Cystic Fibrosis (CF)NCT07157644
Exercise Capacity, Muscle Oxygenation, Respiratory Muscle Strength, and Physical Activity Level in Pediatric CF and PCD
An observational study of Cystic Fibrosis (CF) and Primary Ciliary Dyskinesia (PCD), run by Gazi University at 1 site in Turkey (Türkiye).
Trial summary
Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are genetic diseases characterized by chronic respiratory tract infections. In both diseases, impaired mucociliary clearance, recurrent respiratory infections, and persistent inflammation lead to progressive deterioration in respiratory function. Read more about this study
- Who is it for?
- Ages 6 to 18 yearsHealthy volunteers can take part
- Conditions the study is for
- Cystic Fibrosis (CF), Primary Ciliary Dyskinesia (PCD)
- How many people
- 88 participants
- Where
- 1 siteTurkey (Türkiye)
- Study dates
- 1 Jan 2021to 1 Mar 2025
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 to 18 years
- All sexes
- Healthy volunteers accepted
- Child, Adult
Study population: Thirty-one patients with CF, 27 patients with PSD were included in the study. There are 30 healthy data.
Who may be able to join
- Cystic fibrosis patients;
- Patients diagnosed with cystic fibrosis according to the American Cystic Fibrosis Association consensus report
- Between the ages of 6 and 18
- Clinically stable conditions
- Primary ciliary dyskinesia patients;
- Patients diagnosed with primary ciliary dyskinesia according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) guidelines
Show all 11Show fewer
- Between the ages of 6 and 18
- Clinically stable conditions
- Healthy controls;
- Agreeing to participate voluntarily in the study
- Between the ages of 6 and 18
Who cannot join
- Patients;
- Uncooperative
- Orthopedic or neurological disorders that will affect functional capacity
- Pneumonia or any acute infection
- Healthy controls;
- Chronic disease
- Uncooperative
- Orthopedic or neurological disorders that will affect functional capacity
Trial locations and contacts
1 site in 1 country.
Turkey (Türkiye)1 site
Gazi University Faculty of Health Sciences Department of Cardiopulmonary Physiotherapy and Rehabilitation
Ankara, Çankaya, 06490
About this study
Official title: Comparison of Functional Exercise Capacity, Muscle Oxygenation, Respiratory Muscle Strength, and Physical Activity Level in Pediatric Cystic Fibrosis and Primary Ciliary Dyskinesia
Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are genetic diseases characterized by chronic respiratory tract infections. In both diseases, impaired mucociliary clearance, recurrent respiratory infections, and persistent inflammation lead to progressive deterioration in respiratory function. This condition limits patients' activities of daily living, leading to physical inactivity and exercise intolerance. Functional exercise capacity in patients with CF and PCD is reduced due to increased respiratory load, musculoskeletal involvement, and nutritional deficiencies. In exercise tests involving the upper and lower extremities, both patient groups exhibited significantly lower performance compared to healthy individuals. Muscle oxygenation is particularly reduced in patients with cystic fibrosis and is associated with inadequate oxygen delivery to peripheral muscles, mitochondrial dysfunction, and increased muscle fatigue. Although studies on muscle oxygenation in PCD patients are limited, it is thought to be affected by similar pathophysiological mechanisms. Respiratory muscle strength is weakened in both patient groups due to chronic cough, hyperinflation, and increased respiratory effort. This is particularly evident in a significant decrease in inspiratory and expiratory muscle strength. The number of studies in the literature evaluating muscle oxygenation, respiratory muscle strength, and physical activity levels in patients with CF and PCD is limited. There are no studies comparing muscle oxygenation between patients with CF and PCD.
