Biliary atresia (BA) is a rare disorder, occurring in approximately 1 in 10,000 to 20,000 births per year in the United States. Yet, it is the most frequent cause of end-stage liver disease in pediatric populations, the most common indication for liver transplant, and the most frequent cause of cholestasis in children younger than 2 months. Early identification and referral are key because the most important prognostic factor in BA is the age at which patients are treated with a hepatoporto-enterostomy or Kasai procedure, ideally, by 45 to 60 days after birth. Early surgical management may mitigate the need for liver transplant, although 80% of patients in recent studies require transplant later in life. This In Brief article reviews strategies for diagnosis and management, discusses subcategories and etiologic factors, and addresses management of postoperative patients with BA within primary care practices.

The initial diagnosis of BA is challenging because there...

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