Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland Both authors contributed equally.
Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Eur Respir J. 2016 Oct;48(4):1081-1095. doi: 10.1183/13993003.00736-2016. Epub 2016 Aug 4.
Few original studies have described the prevalence and severity of clinical symptoms of primary ciliary dyskinesia (PCD). This systematic review and meta-analysis aimed to identify all published studies on clinical manifestations of PCD patients, and to describe their prevalence and severity stratified by age and sex.We searched PubMed, Embase and Scopus for studies describing clinical symptoms of ≥10 patients with PCD. We performed meta-analyses and meta-regression to explain heterogeneity.We included 52 studies describing a total of 1970 patients (range 10-168 per study). We found a prevalence of 5% for congenital heart disease. For the rest of reported characteristics, we found considerable heterogeneity (I range 68-93.8%) when calculating the weighted mean prevalence. Even after taking into account the explanatory factors, the largest part of the between-studies variance in symptom prevalence remained unexplained for all symptoms. Sensitivity analysis including only studies with test-proven diagnosis showed similar results in prevalence and heterogeneity.Large differences in study design, selection of study populations and definition of symptoms could explain the heterogeneity in symptom prevalence. To better characterise the disease, we need larger, multicentre, multidisciplinary, prospective studies that include all age groups, use uniform diagnostics and report on all symptoms.
很少有原始研究描述原发性纤毛运动障碍（PCD）的临床症状的患病率和严重程度。本系统评价和荟萃分析旨在确定所有发表的关于 PCD 患者临床表现的研究，并按年龄和性别描述其患病率和严重程度。我们在 PubMed、Embase 和 Scopus 中搜索描述了≥10 例 PCD 患者临床症状的研究。我们进行了荟萃分析和荟萃回归分析，以解释异质性。我们纳入了 52 项研究，共描述了 1970 例患者（每项研究范围为 10-168 例）。我们发现先天性心脏病的患病率为 5%。对于其他报告的特征，我们发现计算加权平均患病率时存在相当大的异质性（I 范围为 68-93.8%）。即使考虑到解释因素，所有症状的研究间方差的大部分仍然无法解释。包括仅具有经证实诊断的研究的敏感性分析显示，患病率和异质性相似。研究设计、研究人群选择和症状定义的差异很大，可能解释了症状患病率的异质性。为了更好地描述疾病，我们需要更大、多中心、多学科、前瞻性研究，包括所有年龄组，使用统一的诊断方法并报告所有症状。
