Muscle Function, Exercise Capacity and Physical Activity Level in Primary Ciliary Dyskinesia and Kartagener Syndrome

NCT05816876 | Completed

• 1 other identifier: Gazi University 32
• Study Type: observational
• Target: 65
• Locations: 1 country
• Sites: 1

Brief Summary

Studies evaluating respiratory and peripheral muscle functions in PCD patients and comparing them with healthy children are limited in the literature. There is no study investigating pulmonary and extrapulmonary effects in Kartagener syndrome, which is a form of PCD. The aim of our study is to compare respiratory functions, respiratory muscle strength and endurance, exercise capacity, peripheral muscle strength, physical activity level and quality of life in patients with PCD, Kartagener syndrome and healthy children.

Conditions

Primary Ciliary Dyskinesia

Keywords

primary ciliary dyskinesia; exercise capacity; muscle strength; physical activity

Outcome Measures

Primary Outcomes (1)

• Functional exercise capacity

  Functional exercise capacity will be evaluated with the 6- Minute Walk Test. 6- Minute Walk Test will be applied according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) criteria.

  First Day

Secondary Outcomes (16)

• Physical activity (Total energy expenditure)

  Second Day

• Physical activity (Active energy expenditure (joule / day))

  Second Day

• Physical activity (Physical activity time (min / day))

  Second Day

• Physical activity (Average metabolic equivalent (MET / day))

  Second Day

• Physical activity (Number of steps (steps / day))

  Second Day

Study Arms (3)

Patients with primary ciliary dyskinesia

Exercise capacity assessed using six minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version).

Patients with Kartagener Syndrome

Exercise capacity assessed using six minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version).

Healthy controls

Exercise capacity assessed using six minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version).

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

Forty patients with primary ciliary dyskinesia/Kartagener's Syndrome were recruited. It also has 60 healthy data.

You may qualify if:

• Patients;
• Individuals aged 6-18 years, who were diagnosed with PCD and Kartagener syndrome, and received standard medical treatment, were included in the study.
• Healthy controls;
• Individuals between the ages of 6 and 18 without a known chronic disease 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 Facutly of Health Sciences Department of Physiotheraphy and Rehabilitation, Cardiopulmonary Rehabilitation Unit

Ankara, Çankaya, 06490, Turkey (Türkiye)

Location

Related Publications (9)

• Hogg C. Primary ciliary dyskinesia: when to suspect the diagnosis and how to confirm it. Paediatr Respir Rev. 2009 Jun;10(2):44-50. doi: 10.1016/j.prrv.2008.10.001. Epub 2009 Apr 9.

  PMID: 19410200 BACKGROUND

• Ortega HA, Vega Nde A, Santos BQ, Maia GT. Primary ciliary dyskinesia: considerations regarding six cases of Kartagener syndrome. J Bras Pneumol. 2007 Sep-Oct;33(5):602-8. doi: 10.1590/s1806-37132007000500017. English, Portuguese.

  PMID: 18026660 BACKGROUND

• Takeuchi K, Kitano M, Ishinaga H, Kobayashi M, Ogawa S, Nakatani K, Masuda S, Nagao M, Fujisawa T. Recent advances in primary ciliary dyskinesia. Auris Nasus Larynx. 2016 Jun;43(3):229-36. doi: 10.1016/j.anl.2015.09.012. Epub 2015 Oct 31.

  PMID: 26527516 BACKGROUND

• Marthin JK, Petersen N, Skovgaard LT, Nielsen KG. Lung function in patients with primary ciliary dyskinesia: a cross-sectional and 3-decade longitudinal study. Am J Respir Crit Care Med. 2010 Jun 1;181(11):1262-8. doi: 10.1164/rccm.200811-1731OC. Epub 2010 Feb 18.

  PMID: 20167855 BACKGROUND

• Arikan H, Yatar I, Calik-Kutukcu E, Aribas Z, Saglam M, Vardar-Yagli N, Savci S, Inal-Ince D, Ozcelik U, Kiper N. A comparison of respiratory and peripheral muscle strength, functional exercise capacity, activities of daily living and physical fitness in patients with cystic fibrosis and healthy subjects. Res Dev Disabil. 2015 Oct-Nov;45-46:147-56. doi: 10.1016/j.ridd.2015.07.020. Epub 2015 Aug 1.

  PMID: 26241869 BACKGROUND

• Firat M, Bosnak-Guclu M, Sismanlar-Eyuboglu T, Tana-Aslan A. Respiratory muscle strength, exercise capacity and physical activity in patients with primary ciliary dyskinesia: A cross-sectional study. Respir Med. 2022 Jan;191:106719. doi: 10.1016/j.rmed.2021.106719. Epub 2021 Dec 17.

  PMID: 34952415 BACKGROUND

• Simsek S, Inal-Ince D, Cakmak A, Emiralioglu N, Calik-Kutukcu E, Saglam M, Vardar-Yagli N, Ozcelik HU, Sonbahar-Ulu H, Bozdemir-Ozel C, Kiper N, Arikan H. Reduced anaerobic and aerobic performance in children with primary ciliary dyskinesia. Eur J Pediatr. 2018 May;177(5):765-773. doi: 10.1007/s00431-018-3121-2. Epub 2018 Feb 27.

  PMID: 29487997 BACKGROUND

• Denizoglu Kulli H, Gurses HN, Zeren M, Ucgun H, Cakir E. Do pulmonary and extrapulmonary features differ among cystic fibrosis, primary ciliary dyskinesia, and healthy children? Pediatr Pulmonol. 2020 Nov;55(11):3067-3073. doi: 10.1002/ppul.25052. Epub 2020 Sep 11.

  PMID: 32877003 BACKGROUND

• Madsen A, Green K, Buchvald F, Hanel B, Nielsen KG. Aerobic fitness in children and young adults with primary ciliary dyskinesia. PLoS One. 2013 Aug 19;8(8):e71409. doi: 10.1371/journal.pone.0071409. eCollection 2013.

  PMID: 23977038 BACKGROUND

MeSH Terms

Conditions

Ciliary Motility Disorders; Motor Activity

Condition Hierarchy (Ancestors)

Respiratory Tract Diseases; Otorhinolaryngologic Diseases; Ciliopathies; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Behavior

Study Officials

• Meral Boşnak Güçlü, Prof. Dr.

  Gazi University

  STUDY DIRECTOR

• Merve Fırat, MD

  Hacettepe University

  STUDY CHAIR

• Şeyma Mutlu, MD

  Baskent University

  PRINCIPAL INVESTIGATOR

• Betül Yoleri, MD

  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. Dr.

Study Record Dates

• First Submitted: April 4, 2023
• First Posted: April 18, 2023
• Study Start: November 10, 2016
• Primary Completion: January 20, 2023
• Study Completion: February 1, 2023
• Last Updated: April 18, 2023

Record last verified: 2023-04

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)