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

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT03370029
Recruitment Status : Completed
First Posted : December 12, 2017
Last Update Posted : February 15, 2019
Information provided by (Responsible Party):
Meral Boşnak Güçlü, Gazi University

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.

Condition or disease
Primary Ciliary Dyskinesia

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.

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Study Type : Observational
Actual Enrollment : 26 participants
Observational Model: Case-Control
Time Perspective: Cross-Sectional
Official Title: Comparison of Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children With Primary Ciliary Dyskinesia and Healthy Controls
Actual Study Start Date : October 2016
Actual Primary Completion Date : February 25, 2018
Actual Study Completion Date : June 30, 2018

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.

Primary Outcome Measures :
  1. Respiratory muscle strength [ Time Frame: first day ]
    Evaluated using mouth pressure device

  2. Functional exercise capacity [ Time Frame: first day ]
    Evaluated using 6-minute walking test

  3. Anaerobic exercise capacity [ Time Frame: first day ]
    Evaluated using 3-minute step test

  4. Physical activity [ Time Frame: first day ]
    Evaluated using a metabolic holter

Secondary Outcome Measures :
  1. Pulmonary functions [ Time Frame: first day ]
    Evaluated using spirometer

  2. Muscle strength [ Time Frame: second day ]
    Evaluated using hand-held dynamometer for peripheral muscles (shoulder abduction, elbow flexion, knee extension, hand grip strength)

  3. Cough strength [ Time Frame: second day ]
    Evaluated using PEFmeter

  4. Respiratory muscle endurance [ Time Frame: second day] ]
    Evaluated using respiratory muscle trainer (POWERbreathe)

  5. Activity dyspnea [ Time Frame: first day ]
    Evaluated using Modified Borg Scale (0-10 scores, higher values represent a worse outcome)

  6. Generic Quality of life [ Time Frame: second day ]

    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.)

  7. Disease- specific quality of life [ Time Frame: second day ]
    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.

  8. Depression [ Time Frame: second day ]
    Children's Depression Inventory Turkish Version (Children's Depression Inventory is a 27-item, self-rated, symptom-oriented scale.

Information from the National Library of Medicine

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Ages Eligible for Study:   6 Years to 18 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
Out- patients and healthy volunteers

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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT03370029

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Gazi University Faculty of Health Sciences Department of Physical Therapy and Rehabilitation
Yenimahalle, Turkey
Sponsors and Collaborators
Gazi University
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Study Chair: Merve Fırat, Pt Research assistant
Study Director: Meral Boşnak Güçlü, Pt,Phd Associate professor
Principal Investigator: Tuğba Şişmanlar Eyüboğlu, MD Medical doctor
Principal Investigator: Ayşe Tana Aslan, MD,Phd Professor
Additional Information:
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Responsible Party: Meral Boşnak Güçlü, Associate professor, Gazi University Identifier: NCT03370029    
Other Study ID Numbers: Gazi University5
First Posted: December 12, 2017    Key Record Dates
Last Update Posted: February 15, 2019
Last Verified: February 2019
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No
Plan Description: There is not a plan to make individual participant data but when the statistical analyses of all data are made, all results will be shared.
Keywords provided by Meral Boşnak Güçlü, Gazi University:
Exercise capacity
Respiratory muscle strength
Physical inactivity
Quality of life
Additional relevant MeSH terms:
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Ciliary Motility Disorders
Movement Disorders
Central Nervous System Diseases
Nervous System Diseases
Neurologic Manifestations
Respiratory Tract Diseases
Otorhinolaryngologic Diseases
Abnormalities, Multiple
Congenital Abnormalities
Genetic Diseases, Inborn