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Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis

Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis
Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis
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.
0903-1936
1-15
Goutaki, Myrofora
60fbeefc-dbb1-429c-b81a-3c35d368db64
Meier, Anna Bettina
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Halbeisen, Florian S.
d4282cb2-a4d2-4f60-a471-daf86fc41020
Lucas, Jane S.
5cb3546c-87b2-4e59-af48-402076e25313
Dell, Sharon D.
736623b0-32d7-45a4-811b-af3debee2eae
Maurer, Elisabeth
1edb44f7-8bc4-4eb1-9f20-66f13e40f1a7
Casaulta, Carmen
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Jurca, Maja
dcb4db61-e10a-43c7-b97b-d2bc1c129c40
Spycher, Ben D.
e92b6bc1-ad6b-4a2a-a907-8dc0fd3614d9
Kuehni, Claudia E.
ac67c925-ee32-429d-a3b5-c244daa314b4
Goutaki, Myrofora
60fbeefc-dbb1-429c-b81a-3c35d368db64
Meier, Anna Bettina
76f200ac-3320-4988-a4b6-c2edff3eae77
Halbeisen, Florian S.
d4282cb2-a4d2-4f60-a471-daf86fc41020
Lucas, Jane S.
5cb3546c-87b2-4e59-af48-402076e25313
Dell, Sharon D.
736623b0-32d7-45a4-811b-af3debee2eae
Maurer, Elisabeth
1edb44f7-8bc4-4eb1-9f20-66f13e40f1a7
Casaulta, Carmen
0a57a56c-3780-4fd4-a616-2e1348742348
Jurca, Maja
dcb4db61-e10a-43c7-b97b-d2bc1c129c40
Spycher, Ben D.
e92b6bc1-ad6b-4a2a-a907-8dc0fd3614d9
Kuehni, Claudia E.
ac67c925-ee32-429d-a3b5-c244daa314b4

Goutaki, Myrofora, Meier, Anna Bettina, Halbeisen, Florian S., Lucas, Jane S., Dell, Sharon D., Maurer, Elisabeth, Casaulta, Carmen, Jurca, Maja, Spycher, Ben D. and Kuehni, Claudia E. (2016) Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. European Respiratory Journal, 1-15. (doi:10.1183/13993003.00736-2016). (PMID:27492829)

Record type: Article

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.

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__filestore.soton.ac.uk_users_jlucas1_mydocuments_Documents_Publications in preparation_respiratory writeups_Clin Manifestations Bern_Clinical manifestations in Primary Ciliary Dyskinesia systematic review and meta-analysis_revised_CLEAN.docx - Accepted Manuscript
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Accepted/In Press date: 24 May 2016
e-pub ahead of print date: 4 August 2016
Organisations: Clinical & Experimental Sciences

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Local EPrints ID: 399377
URI: https://eprints.soton.ac.uk/id/eprint/399377
ISSN: 0903-1936
PURE UUID: f846950c-4d37-4fe4-a099-444aec6177bd

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Date deposited: 15 Aug 2016 12:17
Last modified: 10 Dec 2019 06:27

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Contributors

Author: Myrofora Goutaki
Author: Anna Bettina Meier
Author: Florian S. Halbeisen
Author: Jane S. Lucas
Author: Sharon D. Dell
Author: Elisabeth Maurer
Author: Carmen Casaulta
Author: Maja Jurca
Author: Ben D. Spycher
Author: Claudia E. Kuehni

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