Read the detailed description
In patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD), lower extremity exercise capacity, skeletal muscle function, respiratory muscle strength, and physical activity levels are limited by various pathophysiological mechanisms. In CF patients, lower extremity exercise capacity is significantly reduced due to ventilation limitation, respiratory muscle fatigue, and mitochondrial dysfunction. Early fatigue findings such as delayed oxygen uptake and lactate accumulation have been reported in lower extremity-specific exercise tests. In PCD patients, respiratory workload increases due to ventilation-perfusion mismatch and impaired mucociliary clearance, which can limit muscle oxygen utilization during exercise. Recent studies have shown that PCD patients have lower resting muscle oxygen saturation compared to healthy individuals, but these values are relatively maintained during exercise. In CF, respiratory muscle strength is weakened, particularly at the diaphragm and intercostal muscles, leading to a decrease in ventilatory reserve during exercise. Similarly, submaximal respiratory muscle fatigue and decreased inspiratory muscle strength have been reported in patients with PCD. Regarding physical activity levels, daily activity levels in both patient groups are significantly lower than in healthy peers, and this has been associated with disease progression, muscle dysfunction, and exercise intolerance. Objectively measured studies in children and adolescents with CF have reported that they fall below the recommended daily activity level, and this inadequacy negatively impacts muscle function over time. A similar tendency toward physical inactivity is also found in PCD patients, and this is considered directly related to exercise capacity. The number of studies in the literature evaluating muscle oxygenation, respiratory muscle strength, and physical activity levels in patients with CF and PCD is limited. There are no studies comparing muscle oxygenation in patients with CF and PCD. The aim of our study was to compare functional exercise capacity, muscle oxygenation, respiratory muscle strength, and physical activity in children with CF, PCD, and healthy children.
- Keywords
- cystic fibrosis, primary ciliary dyskinesia, muscle oxygenation, exercise tolerance, physical activity
- Medical subject headings
- Cystic Fibrosis, Ciliary Motility Disorders, Motor Activity
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Patient with Primary Ciliary Dyskinesia | Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), medical history (past and present history, family history, diagnosis, disease duration, smoking and environmental exposure, exacerbation status within the last year, number of hospital admissions and hospitalizations, medications used, parental consanguinity, socioeconomic status, number of siblings diagnosed with primary ciliary dyskinesia (PCD) or, cystic fibrosis (CF) body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals. | |
| Patient with Cystic Fibrosis | Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), medical history (past and present history, family history, diagnosis, disease duration, smoking and environmental exposure, exacerbation status within the last year, number of hospital admissions and hospitalizations, medications used, parental consanguinity, socioeconomic status, number of siblings diagnosed with primary ciliary dyskinesia (PCD) or, cystic fibrosis (CF) body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals. | |
| Health Controls | Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals. |
What is being measured
The primary outcome is the main question the study is designed to answer.
Functional Exercise Capacity
The six minute walk test (six-MWT) was used to assess functional exercise capacity. The six-MWT was administered according to the criteria of the American Thoracic Society and the European Respiratory Society. Heart rate at rest, after the test, and at the first minute of recovery were assessed using a heart rate monitor (Polar FTI00, China), blood pressure using a sphygmomanometer (Erka Perfect Aneroid, Germany), oxygen saturation using a portable pulse oximeter (Nonin Onyx Vantage 9590, Minnesota, USA), and respiratory frequency (counting the number of breaths taken per minute). The severity of dyspnea and body and leg fatigue was determined using the modified Borg Scale. The six-MWT was repeated twice. Walking distance was expressed in meters and as a percentage of the predicted value. The best walking distance result was selected for analysis. The percentage of the predicted walking distance values was calculated using the reference equation of Gibbons et al.
Measured: First Day
Muscle oxygenation (Resting muscle oxygen saturation (SmO2rest))
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the six-MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (Minimum muscle oxygen saturation (SmO2min))
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (Maximum muscle oxygen saturation (SmO2max))
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (ΔSmO2)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (SmO2averaged-min)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (SmO2averaged -max)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (ΔSmO2averaged)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (SmO2recovery)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (SmO2recovery-averaged)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (Resting total hemoglobin level (THbrest))
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded
Measured: First Day
Muscle oxygenation (Minumum total hemoglobin level (THbmin))
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (Maximum total hemoglobin level (Thbmax))
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (ΔTHb)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Muscle oxygenation (THbrecovery)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Measured: First Day
Secondary outcomes (20)
Heart rate
Heart rate at rest, after the test, and at the first minute of recovery were assessed using a heart rate monitor (Polar FTI00, China).
Measured: First day
Blood pressure
Blood pressure at rest, after the test, and at the first minute of recovery were assessed using a sphygmomanometer (Erka Perfect Aneroid).
Measured: First day
Oxygen saturation
Oxygen saturation at rest, after the test, and at the first minute of recovery were assessed using a portable pulse oximeter (Nonin Onyx Vantage 9590, Minnesota, USA).
Measured: First day
Breathing frequency
Breathing frequency at rest, after the test, and at the first minute of recovery were assessed using a counting the number of breaths taken per minute.
