title: Successful treatment of fulminant Wilson's disease without liver transplantation.
abstract: Fulminant Wilson's disease (WD) is life-threatening. The revised WD prognostic index (RWPI) has been used to predict the severity of the disease, with a score ≥11 indicating fatal outcome without liver transplantation (LTx). We here report the case of a 10-year-old female patient with fulminant WD (RWPI, 16) who recovered fully after plasma exchange and continuous hemodiafiltration, followed by treatment with copper chelate agents. To the best of our knowledge, there have been five fulminant WD patients with RWPI ≥ 11 including the present patient, in whom LTx was not done. Based on the therapeutic modalities in these five cases, non-surgical treatment (blood purification and copper chelate agents) may be able to avoid LTx in fulminant WD even with very high RWPI, although preparation for LTx is necessary.
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Mitsuo
authors.initials: M
authors.lastname: Motobayashi
authors.forename: Tetsuhiro
authors.initials: T
authors.lastname: Fukuyama
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Nakayama
authors.forename: Kenji
authors.initials: K
authors.lastname: Sano
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Noda
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Hidaka
authors.forename: Yoshiro
authors.initials: Y
authors.lastname: Amano
authors.forename: Shu-Ichi
authors.initials: S
authors.lastname: Ikeda
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
authors.forename: Yuji
authors.initials: Y
authors.lastname: Inaba
mesh_terms: D002648:Child; D005260:Female; D006438:Hemodilution; D006527:Hepatolenticular Degeneration / Q000628:therapy*; D006801:Humans; D010951:Plasma Exchange
keywords: continuous hemodiafiltration; fulminant Wilson's disease; liver transplantation; plasma exchange; revised Wilson's disease prognostic index