Do pulmonary and extrapulmonary features differ among cystic fibrosis, primary ciliary dyskinesia, and healthy children?

Abstract

Background: Primary ciliary dyskinesia (PCD) is generally likened to cystic fibrosis (CF) due to similarities in impaired mucociliary clearance and some other symptoms. The aim of our study was to investigate pulmonary and extrapulmonary characteristics of children with CF and PCD since no studies have addressed respiratory muscle strength in children with PCD and to compare the results to those obtained from healthy age-matched controls. Methods: Pulmonary and extrapulmonary characteristics were assessed by 6-min walk test, spirometry, maximum inspiratory and expiratory pressure measurements, and knee extensor strength test in the children with CF, PCD, and healthy controls. Results: Children with PCD and CF had similar PFT results, except forced expiratory flow between 25% and 75% of vital capacity (FEF 25-75) which was lower in PCD (p = .04). Maximum inspiratory pressure (MIP) value was lower in the children with CF compared with the healthy controls (p = .016), MEP value of the children with PCD was worse than those with CF and healthy controls (p = .013 and p = .013), respectively. 6-min walk test (6MWT) distance of the children with CF was lower than their healthy counterparts (p = .003). Knee extensor muscle strength differed among the children with PCD, CF, and healthy control groups, but post hoc test failed to show statistical significance (p = .010). Conclusion: Children with CF and PCD had some impairments in pulmonary functions, respiratory muscle strength, functional capacity, and peripheral muscle strength compared with healthy children. However, the unique characteristics of each disease should be considered during physiotherapy assessment and treatment. The clinicians may especially focus on the respiratory and peripheral muscle strength of the children with PCD.

Aerobic exercise capacity, cough strength, posture, and depression in primary ciliary dyskinesia

“…The patients with PCD had lower completed steps (86 vs. 90 steps) at the end of the test in the present study. A few studies previously evaluated exercise capacity in patients with PCD and the findings are contradictory 3–7 . Similar to our results, Sonbahar‐Ulu et al, Simşek et al, and Madsen et al reported decreased exercise capacity in PCD patients than healthy controls.…”

In children with primary ciliary dyskinesia, which type of lung function test is the earliest determinant of decline in lung health: A systematic review

“…Additionally, some studies provided additional details, that is, Kinghorn et al included those who had FEV1 > 60% with no cough or wheeze in the last 2 weeks; Denizoglu Kulli et al excluded those with any hospitalization in the last 1 month or with a history of cardiovascular disease; Magnin et al excluded anyone with an acute exacerbation in the last 6 weeks, however, included some who were toward the end of 2 weeks antibiotics course. In five studies, PCD was diagnosed using transmission electron microscopy (TEM) and genetic testing 32–34,38,39 . In the remaining three studies, TEM along with clinical phenotype formed the basis of diagnosis.…”

“…All eight studies included in this systematic review were observational studies 32–39 . Apart from one study, there was no computer‐generated randomization or allocation concealment of the subjects doing different lung function tests, 39 leading to selection bias.…”

“…The studies were classified according to the OCEBM (Oxford Center for Evidence‐Based Medicine) 40 levels of evidence and the quality was scored using the McGrath quality assessment 41 . Only one study was classified as Grade 2, 37 while the rest were Grade 3 32–38 …”

Video game‐based exercise in children and adolescents with non‐cystic fibrosis bronchiectasis: A randomized comparative study of aerobic and breathing exercises

“…Permanent obstruction and impaired gas exchange mechanism in the airways that develop due to chronic inflammation and sputum cause the decrease of respiratory function and respiratory muscle strength 3,4 . Also, impairment of functional capacity and balance are extrapulmonary manifestations of NCFB 4,5 …”