Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. Issue 4 (4th August 2016)

• Record Type: Journal Article
• Authors: Goutaki, Myrofora; Meier, Anna Bettina; Halbeisen, Florian S.; Lucas, Jane S.; Dell, Sharon D.; Maurer, Elisabeth; Casaulta, Carmen; Jurca, Maja; Spycher, Ben D.; Kuehni, Claudia E.
• Is Part Of: European respiratory journal. Volume 48:Issue 4(2016)
• Page Start: 1081
• Page End: 1095
• Publication Date: 2016-08-04
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.00736-2016
• Languages: English
• ISSNs: 0903-1936

Abstract

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 2 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. Review of the clinical manifestations of PCD found between-study variation; large prospective studies needed http://ow.ly/Y5GC300Sw73