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

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Primary Ciliary Dyskinesia

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Primary ciliary dyskinesia patients<br>Primary ciliary dyskinesia patients will be included in study. Inclusion and exclusion criteria were considered. | |
| Healthy individuals<br>Those without diagnosed chronic disease will be included in study. Inclusion and exclusion criteria were considered. | |

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Exercise capacity, Respiratory muscle strength, Physical inactivity, Quality of life