Biliary atresia, liver transplantation

Unlabeled Uses Prophylaxis of liver transplant rejection, treatment of alcoholic cirrhosis, biliary atresia, chronic hepatitis, gallstone formation, sclerosing cholangitis... 

Baerg J, Zuppan C, Klooster M. Biliary atresia - a fifteen-year review of clinical and pathologic factors associated with liver transplantation. J Pediatr Surg 2004 39 800-803. 

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 a case report, a 14-month-old girl with biliary atresia who had an orthotopic liver transplant was diagnosed with TAC-associated PRES with leptomeningeal enhancement 17days later. The patient improved after TAC was discontinued [59 ]. 

The conditions for which hepatic transplantation has been applied differ in pediatric and adult cases and are shown in Tables 1 and 2. 50% of the pediatric cases have been transplanted for biliary atresia.The majority of the other pediatric recipients have been transplanted for alpha 1 antitrypsin deficiency or chronic liver disease of unknown ethiology. In contrast, the leading conditions for which hepatic transplantation has been applied in adult recipients are postnecrotic cirrhosis, primary biliary cirrhosis and primary hepatic malignancy. 

Falchetti D, de Carvalho FB, Clapuyt P, de Ville de Goyet, de Hemptinne B, Claus D, Otte JB (1991) Liver transplantation in children with biliary atresia and polysplenia syndrome. J Pediatr Surg 5 528-531 Gonzalez et al. (1993)... 

---