Clinical trial NCT03370029: Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children Primary Ciliary Dyskinesia
Official title: Comparison of Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children With Primary Ciliary Dyskinesia and Healthy Controls
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Primary Ciliary Dyskinesia
Keywords: Exercise capacity
Respiratory muscle strength
Physical inactivity
Quality of life
Lead sponsor: Gazi University
Sponsor class: OTHER
First posted: 2017-12-12
Enrollment: 26
Sex: ALL
Minimum age (years): 6
Maximum age (years): 18
Standard ages: CHILD
ADULT
Accepts healthy volunteers: yes
Observational model: CASE_CONTROL
Time perspective: CROSS_SECTIONAL
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Out- patients and healthy volunteers

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.

Detailed description: According to sample size calculation 26 diagnosed primary ciliary dyskinesia patients and 26 healthy individuals will be included. Respiratory muscle strength, anaerobic and aerobic exercise capacity, physical activity, pulmonary functions, peripheral muscle strength, cough strength, respiratory muscle endurance, activity dyspnea perception, quality of life and depression will be evaluated. Primary outcome measurements are respiratory muscle strength, exercise capacity, physical activity; secondary outcomes are pulmonary functions, peripheral muscle strength, cough strength, respiratory muscle endurance, activity dyspnea perception, quality of life and depression.

Eligibility criteria: Inclusion Criteria:

* Diagnosed primary ciliary dyskinesia patients,
* 6-18 years of age,
* under standard medications,
* stable patients without exacerbation or infection

Exclusion Criteria:

* 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

Primary outcomes:
measure: Respiratory muscle strength
timeFrame: first day
measure: Functional exercise capacity
timeFrame: first day
measure: Anaerobic exercise capacity
timeFrame: first day
measure: Physical activity
timeFrame: first day
Secondary outcomes:
measure: Pulmonary functions
timeFrame: first day
measure: Muscle strength
timeFrame: second day
measure: Cough strength
timeFrame: second day
measure: Respiratory muscle endurance
timeFrame: second day]
measure: Activity dyspnea
timeFrame: first day
measure: Generic Quality of life
timeFrame: second day
measure: Disease- specific quality of life
timeFrame: second day
measure: Depression
timeFrame: second day
Other outcomes:
Locations:
city: Yenimahalle
country: Turkey
facility: Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation
Study officials:
affiliation: Research assistant
name: Merve Fırat, Pt
role: STUDY_CHAIR
affiliation: Associate professor
name: Meral Boşnak Güçlü, Pt,Phd
role: STUDY_DIRECTOR
affiliation: Medical doctor
name: Tuğba Şişmanlar Eyüboğlu, MD
role: PRINCIPAL_INVESTIGATOR
affiliation: Professor
name: Ayşe Tana Aslan, MD,Phd
role: PRINCIPAL_INVESTIGATOR
References:
citation: 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.
citation: 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.