Measured: First day
Dyspnea
The severity of dyspnea was determined using the modified Borg Scale.
Measured: First day
Body and leg fatigue
The severity of body and leg fatigue was determined using the modified Borg Scale.
Measured: First day
Pulmonary function (Forced vital capacity (FVC))
Pulmonary function was assesed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced vital capacity (FVC) was assessed.
Measured: First Day
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced expiratory volume in the first second (FEV1) was assessed.
Measured: First Day
Pulmonary function (FEV1 / FVC)
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, FEV1 / FVC was assessed.
Measured: First Day
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, flow rate 25-75% of forced expiratory volume (FEF 25-75%) was assessed.
Measured: First Day
Pulmonary function (Peak flow rate (PEF))
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, peak flow rate (PEF) was assessed.
Measured: First Day
Respiratory Muscle Strength
Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength were measured using a portable mouth pressure measuring device according to American Thoracic Society and European Respiratory Society criteria
Measured: Second Day
Respiratory Muscle Endurance
Incremental threshold loading test
Measured: Second Day
Physical Activity Level (Total energy expenditure)
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second Day
Physical activity (Active energy expenditure (joule / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Active energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second Day
Physical activity (Physical activity time (min / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Physical activity time (min / day)will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second day
Physical activity (Average metabolic equivalent (MET / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Average metabolic equivalent (MET / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second Day
Physical activity (Number of steps (steps / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Number of steps (steps / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second Day
Physical activity (Time spent lying down (min / day) days))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Time spent lying down (min / day) days) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second Day
Physical activity (Sleep time (min / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient will be informed about removing the device while taking a bath. Sleep time (min / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Measured: Second Day
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted5 Sep 2025
- Study start1 Jan 2021
- Primary completion1 Feb 2025
- Study completion1 Mar 2025
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (9)Papers the sponsor has linked to this study.
- Other trials for Cystic Fibrosis (CF)Studies of the same condition, open and under way.
- More trials run by Gazi UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Other
- Time perspective
- Retrospective
- Enrolment
- 88 participants (actual)
Sponsor and investigators
- Lead sponsor
- Gazi University
- Sponsor type
- University, hospital or other
- Responsible party
- Principal investigator, Meral Boşnak Güçlü, Gazi University
- Investigators
- Şeyma MUTLU KAYAARSLAN, MSc, Principal investigator, Baskent UniversityMeral BOŞNAK GÜÇLÜ, Prof. Dr, Study director, Gazi UniversityBetül YOLERİ, MSc, Study chair, Gazi University
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07157644
- Sponsor's protocol ID
- Gazi2707
- Status last verified
- Jan 2026
- First submitted
- 23 Aug 2025
- Last updated
- 3 Feb 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Nakagomi A, Shoji T, Okada S, Ohno Y, Kobayashi Y. Validity of the augmentation index and pulse pressure amplification as determined by the SphygmoCor XCEL device: a comparison with invasive measurements. Hypertens Res. 2018 Jan;41(1):27-32. doi: 10.1038/hr.2017.81. Epub 2017 Oct 5.
PubMed 28978987Harun SN, Wainwright C, Klein K, Hennig S. A systematic review of studies examining the rate of lung function decline in patients with cystic fibrosis. Paediatr Respir Rev. 2016 Sep;20:55-66. doi: 10.1016/j.prrv.2016.03.002. Epub 2016 Mar 14.
PubMed 27259460Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, Bush A. Cystic fibrosis. Nat Rev Dis Primers. 2015 May 14;1:15010. doi: 10.1038/nrdp.2015.10.
PubMed 27189798Mutlu S, Bosnak Guclu M, Sismanlar Eyuboglu T, Aslan AT. Upper Extremity Exercise Capacity and Muscle Oxygenation in Patients With Primary Ciliary Dyskinesia. Pediatr Pulmonol. 2025 Jan;60(1):e27470. doi: 10.1002/ppul.27470.
PubMed 39785198Gosselink R, Troosters T, Decramer M. Peripheral muscle weakness contributes to exercise limitation in COPD. Am J Respir Crit Care Med. 1996 Mar;153(3):976-80. doi: 10.1164/ajrccm.153.3.8630582.
