This study aims to compare the maximal exercise capacity, functional exercise capacity, respiratory functions, physical activity level, quality of life, respiratory muscle strength and endurance, peripheral muscle strength, muscle oxygenation and dyspnea in children with primary immunodeficiency (PID) and healthy individuals. The study was designed as a cross-sectional study on 26 patients diagnosed with primary immunodeficiency and 26 age- and sex-matched healthy individuals. Differences between both groups will be evaluated statistically. The results of this study aim to reveal how primary immunodeficiency patients are affected by factors such as respiratory functions, physical activity level and quality of life
Primary immune deficiencies (PIDs) are a heterogeneous group of diseases resulting from various abnormalities that affect the development, differentiation, and/or function of cells and components of the immune system. PIDs are clinically characterized by increased susceptibility to infections, autoimmune diseases, auto-inflammatory disorders, allergies, bone marrow failure, and/or malignancies. Although individually rare, the total number of individuals with PIDs constitutes a significant health burden. The prevalence and distribution of PIDs vary among populations, with an estimated prevalence of 1/1,000 to 1/5,000. The specific prevalence of PIDs in Turkey is not well known due to the lack of extensive studies and a national registry system. However, a study encompassing two centers in Turkey reported a prevalence of 30.5/100,000. Currently, approximately 500 PID diseases have been identified, with selective immunoglobulin A (IgA) deficiency being the most common and common variable immunodeficiency (CVID) being the most common symptomatic PID. Pulmonary complications are highly prevalent among PID patients and significantly contribute to morbidity and mortality. Recurrent respiratory tract infections often serve as the initial warning sign in some PIDs and are a leading cause of mortality in adult PID patients. The presence of two or more pneumonia episodes per year is considered one of the ten warning signs of PID. Acute and chronic infections primarily constitute respiratory system diseases, while non-infectious respiratory complications include asthma, bronchiectasis, bronchiolitis obliterans, interstitial lung disease, granulomatous lung disease, and malignancies. These diseases significantly impact the quality of life of PID patients, limit their working abilities, and restrict their physical and social activities. Health-related quality of life in PID patients is also significantly affected by delays in the diagnosis and treatment of infections. As survival from infections improves, non-infectious pulmonary complications become more common in PID patients. Particularly in PIDs characterized by antibody deficiencies, permanent lung damage is observed in 20-40% of patients. Physical activity levels are also affected in PID patients, but there are only survey studies on this topic. Therefore, it is expected that respiratory muscle strength, as well as respiratory muscle endurance, functional exercise capacity, and peripheral muscle strength, would be affected in these patients. There is no research available on maximal exercise capacity, functional exercise capacity, respiratory muscle strength and endurance, and peripheral muscle strength. The aim of this study is to compare the maximal exercise capacity, functional exercise capacity, respiratory functions, physical activity level, quality of life, respiratory muscle strength and endurance, peripheral muscle strength, muscle oxygenation and dyspnea in patients with primary immune deficiencies with healthy individuals.
Study Type
OBSERVATIONAL
Enrollment
52
Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
Ankara, Çankaya, Turkey (Türkiye)
RECRUITINGMaximal Exercise capacity
Maximal Exercise capacity will be evaluated with Cardiopulmonary Exercise testing. The Cardiopulmonary Exercise Testing will be applied according to American Thoracic Society (ATS) and European Respiratory Society (ERS) criteria.
Time frame: through study completion, an average of 1 year
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: through study completion, an average of 1 year
Pulmonary function
Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be measured according to ATS and ERS criteria. With the device, forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and FEV1 / FVC will be evaluated.
Time frame: through study completion, an average of 1 year
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) will be evaluated.
Time frame: through study completion, an average of 1 year
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%) will be evaluated.
Time frame: through study completion, an average of 1 year
Respiratory muscle endurance
Respiratory muscle endurance will be assessed by the POWERbreathe Wellness (POWERbreathe, Inspiratory Muscle Training (IMT) Technologies Ltd., Birmingham, UK) device and the respiratory muscle endurance test with increased threshold load. The test will start with 20% of the maximal inspiratory pressure and the pressure will be increased to 40%, 60%, 80% and 100% every two minutes. Patients will be asked to continue breathing through the device during the whole time. During the test, the number of breaths delivered and the maximal time reached during each 2-minute period will be recorded. If the individual can't breathe 3 consecutive times, the test will be 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 will be multiplied. The value found will be recorded as the respiratory muscle endurance value.
Time frame: through study completion, an average of 1 year
Respiratory muscle strength
Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength will be measured with a portable mouth pressure measuring device according to ATS and ERS criteria.
Time frame: through study completion, an average of 1 year
Peripheral muscle strength
Isometric peripheral muscle strength will be measured with a portable hand dynamometer (JTECH Commander, USA). Measurements will be repeated on the shoulder abductors and knee extensors three times on the right and left.
Time frame: through study completion, an average of 1 year
Muscle Oxygenation (Local oxygen saturation (SmO2))
Muscle Oxygenation will be measured with Moxy device monitor (Moxy, Fortiori Design LLC, Minnesota, USA). The device will be placed on the motor point of quadriceps muscle during Cardiopulmonary Exercise Testing as well as during the six minute walk test. Local oxygen saturation (SmO2) levels will be measured.
Time frame: through study completion, an average of 1 year
Muscle Oxygenation (Total haemoglobin (THb))
Muscle Oxygenation will be measured with Moxy device monitor (Moxy, Fortiori Design LLC, Minnesota, USA). The device will be placed on the motor point of quadriceps muscle during Cardiopulmonary Exercise Testing as well as during the six minute walk test. Total haemoglobin (THb) levels will be measured.
Time frame: through study completion, an average of 1 year
Physical activity (Total energy expenditure)
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Physical activity (Active energy expenditure (joule / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Active energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Physical Activity (Physical activity time (min / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Physical activity time (min / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Physical Activity (Average metabolic equivalent (MET / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Average metabolic equivalent (MET / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Physical Activity (Number of steps (steps / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Number of steps (steps / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Physical Activity (Time spent lying down (min / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Time spent lying down (min / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Physical Activity (Sleep time (min / day))
Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multi sensor physical activity monitor over the triceps muscle of the non-dominant arm for 4 continuous days. The patient was informed about removing the device while taking a bath. Sleep time (min / day) will be measured with the multi-sensor physical activity monitor. The measured parameter will be averaged and analyzed with the "SenseWear® 7.0 Software" program.
Time frame: through study completion, an average of 1 year
Pediatric Quality of Life Inventory™ 4.0 (Quality of Life Questionnaire)
The quality of life will be measured with the Turkish version of Pediatric Quality of Life Inventory™ 4.0 (PedsQL™ 4.0). PedsQL measures health related quality of life in children and adolescents. The measure can be completed by parents (the Proxy Report) as well as children and young people (the Self-Report). The 23-item PedsQL Generic Core Scales were designed to measure the core dimensions of health as delineated by the World Health Organization, as well as role (school) functioning. The four scales that it measures are Physical Functioning (8 items), Emotional Functioning (5 items), Social Functioning (5 items) and School Functioning (5 items). Each item of the instrument is scored on a 5-point scale. Scale scores are computed as the sum of the items over the number of items answered.
Time frame: through study completion, an average of 1 year
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