title: Embryonic biliary atresia in a very-low-birth-weight premature infant.
abstract: Two major forms of biliary atresia, the embryonic and perinatal type, are considered to have different pathogeneses and distinct prognoses. Embryonic biliary atresia is associated with worse prognosis. We report a case of embryonic biliary atresia in a preterm male infant of 31 weeks of gestation and weighing 1375 g, with the initial manifestation of intermittent acholic stool 5 days after birth. Kasai portoenterostomy was per-formed at the age of 51 days when he weighed 2164 g. Nevertheless, poorly restored bile flow and progressive cholestasis led to early liver transplantation at the age of 8 months. Liver function had recovered to normal levels by the age of 12 months. Diagnosis of biliary atresia in preterm infants is difficult and requires a high index of suspicion and careful workup. This case illustrates the poor outcome of embryonic biliary atresia and that early liver transplantation may be necessary to improve the prognosis.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
authors.forename: Hung-Wen
authors.initials: HW
authors.lastname: Chen
authors.forename: Wen-Ming
authors.initials: WM
authors.lastname: Hsu
authors.forename: Mei-Hwei
authors.initials: MH
authors.lastname: Chang
authors.forename: Chien-Yi
authors.initials: CY
authors.lastname: Chen
authors.forename: Hung-Chieh
authors.initials: HC
authors.lastname: Chou
authors.forename: Po-Nien
authors.initials: PN
authors.lastname: Tsao
authors.forename: Wu-Shiun
authors.initials: WS
authors.lastname: Hsieh
mesh_terms: D001656:Biliary Atresia / Q000196:embryology* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D019102:Infant, Very Low Birth Weight; D016031:Liver Transplantation; D008297:Male