key: cord-0038615-oeo2yt7v authors: nan title: Answer to Picture Quiz date: 2011-07-21 journal: Med J Armed Forces India DOI: 10.1016/s0377-1237(10)80134-0 sha: 2d03852f9c128683709c948388153fd44d636268 doc_id: 38615 cord_uid: oeo2yt7v nan Kawasaki disease was first described in 1967 by the Japanese paediatrician Tomisaku Kawasaki. In 80% cases affected children are aged less than five years. Older children are only rarely affected [1] . Clinically Kawasaki disease is an acute onset illness characterized by high fever with mucocutaneous changes and lymphadenitis. Fever is a constant feature in Kawasaki disease and usually ranges between 38-40°C with irregular spikes. Mucocutaneous changes are seen in 90% cases. The five principal criteria along with fever of five days duration for diagnosis of Kawasaki disease are (i) bilateral bulbar non exudative conjunctivitis (ii) changes of mucosa of oropharynx including dry fissured lips, injected pharynx, strawberry tongue (iii) changes of the peripheral extremities such as edema or erythema of hands and feet, desquamation usually beginning periungually (iv) rash which is non vesicular and primarily truncal (v) cervical lymphadenopathy [2] . Other associated features of Kawasaki disease are arthritis, irritability, aseptic meningitis, urethritis and diarrhea. Kawasaki disease closely mimics other infections and syndromes such as viral infections (measles, adenovirus, enterovirus, and Epstein-Barr virus), bacterial cervical lymphadenitis, drug hypersensitivity reactions, Stevens-Johnson syndrome and juvenile rheumatoid arthritis. Though the etiology remains unknown, recently corona virus infection and genetic polymorphism have been implicated [3] . Elevated leucocyte counts with increased neutrophils, elevated ESR and C-reactive protein are also present along with thrombocytosis. Kawasaki disease typically has a triphasic course. The acute phase with fever and signs of inflammation subsides by one to two weeks. The subsequent subacute phase characterized by desquamation of tips of fingers of hands and feet lasts one to two weeks. The convalescent phase starts usually four to six weeks after onset of the disease. Coronary aneurysms are the most typical cardiovascular feature of Kawasaki disease occurring in 20% of untreated patients. The coronary artery disease may lead to myocardial infarction or aneurysmal rupture with mortality usually within two months of disease onset in untreated patients. The standard management of Kawasaki disease combines high dose intravenous immunoglobulin (2g/kg) and aspirin in anti-inflammatory doses (80-100mg/kg/day) given in four divided doses for 14 days or up to 48-72 hours of cessation of fever [2] . This treatment protocol has been shown to reduce the duration of the acute disease and also reduce the occurrence of coronary aneurysms [2] . Aspirin is usually reduced to a dose of 5-10 mg/kg once fever subsides and continued for about six weeks. Early initiation of treatment reduces the incidence of coronary complications from 45% to 20%. This reduction is possibly due to rapid fall in levels of pro-inflammatory cytokines immediately after immunoglobulin infusion which prevents endothelial damage [4] . The Kawasaki disease population should be monitored at regular intervals and should also be assessed and counselled for atherosclerotic risk. About one half of the coronary artery aneurysms associated with Kawasaki disease resolve within one to two years, particularly those that are smaller(<6mm) and fusiform [5] . Patients with "incomplete" Kawasaki disease exhibit fever and some of the classic symptoms but not enough to meet the diagnostic criteria (less than four of the five principal clinical findings). The finding most consistently absent in incomplete Kawasaki disease is cervical lymphadenopathy. Mucous membrane changes are the most consistent finding. When a patient with Kawasaki disease exhibits features such as hypertension, nephritis, seizures or cerebrovascular accident it is termed as "atypical" Kawasaki disease [6] . Nelson Text Book of Paediatrics Diagnosis, Treatment and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young Association between a novel human corona virus and Kawasaki disease Cytokine modulation with immune gamma globulin in peripheral blood of normal children and its implications in Kawasaki disease treatment Cardiovascular complications in Kawasaki disease: coronary artery lumen and long-term consequences Kawasaki disease-An Indian Perspective None identified