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

Primary Ciliary Dyskinesia Clinical Trial

Official title:

Comparison of Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children With Primary Ciliary Dyskinesia and Healthy Controls

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT03370029
• Other study ID #: Gazi University5
• Status: Completed
• Start date: October 2016
• Est. completion date: June 30, 2018

Study information

• Verified date: February 2019
• Source: Gazi University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 26
• Est. completion date: June 30, 2018
• Est. primary completion date: February 25, 2018
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 6 Years to 18 Years

Eligibility

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

Related Conditions & MeSH terms

• Ciliary Motility Disorders
• Dyskinesias
• Kartagener Syndrome
• Primary Ciliary Dyskinesia

Locations

Country	Name	City	State
Turkey	Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation	Yenimahalle

Sponsors (1)

Lead Sponsor	Collaborator
Gazi University

Country where clinical trial is conducted

Turkey, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Respiratory muscle strength	Evaluated using mouth pressure device	first day
Primary	Functional exercise capacity	Evaluated using 6-minute walking test	first day
Primary	Anaerobic exercise capacity	Evaluated using 3-minute step test	first day
Primary	Physical activity	Evaluated using a metabolic holter	first day
Secondary	Pulmonary functions	Evaluated using spirometer	first day
Secondary	Muscle strength	Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength)	second day
Secondary	Cough strength	Evaluated using PEFmeter	second day
Secondary	Respiratory muscle endurance	Evaluated using respiratory muscle trainer (POWERbreathe)	second day]
Secondary	Activity dyspnea	Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome)	first day
Secondary	Generic Quality of life	Cystic Fibrosis Questionnaire Revised (CFQ-R) Turkish version (Scores range from 0 to 100, with higher scores indicating better health. 9 Quality of life domains: Physical, role/school, vitality, emotion, social, body image, eating, treatment burden, health perceptions.3 symptom scales: Weight, respiratory, and digestion.Number of items in CFQ-R CFQ-R Teen/Adult: 50. CFQ-R Parent: 44. CFQ-R Child: 35.)	second day
Secondary	Disease- specific quality of life	Health-related quality-of-life questionnaires-Primary Ciliary Dyskinesia ( PCD-QOL) Turkish version ( PCD-QOL questionnaire developed different domains for different age groups: pediatric, adolescent, and adult patients as well as parents. These domains mainly evaluate physical, emotional, and social aspects of PCD related to QOL. Moreover, there are different domains for various symptoms at different age groups. The total numbers of items in the questionnaires are 37 in the questionnaire for children, 43 in the one for adolescents, 49 in the one for adults, 41 in the parents' questionnaire.	second day
Secondary	Depression	Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale.	second day

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