ASPHYXIATING THORACIC DYSTROPHY WITH END-STAGE RENAL-FAILURE - A REPORT OF 2 CASES

Two siblings with asphyxiating thoracic dystrophy developed rapidly-pr ogressive renal failure at four years of age. Their parents were first cousins. Both were treated by a kidney transplant from a family membe r. The renal outcome was satisfactory. Liver function tests done after the transplant procedure showed cytolysis and cholestasis. There was no evidence of viral hepatitis, and the liver biopsy was normal, with no fibrosis. Liver function tests returned to normal one year later at the same time as the cyclosporin dose was decreased. At last follow-u p one and two years after kidney transplantation, respectively, both c hildren had normal kidney and liver function tests.