Favourable long term effect of ursodeoxycholic acid treatment on congenital vanishing bile duct syndromes

Vanishing bile duct syndromes (VBDS) constitute a heterogeneous group of diseases characterized by progressive disappearance of intrahepatic interlobular bile ductules (1). Several conditions have been related with VBDS, both congenital and acquired. Congenital abnormalities include Alagille syndrome, cystic fibrosis, a1 antitrypsin deficiency and progressive familial intrahepatic cholestasis (PFIC), while infections and medications can also cause VBDS later in life (2, 3). PFIC is a group of autosomal recessive disorders of bile formation that present with cholestasis, itching and jaundice (4). Congenital VBDS lead to chronic cholestasis, development of cirrhosis and death from liver failure (2, 3). Primary biliary cirrhosis (or primary biliary cholangiopathy as has been recently renamed) is probably the best known example of these entities where both inherited and environmental factors are implicated (2). Ursodeoxycholic acid (UDCA) is a hydrophilic dihy- droxy bile acid and is recommended for patients with primary biliary cirrhosis (5). AlsO. UDCA can be used as a treatment for other cholestatic syndromes due to its anticholestatic effects (5). In this article, we report a favorable long term effect of UDCA treatment in two patients with congenital vanishing bile duct syndromes. The first patient has been followed up in our clinic for the last 27 years whereas the second patient was first evaluated eight years ago.