Genetics

Progress in biomarkers related to biliary atresia

Biliary atresia (BA) is a severe neonatal liver disease characterized by inflammatory and fibrotic obliteration of intrahepatic and extrahepatic bile ducts. This condition often leads to neonatal jaundice, cirrhosis, and ...

Diseases, Conditions, Syndromes

Scientists identify genes associated with biliary atresia survival

Scientists at Cincinnati Children's Hospital Medical Center have identified an expression pattern of 14 genes at the time of diagnosis that predicts two year, transplant-free survival in children with biliary atresia—the ...

Diseases, Conditions, Syndromes

First genetic clue for elusive pediatric liver disease

A nationwide consortium of researchers has identified the first genetic defect linked to biliary atresia, a mysterious liver disease that is the leading cause for liver transplantation in children.

Medical research

Scientists discover how the liver unclogs itself

A multi-disciplinary team of researchers from the Mechanobiology Institute, Singapore (MBI) at the National University of Singapore (NUS), the Institute of Bioengineering and Nanotechnology (IBN) of A*STAR, and BioSyM, Singapore-MIT ...

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Biliary atresia, also known as "extrahepatic ductopenia" 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. As a birth defect in newborn infants, it has an occurrence of 1/10,000 to 1/15,000 cases in live births in the United States. In the congenital form, the common bile duct between the liver and the small intestine is blocked or absent. 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.

Infants and children with biliary atresia have progressive cholestasis with all the usual concomitant features: pruritus, malabsorption with growth retardation, 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. The cause of the condition is unknown. The only effective treatments are certain surgeries such as the kasai procedure, or liver transplantation.

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