title: Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders.
abstract: A retrospective data analysis on liver transplantation for Wilson's disease (WD) was performed among Italian Liver Transplant Centers. Thirty-seven cases were identified. The main indication for liver transplantation was chronic advanced liver disease in 78% of patients. Mixed hepatic and neuropsychiatric symptoms were recorded in 32.3%. Eight patients presented with fulminant liver failure; 44.8% were on medical treatment. Patient and graft survival at 3 months, 12 months, 3 years, 5 years, and 10 years after transplantation were, respectively, 91.8%, 89.1%, 82.9%, 75.6%, and 58.8%, and 85.3%, 83.0%, 77.1%, 70.3%, and 47.2%. Neurological symptoms significantly improved after orthotopic liver transplantation (OLT), but the survival of patients with mixed hepatic and neuropsychiatric involvement was significantly lower than in patients with liver disease alone (P = 0.04). WD characterized by hepatic involvement alone is a rare but good indication for liver transplantation when specific medical therapy fails. Patients with neuropsychiatric signs have a significantly shorter survival even though liver transplantation has a positive impact on neurological symptoms. In conclusion, a combination of hepatic and neuropsychiatric conditions deserves careful neurological evaluation, which should contraindicate OLT in case of severe neurological impairment.
authors.affiliation: Department of Surgical and Gastroenterological Sciences, Gastroenterology and Liver Transplant Sections, University of Padua, Padua, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Medici
authors.forename: Vincenzo G
authors.initials: VG
authors.lastname: Mirante
authors.forename: Luigi R
authors.initials: LR
authors.lastname: Fassati
authors.forename: Maurizio
authors.initials: M
authors.lastname: Pompili
authors.forename: Domenico
authors.initials: D
authors.lastname: Forti
authors.forename: Massimo
authors.initials: M
authors.lastname: Del Gaudio
authors.forename: Carlo P
authors.initials: CP
authors.lastname: Trevisan
authors.forename: Umberto
authors.initials: U
authors.lastname: Cillo
authors.forename: Giacomo C
authors.initials: GC
authors.lastname: Sturniolo
authors.forename: Stefano
authors.initials: S
authors.lastname: Fagiuoli
mesh_terms: D000328:Adult; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D001523:Mental Disorders / Q000209:etiology*; D009422:Nervous System Diseases / Q000209:etiology*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome