title: 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.
authors.affiliation: Department of Biometry, Nephrology Division, Medical University of South Carolina, Charleston, SC 29425, USA.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Byrne
authors.forename: Milos N
authors.initials: MN
authors.lastname: Budisavljevic
authors.forename: Zihong
authors.initials: Z
authors.lastname: Fan
authors.forename: Sally E
authors.initials: SE
authors.lastname: Self
authors.forename: David W
authors.initials: DW
authors.lastname: Ploth
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications / Q000453:epidemiology / Q000473:pathology; D005260:Female; D006085:Graft Survival; D006801:Humans; D016030:Kidney Transplantation / Q000401:mortality / Q000473:pathology; D008297:Male; D009394:Nephritis, Hereditary / Q000150:complications* / Q000473:pathology