Upper Extremity Exercise Capacity, Muscle Oxygenation, and Balance in Pediatric CF and PCD

NCT07148362 | Completed

• 1 other identifier: 2025-557
• Study Type: observational
• Target: 88
• Locations: 1 country
• Sites: 1

Brief Summary

The number of studies evaluating respiratory functions and upper extremity functional exercise capacity in patients with CF and PCD is limited in the literature. There are no studies comparing upper extremity functional exercise capacity and upper extremity muscle oxygenation in patients with CF and PCD. The aim of our study is to compare respiratory function, upper extremity functional exercise capacity, peripheral muscle strength, muscle oxygenation, and balance in CF, PCD, and healthy children.

Conditions

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

Keywords

cystic fibrosis; primary ciliary dykinesia; balance; muscle oxygenation; exercise tolerance; upper extremity

Outcome Measures

Primary Outcomes (1)

• Upper extremity functional exercise capacity

  Upper extremity functional exercise capacity was assessed with a 6-minute pegboard and ring test (6-PBRT). Subjects were instructed to move as many rings as possible for six minutes. Before and after each test and during the first minute of recovery, HR, SpO2, blood pressure, respiratory frequency, perceived dyspnea, and fatigue were recorded. Total ring counts were recorded as the outcome measure.

  First day

Secondary Outcomes (22)

• Pulmonary function (Forced vital capacity (FVC))

  First day

• Pulmonary function (Forced expiratory volume in the first second (FEV1))

  First day

• Pulmonary function (FEV1 / FVC)

  First day

• Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))

  First day

• Pulmonary function (Peak flow rate (PEF))

  First day

Study Arms (3)

Patients with Cystic fibrosis

Upper extremity functional exercise capacity was evaluated using the 6-minute pegboard and ring test, pulmonary function using spirometry, peripheral muscle strength using a hand-held dynamometer, muscle oxygenation using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD), static balance using the "Biosway Portable Balance System", and dynamic balance assessment using the Y balance test.

Patients with Primary ciliary dyskinesia

Upper extremity functional exercise capacity was evaluated using the 6-minute pegboard and ring test, pulmonary function using spirometry, peripheral muscle strength using a hand-held dynamometer, muscle oxygenation using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD), static balance using the "Biosway Portable Balance System", and dynamic balance assessment using the Y balance test.

Healthy controls

Upper extremity functional exercise capacity was evaluated using the 6-minute pegboard and ring test, pulmonary function using spirometry, peripheral muscle strength using a hand-held dynamometer, muscle oxygenation using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD), static balance using the "Biosway Portable Balance System", and dynamic balance assessment using the Y balance test.

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 between the ages of 6-18 years who are diagnosed with cystic fibrosis according to the American Cystic Fibrosis Association consensus report and whose clinical condition is stable will be included in the study.
• Primary ciliary dyskinesia patients • Patients between the ages of 6-18 years who are diagnosed with primary ciliary dyskinesia according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) guidelines and whose clinical condition was stable will be included in the study.
• Healthy controls
• Healthy individuals of both sexes between the ages of 6-18 who voluntarily agreed to participate in the study will be included.

You may not qualify if:

• Patients
• Patients who are uncooperative, have orthopedic or neurological disorders that will affect functional capacity, and have pneumonia or any acute infection during the evaluation will be excluded from the study.
• Healthy controls;
• Those with a known chronic disease, uncooperative and orthopedic or neurological disorders that will affect functional capacity will not be included.

Sponsors & Collaborators

• Gazi University: lead

Study Sites (1)

Gazi University Faculty of Health Sciences Department of Physiotherapy ana Rehabilitation, Cardiopulmonary Rehabilitation Unit

Ankara, Çankaya, 06490, Turkey (Türkiye)

Location

Related Publications (8)

• 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 BACKGROUND

• 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 BACKGROUND

• 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 BACKGROUND

• 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 BACKGROUND

• Souza RP, Donadio MVF, Heinzmann-Filho JP, Baptista RR, Pinto LA, Epifanio M, Marostica PJC. THE USE OF ULTRASONOGRAPHY TO EVALUATE MUSCLE THICKNESS AND SUBCUTANEOUS FAT IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS. Rev Paul Pediatr. 2018 Oct-Dec;36(4):457-465. doi: 10.1590/1984-0462/;2018;36;4;00015.

  PMID: 30540111 BACKGROUND

• Halbeisen FS, Goutaki M, Spycher BD, Amirav I, Behan L, Boon M, Hogg C, Casaulta C, Crowley S, Haarman EG, Karadag B, Koerner-Rettberg C, Loebinger MR, Mazurek H, Morgan L, Nielsen KG, Omran H, Santamaria F, Schwerk N, Thouvenin G, Yiallouros P, Lucas JS, Latzin P, Kuehni CE. Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study. Eur Respir J. 2018 Aug 23;52(2):1801040. doi: 10.1183/13993003.01040-2018. Print 2018 Aug.

  PMID: 30049738 BACKGROUND

• 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 BACKGROUND

• 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 BACKGROUND

MeSH Terms

Conditions

Cystic Fibrosis; Ciliary Motility Disorders

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

Study Officials

• Şeyma MUTLU KAYAARSLAN, MSc

  Baskent University

  STUDY CHAIR

• Meral BOŞNAK GÜÇLÜ, Prof. Dr

  Gazi University

  STUDY DIRECTOR

• Betül YOLERİ, MSc

  Gazi University

  PRINCIPAL INVESTIGATOR

Study Design

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

Study Record Dates

• First Submitted: August 22, 2025
• First Posted: August 29, 2025
• Study Start: January 1, 2021
• Primary Completion: February 1, 2025
• Study Completion: March 1, 2025
• Last Updated: September 8, 2025

Record last verified: 2025-09

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)