Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis

Yohei Yamada, Ken Hoshino, Naoki Shimojima, Masahiro Shinoda, Hideaki Obara, Shigeyuki Kawachi, Yasushi Fuchimoto, Minoru Tanabe, Yuko Kitagawa, Yasuhide Morikawa

研究成果: Article査読

3 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)e49-e53
ジャーナルPediatric Transplantation
14
5
DOI
出版ステータスPublished - 2010 8月 1

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康
  • 移植

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