Abstract
A retrospective clinicopathologic study was made of 17 children with focal sclerosing glomerulonephropathy with segmental hyalinosis (FSGNH). Renal morphologic changes include segmental glomerular sclerosis with local hyaline, eosinophilic deposits. Segmental glomerular deposits of IgM, IgG, C3, and C4 were commonly observed. Ultrastructural evaluation revealed small paramesangial as well as large and small local subendothelial glomerular capillary electrodense deposits. Inital symptoms were mixed nephritic/nephrotic manifestations or proteinuria. Serum B1C was normal. Persistent hypertension and renal failure occurred early. Corticosteroid therapy was of no apparent, benefit. Five patients with serial renal biopsies showed histologic progression from minimal glomerular alterations that would have been interpreted as minimalchange glomerulonephropathy to FSGNH and finally chronic glomerulonephritis. FSGNH appears to be a specific glomerulonephropathy that may initially present as steriod-resistant nephrotic syndrome with minimal glomerular alterations and progress to chronic end-stage glomerulonephritis.
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