title: Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation.
abstract: This report describes a case of Abernethy malformation associated with hepatopulmonary syndrome, which was resolved after shunt ligation. The clinical course indicated that hepatopulmonary syndrome can develop in Abernethy malformation in which liver function and portal pressure is normal, and liver transplantation is not the exclusive therapy for hepatopulmonary syndrome. The levels of endotoxin and endothelin 1 in the shunt blood were high, whereas those of tumor necrosis factor alpha and interleukin-1beta were within reference range. Although pathogenesis of hepatopulmonary syndrome remains unknown, the findings in this case suggest that bacterial translocation as well as elevated endothelin 1 may play a causal role in development of hepatopulmonary syndrome.
authors.affiliation: Division of General Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan. morikawa-n@ncchd.go.jp
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Morikawa
authors.forename: Toshiro
authors.initials: T
authors.lastname: Honna
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Kitano
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Fuchimoto
authors.forename: Noriko
authors.initials: N
authors.lastname: Kawashima
authors.forename: Kazuteru
authors.initials: K
authors.lastname: Kawasaki
mesh_terms: D015415:Biomarkers / Q000032:analysis; D001784:Blood Gas Analysis; D002675:Child, Preschool; D000013:Congenital Abnormalities / Q000175:diagnosis / Q000601:surgery*; D019332:Endothelin-1 / Q000032:analysis / Q000378:metabolism*; D005500:Follow-Up Studies; D020065:Hepatopulmonary Syndrome / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008026:Ligation / Q000379:methods; D008297:Male; D011168:Portal System / Q000002:abnormalities*; D011169:Portal Vein / Q000002:abnormalities / Q000601:surgery*; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler
chemical_list: D015415:Biomarkers; D019332:Endothelin-1