Journal
FRONTIERS IN PHARMACOLOGY
Volume 13, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fphar.2022.847436
Keywords
Wilson disease; symptomatic; pharmacological therapy; meta-analysis; systematic review
Categories
Funding
- Key Medical Professional Development Plan of Beijing Hospital Management Center (Beijing, China) [ZYLX202125]
- Digestive Medical Coordinated Development Center of Beijing Municipal Administration of Hospitals (Beijing, China) [XXZ0503]
- Beijing Municipal Natural Science Foundation (Beijing, China) [7202068]
- National Science and Technology Major Project during the Thirteenth five-year Plan period [2017ZX10202203-006, 2017ZX10302201-004]
- Beijing Advanced Innovation Center for Big Data-Based Precision Medicine [1212040205]
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This study evaluated the efficacy and safety of common treatment regimens in patients with Wilson disease. The results showed that symptomatic patients treated with d-penicillamine had a higher incidence of adverse effects and neurological deterioration compared to those treated with zinc salts. The therapeutic effectiveness of these two regimens did not appear to be significantly different.
Background: Pharmacological therapy is currently the main treatment method for patients with Wilson disease (WD). We aimed to evaluate the efficacy and safety of the common treatment regimens in these patients.& nbsp;Methods: We conducted a systemic review and meta-analysis by searching multiple databases for studies from inception to October 2021. Outcomes of interest were the improved rate and safety of d-penicillamine and zinc salts treatment in WD patients. Two independent reviewers performed the study selection and data extraction.& nbsp;Results: Sixteen studies were included in this meta-analysis. The pooled improved rate for all included symptomatic WD patients was 78.0% (95% CI: 70.8%-85.2%). In symptomatic hepatic WD patients, there is no difference in the treatment efficiency of d-penicillamine and zinc salts (RR: 0.98, 95% CI: 0.86%-1.12%; p = 0.765). In neurological WD patients, the pooled improved rate of those who received d-penicillamine and zinc salts was 56.3% (95% CI: 37.5%-75.1%) and 80.2% (95% CI: 67.2%-93.2%), respectively. The incidence of adverse effects (RR: 2.42, 95% CI: 1.20%-4.88%; p = 0.014) and neurological deterioration (RR: 1.96, 95% CI: 1.31%-2.93%; p = 0.001) in all symptomatic WD patients treated with d-penicillamine was both higher than that of patients treated with zinc salts.& nbsp;Conclusion: Our analysis suggests that symptomatic WD patients treated with d-penicillamine have higher incidence of adverse effects and neurological deterioration than that of zinc salts. The therapeutic effectiveness of these two regimens does not seem to be significantly different, and these results must be interpreted with caution.& nbsp;
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