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
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.
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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