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<p>Biliary atresia is a&#xa0;rare disease. The prevalence in Europe is ca. 1:17,000 live births [1, 2], much lower than in Asia where the incidence is 1:5000 live births [3]. The cause appears to have a&#xa0;multifactorial genesis including genetic predisposition and a&#xa0;virus-triggered excessive immune response. Those affected clinically present with prolonged neonatal jaundice, acholic stools, failure to thrive or symptoms of a vitamin&#xa0;K deficiency. Since 1965 Kasai portoenterostomy has been an established surgical treatment [4]. Biliary atresia is the most common indication for pediatric liver transplantation (LTx) worldwide. An important risk factor for LTx is the age of the patient at the time of Kasai portoenterostomy: the older the child, the more advanced is the liver remodelling and the more likely that the patients will require an LTx later on. Postoperatively, cholangitis is a&#xa0;frequent complication, which can present as cholestasis and fever.</p>

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Gallengangatresie

  • 期刊论文

  • 发表日期：

    2026年3月10日

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  • Nagoud Schukfeh,
  • Eva-Doreen Pfister

摘要

Biliary atresia is a rare disease. The prevalence in Europe is ca. 1:17,000 live births [1, 2], much lower than in Asia where the incidence is 1:5000 live births [3]. The cause appears to have a multifactorial genesis including genetic predisposition and a virus-triggered excessive immune response. Those affected clinically present with prolonged neonatal jaundice, acholic stools, failure to thrive or symptoms of a vitamin K deficiency. Since 1965 Kasai portoenterostomy has been an established surgical treatment [4]. Biliary atresia is the most common indication for pediatric liver transplantation (LTx) worldwide. An important risk factor for LTx is the age of the patient at the time of Kasai portoenterostomy: the older the child, the more advanced is the liver remodelling and the more likely that the patients will require an LTx later on. Postoperatively, cholangitis is a frequent complication, which can present as cholestasis and fever.