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NCT Number: NCT03370029

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

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.

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Key information

Conditions

Primary Ciliary Dyskinesia Abnormalities, Multiple Behavior Ciliary Motility Disorders Ciliopathies Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Otorhinolaryngologic Diseases Respiratory Tract Diseases Sedentary Behavior

Age range

6 year–18 year

Sex eligibility

All sexes

Study type

Observational

Primary location

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

Yenimahalle, Turkey (Türkiye)

About this study

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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Primary outcomes

  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 outcomes

  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.

Sponsors and collaborators

Lead sponsor

Gazi University

Other

Registry information

Official study title

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

Important dates

Study start
2016
Primary completion
2018
Study completion
2018
First posted
Dec 12, 2017
Registry last updated
Feb 15, 2019

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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