id author title date pages extension mime words sentences flesch summary cache txt cord-006728-bejrttyk Rozmus, Jacob Severe Combined Immunodeficiency (SCID) in Canadian Children: A National Surveillance Study 2013-10-12 .txt text/plain 3509 163 50 Following the identification of disseminated Bacille Calmette-Guérin (BCG) infections in Canadian First Nations, Métis and Inuit (FNMI) children with unrecognized primary immune deficiencies, a national surveillance study was initiated in order to determine the incidence, diagnosis, treatment and outcome of children with SCID in Canada. The objectives of this study were to determine: 1) the incidence and type of SCID in the Canadian FNMI and general populations; 2) the incidence of disseminated BCG disease in SCID patients; 3) the average time delay to diagnosis; 4) known risk factors and initial clinical presentations; 5) whether patients received a HSCT or gene therapy; and 6) the causes of any deaths. The CPSP case definition for SCID was any child less than 2 years of age with the clinical features of SCID (including chronic diarrhea, recurrent pneumonia, failure to thrive, persistent thrush, and opportunistic infections) and at least one of the following: 1) an absolute lymphocyte count of less than 3000/ mm 3 or less than 20 % CD3+ T cells, 2) familial history of primary immunodeficiency. ./cache/cord-006728-bejrttyk.txt ./txt/cord-006728-bejrttyk.txt