RT Journal Article
SR Electronic
T1 Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1081
OP 1095
DO 10.1183/13993003.00736-2016
VO 48
IS 4
A1 Myrofora Goutaki
A1 Anna Bettina Meier
A1 Florian S. Halbeisen
A1 Jane S. Lucas
A1 Sharon D. Dell
A1 Elisabeth Maurer
A1 Carmen Casaulta
A1 Maja Jurca
A1 Ben D. Spycher
A1 Claudia E. Kuehni
YR 2016
UL http://erj.ersjournals.com/content/48/4/1081.abstract
AB 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 (I2 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