Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children Primary Ciliary Dyskinesia
|The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.|
|ClinicalTrials.gov Identifier: NCT03370029|
Recruitment Status : Completed
First Posted : December 12, 2017
Last Update Posted : February 15, 2019
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.
|Condition or disease|
|Primary Ciliary Dyskinesia|
|Study Type :||Observational|
|Actual Enrollment :||26 participants|
|Official Title:||Comparison of Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children With Primary Ciliary Dyskinesia and Healthy Controls|
|Actual Study Start Date :||October 2016|
|Actual Primary Completion Date :||February 25, 2018|
|Actual Study Completion Date :||June 30, 2018|
Primary ciliary dyskinesia patients
Primary ciliary dyskinesia patients will be included in study. Inclusion and exclusion criteria were considered.
Those without diagnosed chronic disease will be included in study. Inclusion and exclusion criteria were considered.
- Respiratory muscle strength [ Time Frame: first day ]Evaluated using mouth pressure device
- Functional exercise capacity [ Time Frame: first day ]Evaluated using 6-minute walking test
- Anaerobic exercise capacity [ Time Frame: first day ]Evaluated using 3-minute step test
- Physical activity [ Time Frame: first day ]Evaluated using a metabolic holter
- Pulmonary functions [ Time Frame: first day ]Evaluated using spirometer
- Muscle strength [ Time Frame: second day ]Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength)
- Cough strength [ Time Frame: second day ]Evaluated using PEFmeter
- Respiratory muscle endurance [ Time Frame: second day] ]Evaluated using respiratory muscle trainer (POWERbreathe)
- Activity dyspnea [ Time Frame: first day ]Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome)
- Generic Quality of life [ Time Frame: 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.)
- Disease- specific quality of life [ Time Frame: 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.
- Depression [ Time Frame: second day ]Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale.
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03370029
|Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation|
|Study Chair:||Merve Fırat, Pt||Research assistant|
|Study Director:||Meral Boşnak Güçlü, Pt,Phd||Associate professor|
|Principal Investigator:||Tuğba Şişmanlar Eyüboğlu, MD||Medical doctor|
|Principal Investigator:||Ayşe Tana Aslan, MD,Phd||Professor|