Biliary atresia

Biliary atresia is also known as “extrahepatic ductopenia” , “infantile cholangiopathies” and “progressive obliterative cholangiopathy” is a congenital or acquired disease of the liver and one of the principal forms of chronic rejection of a transplanted liver allograft.  In the congenital form, biliary atresias are a group of intrauterine developmental abnormalities of the biliary system, the common bile duct between the liver and the small intestine is blocked or absent. The abnormality of development in most instances is extraneous infectionduring the intrauterine development or shortly after birth that brings about obstruction of the bile ducts. The acquired type most often occurs in the setting of autoimmune disease, and is one of the principal forms of chronic rejection of a transplanted liver allograft. The condition therefore may have various grades of obstruction ranging from complete absence of bile ducts termed atresia, to reduction  in their numbers called paucity of bile ducts.

Extrahepatic biliary atresia

The extrahepatic bile ducts fail to develop normally so that in some the bile ducts are absent at birth, while in some cases bile ducts may have been formed but start undergoing sclerosis in the perinatal period. Infants and children with biliary atresia have progressive cholestasis with all the usual concomitant features: jaundice, pruritus, malabsorption with growth retardation,elevated liver transaminases,  fat-soluble vitamin deficiencies, hyperlipidemia, and eventually cirrhosis with portal hypertension. If unrecognized, the condition leads to liver failure—but not kernicterus, as the liver is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood–brain barrier. Initially, the symptoms are indistinguishable from neonatal jaundice, a common phenomenon. Symptoms are usually evident between one and six weeks after birth. Besides jaundice, other symptoms include pale stools, dark urine, swollen abdominal region and large hardened liver (which may or may not be observable by the naked eye). In some cases condition is correctable by surgery, while in majority of cases the atresia is not correctableand in such cases hepatic portoenterostomy (kasai procedure) or hepatic transplantation must be considered. Death is usually due to intercurrent infection, liver failure, and bleeding due to vitamin K deficiency or oesophageal varices. Cirrhosis and ascites are late complication appearing within 2 years of age.

Intrahepatic biliary atresia

Characterized by biliary hypoplasia so that there is paucity of bile ductsrather than there complete absence. The condition is probably due to viral infection acquired during intrauterine period or neonatal period. Cholestatic jaundice usually appears within the first few days of birth and is characterized by high serum bile acids with associated pruritus, and hypercholesterolaemia with appearance of xanthomas by first year of life. Hepatic as well as urinary copper concentrations are are elevated. In some cases, intrahepatic biliary atresia is related to α-1-antitrypsin deficiency.

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