having the nephrogenic syndrome of inappropriate antidiuresis (NSIAD) exhibit hyponatremia and inappropriate elevated urinary osmolality associated to low plasma vasopressin levels. All patients reported to date present either the R137C or R137L V2 mutated receptor. While the clinical features have been characterized, the molecular mechanisms of functioning of these two mutants remain elusive. In the present study, we compare the pharmacological properties of R137C and R137L mutants with the wild-type and the V2 D136A receptor, the latter being reported as a highly constitutively active receptor. Methods: We have performed binding studies, second messenger measurements and BRET experiments in order to evaluate the affi nities of V2 receptor ligands, their agonist and antagonist properties and the ability of the mutated receptors to recruit β-arrestins, respectively. Results: The R137C and R137L V2 receptors exhibit small constitutive activities regarding the G s protein activation. In addition, these two mutants induce a constitutive β-arrestin recruitment. Of interest, they also exhibit weak sensitivities to V2 -agonist and to V2 -inverse agonist in term of G s protein coupling and β-arrestin recruitment.

The small constitutive activities of the mutants and the weak regulation of their functioning by agonist suggest a poor ability of the antidiuretic function to be adapted to the external stimuli, giving to the environmental factors an importance which can explain the phenotypic variability in patients having NSIAD. Objectives: Mizoribine (MZR) is an inhibitor of purine synthesis for an immunosuppressive agent. We empirically noted that the clinical effi cacy of MZR is relatively lower in pediatric patients compared with adults, but there have been few pediatric pharmacokinetic studies on MZR reported. Methods: One hundred and fi ve pediatric patients in our institutions were enrolled. The most frequent disease was idiopathic nephrotic syndrome in 74 patients, followed by secondary glomerulonephritis and IgA nephropathy. The single dose of MZR was 1-14 mg/kg. Population pharmacokinetic (PPK) analysis was performed based on the blood MZR levels in the patients. Results: The mean absorption lag time and absorption rate constant estimated to be 0.501 h and 0.761 h -1 , respectively, showing that MZR slowly appeared in the circulation and blood level elevation was also slow compared to those in adults. Using the calculated PPK parameters, the AUC ∞ and C max were compared based on Bayes' theorem among individual patients. A signifi cant correlation was noted between the age and AUC ∞ (P<0.0001), and the C max was also signifi cantly different among the age groups (P<0.05). Based on these fi ndings, the required MZR dose per body weight increased as the age decreased. Conclusions: The regular dose of MZR (2-3 mg/kg/day) is insuffi cient for pediatric patients. To obtain the expected clinical effect, high-dose treatment should be applied using the MZR-PPK model established in this study.

