RENAL-TRANSPLANTATION IN ALPORTS-SYNDROME

Abstract

Nineteen patients (3 women and 16 men) with Alport''s Syndrome and endstage renal failure received 23 allograft kidneys at 2 medical centers between 1972 and 1983. Ten patients had pretransplant splenectomies, and 4 patients had pretransplant thoracic duct drainage. After a mean follow-up time of 49 mo., analysis revealed total allograft survival was 65% at 1 yr, 50% at 2 yr and 57% at 5 yr. Pretransplant splenectomy resulted in 60% allograft survival at 24 mo. mean follow-up. Pretransplant thoracic duct drainage resulted in 100% allograft survival at 15.6 mo. mean follow-up. The overall allograft survival was greatest for 3 and 4 antigen-matched kidneys and for living related donor kidneys. Evidently, 50% of all allografts in men were functional at 50.8 mo. mean follow-up. All allografts in women were functional at 48.3 mo. mean follow-up. Three of 4 patients who expired had pretransplant splenectomies. Renal transplantation is evidently the preferred method of treatment for patients with Alport''s Syndrome.