A case of living related liver transplantation for Wilsonian fulminant hepatitis

We reported a 13-year-old girl with Wilsonian fulminant hepatitis. The diagnosis was suspected clinically and was later confirmed by chemical and pathologic studies. She was admitted to our hospital with acute hepatic failure and severe hemolysis. Plasmapheresis as will as oral D-penicillamine intak...

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Published inKanzo Vol. 37; no. 4; pp. 244 - 249
Main Authors INUI, Ayano, OHKAWA, Takashi, FUJISAWA, Tomoo, KOMATSU, Haruki, MIYAGAWA, Yoshihiro, UEMOTO, Shinji, TANAKA, Koichi, INOMATA, Yukihiro
Format Journal Article
LanguageJapanese
Published The Japan Society of Hepatology 1996
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ISSN0451-4203
1881-3593
1881-3593
DOI10.2957/kanzo.37.244

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Summary:We reported a 13-year-old girl with Wilsonian fulminant hepatitis. The diagnosis was suspected clinically and was later confirmed by chemical and pathologic studies. She was admitted to our hospital with acute hepatic failure and severe hemolysis. Plasmapheresis as will as oral D-penicillamine intake did not show any improvement. On the 94th day, the patient received successful living related liver transplantation (LRLT). Donor was her mother. The patients who present severe intravascular hemolysis as a initial manifestation of Wilson's disease have very poor prognosis, because almost all these patiemts are in the state of decompensated liver cirrhosis and chelating agents such as D-penicillamine are not effective. In foreign countries, liver transplantation is indicated for such cases and has been able to rescue many patients In conclusion, LRLT is a effective therapy for Wilsonian fuluminant hepatitis in Japan.
ISSN:0451-4203
1881-3593
1881-3593
DOI:10.2957/kanzo.37.244