PubMed 8630582Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, Dell S, Eber E, Escudier E, Hirst RA, Hogg C, Jorissen M, Latzin P, Legendre M, Leigh MW, Midulla F, Nielsen KG, Omran H, Papon JF, Pohunek P, Redfern B, Rigau D, Rindlisbacher B, Santamaria F, Shoemark A, Snijders D, Tonia T, Titieni A, Walker WT, Werner C, Bush A, Kuehni CE. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017 Jan 4;49(1):1601090. doi: 10.1183/13993003.01090-2016. Print 2017 Jan.
PubMed 27836958Sharma R, Florea VG, Bolger AP, Doehner W, Florea ND, Coats AJ, Hodson ME, Anker SD, Henein MY. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax. 2001 Oct;56(10):746-50. doi: 10.1136/thorax.56.10.746.
PubMed 11562511Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. Eur Respir J Suppl. 1993 Mar;16:5-40. No abstract available.
PubMed 8499054de Onis M, Onyango AW, Borghi E, Siyam A, Nishida C, Siekmann J. Development of a WHO growth reference for school-aged children and adolescents. Bull World Health Organ. 2007 Sep;85(9):660-7. doi: 10.2471/blt.07.043497.
PubMed 18026621
Study at a glance
From the registry record, last updated by the sponsor on 3 Feb 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Comparison of Functional Exercise Capacity, Muscle Oxygenation, Respiratory Muscle Strength, and Physical Activity Level in Pediatric Cystic Fibrosis and Primary Ciliary Dyskinesia
- ClinicalTrials.gov ID
- NCT07157644
- Acronym / sponsor ID
- Gazi2707
- Keywords
- cystic fibrosis, primary ciliary dyskinesia, muscle oxygenation, exercise tolerance, physical activity
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 88 participants (actual)
- Lead sponsor
- Gazi UniversityUniversity, hospital or other
- Study start
- 1 Jan 2021 (actual)
- Primary completion
- 1 Feb 2025 (actual)
- Study completion
- 1 Mar 2025 (actual)
- First posted
- 5 Sep 2025 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are genetic diseases characterized by chronic respiratory tract infections. In both diseases, impaired mucociliary clearance, recurrent respiratory infections, and persistent inflammation lead to progressive deterioration in respiratory function. This condition limits patients' activities of daily living, leading to physical inactivity and exercise intolerance. Functional exercise capacity in patients with CF and PCD is reduced due to increased respiratory load, musculoskeletal involvement, and nutritional deficiencies. In exercise tests involving the upper and lower extremities, both patient groups exhibited significantly lower performance compared to healthy individuals. Muscle oxygenation is particularly reduced in patients with cystic fibrosis and is associated with inadequate oxygen delivery to peripheral muscles, mitochondrial dysfunction, and increased muscle fatigue. Although studies on muscle oxygenation in PCD patients are limited, it is thought to be affected by similar pathophysiological mechanisms. Respiratory muscle strength is weakened in both patient groups due to chronic cough, hyperinflation, and increased respiratory effort. This is particularly evident in a significant decrease in inspiratory and expiratory muscle strength. The number of studies in the literature evaluating muscle oxygenation, respiratory muscle strength, and physical activity levels in patients with CF and PCD is limited. There are no studies comparing muscle oxygenation between patients with CF and PCD.
Detailed description
In patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD), lower extremity exercise capacity, skeletal muscle function, respiratory muscle strength, and physical activity levels are limited by various pathophysiological mechanisms. In CF patients, lower extremity exercise capacity is significantly reduced due to ventilation limitation, respiratory muscle fatigue, and mitochondrial dysfunction. Early fatigue findings such as delayed oxygen uptake and lactate accumulation have been reported in lower extremity-specific exercise tests. In PCD patients, respiratory workload increases due to ventilation-perfusion mismatch and impaired mucociliary clearance, which can limit muscle oxygen utilization during exercise. Recent studies have shown that PCD patients have lower resting muscle oxygen saturation compared to healthy individuals, but these values are relatively maintained during exercise. In CF, respiratory muscle strength is weakened, particularly at the diaphragm and intercostal muscles, leading to a decrease in ventilatory reserve during exercise. Similarly, submaximal respiratory muscle fatigue and decreased inspiratory muscle strength have been reported in patients with PCD. Regarding physical activity levels, daily activity levels in both patient groups are significantly lower than in healthy peers, and this has been associated with disease progression, muscle dysfunction, and exercise intolerance. Objectively measured studies in children and adolescents with CF have reported that they fall below the recommended daily activity level, and this inadequacy negatively impacts muscle function over time. A similar tendency toward physical inactivity is also found in PCD patients, and this is considered directly related to exercise capacity. The number of studies in the literature evaluating muscle oxygenation, respiratory muscle strength, and physical activity levels in patients with CF and PCD is limited. There are no studies comparing muscle oxygenation in patients with CF and PCD. The aim of our study was to compare functional exercise capacity, muscle oxygenation, respiratory muscle strength, and physical activity in children with CF, PCD, and healthy children.
