Congenital nephrotic syndrome: a clinico-pathologic study of thirty children.

摘要: BACKGROUND Congenital nephrotic syndrome is an uncommon disorder that may be caused by several diseases. These inherited, sporadic, acquired or part of a general malformation syndrome. METHODS We reviewed the clinical characteristics, pathologic findings, and results medical management in 30 infants who presented to Jordan University Hospital with congenital years 1989 1999. RESULTS Most patients (80%) had parents were consanguineous. born premature, average gestational age 36 weeks. (77%) first three months life 26 (87%) significant growth retardation. Twenty-five verified episodes serious bacterial infections occurred 18 patients. Antibiotic therapy however was successful all these episodes. Light microscopy renal biopsies consistent Finnish type nephrosis most (83%). Chronic insufficiency developed 17, five them needed chronic peritoneal dialysis. given albumin transfusion diuretic especially during severe edema. Captopril alone combination ibuprofen eight patients, but without response any them. All died before 5 years. deaths at 15 (range 1-60). CONCLUSION The common our whom within few onset disease. In developing countries, have different from countries view high cost management, poor outcome, risk complications, mortality rate.