Primary Ciliary Dyskinesia
Conditions
Keywords
Exercise capacity, Respiratory muscle strength, Physical inactivity, Quality of life
Brief summary
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.
Detailed description
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.
Interventions
None listed
Sponsors
Gazi University
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
* Diagnosed primary ciliary dyskinesia patients, * 6-18 years of age, * under standard medications, * stable patients without exacerbation or infection
Exclusion criteria
* having cognitive disorder, * orthopedic or neurological disease with a potential to affect functional capacity, * acute infections or pneumonia, * problems which may prevent evaluating such as visual problems
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Respiratory muscle strength | first day | Evaluated using mouth pressure device |
| Functional exercise capacity | first day | Evaluated using 6-minute walking test |
| Anaerobic exercise capacity | first day | Evaluated using 3-minute step test |
| Physical activity | first day | Evaluated using a metabolic holter |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Activity dyspnea | first day | Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome) |
| Generic Quality of life | 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.) |
| Pulmonary functions | first day | Evaluated using spirometer |
| Depression | second day | Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale. |
| Disease- specific quality of life | 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. |
| Muscle strength | second day | Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength) |
| Cough strength | second day | Evaluated using PEFmeter |
| Respiratory muscle endurance | second day] | Evaluated using respiratory muscle trainer (POWERbreathe) |
Countries
Turkey (Türkiye)
Outcome results
None listed