Renal transplant in patients with Alport's syndrome

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
Michael C ByrneDavid W Ploth

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)....Continue Reading

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Citations

Dec 18, 2013·Current Rheumatology Reports·Rebekah JoblingPeter Kannu
Jun 20, 2003·The New England Journal of Medicine·Billy G HudsonEric G Neilson
Jul 26, 2014·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Andrew MallettDavid W Johnson
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Sep 3, 2019·Frontiers in Immunology·Wai H LimGermaine Wong
Oct 5, 2018·BMC Nephrology·Ai KatsumaUNKNOWN Japan Academic Consortium of Kidney Transplantation (JACK)
Apr 15, 2015·Journal of Clinical Pathology·Gabriela D Moyano CrespoJorge H Mukdsi
Sep 30, 2020·Advances in Anatomic Pathology·Mohammed AkhtarMuhammad Asim
Feb 27, 2021·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Moumita Barua, Andrew D Paterson
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