Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children Primary Ciliary Dyskinesia

NCT03370029 | Completed

• 1 other identifier: Gazi University5
• Study Type: observational
• Target: 26
• Locations: 1 country
• Sites: 1

Brief Summary

Primary ciliary dyskinesia is an autosomal recessive disorder characterized by abnormal ciliary movement and disrupted mucociliary clearance. In uncleaned airways, microorganisms and respiratory irritants cause inflammation and infection. Permanent rhinitis and chronic sputum cough are typical features in primary ciliary dyskinesia patients. Primary ciliary dyskinesia is a disease that threatens lung function from pre-school age. One of the main causes of respiratory muscle weakness in chronic lung diseases diseases is worsening of lung function. Such a weakness causes alveolar hypoventilation, microatelectasis, reduction of the cough strength .The cough strength is important for airway cleaning. Exercise capacity is affected in chronic lung diseases. Assessment of exercise capacity in chronic lung diseases is prognostically important. Reduced exercise capacity and pulmonary function in PCD cause decrease in physical activity level. PCD patients have low quality of life and early recognition has been found to affect the quality of life positively. Children with chronic illness have higher level of depression than healthy children. In literature, no study investigated respiratory muscle strength, exercise capacity and physical activity PCD patients. Therefore, the investigators aimed to compare aforementioned outcomes in PCD patients and healthy controls.

Conditions

Primary Ciliary Dyskinesia

Keywords

Exercise capacity; Respiratory muscle strength; Physical inactivity; Quality of life

Outcome Measures

Primary Outcomes (4)

• Respiratory muscle strength

  Evaluated using mouth pressure device

  first day

• Functional exercise capacity

  Evaluated using 6-minute walking test

  first day

• Anaerobic exercise capacity

  Evaluated using 3-minute step test

  first day

• Physical activity

  Evaluated using a metabolic holter

  first day

Secondary Outcomes (8)

• Pulmonary functions

  first day

• Muscle strength

  second day

• Cough strength

  second day

• Respiratory muscle endurance

  second day]

• Activity dyspnea

  first day

Study Arms (2)

Primary ciliary dyskinesia patients

Primary ciliary dyskinesia patients will be included in study. Inclusion and exclusion criteria were considered.

Healthy individuals

Those without diagnosed chronic disease will be included in study. Inclusion and exclusion criteria were considered.

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

Out- patients and healthy volunteers

You may qualify if:

• Diagnosed primary ciliary dyskinesia patients,
• years of age,
• under standard medications,
• stable patients without exacerbation or infection

You may not qualify if:

• having cognitive disorder,
• orthopedic or neurological disease with a potential to affect functional capacity,
• acute infections or pneumonia,
• problems which may prevent evaluating such as visual problems

Sponsors & Collaborators

• Gazi University: lead

Study Sites (1)

Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation

Yenimahalle, Turkey (Türkiye)

Location

Related Publications (2)

• Firat M, Bosnak-Guclu M, Sismanlar-Eyuboglu T, Aslan AT. Aerobic exercise capacity, cough strength, posture, and depression in primary ciliary dyskinesia. Pediatr Pulmonol. 2023 Jan;58(1):73-80. doi: 10.1002/ppul.26158. Epub 2022 Sep 28.

  PMID: 36114723 DERIVED

• 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 DERIVED

Related Links

• Related Info
• Related Info

MeSH Terms

Conditions

Ciliary Motility Disorders; Sedentary Behavior

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

• Merve Fırat, Pt

  Research assistant

  STUDY CHAIR

• Meral Boşnak Güçlü, Pt,Phd

  Associate professor

  STUDY DIRECTOR

• Tuğba Şişmanlar Eyüboğlu, MD

  Medical doctor

  PRINCIPAL INVESTIGATOR

• Ayşe Tana Aslan, MD,Phd

  Professor

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Associate professor

Study Record Dates

• First Submitted: November 10, 2017
• First Posted: December 12, 2017
• Study Start: October 1, 2016
• Primary Completion: February 25, 2018
• Study Completion: June 30, 2018
• Last Updated: February 15, 2019

Record last verified: 2019-02

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)