Primary Ciliary Dyskinesia
Conditions
Brief summary
The individuals with primary ciliary dyskinesia (PCD) have lower aerobic fitness and anaerobic performance than healthy individuals. Cardiopulmonary exercise test (CPET) provides an integrated assessment of integrative exercise responses involving the pulmonary, cardiovascular, hematopoietic, neuropsychological, and skeletal muscle systems that are not adequately reflected by measurement of the function of organ systems. Maximum oxygen consumption is measured by performing gas exchange analysis with CPET, offering an objective measure of cardiorespiratory fitness. The six-minute walk test (6MWT) and the shuttle walk test (SWT) are field tests that produce oxygen consumption results similar to those during CPET. There is a need to determine the physiological responses to CPET, SWT and 6MWT in individuals with PCD. Additionally, the physiological responses of individuals with PCD to different exercise tests will be compared with the responses of healthy individuals. Low aerobic capacity in individuals with PCD may affect the physical, emotional, and social lives of individuals. This influence may cause a decrease in the quality of life of individuals with PCD. The relationship between exercise capacity and quality of life in individuals with PSD will be investigated with this study.
Interventions
OTHERNo intervention
No intervention
Sponsors
Hacettepe University
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
8 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
* Having been diagnosed with PCD in the Pediatrics Department, Pediatric Chest Diseases Unit of Hacettepe University Faculty of Medicine and being routinely referred to Hacettepe University, Faculty of Physical Therapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit for physiotherapy applications and pulmonary rehabilitation program, * Being clinically stable, * Being between 8-18 years old, * Having a forced expiratory volume in the first second (FEV1) ≥ 40%, The healthy group will be composed of individuals who do not have any known disease and volunteer to participate in the study. For healthy individuals, volunteers from acquaintances and/or relatives of the researchers will be included in the study.
Exclusion criteria
* Having unstable clinical condition * Having severe neuromuscular and musculoskeletal problems, * Having any congenital heart diseases other than situs inversus, * Unable to cooperate
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Maximal oxygen uptake | up to 3 weeks | Maximal oxygen uptake will be determined using cardiopulmonary exercise test. |
| 6 minute walk test distance | up to 3 weeks | 6 minute walk test distance will be determined using 6 minute walk test |
| Shuttle walk test distance | up to 3 weeks | Shuttle walk test distance will be determined using shuttle walk test |
| Quality of life score | 1st day | Quality of life score will be determined using multidimensional measure to assess health-related quality of life in primary ciliary dyskinesia (QoL-PCD). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary function test - Peak expiratory flow | 1st day | Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. |
| Pulmonary function test - forced vital capacity | 1st day | Pulmonary function test using a spirometer will be performed. Forced vital capacity will be recorded. |
| Pulmonary function test - Forced mid-expiratory flow (FEF25-75) | 1stday | Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. |
| Pulmonary function test - Forced expiratory volume in one second | 1st day | Pulmonary function test using a spirometer will be performed. Forced expiratory volume in one second will be recorded. |
| Maximal inspiratory pressure | 1st day | Maximal inspiratory pressure will be measured using a mouthpiece device. |
| Maximal expiratory pressure | 1st day | Maximal expiratory pressure will be measured using a mouthpiece device. |
Countries
Turkey (Türkiye)
Outcome results
None listed