Inappropriate Antidiuresis without Elevation of Anti-Diuretic Hormone A. Kogon, R. McDonald. Pediatrics, Seattle Childrens Hospital, Seattle, WA, United States. Objectives: To describe a child with hyponatremic seizures and apparent syndrome of inappropriate antidiuresis (SIADH), with a mutation in the arginine vasopressin receptor 2 (AVPR2) gene. Methods: A 13 month old previously healthy boy presented with a generalized tonic clonic seizure. On exam, the patient was post-ictal, euvolemic and normotensive. His only laboratory abnormality was a serum sodium of 122 mEq/L. Initially, he was treated with a normal saline infusion for 24 hours and serum sodium improved to 128 mEq/L. His intravenous fl uids were then held and he maintained hydration with oral fl uids. Twelve hours later, his serum sodium was 118 mEq/L. Concurrently, all other serum electrolytes, blood urea nitrogen and creatinine were normal. His fractional excretion of sodium was 1.2%, plasma and urine osmolalities were 252 and 392 mOsm/L respectively, anti-diuretic hormone level was 1.8 pg/ml (range:1-13.3), serum aldosterone was 13 ng/dl (range:2-37) and serum renin was <0.1 ng/ml/hr. Subsequently, he was successfully treated with mild fl uid restriction and table salt supplements approximating 10 meq/kg/day of sodium chloride, to maintain serum sodium >130 mEq/L. Results: AVPR2 gene sequencing revealed a p.Arg137Cys mutation. This gene mutation, located on the long arm of the X chromosome, causes a gain of function of the arginine vasopressin receptor that induces constitutive receptor activation in the absence of arginine vasopressin. Over the subsequent 6 months, the patient's table salt supplements were decreased gradually, maintaining serum sodium in the low normal range. Conclusions: AVPR2 mutations should be considered in infants and children presenting with symptomatic hyponatremia without laboratory evidence of SIADH. Objectives: Autosomal-recessive Steroid-resistant nephritic syndrome is a common cause of ESRD in children. Mutations in the TRPC6 gene are a major cause of focal segmental glomerulosclerosis. However, whether the mutations of TRPC6 are also responsible for Chinese children with SRNS? And what's the differentiation of the TRPC6 expression between SRNS children and SSNS children? It's still unknown. Methods: Genomic DNA was isolated from blood samples of 37 SRNS patients, 22 steroid sensitive (SSNS) patients and 16 normal children, and we used PCR-amplifi ed each of 13 exons of TRPC6 gene sequence analysis. Immunohistochemistry method was used to detect the variance of TRPC6 expression between SRNS and SSNS children who had renal biopsy. Results: One novel synonymous mutation G42G (1151C>A) was identifi ed in three SRNS patients, which had not been found in the other two groups. Three biallelic SNPs were also found, including P15S (40C>T, rs3802829), -254C>G (rs3824934), and 237G>A. P15S and -254C>G were identifi ed in the promoter region, maybe they could take part in the regulation of this gene, and it's need further study. Immunohistochemistry showed that the expression of TRPC6 in nephric tubule were signifi cantly higher in SRNS patients than in SSNS children (P<0.01). Conclusions: As far, we had not found causative mutation in TRPC6 gene in Chinese children with SRNS. Maybe TRPC6 mutations were not a major cause of SRNS children in China, though the cohort was small. But, the expression of TRPC6 was higher in SRNS children than in SSNS children. And the two SNPs might act like a regulator of the promoter region of this gene. extracted from cultured cells, and RT-PCR detection of CD133 and CD146 mRNAs was performed. CD133-CD146+ cells were sorted by FACS and cultured to determine their ability to differentiate into osteogenic and adipogenic cells on media specifi cally suitable for differentiation into each cell type. Results: Cells could be cultured very effectively from the urine of patients with hematuria and/or proteinuria. RT-PCR showed expression of CD133 and CD146 mRNAs irrespective of whether the patients had nephropathy. Histochemical staining showed that the cells could differentiate into adipocytes and osteoblasts on the appropriate culture media. Conclusions: Renal mesenchymal stem cells can be cultured from the urine of pediatric patients with nephropathy. Urine may be a useful tool for regenerative medicine for kidney disease. 4 hours after IVP, slow increase thereafter, low physiologic level at night and achievement of an average level before 24 hours after IVP. Urine output was higher than previous days since patients were advised to keep suffi cient hydration. Conclusions: We suppose, that the decrease of FENa after IVP may result from renin-angiotensin-aldosterone system (RAAS) activation in response to hypotension and renal hypoperfusion following CM injection. That may also support a hypothesis of predominant role of prerenal mechanism possibly responsible for a precipitation of CIN.

Effect of Metabolic Acidosis on Neonatal Mouse Proximal Tubule Acidifi cation K. Twombley, J. Gattineni, M. Baum. UT Southwestern, Dallas, TX, United States. Objectives: NHE8 is the predominate sodium hydrogen exchanger in neonates and there is an isoform change at weaning to NHE3. The serum bicarbonate in neonates is lower than adults largely due to a lower rate of proximal tubule acidifi cation. It is unclear if the neonatal proximal tubule is functioning at maximal capacity or if it can respond to metabolic acidosis as in adults. The purpose of this study was to examine the effects of metabolic acidosis in neonatal proximal tubules. Methods: Mice were gavaged orally with 1 mMol/100grams of body weight starting at 5 days of age with either NH 4 Cl or NaCl twice daily for 7 doses. The kidneys were harvested for brush border membrane (BBM) vesicle isolation, RNA for cDNA synthesis, or for proximal tubule isolation for in vitro microperfusion. Results: Acid gavaged neonates decreased their serum bicarbonate from 19.5 + 1.0 to 8.9 + 0.6 mEq/l (p<0.001). Proximal convoluted tubule Na + /H + exchanger activity (dpH/dt) was 1.68 + 0.19 pH units/min in control tubules and 2.49 + 0.60 pH units/min in acidemic neonatal mice (p<0.05) indicating an increase in Na + / H + exchanger activity. There was an increase in both NHE3 (vehicle 0.35 ± 0.07 vs acid 0.73 ± 0.07; p<0.01) and NHE8 BBM protein abundance (vehicle 0.41 ± 0.05 vs acid 0.73 ± 0.06; p<0.001) in acidemic neonates compared to controls, but no signifi cant difference in mRNA levels in either exchanger. Conclusions: Neonatal mice can adapt to metabolic acidosis by increasing the BBM expression of both NHE3 and NHE8 as well as increasing proximal tubule acidifi cation with an increase in proximal tubule Na + /H + exchange activity. This study shows that in neonates, NHE8 can play an adaptive role in response to acidosis.

