Abstract
Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.
Highlights
Introduction to Biliary AtresiaBiliary atresia (BA) is a rare neonatal disease usually manifesting in the first months of life, when ascending obstruction of the biliary tree causes severe cholestasis and rapidly progressing biliary cirrhosis.1.1
In type I, atresia is limited to the common bile duct, and the gallbladder and hepatic ducts are patent (i.e., “distal” BA)
In the mouse model, genetic inactivation of hepatocyte nuclear factor 1 beta resulted in morphological anomalies in intrahepatic bile ducts and in the gallbladder [38], suggesting that mutations in genes that regulate hepatobiliary development may play a role in BA
Summary
Biliary atresia (BA) is a rare neonatal disease usually manifesting in the first months of life, when ascending obstruction of the biliary tree causes severe cholestasis and rapidly progressing biliary cirrhosis. It is plausible that a pre- or perinatal hepatobiliary viral infection might secondarily lead to an autoreactive T cell-mediated bile duct injury [28], whereby infection with a cholangiotropic virus results in initial bile duct epithelial damage, followed by persistent autoimmunemediated inflammation and injury to bile duct epithelia despite clearance of the virus Supporting this hypothesis, in murine models of BA, both cellular and humoral indicators of autoimmunity can be detected, and the progressive bile duct injury is due in part to a bile duct epithelia-specific T cell-mediated immune response [29]. Challenging the infectious hypothesis, a recent and extensive study failed to detect virus-specific sequences in liver samples from 74 BA patients analyzed by PCR, and the overall virus incidence of 42% failed to verify the hypothesis of a viral etiology of BA. In the mouse model, genetic inactivation of hepatocyte nuclear factor 1 beta resulted in morphological anomalies in intrahepatic bile ducts and in the gallbladder [38], suggesting that mutations in genes that regulate hepatobiliary development may play a role in BA
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