Comparison of Exercise Capacity, Physical Activity Level and Quality of Life of Children With Primary Immunodeficiency With Healthy Children
Overview
- Phase
- N/A
- Intervention
- Not specified
- Conditions
- Primary Immunodeficiency Diseases
- Sponsor
- Gazi University
- Enrollment
- 52
- Locations
- 1
- Primary Endpoint
- Maximal Exercise capacity
- Status
- Recruiting
- Last Updated
- 2 years ago
Overview
Brief Summary
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
Detailed Description
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.
Investigators
Meral Boşnak Güçlü
Study director, PT, PhD, Prof. Dr. Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Head of Cardiopulmonary Rehabilitation Clinic
Gazi University
Eligibility Criteria
Inclusion Criteria
- •Individuals aged 6-18 years, diagnosed with primary immune deficiency disease and receiving standard medical treatment, will be included in the study.
- •Healthy controls:
- •Individuals between the ages of 6 and 18 without a known chronic disease will be included.
Exclusion Criteria
- •Patients who are uncooperative, have orthopedic or neurological disorders that will affect their exercise capacity, and have pneumonia or any acute infection during the evaluation will be excluded from the study.
- •Healthy controls:
- •Those with a known chronic disease, uncooperative and orthopedic or neurological disorders that will affect their exercise capacity will not be included.
Outcomes
Primary Outcomes
Maximal Exercise capacity
Time Frame: through study completion, an average of 1 year
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.
Secondary Outcomes
- Pulmonary function(through study completion, an average of 1 year)
- Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))(through study completion, an average of 1 year)
- Respiratory muscle endurance(through study completion, an average of 1 year)
- Functional exercise capacity(through study completion, an average of 1 year)
- Physical Activity (Physical activity time (min / day))(through study completion, an average of 1 year)
- Physical Activity (Time spent lying down (min / day))(through study completion, an average of 1 year)
- Pulmonary function (Peak flow rate (PEF))(through study completion, an average of 1 year)
- Respiratory muscle strength(through study completion, an average of 1 year)
- Muscle Oxygenation (Local oxygen saturation (SmO2))(through study completion, an average of 1 year)
- Physical Activity (Average metabolic equivalent (MET / day))(through study completion, an average of 1 year)
- Physical Activity (Number of steps (steps / day))(through study completion, an average of 1 year)
- Peripheral muscle strength(through study completion, an average of 1 year)
- Muscle Oxygenation (Total haemoglobin (THb))(through study completion, an average of 1 year)
- Physical activity (Total energy expenditure)(through study completion, an average of 1 year)
- Physical Activity (Sleep time (min / day))(through study completion, an average of 1 year)
- Pediatric Quality of Life Inventory™ 4.0 (Quality of Life Questionnaire)(through study completion, an average of 1 year)
- Physical activity (Active energy expenditure (joule / day))(through study completion, an average of 1 year)