title: Primary nephrosis in childhood associated with focal glomerular sclerosis: is long-term prognosis that severe?
abstract: In our study, 32 nephrotic children with focal glomerular sclerosis were observed for an average period of 8 years (ranging 1-19 years of age). Of the 32, 25 children showed histological lesions of focal segmental sclerosis (FSG) and 7 of focal global glomerulosclerosis (FGG). All patients were reevaluated in 1979, creating the most recent status as follows: for children with FSG, 6 (24%) are in remission, 10 (40%) have a relatively normal renal function but exhibit either a persisting proteinuria (PP) or a recurrent nephrotic syndrome (NS), 1 (4%) is in chronic renal failure, 5 (20%) required dialysis and transplantation, and 3 (12%) died from non-renal causes. For children with FGG, 4 (57%) are in remission, 2 (29%) have a good renal function but display either PP or NS, and 1 patient (14%) is in chronic renal failure. The long-term observation of our study shows a more favorable prognosis than the one reported in researched literature. We believe that such results reflect a difference in the type of population encountered in our institution. Our population of patients represents less a highly referred population than the one of the centers who reported similar long-term studies, and we believe therefore that our study may represent a wider spectrum of the natural history of the disease. Moreover, the group of patients with PP or NS does not show a progressive decrease of glomerular filtration rate with time, which suggests that the disease may progress in a stepladder fashion.
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000150:complications*; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D016030:Kidney Transplantation; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D006435:Renal Dialysis
chemical_list: D003520:Cyclophosphamide; D011241:Prednisone