Primary nephrosis in childhood associated with focal glomerular sclerosis: Is long-term prognosis that severe?

Based on the histologic aspects of the lesions, primary nephrosis with focal glomerular sclerosis has been classified into focal segmental glomerulosclerosis (FSG) and focal global glomerulosclerosis (FGG).Several reports [1–14] define its clinical presentation and attempt to identify the initial signs that indicate a poor prognosis. The criteria indicating poor prognosis were reported to be age of onset [15, 16], the presence of a nephrotic syndrome [10, 15, 17], mesangial proliferation [14], the presence of focal segmental glomerulosclerosis on early biopsies [17], the association of glomerulotubular imbalance [18], the severity of the lesions on an early biopsy [1–3, 5, 6, 8, 10, 12, 13, 17], and especially the resistance of these patients to corticosteroid and immunosuppressive therapy [1–3, 6, 8, 9, 11–13, 16, 17].The long-term followup of children with focal glomerular sclerosis has been analyzed in detail, however, only in a few articles [2, 3, 17], and these reports have concluded that the prognosis is generally poor.The purpose of our study is to report the outcome of observing 32 children with primary nephrosis and focal glomerular sclerosis over a period of several years. We observed that the long-term prognosis in our series is more favorable than that described by other reports. We believe that this discrepancy is due to the referral profile of our institution and that our population of patients more accurately reflects the full spectrum of the natural history of the disease