Exercise Capacity, Muscle Oxygenation, Respiratory Muscle Strength, and Physical Activity Level in Pediatric CF and PCD

NCT07157644 | Completed

• 1 other identifier: Gazi2707
• Study Type: observational
• Target: 88
• Locations: 1 country
• Sites: 1

Brief 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. 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.

Conditions

Primary Ciliary Dyskinesia (PCD); Cystic Fibrosis (CF)

Keywords

cystic fibrosis; primary ciliary dyskinesia; muscle oxygenation; exercise tolerance; physical activity

Outcome Measures

Primary Outcomes (15)

• 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.

  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.

  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.

  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.

  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.

  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.

  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.

  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.

  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.

  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.

  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

  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.

  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.

  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.

  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.

  First Day

Secondary Outcomes (20)

• Heart rate

  First day

• Blood pressure

  First day

• Oxygen saturation

  First day

• Breathing frequency

  First day

• Dyspnea

  First day

Study Arms (3)

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.

Eligibility Criteria

• Age: 6 Years - 18 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

Thirty-one patients with CF, 27 patients with PSD were included in the study. There are 30 healthy data.

You may qualify if:

• 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
• 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

You may not qualify if:

• 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

Sponsors & Collaborators

• Gazi University: lead

Study Sites (1)

Gazi University Faculty of Health Sciences Department of Cardiopulmonary Physiotherapy and Rehabilitation

Ankara, Çankaya, 06490, Turkey (Türkiye)

Location

Related Publications (9)

• 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.

  PMID: 28978987 RESULT

• Harun 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.

  PMID: 27259460 RESULT

• Ratjen 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.

  PMID: 27189798 RESULT

• Mutlu 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.

  PMID: 39785198 RESULT

• Gosselink 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.

  PMID: 8630582 RESULT

• Lucas 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.

  PMID: 27836958 RESULT

• Sharma 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.

  PMID: 11562511 RESULT

• Quanjer 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.

  PMID: 8499054 RESULT

• de 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.

  PMID: 18026621 RESULT

MeSH Terms

Conditions

Cystic Fibrosis; Ciliary Motility Disorders; Motor Activity

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases; Otorhinolaryngologic Diseases; Ciliopathies; Abnormalities, Multiple; Congenital Abnormalities; Behavior

Study Officials

• Şeyma MUTLU KAYAARSLAN, MSc

  Baskent University

  PRINCIPAL INVESTIGATOR

• Meral BOŞNAK GÜÇLÜ, Prof. Dr

  Gazi University

  STUDY DIRECTOR

• Betül YOLERİ, MSc

  Gazi University

  STUDY CHAIR

Study Design

• Study Type: observational
• Observational Model: OTHER
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Prof. Dr.

Study Record Dates

• First Submitted: August 23, 2025
• First Posted: September 5, 2025
• Study Start: January 1, 2021
• Primary Completion: February 1, 2025
• Study Completion: March 1, 2025
• Last Updated: February 3, 2026

Record last verified: 2026-01

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)