Biliary atresia (BA) is a rare, life-threatening neonatal liver disease characterized by the progressive obliteration of the extrahepatic bile ducts, leading to cholestasis, liver fibrosis, and cirrhosis. The etiology of biliary atresia remains largely unknown, though it is hypothesized to involve genetic predisposition, viral infections, and immune-mediated mechanisms. Early diagnosis is critical for improving outcomes, with Kasai portoenterostomy being the primary surgical intervention aimed at restoring bile flow. Despite surgical efforts, many patients require liver transplantation due to progressive liver damage.

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Biliary Atresia

  • Megan Anderson Adams,
  • Dor Yoeli,
  • Elizabeth Anne Pomfret

摘要

Biliary atresia (BA) is a rare, life-threatening neonatal liver disease characterized by the progressive obliteration of the extrahepatic bile ducts, leading to cholestasis, liver fibrosis, and cirrhosis. The etiology of biliary atresia remains largely unknown, though it is hypothesized to involve genetic predisposition, viral infections, and immune-mediated mechanisms. Early diagnosis is critical for improving outcomes, with Kasai portoenterostomy being the primary surgical intervention aimed at restoring bile flow. Despite surgical efforts, many patients require liver transplantation due to progressive liver damage.