Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders.
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.
Medici
Valentina
V
Department of Surgical and Gastroenterological Sciences, Gastroenterology and Liver Transplant Sections, University of Padua, Padua, Italy.
Mirante
Vincenzo G
VG
Fassati
Luigi R
LR
Pompili
Maurizio
M
Forti
Domenico
D
Del Gaudio
Massimo
M
Trevisan
Carlo P
CP
Cillo
Umberto
U
Sturniolo
Giacomo C
GC
Fagiuoli
Stefano
S
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