■本系统评价的目的是调查世界各地眼皮肤白化病(OCA)流行病学的可用数据,并确定一个可推广的,可以提出世界范围的患病率数字。
■进行了广泛的文献检索策略,审问PubMed,Scopus,和WebofScience,查找相关文献。最终包括34项报告原始数据的研究进行分析。
■研究结果表明,大多数数据已经过时,在2010年之后,34篇文章中只有6篇(18%)发表。很少有好的研究有合理的方法和大,明确定义的人口样本。世界上只有一小部分国家(26/193[13%])提供了OCA的患病率数据。按大陆,非洲研究的比例不成比例(15/34[44%])。患病率最高(范围,1/22到1/1300;平均,在人口分离株中报告了464个中的1个)。四个非洲国家的平均患病率为4264人中的1人(范围,1755年的1到7900年的1)。欧洲三个国家的患病率(平均值,1/12,000;范围,1/10,000到1/15,000)可能被低估,作为表型,在皮肤白皙的人群中,可能漏诊或误诊为眼白化病或孤立性视力障碍。人口比率可能因当地文化因素而异(例如,近亲交配),可能会随着时间的推移而改变。
■OCA的患病率在各大洲和人群之间差异很大,它经常受到当地因素的影响。这是不可能的,因此,为了确定一个,OCA的可推广的全球患病率,尽管非洲大陆和欧洲的汇率是有用的。
UNASSIGNED: The aim of this systematic review was to investigate the available data on the epidemiology of oculocutaneous albinism (OCA) around the world, and to determine whether a generalizable, worldwide prevalence figure could be proposed.
UNASSIGNED: Extensive literature search strategies were conducted, interrogating PubMed, Scopus, and Web of Science, to locate relevant literature. Ultimately 34 studies reporting original data were included for analysis.
UNASSIGNED: Findings showed that most data were outdated, and only 6 of 34 articles (18%) were published after 2010. There were few good studies with sound methodology and large, clearly defined population samples. Only a small proportion of countries worldwide (26/193 [13%]) have produced prevalence figures for OCA. By continent, African studies were disproportionately represented (15/34 [44%]). The highest prevalence rates (range, 1 in 22 to 1 in 1300; mean, 1 in 464) were reported in population isolates. The mean prevalence from four African countries was 1 in 4264 (range, 1 in 1755 to 1 in 7900). Prevalence for three countries in Europe (mean, 1 in 12,000; range, 1 in 10,000 to 1 in 15,000) may be underestimated, as the phenotype, in fair-skinned populations, may be missed or misdiagnosed as ocular albinism or isolated visual impairment. Population rates may vary depending on local cultural factors (e.g., consanguineous matings) and may change over time.
UNASSIGNED: The prevalence of OCA varies widely between continents and population groups, and it is often influenced by local factors. It was not possible, therefore, to determine a single, generalizable worldwide prevalence rate for OCA, although continental rates for Africa and Europe are useful.