Intrahepatic biliary atresia

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. 

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). 

However, the intrahepatic bile-duct system is indeed still immature at birth, so that the final development of the smallest ramifications takes place during the first few neonatal weeks. At this stage, the intrahepatic biliary system is therefore very susceptible to noxae, which can lead to paucity or even atresia of the bile ducts. 

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. 

---