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Outcomes of Liver Transplant for Adults With Wilson’s Disease
Author(s) -
Ferrarese Alberto,
Morelli Maria Cristina,
Carrai Paola,
Milana Martina,
Angelico Mario,
Perricone Giovanni,
Belli Luca Saverio,
Marrone Giuseppe,
Grieco Antonio,
Martini Silvia,
Manini Matteo Angelo,
Fagiuoli Stefano,
Toniutto Pierluigi,
Galeota Lanza Alfonso,
Bhoori Sherrie,
Petta Salvatore,
Giannini Edoardo G.,
Burra Patrizia
Publication year - 2020
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.25714
Subject(s) - medicine , liver transplantation , disease , liver disease , retrospective cohort study , observational study , stage (stratigraphy) , gastroenterology , transplantation , surgery , pediatrics , paleontology , biology
Wilson’s disease (WD) is a rare genetic disorder with protean manifestations. Even if liver transplantation (LT) could represent an effective therapeutic option for patients with end‐stage liver disease, it has remained controversial in the presence of neuropsychiatric involvement. This study aimed to examine the frequency of adult LT for WD in Italy, focusing on the disease phenotype at the time of LT. A retrospective, observational, multicenter study was conducted across Italy exploring the frequency and characteristics of adults transplanted for WD between 2006 and 2016. A total of 29 adult WD patients underwent LT during the study period at 11 Italian LT centers (accounting for 0.4% of all LTs performed), and 27 of them were considered in this analysis (male/female, n = 9/18; age at LT, 29 years [19‐60 years]; median Model for End‐Stage Liver Disease score at LT, 27 [6‐49]). Isolated hepatic phenotype was the indication for LT in 17 (63%) patients, whereas 2 (7%) patients underwent LT for neurological impairment on compensated liver disease. Overall 1‐ and 5‐year patient survival was excellent (88% and 83%, respectively). Neuropsychiatric symptoms early after LT completely recovered in only a few patients. In conclusion, WD remains an uncommon, unusual indication for LT in Italy, displaying good post‐LT graft and patient survival. Because isolated neuropsychiatric involvement represents a rare indication to LT, more data are needed to properly assess the value of LT for WD in this subset of patients.