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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| The children with primary ciliary dyskinesia | The children with primary ciliary dyskinesia |
| |
| Healthy controls | Healthy children |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| No intervention | Other | No intervention |
|
| Measure | Description | Time Frame |
|---|---|---|
| Shuttle walk test distance | Shuttle walk test distance will be determined using shuttle walk test | up to 3 weeks |
| Quality of life score | Quality of life score will be determined using multidimensional measure to assess health-related quality of life in primary ciliary dyskinesia (QoL-PCD). | 1st day |
| Maximal oxygen uptake | Maximal oxygen uptake will be determined using cardiopulmonary exercise test. | up to 3 weeks |
| 6 minute walk test distance | 6 minute walk test distance will be determined using 6 minute walk test | up to 3 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| Pulmonary function test - forced vital capacity | Pulmonary function test using a spirometer will be performed. Forced vital capacity will be recorded. | 1st day |
| Pulmonary function test - Forced expiratory volume in one second |
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Inclusion Criteria:
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:
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The children with primary ciliary dyskinesia and healthy children
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| Name | Affiliation | Role |
|---|---|---|
| Deniz Inal-Ince | Hacettepe University | Study Chair |
| Ugur Ozcelik | Hacettepe University | Principal Investigator |
| Nagehan Emiralioglu | Hacettepe University | Principal Investigator |
| Melda Saglam | Hacettepe University | Principal Investigator |
| Naciye Vardar-Yagli | Hacettepe University | Principal Investigator |
| Ebru Calik-Kutukcu | Hacettepe University | Principal Investigator |
| Birce Sunman | Hacettepe University | Principal Investigator |
| Hazal Sonbahar-Ulu | Akdeniz University | Principal Investigator |
| Aslihan Cakmak | Hacettepe University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hacettepe University Faculty of Physical Therapy and Rehabilitation | Ankara | 06100 | Turkey (Türkiye) |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 41186743 | Derived | Cakmak-Onal A, Kocaaga E, Sonbahar-Ulu H, Calik E, Vardar-Yagli N, Saglam M, Sunman B, Emiralioglu N, Ozcelik U, Inal-Ince D. Physiological responses to cardiopulmonary and field exercise tests in primary ciliary dyskinesia compared with healthy peers. Eur J Appl Physiol. 2026 Apr;126(4):2051-2061. doi: 10.1007/s00421-025-06019-2. Epub 2025 Nov 4. |
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| ID | Term |
|---|---|
| D002925 | Ciliary Motility Disorders |
| ID | Term |
|---|---|
| D012140 | Respiratory Tract Diseases |
| D010038 | Otorhinolaryngologic Diseases |
| D000072661 | Ciliopathies |
| D000015 | Abnormalities, Multiple |
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Pulmonary function test using a spirometer will be performed. Forced expiratory volume in one second will be recorded.
| 1st day |
| Maximal inspiratory pressure | Maximal inspiratory pressure will be measured using a mouthpiece device. | 1st day |
| Maximal expiratory pressure | Maximal expiratory pressure will be measured using a mouthpiece device. | 1st day |
| Pulmonary function test - Peak expiratory flow | Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. | 1st day |
| Pulmonary function test - Forced mid-expiratory flow (FEF25-75) | Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded. | 1stday |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D030342 | Genetic Diseases, Inborn |