Renal transplantation in Alport's syndrome: anti-glomerular basement membrane glomerulonephritis in the allograft.
Eleven patients with Alport's syndrome had renal transplantation between 1963 and 1980. The mean follow-up is 6 years (6 months to 14 years). All 11 patients are currently alive. Ten have functioning grafts, and in nine the serum creatinine concentration is less than 2 mg/dl. Three patients received a second allograft. Histologic study of the allografts that were lost revealed a diffuse crescentic glomerulonephritis with strongly positive linear IgG staining of the glomerular basement membrane in the allografts of two of the three patients; circulating antiglomerular basement membrane antibodies were present in one patient. These two patients had successful retransplantation and they remain well 1 1/2 and 4 1/2 years later. Renal transplantation in patients with Alport's syndrome is a worthwhile and very satisfactory procedure.
D000293:Adolescent; D000328:Adult; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D005069:Evaluation Studies as Topic; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006084:Graft Rejection; D006801:Humans; D007668:Kidney / Q000276:immunology; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary / Q000276:immunology / Q000601:surgery*; D012086:Reoperation; D014184:Transplantation, Homologous