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Renal transplant in patients with Alport's syndrome.

Abstract
We evaluated 52 renal grafts transplanted into 41 patients with a pretransplantation diagnosis of Alport's syndrome. Overall 1-, 5-, and 10-year patient and graft survival rates were 95.1%, 90.2%, and 80.5% and 86.8%, 66%, and 45.3%, respectively. Although 14% of renal graft biopsy specimens examined with immunofluorescent microscopy showed linear glomerular basement membrane (GBM) immunoglobulin G deposits, only 1 of 41 patients (2.4%) or 52 grafts (1.9%) developed posttransplantation anti-GBM disease. The incidence of anti-GBM disease was 3.1% (1 of 32 patients) in a subgroup of male transplant recipients. Our analysis suggests that the incidence of anti-GBM disease in transplant recipients with Alport's syndrome is less than previously reported. In addition, it does not appear that HLA-DR alleles, which predispose to the development of anti-GBM disease in native kidneys, have a role in transplant recipients with Alport's syndrome posttransplantation. However, immunosuppression level may have a pathophysiological role in the development of anti-GBM disease. The majority of grafts in transplant recipients with Alport's syndrome failed because of chronic allograft nephropathy (69% of grafts) and acute rejection (22% of grafts). A history of previous acute rejection was the only factor that significantly affected graft outcome.
AuthorsMichael C Byrne, Milos N Budisavljevic, Zihong Fan, Sally E Self, David W Ploth
JournalAmerican journal of kidney diseases : the official journal of the National Kidney Foundation (Am J Kidney Dis) Vol. 39 Issue 4 Pg. 769-75 (Apr 2002) ISSN: 1523-6838 [Electronic] United States
PMID11920343 (Publication Type: Journal Article)
CopyrightCopyright 2002 by the National Kidney Foundation, Inc.
Topics
  • Anti-Glomerular Basement Membrane Disease (complications, epidemiology, pathology)
  • Female
  • Graft Survival
  • Humans
  • Kidney Transplantation (mortality, pathology)
  • Male
  • Nephritis, Hereditary (complications, pathology)

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