Acute rejection rates and survival of renal transplant recipients with Alport's Syndrome

Transplant Proc. 2008 Jan-Feb;40(1):120-2. doi: 10.1016/j.transproceed.2007.11.008.


Alport's syndrome, a hereditary disorder characterized by a combination of nephritis and deafness, was first described in 1927. Herein, we have presented 20 cases of Alport's syndrome in renal transplant recipients. Between November 1975 and September 2006, we performed 1602 transplantations. 22 including 20 recipients (1.24%) with Alport's syndrome. The recipients were 16 males and 4 females of overall mean age 21.3 +/- 5.6 years (range, 14-35 years). Seventeen received kidneys from living-related and 5 from cadaveric donors. We retrospectively assessed recipient features: age, gender, physical examination, routine blood biochemistry, histopathological results, and audiometric test results, as well as postoperative complications in each of these 20 recipients. Ten instances of acute rejection occurred in 8 recipients. There were 3 postoperative complications, all of which were lymphoceles. We had no vascular or urinary system complications. At the time of this report, 19 recipients are alive; the other 1 died due to Kaposi's sarcoma. Sixteen recipients display good renal function and creatinine levels ranging from 0.8 to 2.9 mg/dL during a mean follow-up of 8.4 +/- 4.8 years (range, 1 to 20 years). Three of 19 recipients returned to hemodialysis at 17, 13, and 6 years after their first graft, respectively. Retransplantation was performed on 2 recipients at 18 and 7 years, respectively, after their first transplantation. In conclusion, although the number of patients in our series was small, in light of their uneventful postoperative periods and the good posttransplantation renal function in our recipients, we consider Alport's syndrome recipients as good candidates for transplantation.

MeSH terms

  • Acute Disease
  • Adolescent
  • Adult
  • Female
  • Graft Rejection / epidemiology*
  • Graft Survival
  • Humans
  • Immunosuppression Therapy / methods
  • Immunosuppressive Agents / therapeutic use
  • Kidney Failure, Chronic / etiology
  • Kidney Failure, Chronic / surgery
  • Kidney Transplantation / adverse effects*
  • Kidney Transplantation / mortality
  • Living Donors
  • Male
  • Nephritis, Hereditary / surgery*
  • Retrospective Studies
  • Survival Analysis


  • Immunosuppressive Agents