Pulmonary functions, nasal symptoms, and quality of life in patients with primary ciliary dyskinesia (PCD)

Guy Gut, Ronen Bar-Yoseph, Moneera Hanna, Noa Brandl, Irit Alisha, Suha Rizik, Mordechai Pollak, Fahed Hakim, Israel Amirav, Lea Bentur, Michal Gur

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Several factors may influence quality of life (QOL) for patients with primary ciliary dyskinesia (PCD). We aimed to evaluate the association between pulmonary functions, nasal symptoms and QOL in PCD patients. Methods: A prospective single center study. Patients performed spirometry, whole body plethysmography, forced oscillation technique (FOT), lung clearance index (LCI), 6-min walk test (6MWT), and filled two questionnaires: a specific PCD QOL questionnaire (PCD-QOL) and Sino-nasal outcome test (SNOT-22) questionnaire, assessing symptoms of chronic rhinosinusitis and health related QOL. Results: Twenty-seven patients (56% females), age 19.4 ± 10.5 years were included; their, FEV1 was 74.6 ± 22.7%, and RV/TLC was (157.3 ± 39.3% predicted). Health perception and lower respiratory symptoms domains of PCD-QOL had the lowest score (median [IQR]: 50 [33.3−64.6] and 57.1 [38.9−72.2], respectively). FOT parameters correlated with several PCD-QOL domains. R5 z-score (indicating total airway resistance) and AX z-score (indicating airway reactance) correlated negatively with physical domain (r = −0.598, p =.001, and r = −0.42, p =.03, respectively); R5 z-score also correlated negatively with hearing domain (r = −0.57, p =.002). R5-20 z-score (indicating small airway resistance) correlated negatively with role domain (r = −0.49, p =.03). SNOT-22 score correlated negatively with several PCD-QOL domains (lower respiratory symptoms r = −0.77, p <.001; physical r = −0.72, p <.001; upper respiratory symptoms r = −0.66, p <.001). No correlations were found between spirometry values, LCI, 6MWT, and PCD-QOL. Conclusions: FOT suggested small airway dysfunction, and correlated negatively with several PCD-QOL domains. Nasal symptoms had strong negative correlations with PCD-QOL. Larger longitudinal studies will further elucidate factors affecting QOL in PCD.

Original languageEnglish
Pages (from-to)688-694
Number of pages7
JournalPediatric Pulmonology
Volume59
Issue number3
Early online date20 Dec 2023
DOIs
StatePublished - Mar 2024
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2023 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.

Keywords

  • SNOT-22
  • forced oscillation technique
  • nasal symptoms
  • primary ciliary dyskinesia
  • quality of life

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