Study design
- Observational model
- Other
- Time perspective
- Retrospective
- Number of arms
- 3
| Arm | Type | Receives |
|---|---|---|
| Patient with Primary Ciliary Dyskinesia | – | –Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), medical history (past and present history, family history, diagnosis, disease duration, smoking and environmental exposure, exacerbation status within the last year, number of hospital admissions and hospitalizations, medications used, parental consanguinity, socioeconomic status, number of siblings diagnosed with primary ciliary dyskinesia (PCD) or, cystic fibrosis (CF) body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals. |
| Patient with Cystic Fibrosis | – | –Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), medical history (past and present history, family history, diagnosis, disease duration, smoking and environmental exposure, exacerbation status within the last year, number of hospital admissions and hospitalizations, medications used, parental consanguinity, socioeconomic status, number of siblings diagnosed with primary ciliary dyskinesia (PCD) or, cystic fibrosis (CF) body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals. |
| Health Controls | – | –Demographic information (age, gender, education level), physical characteristics (weight, height, BMI (Body Mass Index)), body weight, height, and BMI Z scores were recorded. Pulmonary function, respiratory muscle strength and endurance, functional exercise capacity, muscle oxygenation, and physical activity level were assessed for all individuals. |
Who may qualify
- Ages
- 6 to 18 years
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult
- Study population
- Thirty-one patients with CF, 27 patients with PSD were included in the study. There are 30 healthy data.
Key inclusion criteria (11 listed)
- Cystic fibrosis patients;
- Patients diagnosed with cystic fibrosis according to the American Cystic Fibrosis Association consensus report
- Between the ages of 6 and 18
- Clinically stable conditions
- Primary ciliary dyskinesia patients;
Key exclusion criteria (8 listed)
- Patients;
- Uncooperative
- Orthopedic or neurological disorders that will affect functional capacity
- Pneumonia or any acute infection
- Healthy controls;
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Functional Exercise Capacity
Time frame: First Day
The six minute walk test (six-MWT) was used to assess functional exercise capacity. The six-MWT was administered according to the criteria of the American Thoracic Society and the European Respiratory Society. Heart rate at rest, after the test, and at the first minute of recovery were assessed using a heart rate monitor (Polar FTI00, China), blood pressure using a sphygmomanometer (Erka Perfect Aneroid, Germany), oxygen saturation using a portable pulse oximeter (Nonin Onyx Vantage 9590, Minnesota, USA), and respiratory frequency (counting the number of breaths taken per minute). The severity of dyspnea and body and leg fatigue was determined using the modified Borg Scale. The six-MWT was repeated twice. Walking distance was expressed in meters and as a percentage of the predicted value. The best walking distance result was selected for analysis. The percentage of the predicted walking distance values was calculated using the reference equation of Gibbons et al.
Muscle oxygenation (Resting muscle oxygen saturation (SmO2rest))
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the six-MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Minimum muscle oxygen saturation (SmO2min))
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Maximum muscle oxygen saturation (SmO2max))
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (ΔSmO2)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2averaged-min)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2averaged -max)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (ΔSmO2averaged)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2recovery)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (SmO2recovery-averaged)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Resting total hemoglobin level (THbrest))
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded
Muscle oxygenation (Minumum total hemoglobin level (THbmin))
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (Maximum total hemoglobin level (Thbmax))
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (ΔTHb)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT, the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
Muscle oxygenation (THbrecovery)
Time frame: First Day
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6MWT the device was placed on the quadriceps muscle of the dominant leg, and measurements were recorded.
