Physical Fitness, Exercise Capacity and Activities of Daily Living in Primary Ciliary Dyskinesia: a Retrospective Study

Study Overview
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Brief Summary
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Decreased pulmonary function, peripheral muscle strength, and exercise capacity were reported in primary ciliary dyskinesia (PCD) in recent studies. We aimed to investigate the data conducted between 10 July 2015 and 10 January 2015 of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL) in PCD and healthy counterparts retrospectively at the first stage of the study and the effects of inspiratory muscle training on pulmonary function, respiratory muscle strength, and exercise capacity in PCD patients with decreased inspiratory muscle strength from the database recorded between 10 July 2015 and 10 January 2015 retrospectively at the second stage of the study.

Official Title
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Physical Fitness, Exercise Capacity and Activities of Daily Living in Primary Ciliary Dyskinesia: a Retrospective Study

Conditions
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Primary Ciliary Dyskinesia, Kartagener Syndrome, Immotile Cilia Syndrome

Intervention / Treatment
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* Other: Data Collection
* Other: Data Collection


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Clinically stable Being able to cooperate with the assessments Not having any cardiovascular, orthopedic, or neurological problems Exclusion Criteria: Not being clinically stable Not being able to cooperate with the assessments Having any cardiovascular, orthopedic, or neurological problems

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 |
| --- | --- |
| Primary Ciliary Dyskinesia (PCD)<br>The first stage of the study: Data of 20 PCD patients from the database recorded between 10 July 2015 and 10 January 2017. The second stage of the study: Data of 14 PCD patients who had lower inspiratory muscle strength from the same database underwent supervised inspiratory muscle training (IMT) at our research unit and airway clearance techniques (ACT) as a home-based therapy for 6 weeks from the database recorded between 10 July 2015 and 10 January 2017. Data of 14 PCD patients who had lower inspiratory muscle strength from the same database underwent airway clearance techniques (ACT) as a home-based therapy for 6 weeks from the database recorded between 10 July 2015 and 10 January 2017. | Other: Data Collection<br>* The first stage of the study: Retrospective data collection of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL). The second stage of the study Retrospective data of pulmonary function, respiratory muscle strength, functional capacity before and after supervised inspiratory muscle training with POWERbreathe K5 device and airway clearance techniques performed for 6 weeks in PCD with decreased respiratory muscle strength (Intervention Group). Retrospective data of pulmonary function, respiratory muscle strength, and functional capacity before and after airway clearance techniques performed for 6 weeks as home-based therapy in PCD with decreased respiratory muscle strength (Control group).<br>|
| Healthy Group<br>Data of 20 healthy subjects from the database recorded between 10 July 2015 and 10 January 2017. | Other: Data Collection<br>* -Retrospective data collection of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| A primer outcome for the first stage of the study: Physical fitness | Munich Physical Fitness Test (MFT) was used to evaluate physical fitness. It is a test with six parameters including ball bounce, sandbag throwing, bending, vertical jumping, hanging and step test. | 10 July 2015-10 January 2015-retrospectively | 
| A primer outcome for the second stage of the study: Functional capacity | Functional capacity was evaluated with six minute walk test (6MWT). | 10 July 2015-10 January 2015-retrospectively | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Demographic features | The age was recorded in years. | 10 July 2015-10 January 2015-retrospectively | 
| Demographic features | Weight was recorded in kilograms. | 10 July 2015-10 January 2015-retrospectively | 
| Demographic features | The length was saved in meters. | 10 July 2015-10 January 2015-retrospectively | 
| Demographic features | Body mass index was calculated by dividing the weight by the square of the height (kg/m^2) and recorded. | 10 July 2015-10 January 2015-retrospectively | 
| Pulmonary Function | Pulmonary function (FEV1 (forced expiratory volume in one second), FVC (forced vital capacity), FEV1/FVC, FEF25-75 (%) (forced expiratory volume 25-75 %) and PEF (peak expiratory flow)) assessed with a portable spirometer (Spirobank MIR, Italy). | 10 July 2015-10 January 2015-retrospectively | 
| Respiratory muscle strength | Respiratory muscle strength (maximal inspiratory pressure (MIP);maximal expiratory pressure (MEP), respectively) was measured with a portable, electronic mouth pressure monitor (Micro Medical MicroMPM, UK) and recorded. | 10 July 2015-10 January 2015-retrospectively | 
| Exercise Capacity | Exercise capacity was assessed with the modified shuttle walk test. | 10 July 2015-10 January 2015-retrospectively | 
| Activities of Daily Living | Glittre activities of daily living (ADL) test was used to evaluate daily life activity. | 10 July 2015-10 January 2015-retrospectively | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Primary Ciliary Dyskinesia, Kartagener Syndrome, Immotile Cilia Syndrome, Pulmonary Function, Respiratory Muscle Strength, Exercise Capacity, Physical Fitness, Inspiratory Muscle Strength Training