Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children Primary Ciliary Dyskinesia

Comparison of Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children With Primary Ciliary Dyskinesia and Healthy Controls

Primary ciliary dyskinesia is an autosomal recessive disorder characterized by abnormal ciliary movement and disrupted mucociliary clearance. In uncleaned airways, microorganisms and respiratory irritants cause inflammation and infection. Permanent rhinitis and chronic sputum cough are typical features in primary ciliary dyskinesia patients. Primary ciliary dyskinesia is a disease that threatens lung function from pre-school age. One of the main causes of respiratory muscle weakness in chronic lung diseases diseases is worsening of lung function. Such a weakness causes alveolar hypoventilation, microatelectasis, reduction of the cough strength .The cough strength is important for airway cleaning.

Exercise capacity is affected in chronic lung diseases. Assessment of exercise capacity in chronic lung diseases is prognostically important. Reduced exercise capacity and pulmonary function in PCD cause decrease in physical activity level. PCD patients have low quality of life and early recognition has been found to affect the quality of life positively. Children with chronic illness have higher level of depression than healthy children.

In literature, no study investigated respiratory muscle strength, exercise capacity and physical activity PCD patients. Therefore, the investigators aimed to compare aforementioned outcomes in PCD patients and healthy controls.

Study Overview

• Status: Completed
• Conditions: Primary Ciliary Dyskinesia

Detailed Description

According to sample size calculation 26 diagnosed primary ciliary dyskinesia patients and 26 healthy individuals will be included. Respiratory muscle strength, anaerobic and aerobic exercise capacity, physical activity, pulmonary functions, peripheral muscle strength, cough strength, respiratory muscle endurance, activity dyspnea perception, quality of life and depression will be evaluated. Primary outcome measurements are respiratory muscle strength, exercise capacity, physical activity; secondary outcomes are pulmonary functions, peripheral muscle strength, cough strength, respiratory muscle endurance, activity dyspnea perception, quality of life and depression.

• Study Type: Observational
• Enrollment (Actual): 26

Contacts and Locations

Study Locations

• Turkey
  • Yenimahalle, Turkey
    • Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Out- patients and healthy volunteers

Description

Inclusion Criteria:

• Diagnosed primary ciliary dyskinesia patients,
• 6-18 years of age,
• under standard medications,
• stable patients without exacerbation or infection

Exclusion Criteria:

• having cognitive disorder,
• orthopedic or neurological disease with a potential to affect functional capacity,
• acute infections or pneumonia,
• problems which may prevent evaluating such as visual problems

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Cross-Sectional

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Primary ciliary dyskinesia patients; Primary ciliary dyskinesia patients will be included in study. Inclusion and exclusion criteria were considered.
Healthy individuals; Those without diagnosed chronic disease will be included in study. Inclusion and exclusion criteria were considered.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Respiratory muscle strength	Evaluated using mouth pressure device	first day
Functional exercise capacity	Evaluated using 6-minute walking test	first day
Anaerobic exercise capacity	Evaluated using 3-minute step test	first day
Physical activity	Evaluated using a metabolic holter	first day

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Pulmonary functions	Evaluated using spirometer	first day
Muscle strength	Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength)	second day
Cough strength	Evaluated using PEFmeter	second day
Respiratory muscle endurance	Evaluated using respiratory muscle trainer (POWERbreathe)	second day]
Activity dyspnea	Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome)	first day
Generic Quality of life	Cystic Fibrosis Questionnaire Revised (CFQ-R) Turkish version (Scores range from 0 to 100, with higher scores indicating better health. 9 Quality of life domains: Physical, role/school, vitality, emotion, social, body image, eating, treatment burden, health perceptions.3 symptom scales: Weight, respiratory, and digestion.Number of items in CFQ-R CFQ-R Teen/Adult: 50. CFQ-R Parent: 44. CFQ-R Child: 35.)	second day
Disease- specific quality of life	Health-related quality-of-life questionnaires-Primary Ciliary Dyskinesia ( PCD-QOL) Turkish version ( PCD-QOL questionnaire developed different domains for different age groups: pediatric, adolescent, and adult patients as well as parents. These domains mainly evaluate physical, emotional, and social aspects of PCD related to QOL. Moreover, there are different domains for various symptoms at different age groups. The total numbers of items in the questionnaires are 37 in the questionnaire for children, 43 in the one for adolescents, 49 in the one for adults, 41 in the parents' questionnaire.	second day
Depression	Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale.	second day

Collaborators and Investigators

• Sponsor: Gazi University
• Investigators: Study Chair: Merve Fırat, Pt, Research assistant; Study Director: Meral Boşnak Güçlü, Pt,Phd, Associate professor; Principal Investigator: Tuğba Şişmanlar Eyüboğlu, MD, Medical Doctor; Principal Investigator: Ayşe Tana Aslan, MD,Phd, PROFESSOR

Publications and helpful links

General Publications

• Firat M, Bosnak-Guclu M, Sismanlar-Eyuboglu T, Aslan AT. Aerobic exercise capacity, cough strength, posture, and depression in primary ciliary dyskinesia. Pediatr Pulmonol. 2022 Sep 17. doi: 10.1002/ppul.26158. Online ahead of print.
• Firat M, Bosnak-Guclu M, Sismanlar-Eyuboglu T, Tana-Aslan A. Respiratory muscle strength, exercise capacity and physical activity in patients with primary ciliary dyskinesia: A cross-sectional study. Respir Med. 2022 Jan;191:106719. doi: 10.1016/j.rmed.2021.106719. Epub 2021 Dec 17.

Helpful Links

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Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2016
• Primary Completion (Actual): February 25, 2018
• Study Completion (Actual): June 30, 2018

Study Registration Dates

• First Submitted: November 10, 2017
• First Submitted That Met QC Criteria: December 11, 2017
• First Posted (Actual): December 12, 2017

Study Record Updates

• Last Update Posted (Actual): February 15, 2019
• Last Update Submitted That Met QC Criteria: February 13, 2019
• Last Verified: February 1, 2019

More Information

Terms related to this study

• Keywords: Quality of life; Physical inactivity; Exercise capacity; Respiratory muscle strength
• Additional Relevant MeSH Terms: Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Neurologic Manifestations; Congenital Abnormalities; Genetic Diseases, Inborn; Otorhinolaryngologic Diseases; Movement Disorders; Abnormalities, Multiple; Ciliopathies; Dyskinesias; Ciliary Motility Disorders
• Other Study ID Numbers: Gazi University5

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No
• IPD Plan Description: There is not a plan to make individual participant data but when the statistical analyses of all data are made, all results will be shared.