[Familial nephrotic syndrome with focal glomerular sclerosis (author's transl)].
This is a report about three siblings (one boy and two girls) suffering from a clinically- and morphologically-identical form of renal disease. The disease began in each case with symptomless proteinuria at the age of 3 years and proceeded after several years to the full-blown picture of idiopathic nephrotic syndrome with the rapid development of renal insufficiency. Histologically, minimal proliferative intercapillary glomerulonephritis with focal sclerosis was found in all 3 cases. This condition was resistant to steroid and immunosuppressive therapy. The incidence and the morphological, clinical and therapeutic peculiarities and the prognosis of familial nephrotic syndrome are discussed on the basis of these case reports.
D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005921:Glomerulonephritis / Q000235:genetics*; D005923:Glomerulosclerosis, Focal Segmental / Q000235:genetics* / Q000473:pathology; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000235:genetics* / Q000473:pathology
Familiäres nephrotisches Syndrom mit fokaler Glomerulosklerose