Haemophilia A and chronic hepatopathy caused by extrahepatic biliary atresia: two congenital diseases cured by orthotopic liver transplantation.

Abstract

Since the publication of the first successful liver transplantation in a patient with severe haemophilia A by Lewis et al. in 1985, different authors have reported clinical cure of haemophilia A by orthotopic liver 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 biliary atresia is the most common cause of obstructive jaundice in the neonatal period and the most common indication for liver transplantation in childhood. In this article we report the first successful orthotopic liver transplantation performed in Spain, carried out in the youngest patient thus far described, a 5-year-old boy with haemophilia A and chronic liver disease secondary to congenital biliary atresia.