Extrahepatic biliary atresia

Extrahepatic biliary atresia may involve all or part of the extrahepatic bifiary tree. The gallbladder is usually absent. Involvement of the hepatic or common duct leads to the characteristic syndrome of severe cholestatic jaundice. It occurs in 1 in 10,000 births, with females more commonly affected than males. Jaundice and pruritus usually appear in the first week. Stools are pale and the urine is tea colored. Jaundice is deep, but the aminotransferases are only mildly elevated. If jaundice persists beyond 14 days of age, a direct or conjugated bilirubin measurement must be performed to exclude biliary atresia. If it is elevated, the urine should be tested for bile and the stool color inspected if the color is not green or yellow, biliary atresia is likely. Early identification of this condition is essential if these infants are to benefit fi"om the operation of portoenterostomy, which should be performed no later than 60 days after birth. If portoenterostomy is not successful, liver transplantation is the treatment of choice. Children rarely live beyond 3 years unless the lesion is surgically correctable. 

In 1971, Makino et al. found that considerable amounts of 3jS-hydroxychol-5-en-24-oic acid were excreted in urine of children with extrahepatic biliary atresia [111]. Since then, the unsaturated C24 bile acid has been identified in human meconium [112,113], amniotic fluid [114,115], gallbladder bile from premature and term infants [116], urine from children and adults, both healthy and with liver disease [82,117], and bile and feces from newborn and fetal guinea pigs [118]. The natural occurrence of 3j8-hydroxychol-5-en-24-oic acid suggests that the side chain of cholesterol is degraded before modification of the steroid ring system (Chapter 9). 

Bayliss EA, Flambidge KM, Sokol RJ, et al. Hepatic concentrations of zinc, copper and manganese in infants with extrahepatic biliary atresia J Trace Elem Med Biol 1995 9 40-43. 

Cholestatic Extrahepatic biliary atresia Primary sclerosing cholangitis ... 

Biliary atresia is a heterogeneous group of acquired disorders that involve either the extrahepatic or intrahepatic bile ducts. Possible etiologies include cytomegalovirus, reovirus III, Epstein-Barr virus, rubella virus, tti-antitrypsin deficiency, Down syndrome, and trisomy 17 or 18. 

Conjugated hyperbilimbinemia is rare during the neonatal period. It can result from impaired hepatocellular function or extrahepatic obstmction. Hepatocellular defects can be caused by bacterial, viral, or parasitic infections, cystic fibrosis, a -antitrypsin deficiency, Dubin-Johnson and Rotor s syndromes, and other genetic disease. Extrahepatic obstruction can be congenital (biliary atresia) or acquired. 

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