TY - JOUR
T1 - Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis
AU - Yamada, Yohei
AU - Hoshino, Ken
AU - Shimojima, Naoki
AU - Shinoda, Masahiro
AU - Obara, Hideaki
AU - Kawachi, Shigeyuki
AU - Fuchimoto, Yasushi
AU - Tanabe, Minoru
AU - Kitagawa, Yuko
AU - Morikawa, Yasuhide
PY - 2010/8/1
Y1 - 2010/8/1
N2 - 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.
AB - 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.
KW - biliary atresia
KW - hypereosinophilic syndrome
KW - liver transplantation
KW - portal vein thrombosis
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U2 - 10.1111/j.1399-3046.2009.01170.x
DO - 10.1111/j.1399-3046.2009.01170.x
M3 - Article
C2 - 20042017
AN - SCOPUS:77954464054
SN - 1397-3142
VL - 14
SP - e49-e53
JO - Pediatric Transplantation
JF - Pediatric Transplantation
IS - 5
ER -