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Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta-analysis of controlled studies

Appenzeller-Herzog, Christian and Mathes, Tim and Heeres, Marlies L. S. and Weiss, Karl Heinz and Houwen, Roderick H. J. and Ewald, Hannah. (2019) Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta-analysis of controlled studies. Liver international, 39 (11). pp. 2136-2152.

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Abstract

BACKGROUND & AIMS: Wilson disease (WD) is a rare disorder of copper metabolism. The objective of this systematic review is to determine the comparative effectiveness and safety of common treatments of WD. METHODS: We included WD patients of any age or stage and the study drugs D-penicillamine, zinc salts, trientine, and tetrathiomolybdate. The control could be placebo, no treatment, or any other treatment. We included prospective, retrospective, randomized, and non-randomized studies. We searched Medline and Embase via Ovid, the Cochrane Central Register of Controlled Trials, and screened reference lists of included articles. Where possible, we applied random-effects meta-analyses. RESULTS: The 23 included studies reported on 2055 patients and mostly compared D-penicillamine to no treatment, zinc, trientine, or succimer. One study compared tetrathiomolybdate and trientine. Post-decoppering maintenance therapy was addressed in one study only. Eleven of 23 studies were of low quality. When compared to no treatment, D-penicillamine was associated with a lower mortality (odds ratio 0.013; 95% CI 0.0010 to 0.17). When compared to zinc, there was no association with mortality (odds ratio 0.73; 95% CI 0.16 to 3.40) and prevention or amelioration of clinical symptoms (odds ratio 0.84; 95% CI 0.48 to 1.48). Conversely, D-penicillamine may have a greater impact on side effects and treatment discontinuations than zinc. CONCLUSIONS: There are some indications that zinc is safer than D-penicillamine therapy while being similarly effective in preventing or reducing hepatic or neurologic WD symptoms. Study quality was low warranting cautious interpretation of our findings.
Faculties and Departments:03 Faculty of Medicine > Departement Klinische Forschung > Clinical Epidemiology and Biostatistics CEB
10 Zentrale universitäre Einrichtungen > Universitätsbibliothek
UniBasel Contributors:Appenzeller-Herzog, Christian and Ewald, Hannah
Item Type:Article, refereed
Article Subtype:Research Article
Publisher:Wiley
ISSN:1478-3223
e-ISSN:1478-3231
Note:Publication type according to Uni Basel Research Database: Journal article
Language:English
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Last Modified:11 Nov 2019 10:26
Deposited On:11 Nov 2019 10:26

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