The Pendrin Gene, PDS, Is Transcriptionally Regulated by the Intestinal Natriuretic Peptide Uroguanylin J. Rozenfeld, 1 O. Tal, 1 L. Adler, 1 E. Efrati, 1 A. Stewart, 2 S. Carrithers, 3 S. Alper, 2 I. Zelikovic. 1 1 Div Pediat Nephrol, Rambam Med Ctr, Technion, Haifa, Israel; 2 Ren Div and Mol Vasc Med Div, Beth Israel Deac Med Ctr, Boston, MA, United States; 3 Sequela, Pewee Valley, KY, United States.

Objectives: Pendrin (SLC26A4), a Cl-/HCO3-exchanger, encoded by the gene PDS and expressed in the CCD, is involved in blood pressure regulation. Uroguanylin (UGN), a peptide produced in the intestine, functions in the kidney as "intestinal natriuretic hormone". We investigated whether UGN exerts its effect on electrolyte homeostasis partly by modulating pendrin activity. Methods: qRT-PCR, immunofl uorescence, transfection experiments. Results: qRT-PCR analysis of HEK293 cells exposed to UGN showed a decrease in endogenous pendrin mRNA. Mice received UGN intravenously. Kidneys were harvested for pendrin mRNA quantifi cation and pendrin protein visualization. UGN-injected animals displayed a decrease in pendrin mRNA and protein expression. Luciferase reporter plasmids containing different length fragments of human PDS (hPDS) promoter were transfected into HEK293 cells. Exposure of cells to UGN decreased hPDS promoter activity compared to activity in control cells. Furthermore, the fi ndings provided evidence for the presence of a hypothetical UGN response element within the 96bp region between -1140bp and -1044bp on the hPDS promoter. This region overlaps with the previously demonstrated hypothetical pH response element. Conclusions: UGN inhibits pendrin expression at the transcriptional level in vitro and in vivo. Characterization of the UGN response element on the PDS promoter and its possible role in extracellular fl uid homeostasis and blood-pressure regulation awaits future studies.

Artery Intima Media Thickness (cIMT) in Children with Chronic Kidney Disease (CKD): A Report from the Chronic Kidney Disease in Children (CKiD) Cohort Study T. Brady

Warady, 5 M. Mitsnefes. 3 1 Johns Hopkins University

United States; 3 Cincinnati Children's Hospital Medical Center

Children's Hospital of Philadelphia

Children's Mercy Hospital

580, 631 van den Heuvel

Keyword Index Numerals refer to abstract number

Adverse effects

Age factors

Area-under-curve (AUC)

Bacterial infection

Glomerular fi ltration rate

Monoclonal antibodies 9

Nuclear factor-kappa B (NF-kB)

Polymerase chain reaction (PCR)

Renal artery stenosis

717 Risk factors

Transforming growth factor-beta (TGF-b)

An Exceptional Case Neonatal Acute Renal Failure, ACE Inhibitors Nephropathy M. Giménez, A.D. Madrid, C. Herrero, C. Yolanda, L. Enrique, V. Ramon, N. Jose, M. Garrido. Pediatric Nephrology Department, Hospital Vall d'Hebron, Barcelona, Barcelona, Spain; Pathological anatomy, Hospital Vall d'Hebron, Barcelona, Barcelona, Spain. Objectives: These drugs now have been shown to be fetotoxic causing profound fetal hypotension, renal tubular dysplasia, anuria-oligohydramnios, growth

Author Index Numerals refer to abstract number.