Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance in Patients With Primary Ciliary Dyskinesia
Condition: Primary Ciliary Dyskinesia
MeSH terms: Ciliary Motility Disorders; Dyskinesias
Study type: Observational

Summary: 
      The main aim of the study is to evaluate upper extremity exercise capacity and muscle
      oxygenation in patients with primary ciliary dyskinesia. The secondary aim of the study is to
      evaluate respiratory function, respiratory muscle strength and endurance, peripheral muscle
      strength, balance, physical activity level and quality of life in patients with primary
      ciliary dyskinesia and compare all parameters with healthy controls.
    

Detailed description: 
      Primary ciliary dyskinesia (PCD) is a genetic, heterogeneous disease caused by cilia absence
      or dysfunction. Impaired function of motile cilia leads to impaired mucociliary clearance,
      recurrent chest infections, and progressive destruction of the lung structure. Clinical
      features include neonatal respiratory distress syndrome, chronic daily productive cough,
      chronic nasal congestion, and chronic or recurrent otitis media.

      Pulmonary functions, exercise capacity, respiratory muscle strength and endurance, peripheral
      muscle strength, physical activity level and quality of life were affected in patients. The
      number of studies on these subjects is limited.

      Pulmonary function and extremity muscle strength are determinants of exercise capacity in
      chronic obstructive pulmonary disease. Decreased strength and endurance in the limb muscles
      is associated with exercise intolerance. Patients have decreased upper extremity muscle
      strength. There is no study in the literature evaluating upper extremity exercise capacity in
      patients with PCD. Measuring the oxygen saturation of cardiorespiratory and peripheral
      muscles is important for understanding the dynamics of oxygen delivery and consumption during
      exercise. There are no studies in the literature evaluating muscle oxygenation in this
      patient population.

      The main aim of the study is to evaluate upper extremity exercise capacity and muscle
      oxygenation in patients with primary ciliary dyskinesia. The secondary aim of the study is to
      evaluate respiratory function, respiratory muscle strength and endurance, peripheral muscle
      strength, balance, physical activity level and quality of life in patients with primary
      ciliary dyskinesia and compare them with healthy controls.

      The study was planned cross-sectional. Patients with primary ciliary dyskinesia referred to
      the Cardiopulmonary Rehabilitation Unit of Gazi University Physiotherapy and Rehabilitation
      Department by the physicians of the Department of Pediatric Pulmonary Diseases of Gazi
      University Faculty of Medicine will be included in the study. At least 18 patients with
      primary ciliary dyskinesia and at least 18 healthy controls of similar age and sex will be
      evaluated in the study. The assessments will be completed in two days.

      Upper extremity exercise capacity (6 minutes Pegboard and Ring Test), muscle oxygenation
      ("Moxy" monitor), functional exercise capacity(6 minutes walk test), respiratory functions
      (spirometer), respiratory muscle strength (mouth pressure measurement), respiratory muscle
      endurance (incremental threshold loading test), peripheral muscle strength (dynamometer),
      balance("Biodex Balance System®" and Y balance test), physical activity level (multi-sensor
      activity monitor) and quality of life (Quality of Life scale for Primary Ciliary Dyskinesia
      (QOL-PCD scale version 4.3) will be evaluated.
    

Observational model: Case-Control
Time perspective: Cross-Sectional
Sampling method: Non-Probability Sample
Study population: 
        At least 18 patients with primary ciliary dyskinesia will be included in patients group and
        18 healthy individuals will be included in control group.
      
Keywords: Primary Ciliary Dyskinesia; Upper Extremity Exercise Capacity; Muscle Oxygenation; Balance; Physical Activity