An observational study in Primary Ciliary Dyskinesia, sponsored by Gazi University. Completed at 1 site in Turkey. Open to participants aged 6 Years to 18 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2019-02-15.
Sponsored by Gazi University · Observational
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.
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.
79 studies on the registry are indexed under Ciliary Motility Disorders; 26 are open to participants now.
This study's enrollment of 26 is below the median of 130 across 55 observational studies indexed under Ciliary Motility Disorders.
Browse Ciliary Motility Disorders studies →Gazi University is the lead sponsor of 551 studies on the registry; 102 are open to participants now.
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Out- patients and healthy volunteers
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Exclusion Criteria:
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
Evaluated using mouth pressure device
Time frame: first day
Functional exercise capacity
Evaluated using 6-minute walking test
Time frame: first day
Anaerobic exercise capacity
Evaluated using 3-minute step test
Time frame: first day
Physical activity
Evaluated using a metabolic holter
Time frame: first day
Pulmonary functions
Evaluated using spirometer
Time frame: first day
Muscle strength
Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength)
Time frame: second day
Cough strength
Evaluated using PEFmeter
Time frame: second day
Respiratory muscle endurance
Evaluated using respiratory muscle trainer (POWERbreathe)
Time frame: second day]
Activity dyspnea
Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome)
Time frame: 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.)
Time frame: 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.
Time frame: second day
Depression
Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale.
Time frame: second day
Plan to share: No — There is not a plan to make individual participant data but when the statistical analyses of all data are made, all results will be shared.
Ran from Oct 2016 to primary completion in Feb 2018, 16 months.
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This study is completed, as verified in Feb 2019. You cannot join it, but the record below documents what was studied.
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