Respiratory symptoms of Swiss people with primary ciliary dyskinesia.

Details

Serval ID
serval:BIB_ECE2A775992A
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Respiratory symptoms of Swiss people with primary ciliary dyskinesia.
Journal
ERJ open research
Author(s)
Goutaki M., Hüsler L., Lam Y.T., Koppe H.M., Jung A., Lazor R., Müller L., Pedersen ESL, Kuehni C.E.
Working group(s)
Swiss PCD Research Group
ISSN
2312-0541 (Print)
ISSN-L
2312-0541
Publication state
Published
Issued date
04/2022
Peer-reviewed
Oui
Volume
8
Number
2
Pages
00673
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Mostly derived from chart reviews, where symptoms are recorded in a nonstandardised manner, clinical data about primary ciliary dyskinesia (PCD) are inconsistent, which leads to missing and unreliable information. We assessed the prevalence and frequency of respiratory and ear symptoms and studied differences by age and sex among an unselected population of Swiss people with PCD.
We sent a questionnaire that included items from the FOLLOW-PCD standardised questionnaire to all Swiss PCD registry participants.
We received questionnaires from 74 (86%) out of 86 invited persons or their caregivers (median age 23 years, range 3-73 years), including 68% adults (≥18 years) and 51% females. Among participants, 70 (94%) reported chronic nasal symptoms; most frequently runny nose (65%), blocked nose (55%) or anosmia (38%). Ear pain and hearing problems were reported by 58% of the participants. Almost all (99%) reported cough and sputum production. The most common chronic cough complications were gastro-oesophageal reflux (n=11; 15%), vomiting (n=8; 11%) and urinary incontinence (n=6; 8%). Only nine (12%) participants reported frequent wheeze, which occurred mainly during infection or exercise, while 49 (66%) reported shortness of breath, and 9% even at rest or during daily activities. Older patients reported more frequent nasal symptoms and shortness of breath. We found no difference by sex or ultrastructural ciliary defect.
This is the first study to describe patient-reported PCD symptoms. The consistent collection of standardised clinical data will allow us to better characterise the phenotypic variability of the disease and study disease course and prognosis.
Pubmed
Web of science
Open Access
Yes
Create date
19/04/2022 14:06
Last modification date
14/09/2022 6:38
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