Twenty-seven children with focal segmental glomerulosclerosis: correlation between the segmental location of the glomerular lesions and prognosis.

Twenty-seven children with focal segmental glomerulosclerosis (FSGS) were studied for clinical and pathological manifestations in order to determine whether the location of the sclerotic lesions bears prognostic implications. There were 11 children with peripheral FSGS and 16 with hilar FSGS. During a mean follow-up period of 6 years, 10 of the 16 with hilar FSGS developed renal failure (GFR less than 20 ml/min/m2) but all of the 11 with peripheral FSGS maintained normal renal function. We suggest that FSGS may be divided into a hilar type with poor prognosis and a peripheral type with benign prognosis. It is conceivable that these two groups represent etiologically different entities.