Renal transplantation in patients with Alport syndrome

For pediatric kidney transplant physicians, two aspects of Alport syndrome set the disease apart from other causes of terminal renal failure. First, an understanding of the genetics of Alport syndrome is needed to make appropriate decisions regarding potential related kidney donors to Alport patients requiring renal transplantation. Second, renal transplantation for Alport syndrome may be complicated by post-transplant anti-GBM nephritis, a problem that is nearly unique to this disease. This review discusses these aspects of Alport syndrome and attempts to provide rational recommendations for clinicians.