Investigation of Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance and Physical Activity Levels in Patients With Primary Ciliary Dyskinesia
Overview
- Phase
- N/A
- Intervention
- Not specified
- Conditions
- Primary Ciliary Dyskinesia
- Sponsor
- Gazi University
- Enrollment
- 54
- Locations
- 2
- Primary Endpoint
- Upper extremity exercise capacity
- Status
- Completed
- Last Updated
- 3 years ago
Overview
Brief 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.
Investigators
Meral Boşnak Güçlü
Prof. Dr.
Gazi University
Eligibility Criteria
Inclusion Criteria
- •6-18 years old
- •Diagnosed with primary ciliary dyskinesia
- •Stability of clinical condition
- •Healthy controls;
- •6-18 years old
Exclusion Criteria
- •Diagnosed neurological, cognitive problem that can affect evaluations,
- •Acute pneumonia or any infection
- •History of exacerbation in the last 1 month
- •More than 10% change in FEV1 in the last 6 months
- •History of coronavirus disease (COVID-19)
- •History of smoking
- •Diagnosed vision, hearing, vestibular, or neurological problems that can affect balance
- •Diagnosed orthopedic problems affecting mobility or a history of musculoskeletal surgery
- •Healthy controls;
- •Diagnosed neurological, cognitive problem that can affect evaluations
Outcomes
Primary Outcomes
Upper extremity exercise capacity
Time Frame: First Day
Upper extremity exercise capacity will be evaluated with the six-minute pegboard and ring test (6-PBRT). A pegboard with two upper and lower bars set at participants shoulder level and above the shoulder level will used. Ten rings will placed on both the lower bars. Patients will be asked to move a single ring at a time with both hands from the lower bars to the upper. The score is the total number of rings moved the six-minute period.
Muscle Oxygenation
Time Frame: First Day
Muscle oxygenation assessment will be performed using the Moxy monitor (Moxy, Fortiori Design LLC, Minnesota, USA). The device will be placed on the 1/3 lower motor point of the quadriceps muscle group of the dominant leg and on the dominant arm deltoid muscle. A minimum of 3 minutes will be waited until the resting measurements and skeletal muscle oxygenation (StO2) signal stabilize. The measurements will be repeated during six minute walking test and six minute pegboard and ring test.
Secondary Outcomes
- Physical activity (Physical activity time (min / day)(Second Day)
- Physical activity (Total energy expenditure)(Second day)
- Physical activity (Sleep time (min / day)(Second Day)
- Pulmonary function (Peak flow rate (PEF))(First Day)
- Physical activity (Average metabolic equivalent (MET / day)(Second Day)
- Respiratory muscle strength(First day)
- Life quality(Second day)
- Physical activity (Active energy expenditure (joule / day)(Second day)
- Functional exercise capacity(First Day)
- Balance(Second day)
- Physical activity (Number of steps (steps / day)(Second Day)
- Pulmonary function (FEV1 / FVC)(First Day)
- Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))(First Day)
- Physical activity (Time spent lying down (min / day) days)(Second Day)
- Respiratory muscle endurance(Second Day)
- Pulmonary function (Forced vital capacity (FVC))(First Day)
- Pulmonary function (Forced expiratory volume in the first second (FEV1))(First Day)