Ear and upper airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review.
Eur Respir Rev
; 32(169)2023 Sep 30.
Article
en En
| MEDLINE
| ID: mdl-37437912
ABSTRACT
BACKGROUND:
Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by pulmonary, otological and sino-nasal manifestations. Well-defined clinical outcome measures are needed in such rare diseases research to improve follow-up and treatments. Pulmonary outcome measures have recently been described. The aim of this study was to identify ear and upper airway outcome measures that could be used for longitudinal follow-up of individuals with PCD.METHODS:
A scoping review was performed by systematically searching MEDLINE, Embase and Cochrane Database of Systematic Reviews online databases for studies published from January 1996 to March 2022 that included at least 10 adult or paediatric PCD patients and reported ear and upper airway outcomes.RESULTS:
33 studies (1794 patients) were included. 10 ear and upper airway outcomes were reported. 17 studies reported audiometry, 16 reported otoscopic findings, and 13 reported rhinoscopic findings and sinus imaging. Health-related quality of life questionnaires were performed in seven studies. There was a high variability in definitions and measurement of outcomes between studies.CONCLUSIONS:
This scoping review highlights the lack of data regarding ear and upper airway outcomes in PCD. It also reports a high heterogeneity in outcome definitions or measures. We provide well-founded specific suggestions to standardise ear and upper airway outcome definitions and reporting for future PCD research studies.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Calidad de Vida
/
Trastornos de la Motilidad Ciliar
Tipo de estudio:
Prognostic_studies
/
Systematic_reviews
Aspecto:
Patient_preference
Límite:
Adult
/
Child
/
Humans
Idioma:
En
Revista:
Eur Respir Rev
Año:
2023
Tipo del documento:
Article
País de afiliación:
Francia