Long Term Outcome of Renal Transplantation for Alport's Syndrome

This is the authors' abstract. We don't add key points for this paper.

581 PURPOSE: To determine the long term outcome of renal transplantation in patients with Alport's Syndrome (AS). METHODS: A single-center, retrospective review of 1531 renal transplants between 1974 and 1998 identified 38 patients with end-stage renal disease secondary to AS. A total of 48 renal transplants (41 CAD; 7 LRD) were performed in these patients (83% Caucasian; 15% African-American; 2% Hispanic). Endpoints examined included patient and graft survival, incidence of delayed graft function and rejection, death with functioning graft, impact of cyclosporine and race, and causes of graft loss. Maintenance immunosuppression consisted of prednisone and azathioprine only until 1984 when CSA was added to the regimen. Mycophenolate mofetil replaced azathioprine in 1995. Mean follow-up was 59 months (range 1 month to 213 months). (Table)TableRESULTS: Patient and graft survival in recipients with AS were not statistically different from all other recipients. There was no difference in patient survival among different AS racial groups. Incidences of delayed graft function and rejection were similar to other recipients during the same time period. A total of 27 grafts failed; 8 grafts (3 pre-CSA era, 1 retransplant, 1 venous thrombosis) were lost within 1 year. Rejection accounted for 90% of graft failures. Two patients died with functioning grafts. Only one graft failure was due to recurrent anti-GBM nephritis. CONCLUSION: Our results substantiate other reports indicating a 3-4% incidence of post-transplant anti-GBM nephritis. We conclude that over a 24 year period, with changing immunosuppressive regimens, patients with Alport's Syndrome can have excellent patient and graft survival with a low incidence of anti-GBM nephritis.