Abstract
Biliary atresia (BA) is a fibrosclerosing cholangiopathy resulting in obstruction of the biliary tree. Diagnosis and early performance of the Kasai hepatic portoenterostomy improves outcomes.1 Current guidelines recommend measurement of direct bilirubin (DB) or conjugated bilirubin levels in neonates who are still jaundiced at age 2 to 3 weeks.2,3 However, recent studies have documented the elevation of DB or conjugated bilirubin levels within the first days after birth in neonates with BA,4,5 suggesting that BA is a developmental cholangiopathy associated with a genetic, prenatal, or developmental event. To test this hypothesis, this case series evaluated DB levels in the first days after birth among neonates who were subsequently diagnosed with BA.
Highlights
Biliary atresia (BA) is a fibrosclerosing cholangiopathy resulting in obstruction of the biliary tree
We identified 8 neonates with BA (6 female; 2 male; gestational age, 34-41 weeks), all of whom had a direct bilirubin (DB) level higher than 1.3 mg/dL within the first week (Figure)
Our findings confirmed published data documenting that neonates with BA manifest direct hyperbilirubinemia within the first week of life and that DB levels increase over time
Summary
Biliary atresia (BA) is a fibrosclerosing cholangiopathy resulting in obstruction of the biliary tree. Diagnosis and early performance of the Kasai hepatic portoenterostomy improves outcomes.[1] Current guidelines recommend measurement of direct bilirubin (DB) or conjugated bilirubin levels in neonates who are still jaundiced at age 2 to 3 weeks.[2,3] recent studies have documented the elevation of DB or conjugated bilirubin levels within the first days after birth in neonates with BA,[4,5] suggesting that BA is a developmental cholangiopathy associated with a genetic, prenatal, or developmental event. This case series evaluated DB levels in the first days after birth among neonates who were subsequently diagnosed with BA.
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