[Focal sclerosing and segmentary nephropathy in children]

The cases of 23 nephrotic children in whom percutaneous renal biopsies or postmortem studies showed focal and segmentary sclerosis were reviewed. It was more common in males and the average age at the onset was 6 years. The main symptoms were edema, hematuria and unspecific general manifestations. On admission, nephrotic syndrome was present in 91% of the cases and in a lesser proportion, edema hematuria and high blood pressure were found. The lesion was diagnosed at the first biopsy in 19 cases and in 4, subsequent biopsies or postmortem studies supported the diagnosis. Only 9 out 21 patients treated showed response to steroids and 5 of the resistant cases were given cyclophosphamide, but only one responded. They were checked at different periods of time ranging from 7 months to 10 years and by the end of the study, 11 patients were still under control showing normal renal function in six of them; 4 other cases showed mild renal insufficiency and in the remaining one, there was moderate renal insufficiency. Two died. A good correlation was evident between response to steroids and conservation of renal function. The findings are compared to those from other authors and emphasis is placed on the need to suspect the entity when a nephrotic syndrome with minimal changes is resistant to steroids, or when persistent hematuria is present; also, in cases showing initial lesions of tubular atrophy or interstitial fibrosis.