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

Completed

• Conditions: Primary Ciliary Dyskinesia

• Registration Number: NCT03370029
• Lead Sponsor: Gazi University

Brief Summary

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.

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 Timeline

• Study Start: 2016-10
• Study First Submitted: 2017-11-10
• Study First Submitted QC: 2017-12-11
• Study First Posted: 2017-12-12
• Primary Completion: 2018-02-25
• Study Completion: 2018-06-30
• Status Verified: 2019-02
• Last Update Submitted: 2019-02-13
• Last Update Posted: 2019-02-15

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 26

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 & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

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

Secondary Outcome Measures

Name	Time	Method
Depression	second day	Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale.
Respiratory muscle endurance	second day]	Evaluated using respiratory muscle trainer (POWERbreathe)
Disease- specific quality of life	second day	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.
Pulmonary functions	first day	Evaluated using spirometer
Muscle strength	second day	Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength)
Cough strength	second day	Evaluated using PEFmeter
Activity dyspnea	first day	Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome)
Generic Quality of life	second day	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.)

Trial Locations

Locations (1)

Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation; 🇹🇷 Yenimahalle, Turkey