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Primary nephrosis in childhood associated with focal glomerular sclerosis: is long-term prognosis that severe?
Insights
This study followed 32 children with focal glomerular sclerosis for 8 years. Results indicate a more favorable prognosis, with many achieving remission or maintaining renal function, suggesting a stepladder progression for some.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Focal glomerular sclerosis (FGS) is a significant cause of nephrotic syndrome in children.
- Long-term prognosis and disease progression patterns require further elucidation.
Purpose of the Study:
- To evaluate the long-term outcomes and natural history of focal glomerular sclerosis in a pediatric cohort.
- To compare prognosis with previously reported data and identify factors influencing outcomes.
Main Methods:
- Longitudinal observation of 32 children diagnosed with focal glomerular sclerosis (25 with focal segmental glomerulosclerosis - FSG, 7 with focal global glomerulosclerosis - FGG).
- Average follow-up period of 8 years, with reevaluation in 1979.
- Assessment of renal function, remission status, proteinuria, and need for renal replacement therapy.
Main Results:
- FSG cohort: 24% remission, 40% persisting proteinuria/recurrent nephrotic syndrome (NS) with stable renal function, 4% chronic renal failure, 20% dialysis/transplantation, 12% non-renal death.
- FGG cohort: 57% remission, 29% proteinuria/NS with good renal function, 14% chronic renal failure.
- Patients with persisting proteinuria or NS did not show progressive decline in glomerular filtration rate, suggesting a stepladder progression.
Conclusions:
- The observed prognosis for pediatric focal glomerular sclerosis appears more favorable than previously reported.
- This may be attributed to the study population representing a wider spectrum of the disease, less highly referred.
- The non-progressive nature of renal function decline in some patients suggests a potential stepladder pattern of disease advancement.
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.