Respiratory Function, Exercise Capacity and Peripheral Muscle Strength Among Patients With CF, PCD and Healthy Children

Study Overview
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Brief Summary
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The aim of this study is to compare pulmonary function, respiratory muscle strength, exercise capacity and peripheral muscle strength of patients with CF, PCD and healthy childrens.

Detailed Description
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The impaired airway clearence and pulmonary functions, exercise intolerance, low physical activity level and decreased peripheral muscle strength make physiotherapy approaches important in the management of CF and PCD.

Official Title
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Comparison of Respiratory Function, Exercise Capacity and Peripheral Muscle Strength Among Patients With Cystic Fibrosis, Primary Ciliary Dyskinesia and Healthy Children

Conditions
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Primary Ciliary Dyskinesia, Cystic Fibrosis

Intervention / Treatment
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* Other: Measurement of functional capacity
* Other: pulmonary function test
* Other: Functional capacity
* Other: Peripheral muscle strength
* Other: Respiratory muscle strength


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of cystic fibrosis or primary ciliary dyskinesia Exclusion Criteria: Hospitalization history in past month Diagnosis of other chronic pediatric diseases which may impair exercise tolerance such as cerebral palsy or neuromuscular disease Candidates for lung transplantation or history of lung transplantation

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 |
| --- | --- |
| Cystic fibrosis<br>children with cystic fibrosis | Other: Measurement of functional capacity<br>* Functional capacity of participants will be measured with six-minute walk test.<br>Other: pulmonary function test<br>* It will be measured using basic spirometry and presented lung volume parameters such as FEV1,FVC,FEV1/FVC,PEF, FEF25-75<br>Other: Functional capacity<br>* Functional capacity of participants will be measured with six-minute walk test and sit-to-stand test<br>Other: Peripheral muscle strength<br>* Isometric M. Quadriceps strength will be measured using electronic hand held dynamometer in sitting position.<br>Other: Respiratory muscle strength<br>* Inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures.<br>|
| primary ciliary dyskinesia<br>children with primary ciliary dyskinesia | Other: Measurement of functional capacity<br>* Functional capacity of participants will be measured with six-minute walk test.<br>Other: pulmonary function test<br>* It will be measured using basic spirometry and presented lung volume parameters such as FEV1,FVC,FEV1/FVC,PEF, FEF25-75<br>Other: Functional capacity<br>* Functional capacity of participants will be measured with six-minute walk test and sit-to-stand test<br>Other: Peripheral muscle strength<br>* Isometric M. Quadriceps strength will be measured using electronic hand held dynamometer in sitting position.<br>Other: Respiratory muscle strength<br>* Inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures.<br>|
| healthy controls<br>Age-matched healthy volunteers | Other: Measurement of functional capacity<br>* Functional capacity of participants will be measured with six-minute walk test.<br>Other: pulmonary function test<br>* It will be measured using basic spirometry and presented lung volume parameters such as FEV1,FVC,FEV1/FVC,PEF, FEF25-75<br>Other: Functional capacity<br>* Functional capacity of participants will be measured with six-minute walk test and sit-to-stand test<br>Other: Peripheral muscle strength<br>* Isometric M. Quadriceps strength will be measured using electronic hand held dynamometer in sitting position.<br>Other: Respiratory muscle strength<br>* Inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Six-minute walk test distance | Distance walked in six minutes will be recorded in meters. Test will be conducted according to the guideline of American Thoracic Society (ATS). | 15 minutes | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Forced Vital Capacity (FVC) | FVC will be measured using basic spirometry and expressed as the percentage of the predicted value according to the guideline of European Respiratory Society (ERS) | 5 minutes | 
| Forced Expiratory Volume in 1 second (FEV1) | FEV1 will be measured using basic spirometry and expressed as the percentage of the predicted value according to the guideline of European Respiratory Society (ERS) | 5 minutes | 
| Peak Expiratory Flow (PEF) | PEF will be measured using basic spirometry and expressed as the percentage of the predicted value according to the guideline of European Respiratory Society (ERS) | 5 minutes | 
| M. Quadriceps strength | Isometric M. Quadriceps strength (kg) will be measured using electronic hand held dynamometer in sitting position. | 5 minutes | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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respiratory functions, exercise capacity, muscle strength