HEMOPHILIA-A AND CHRONIC HEPATOPATHY CAUSED BY EXTRAHEPATIC BILIARY ATRESIA - 2 CONGENITAL DISEASES CURED BY ORTHOTOPIC LIVER-TRANSPLANTATION

Since the publication of the first successful liver transplantation in a patient with severe haemophilia A by Lewis et al. in 1985, differen t authors have reported clinical cure of haemophilia A by orthotopic l iver transplantation. In the published cases liver transplantation was performed due to end-stage chronic liver disease secondary to factor replacement therapy for haemophilia A or haemophilia B. Congenital bil iary atresia is the most common cause of obstructive jaundice in the n eonatal period and the most common indication for liver transplantatio n in childhood. In this article we report the first successful orthoto pic liver transplantation performed in Spain, carried out in the young est patient thus far described,a 5-year-old boy with haemophilia A and chronic liver disease secondary to congenital biliary atresia.