Atresia, biliary

There is very little evidence relating to the role of ROMs in cholestatic liver disease. Serum selenium and glutathione peroxidase activity are decreased in humans with intrahepatic cholestasis of pregnancy (Kauppila et al., 1987). Low levels of vitamin E have been reported in patients with primary biliary cirrhosis, and in children with Alagille s syndrome or biliary atresia (Knight et al., 1986 Jeffrey etal., 1987 Lemonnier etal., 1987 Babin etal., 1988 Kaplan et al., 1988 Sokol etal., 1989). Serum levels of Mn-SOD are increased in patients with all stages of primary biliary cirrhosis compared with patients with other forms of chronic liver disease, although whether this causes or results from the disease process is unclear (Ono etal., 1991). 

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

Vitamin E, a fat-soluble vitamin, is used in vitamin E deficiency in premature infants and in patients with impaired fat absorption (including patients with cystic fibrosis) and in biliary atresia. Sources are eggs, vegetable oils, margarine, and mayonnaise nuts and seeds fortified cereals green leafy vegetables. 

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. 

Alcohol consumption is the leading cause of chronic liver disease in adults. Biliary atresia is a leading cause of chronic liver disease in children. 

A fasting ultrasound should show a full gallbladder, which would be emptied when the patient next ate a meal. If the fasted gallbladder is small and irregular bile may not be flowing into it from the liver, which may suggest obstruction or, in infants, biliary atresia. 

Pale stools are a sign of biliary obstruction. Normally, bile is secreted into the intestine, where the majority is converted to the faecal pigment stercobilin. If there is a biliary obstruction bile secretion is reduced and this conversion cannot take place, and so the stools do not have the usual coloration. Where there is complete obstruction, such as in biliary atresia, the stools may be white. 

Differential diagnosis of neonatal jaundice (below 10-12 mg/dl) vs. neonatal biliary atresia... 

Meunier JF, Goujard E, Dubousset AM, Samii K, Mazoit JX. Pharmacokinetics of bupivacaine after continuous epidural infusion in infants with and without biliary atresia. Anesthesiology 2001 95(l) 87-95. 

Late disease 2-12 weeks Intracranial, skin, gastrointestinal Mainly idiopathic, can be the presenting feature of an underlying disease (for example cystic fibrosis, ai-antitrypsin deficiency, biliary atresia there is often some degree of cholestasis... 

Oka Y et al. Manganese deposits in patients with biliary atresia after hepatic porto-enterostomy. J Pediatr Surg 2000 35 450-3. 

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. 

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. 

Intrahepatic biliary atresia is characterized by a paucity of intrahepatic bile ducts. Jaundice usually appears within the first few days of life. Serum bilirubin is elevated and serum cholesterol may be very high and lead to the formation of xanthomas. The hepatic histology is nonspecific, showing bile duct paucity, giant cells, inflammation, and fibrosis. Survival into adolescence is common, although growth is usually retarded. 

Treatment of intrahepatic biliary atresia is symptomatic, with intramuscular replacement of vitamins A, D, and E. Medium chain triglycerides that do not need bile acids for absorption provide calories in patients with partial atresia. Cholestyramine may relieve pruritus. Ursodeoxychofic acid reduces serum enzyme activities and refieves pruritus in some patients. 

Hussein M, Howard ER, Mieli-Vergani G, Mowat AP. Jaundice at 14 days of age exclude biliary atresia. 

Rocchi E, Gibertini P, Santunione V, Balli F, Ventura E. Faecal and urinary coproporphyrin isomers in biliary atresia and neonatal hepatitis. Ric Clin Lab 1980 10 509-10. 

It must be remembered that xanthelasma is associated with dyslipidemia in 50% of cases. It can also be symptomatic of hepatobiliary disorders (biliary atresia and biliary cirrhosis) when associated with cholesterol deposits that start to build up in the hands and feet before spreading. Monoclonal gammapathies have also been described in association with xanthelasma. 

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