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

Medici, Valentina; Mirante, V.G.; Fassati, L. R.; Pompili, Maurizio; Forti, D; Gaudio, Massimo Del; Trevisan, Carlo P.; Cillo, Umberto; Sturniolo, Giacomo Carlo; Fagiuoli, Stefano

doi:10.1002/lt.20486

Cited by 149 publications

(104 citation statements)

References 21 publications

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“…However this remains a matter of controversy in cases of neurological worsening without liver failure, despite reports of 70% of these patients improving after transplantation [20]. In our experience, after a mean follow-up of five years, 12 of 16 patients (75%) improved when transplanted for neurologic worsening.…”

Section: Wilson's Disease (Wd)mentioning

confidence: 62%

Bioavailable Trace Metals in Neurological Diseases

Poujois

Devedjian

Moreau

et al. 2016

Curr Treat Options Neurol

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Opinion statement  Medical treatment in Wilson's disease includes chelators (D-Penicillamine andTrientine) or zinc salts that have to be maintain all the lifelong. This pharmacological treatment is categorized into two phases; the first being a decoppering phase and the second a maintenance's one. The best therapeutic approach remains controversial, as only a few non-controlled trials have compared these treatments. During the initial phase, progressive increase of chelators' doses adjusted to exchangeable copper and urinary copper might help to avoid neurological deterioration. Liver transplantation is indicated in acute fulminant liver failure and decompensated cirrhosis; in cases of neurologic deterioration, it must be individually discussed. During the maintenance phase, the most important challenge is to obtain a good adherence to lifelong medical therapy. Neurodegenerative diseases that lead to a mislocalization of iron can be caused by a culmination of localized overload (pro-oxidant siderosis) and localized deficiency (metabolic distress). A new therapeutic concept with conservative iron chelation rescues iron-overloaded neurons by scavenging labile iron and, by delivering this chelated metal to endogenous apotransferrin, allows iron redistribution to avoid systemic loss of iron.

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Section: Wilson's Disease (Wd)mentioning

confidence: 62%

Bioavailable Trace Metals in Neurological Diseases

Poujois

Devedjian

Moreau

et al. 2016

Curr Treat Options Neurol

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“…However, OLT in the case of severe neurological impairment is still open to debate. 4 One shortcoming of APOLT for cirrhotic liver disease is the potential risk for carcinogenicity of the remnant native liver. This problem remains to be resolved.…”

Section: Discussionmentioning

confidence: 99%

Auxiliary Partial Orthotopic Living Donor Liver Transplantation in a Patient With Wilson's Disease: A Case Report

Park

Kim

Wang

et al. 2008

Transplantation Proceedings

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“…Liver transplantation is the recommended therapy for patients with fulminant hepatitis, or in those with relentless progression of hepatic dysfunction despite drug therapy, and survival rates are only very slightly inferior to those after transplant for other indications [103][104][105] . Liver transplantation corrects the underlying hepatic metabolic defect in WD [106] , and is one of the few indications for liver transplantation in which there is no risk of recurrence, unless in the unfortunate and improbable hypothesis of receiving a graft from an undiagnosed WD.…”

Section: Liver Transplantationmentioning

confidence: 99%

Wilson’s disease: A review of what we have learned

Rodríguez–Castro

Hevia‐Urrutia

Sturniolo

2015

WJH

136

123

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Wilson's disease (WD), which results from the defective ATP7B protein product, is characterized by impaired copper metabolism and its clinical consequences vary from an asymptomatic state to fulminant hepatic failure, chronic liver disease with or without cirrhosis, neurological, and psychiatric manifestations. A high grade of suspicion is warranted to not miss cases of WD, especially less florid cases with only mild elevation of transaminases, or isolated neuropsychiatric involvement. Screening in first and second relatives of index cases is mandatory, and treatment must commence upon establishment of diagnosis. Treatment strategies include chelators such as D-penicillamine and trientine, while zinc salts act as inductors of methallothioneins, which favor a negative copper balance and a reduction of free plasmatic copper. As an orphan disease, research is lacking in this field, especially regarding therapeutic strategies which are associated with better patient compliance and which could eventually also reverse established injury.

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

Cited by 149 publications

References 21 publications

Bioavailable Trace Metals in Neurological Diseases

Bioavailable Trace Metals in Neurological Diseases

Auxiliary Partial Orthotopic Living Donor Liver Transplantation in a Patient With Wilson's Disease: A Case Report

Wilson’s disease: A review of what we have learned

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