20 secondary outcomes
Heart rate
Time frame: First day
Blood pressure
Time frame: First day
Oxygen saturation
Time frame: First day
Breathing frequency
Time frame: First day
Dyspnea
Time frame: First day
Body and leg fatigue
Time frame: First day
Pulmonary function (Forced vital capacity (FVC))
Time frame: First Day
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Time frame: First Day
Pulmonary function (FEV1 / FVC)
Time frame: First Day
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Time frame: First Day
Pulmonary function (Peak flow rate (PEF))
Time frame: First Day
Respiratory Muscle Strength
Time frame: Second Day
Respiratory Muscle Endurance
Time frame: Second Day
Physical Activity Level (Total energy expenditure)
Time frame: Second Day
Physical activity (Active energy expenditure (joule / day))
Time frame: Second Day
Physical activity (Physical activity time (min / day))
Time frame: Second day
Physical activity (Average metabolic equivalent (MET / day))
Time frame: Second Day
Physical activity (Number of steps (steps / day))
Time frame: Second Day
Physical activity (Time spent lying down (min / day) days))
Time frame: Second Day
Physical activity (Sleep time (min / day))
Time frame: Second Day
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 3 Feb 2026.
- Results publication: PubMed 28978987Nakagomi A, Shoji T, Okada S, Ohno Y, Kobayashi Y. Validity of the augmentation index and pulse pressure amplification as determined by the SphygmoCor XCEL device: a comparison with invasive measurements. Hypertens Res. 2018 Jan;41(1):27-32. doi: 10.1038/hr.2017.81. Epub 2017 Oct 5.
- Results publication: PubMed 27259460Harun SN, Wainwright C, Klein K, Hennig S. A systematic review of studies examining the rate of lung function decline in patients with cystic fibrosis. Paediatr Respir Rev. 2016 Sep;20:55-66. doi: 10.1016/j.prrv.2016.03.002. Epub 2016 Mar 14.
- Results publication: PubMed 27189798Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, Bush A. Cystic fibrosis. Nat Rev Dis Primers. 2015 May 14;1:15010. doi: 10.1038/nrdp.2015.10.
- Results publication: PubMed 39785198Mutlu S, Bosnak Guclu M, Sismanlar Eyuboglu T, Aslan AT. Upper Extremity Exercise Capacity and Muscle Oxygenation in Patients With Primary Ciliary Dyskinesia. Pediatr Pulmonol. 2025 Jan;60(1):e27470. doi: 10.1002/ppul.27470.
- Results publication: PubMed 8630582Gosselink R, Troosters T, Decramer M. Peripheral muscle weakness contributes to exercise limitation in COPD. Am J Respir Crit Care Med. 1996 Mar;153(3):976-80. doi: 10.1164/ajrccm.153.3.8630582.
- Results publication: PubMed 27836958Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, Dell S, Eber E, Escudier E, Hirst RA, Hogg C, Jorissen M, Latzin P, Legendre M, Leigh MW, Midulla F, Nielsen KG, Omran H, Papon JF, Pohunek P, Redfern B, Rigau D, Rindlisbacher B, Santamaria F, Shoemark A, Snijders D, Tonia T, Titieni A, Walker WT, Werner C, Bush A, Kuehni CE. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017 Jan 4;49(1):1601090. doi: 10.1183/13993003.01090-2016. Print 2017 Jan.
- Results publication: PubMed 11562511Sharma R, Florea VG, Bolger AP, Doehner W, Florea ND, Coats AJ, Hodson ME, Anker SD, Henein MY. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax. 2001 Oct;56(10):746-50. doi: 10.1136/thorax.56.10.746.
- Results publication: PubMed 8499054Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. Eur Respir J Suppl. 1993 Mar;16:5-40. No abstract available.
- Results publication: PubMed 18026621de Onis M, Onyango AW, Borghi E, Siyam A, Nishida C, Siekmann J. Development of a WHO growth reference for school-aged children and adolescents. Bull World Health Organ. 2007 Sep;85(9):660-7. doi: 10.2471/blt.07.043497.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Şeyma MUTLU KAYAARSLAN, MSc principal investigator
Baskent University
Meral BOŞNAK GÜÇLÜ, Prof. Dr study director
Gazi University
Betül YOLERİ, MSc study chair
Gazi University
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Cystic Fibrosis (CF) and Primary Ciliary Dyskinesia (PCD), run by Gazi University at 1 site in Turkey (Türkiye).
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 11 inclusion and 8 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 3 Feb 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Turkey (Türkiye). Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Functional Exercise Capacity (time frame: First Day). The study lists 15 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT07157644, which also lists 9 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 3 Feb 2026.