Studies evaluating respiratory and peripheral muscle functions in PCD patients and comparing them with healthy children are limited in the literature. There is no study investigating pulmonary and extrapulmonary effects in Kartagener syndrome, which is a form of PCD. The aim of our study is to compare respiratory functions, respiratory muscle strength and endurance, exercise capacity, peripheral muscle strength, physical activity level and quality of life in patients with PCD, Kartagener syndrome and healthy children.
Primary ciliary dyskinesia (PCD) is an autosomal recessive disease characterized by abnormal ciliary movement and impaired mucociliary clearance. Kartagener syndrome is an autosomal recessive primary ciliary dyskinesia syndrome characterized by situs inversus, bronchiectasis and chronic sinusitis and is the most serious form of PCD, accounting for 50% of all PCD cases. In these syndromes, there is both movement and structural defect in the cilia, and mucociliary clearance is impaired. Symptoms usually appear in childhood. Organ anomalies can also be seen in Kartagener syndrome. Primary ciliary dyskinesia is a disease that threatens lung functions starting from preschool age. One of the main causes of respiratory muscle weakness in chronic lung diseases is worsening of lung functions. Evaluation of exercise capacity in patients with PCD has prognostic importance. Decreased exercise capacity, respiratory function, and cardiopulmonary function cause physical inactivity. In chronic lung diseases, sedentary lifestyle, airway obstruction, malnutrition and decreased exercise capacity cause muscle weakness. Muscle strength and function are important for performing activities of daily living. There is no study in the literature comparing respiratory and physical functions in children with PCD and in children with Kartagener's syndrome, which is the most serious form of PCD. The aim of our study is to compare respiratory functions, respiratory muscle strength and endurance, exercise capacity, peripheral muscle strength, physical activity level and quality of life in patients with PCD, Kartagener syndrome and healthy children. The study was planned as a cross-sectional, retrospective. Individuals aged 6-18 years, who were diagnosed with PCD and Kartagener syndrome, and received standard medical treatment, were included in the study. For the healthy group, individuals aged 6-18 years without a known chronic disease were included. Individuals' exercise capacity, respiratory functions, physical activity levels, peripheral and respiratory muscle strength, respiratory muscle endurance and quality of life will be evaluated. Exercise capacity evaluated using six-minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand-held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version). The assessments will be completed in two days.
Gazi University Facutly of Health Sciences Department of Physiotheraphy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
Ankara, Çankaya, Turkey (Türkiye)
Functional exercise capacity
Functional exercise capacity will be evaluated with the 6- Minute Walk Test. 6- Minute Walk Test will be applied according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) criteria.
Time frame: First Day
Physical activity (Total energy expenditure)
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Active energy expenditure (joule / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Active energy expenditure (joule / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Physical activity time (min / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Physical activity time (min / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
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Study Type
OBSERVATIONAL
Enrollment
65
Time frame: Second Day
Physical activity (Average metabolic equivalent (MET / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Average metabolic equivalent (MET / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Number of steps (steps / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Number of steps (steps / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Time spent lying down (min / day) days))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Time spent lying down (min / day) days) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Physical activity (Sleep time (min / day))
Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Sleep time (min / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: Second Day
Pulmonary function (Forced vital capacity (FVC))
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced vital capacity (FVC) was evaluated.
Time frame: First Day
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced expiratory volume in the first second (FEV1) was evaluated.
Time frame: First Day
Pulmonary function (FEV1 / FVC)
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, FEV1 / FVC was evaluated.
Time frame: First Day
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, flow rate 25-75% of forced expiratory volume (FEF 25-75%) was evaluated.
Time frame: First Day
Pulmonary function (Peak flow rate (PEF))
Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, peak flow rate (PEF) was evaluated.
Time frame: First Day
Respiratory muscle strength
Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength were measured with a portable mouth pressure measuring device according to ATS and ERS criteria.
Time frame: First Day
Peripheral muscle strength
Isometric peripheral muscle strength was measured with a portable hand dynamometer (JTECH Commander, USA). Measurements were repeated on the shoulder abductors and knee extensors three times on the right and left.
Time frame: Second day
Respiratory muscle endurance
Respiratory muscle endurance was assessed by the POWERbreathe Wellness (POWERbreathe, Inspiratory Muscle Training (IMT) Technologies Ltd., Birmingham, UK) device and the respiratory muscle endurance test at increased threshold load. The test was started with 20% of the maximal inspiratory pressure and the pressure was increased to 40%, 60%, 80% and 100% every two minutes. Patients were asked to continue breathing through the device during the test. During the test, the number of breaths delivered and the maximal time reached during each 2-minute period were recorded. If the individual could not breathe 3 consecutive times, the test was terminated by the physiotherapist. The total duration of the test and the maximum pressure value at which it continues to breathe for at least 1 minute was multiplied. The value found was recorded as the respiratory muscle endurance value.
Time frame: Second Day
Life quality
It was evaluated with the Turkish version of the disease-specific Primary Ciliary Dyskinesia Quality of Life scale. The questions were about physical function, emotional function, social function, respiratory symptoms, treatment burden, ear and hearing, sinus symptoms, role function, health perceptions, school functioning, eating and weight problems.
Time frame: Second Day