医学
流行病学
粘多糖病
儿科
加强流行病学观察研究报告
人口
荟萃分析
梅德林
检查表
公共卫生
观察研究
内科学
病理
环境卫生
生物
古生物学
生物化学
作者
Weijing Kong,Shanshan Wu,Jing Zhang,Long Cheng,Ying‐Xue Ding,Yan Meng
标识
DOI:10.1515/jpem-2020-0742
摘要
Mucopolysaccharidosis III, an autosomal recessive lysosomal storage disorder, is characterized by progressive mental retardation and behavioral problems. Meta-analysis of global mucopolysaccharidosis III epidemiology, which serves as a fundamental reference for public health decision-making, was not available prior to this study. To provide a systematic review and meta-analysis of birth prevalence of mucopolysaccharidosis III in multiple countries.MEDLINE and EMBASE databases were searched for original research articles on the epidemiology of mucopolysaccharidosis III from inception until 1st July, 2020. A checklist adapted from STROBE (STrengthening the Reporting of OBservational studies in Epidemiology) was used to assess the quality of all studies involved. Meta-analysis, adopting a random effects logistic model, was performed to estimate pooled birth prevalence of mucopolysaccharidosis III and its subtypes.Twenty-five studies screened out of 1,826 records were included for data extraction. The pooled global mucopolysaccharidosis III birth prevalence was 0.76 cases (95% CI: 0.57-0.96) per 100,000 live births. The pooled global birth prevalence of mucopolysaccharidosis III subtypes (A, B, and C) was 0.52 cases (95% CI: 0.33-0.72), 0.21 cases (95% CI: 0.12-0.30) and 0.01 cases (95% CI: 0.005-0.02) per 100,000 live births, respectively.Based on the global population size (7.8 billion) and the life span of patients, there would be 12-19 thousand mucopolysaccharidosis III patients worldwide. To our knowledge, this is the first comprehensive systematic review that presented quantitative data fundamental for evidence-based public health decision-making by evaluating global epidemiology of mucopolysaccharidosis III.
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