Physiological Responses to Exercise Tests in Primary Ciliary Dyskinesia Compared With Healthy Individuals

Study Overview
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Brief Summary
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The individuals with primary ciliary dyskinesia (PCD) have lower aerobic fitness and anaerobic performance than healthy individuals. Cardiopulmonary exercise test (CPET) provides an integrated assessment of integrative exercise responses involving the pulmonary, cardiovascular, hematopoietic, neuropsychological, and skeletal muscle systems that are not adequately reflected by measurement of the function of organ systems. Maximum oxygen consumption is measured by performing gas exchange analysis with CPET, offering an objective measure of cardiorespiratory fitness. The six-minute walk test (6MWT) and the shuttle walk test (SWT) are field tests that produce oxygen consumption results similar to those during CPET. There is a need to determine the physiological responses to CPET, SWT and 6MWT in individuals with PCD. Additionally, the physiological responses of individuals with PCD to different exercise tests will be compared with the responses of healthy individuals. Low aerobic capacity in individuals with PCD may affect the physical, emotional, and social lives of individuals. This influence may cause a decrease in the quality of life of individuals with PCD. The relationship between exercise capacity and quality of life in individuals with PSD will be investigated with this study.

Official Title
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Physiological Responses to Exercise Tests in Primary Ciliary Dyskinesia Compared With Healthy Children and Investigation of the Relationship Between Exercise Capacity and Quality of Life in Primary Ciliary Dyskinesia

Conditions
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Primary Ciliary Dyskinesia

Intervention / Treatment
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* Other: No intervention


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Having been diagnosed with PCD in the Pediatrics Department, Pediatric Chest Diseases Unit of Hacettepe University Faculty of Medicine and being routinely referred to Hacettepe University, Faculty of Physical Therapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit for physiotherapy applications and pulmonary rehabilitation program, Being clinically stable, Being between 8-18 years old, Having a forced expiratory volume in the first second (FEV1) ≥ 40%, The healthy group will be composed of individuals who do not have any known disease and volunteer to participate in the study. For healthy individuals, volunteers from acquaintances and/or relatives of the researchers will be included in the study. Exclusion Criteria: Having unstable clinical condition Having severe neuromuscular and musculoskeletal problems, Having any congenital heart diseases other than situs inversus, Unable to cooperate

Ages Eligible for Study
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Minimum Age: 8 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 |
| --- | --- |
| The children with primary ciliary dyskinesia<br>The children with primary ciliary dyskinesia | Other: No intervention<br>* No intervention<br>|
| Healthy controls<br>Healthy children | Other: No intervention<br>* No intervention<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Shuttle walk test distance | Shuttle walk test distance will be determined using shuttle walk test | up to 3 weeks | 
| Quality of life score | Quality of life score will be determined using multidimensional measure to assess health-related quality of life in primary ciliary dyskinesia (QoL-PCD). | 1st day | 
| Maximal oxygen uptake | Maximal oxygen uptake will be determined using cardiopulmonary exercise test. | up to 3 weeks | 
| 6 minute walk test distance | 6 minute walk test distance will be determined using 6 minute walk test | up to 3 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Pulmonary function test - forced vital capacity | Pulmonary function test using a spirometer will be performed. Forced vital capacity will be recorded. | 1st day | 
| Pulmonary function test - Forced expiratory volume in one second | Pulmonary function test using a spirometer will be performed. Forced expiratory volume in one second will be recorded. | 1st day | 
| Maximal inspiratory pressure | Maximal inspiratory pressure will be measured using a mouthpiece device. | 1st day | 
| Maximal expiratory pressure | Maximal expiratory pressure will be measured using a mouthpiece device. | 1st day | 
| Pulmonary function test - Peak expiratory flow | Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. | 1st day | 
| Pulmonary function test - Forced mid-expiratory flow (FEF25-75) | Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. | 1stday |