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. Copyright © 2005 by the American Association for the Study of Liver Diseases.

Medici, V., Mirante, V.G., Fassati, L.R., Pompili, M., Forti, D., Del Gaudio, M., et al. (2005). Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders. LIVER TRANSPLANTATION, 11(9), 1056-1063 [10.1002/lt.20486].

Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders

ANGELICO, MARIO;TISONE, GIUSEPPE;
2005

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. Copyright © 2005 by the American Association for the Study of Liver Diseases.
Pubblicato
Rilevanza internazionale
Articolo
Sì, ma tipo non specificato
Settore MED/12 - Gastroenterologia
eng
Con Impact Factor ISI
antivirus agent; cyclosporin A; penicillamine; steroid; tacrolimus; trientine; zinc; adult; article; chronic liver disease; clinical article; controlled study; data analysis; disease severity; female; graft survival; health care facility; human; Italy; liver failure; liver graft rejection; liver transplantation; male; medical documentation; mental disease; neurologic disease; priority journal; retrospective study; statistical significance; survival rate; survival time; symptomatology; treatment failure; treatment indication; Wilson disease; Adult; Female; Hepatolenticular Degeneration; Humans; Liver Transplantation; Male; Mental Disorders; Nervous System Diseases; Retrospective Studies; Survival Analysis; Treatment Outcome
Medici, V., Mirante, V.G., Fassati, L.R., Pompili, M., Forti, D., Del Gaudio, M., et al. (2005). Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders. LIVER TRANSPLANTATION, 11(9), 1056-1063 [10.1002/lt.20486].
Medici, V; Mirante, V; Fassati, L; Pompili, M; Forti, D; Del Gaudio, M; Trevisan, C; Cillo, U; S. t. u. r. n. i. o. l. o., G; Fagiuoli, S; Andriulli, A; Angelico, M; Aresu, G; Burra, P; Caccamo, L; Castagneto, M; D'Amico, Df; Dardano, G; Filla, A; Gasbarrini, A; Gasbarrini, G; Gianni, S; Grazi, Gl; Martines, D; Marzano, A; Melada, E; Nardo, B; Pevere, S; Rapaccini, Gl; Rizzetto, M; Rondinara, G; Salizzoni, M; Slim A. O., Sm; Tisone, G; Valente U., Zg
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2108/29747
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