Renal Transplantation in Patients with Alport Syndrome

Abstract

Objective: Alport syndrome is an inherited disease that occurs in 1/50,000 and is characterized by hematuria, kidney failure, deafness and ocular anomalies. Alport syndrome, which is inherited as X-linked recessive with a rate of 85%, may be inherited also as autosomal dominant or autosomal recessive. Defective type-4 collagen participating in the structure of glomerular basement membrane causes a progressive decline in kidney functions. This research investigates the results of end-stage renal failure in patients with Alport syndrome following kidney transplantation. Methods: In our Hospital Organ Transplant Center 11 kidney transplantations were performed in 10 patients with Alport syndrome between October 2010 and December 2020. The recipients were analyzed retrospectively for acute rejection, complication rate, graft and patient survival. Results: Eleven (0.88%) of 1,251 kidney transplants were performed in patients with Alport syndrome. Acute rejection did not occur in any patient after kidney transplantation and no medical or surgical complications were observed in the early postoperative period. One patient died 19 months after surgery because of pneumonia and sepsis while his graft was functional. Graft loss was observed in two patients. In one of these patients, graft loss developed due to drug incompatibility in the 11th month after kidney transplantation. In the other patient, graft loss was observed due to chronic allograft nephropathy in the 63rd month postoperatively. This patient underwent a second kidney transplant surgery from a living donor. Conclusion: Alport syndrome is a rare cause of chronic kidney failure. Kidney transplantation is an effective and successful treatment method for end-stage renal disease patients with Alport syndrome.