Our study concerned 147 children with chronic renal failure (CRF) (creatinine clearance less than 50 ml/min/1.73 m2). Its goal was to analyse the distribution of primary renal diseases, natural history of renal failure (RF) according to etiology, and long term survival. Renal diseases responsible for RF were: malformations of the urinary tract (38%), glomerular pathology (26%), hereditary renal diseases (20%), isolated renal hypoplasias (11%), and miscellaneous (5%). Corticoresistant nephrosis accounted for 34% of glomerular diseases and nephronophtisis 63% of hereditary renal diseases. On the whole, RF was related with an uropathy or renal hypoplasia in half of cases and with congenital renal disease in almost 3/4 of cases. The natural history varied according to primary renal disease: slow deterioration after a period of relative stability for uropathy or renal hypoplasias, slow and regular deterioration for nephronophtisis, rapid deterioration for glomerular diseases.
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