title: Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis.
abstract: Idiopathic HES is characterlized by prolonged eosinophilia without an identifiable underlying cause and multiple-organ dysfunction. We report a case of a LDLT for a 12-yr-old Japanese girl with BA accompanied by HES. Histological examination of the resected liver showed biliary cirrhosis with dense eosinophilic infiltration of portal tracts and the lobules of the liver. She developed portal vein thrombosis on post-operative day 10 and the histopathological findings of the thrombus revealed dense eosinophilic deposition, suggesting that HES might have influenced the formation of this thrombus. Liver graft biopsies also demonstrated the presence of activated eosinophilils with biliary damage. Blood chemistry findings suggested liver dysfunction as a result of the eosinophilic infiltrations. Prednisolone treatment improved the liver dysfunction. Four years after LDLT, she remains clinically well on prednisolone at 0.3 mg/kg/day, with an eosinophil count ranging from 10 to 15%. A literature review has not shown any previous reports of HES with BA. This case demonstrates the possibility of an association between eosinophilic infiltration and liver dysfunction during follow-up for BA and after LDLT.
authors.affiliation: Department of Surgery, Keio University School of Medicine, Shinano-machi, Shinjuku-ku, Tokyo, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Yamada
authors.forename: Ken
authors.initials: K
authors.lastname: Hoshino
authors.forename: Naoki
authors.initials: N
authors.lastname: Shimojima
authors.forename: Masahiro
authors.initials: M
authors.lastname: Shinoda
authors.forename: Hideaki
authors.initials: H
authors.lastname: Obara
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Kawachi
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Fuchimoto
authors.forename: Minoru
authors.initials: M
authors.lastname: Tanabe
authors.forename: Yuko
authors.initials: Y
authors.lastname: Kitagawa
authors.forename: Yasuhide
authors.initials: Y
authors.lastname: Morikawa
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D002648:Child; D005260:Female; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications*; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects; D011169:Portal Vein; D017131:Thrombectomy; D020246:Venous Thrombosis / Q000209:etiology* / Q000601:surgery