Clinical trial NCT06092528: Investigation of the Effects of Pulmonary Rehabilitation in Children With Primary Immunodeficiency
Official title: Investigation of the Effects of Pulmonary Rehabilitation on Exercise Capacity, Muscle Oxygenation and Physical Activity Level in Children With Primary Immunodeficiency
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: Primary Immunodeficiency
Keywords: primary immunodeficiency
pulmonary rehabilitation
exercise capacity
muscle oxygenation
physical activity
Lead sponsor: Gazi University
Sponsor class: OTHER
First posted: 2023-10-23
Enrollment: 40
Sex: ALL
Minimum age (years): 6
Maximum age (years): 18
Standard ages: CHILD
ADULT
Accepts healthy volunteers: no
Allocation: RANDOMIZED
Intervention model: PARALLEL
Intervention model description: Parallel Assignment
Primary purpose: SUPPORTIVE_CARE

Brief summary: Primary immunodeficiencies (PID) are a heterogeneous group of diseases that occur as a result of disorders that affect the development, differentiation and/or function of various cells and building blocks in the immune system. Among the symptoms and complications of PID, pulmonary complications are very common and significantly increase the morbidity and mortality of the disease.

Detailed description: Among the symptoms and complications of PID, pulmonary complications are very common and significantly increase the morbidity and mortality of the disease. Recurrent respiratory infections are often the first warning sign in some types of PID and is a cause of mortality in adults with PID. Presence of 2 or more pneumonias per year is one of the 10 warning signs of PID. Respiratory system diseases are mainly caused by acute and chronic infections. Non-infectious respiratory system diseases and complications are asthma, bronchiectasis, bronchiolitis obliterans, interstitial lung disease, granulomatous lung disease and malignancies. These diseases significantly affect the quality of life of PID patients, limiting their ability to work and 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 increases, non-infectious pulmonary complications are more common in PID patients. Permanent lung damage is seen at a rate of 20-40%, especially in PID patients with antibody deficiency. The main causes of exercise intolerance in patients with lung disease include isolated or associated factors such as increased symptoms (fatigue and shortness of breath in the lower extremities), development of dynamic hyperinflation, peripheral muscle dysfunction, abnormalities in oxygen transport and progressive loss of physical condition, physical inactivity. There are no studies evaluating exercise capacity, respiratory and peripheral muscle strength, inspiratory muscle endurance, and muscle oxygenation in children with PID. In studies conducted with post-infectious bronchiolitis obliterans patients, it has been shown that exercise capacity is reduced in these patients. There are no studies in the literature on pulmonary rehabilitation practices and efficacy in PID patients.

The primary aim of this study: To investigate the effects of pulmonary rehabilitation on exercise capacity and muscle oxygenation in children with primary immunodeficiency.

The secondary aim of this study: To investigate the effects of pulmonary rehabilitation on physical activity level, respiratory functions, peripheral and respiratory muscle strength, inspiratory muscle endurance, shortness of breath, fatigue and quality of life in children with primary immunodeficiency.

Primary outcome measurement will be oxygen consumption (cardiopulmonary exercise test).

Secondary outcome will be muscle oxygenation (Moxy device), physical activity level (multi sensor activity device), pulmonary function (spirometer), functional exercise capacity (six-minute walk test), respiratory (mouth pressure device) and peripheral muscle (hand-held dynamometer) strength, inspiratory muscle endurance (incremental threshold loading test), dyspnea (Modified Borg Scale (MBS)), fatigue (Modified Borg Scale) and quality of life (The Pediatric Quality of Life Inventory (PedsQL)).

Eligibility criteria: Inclusion Criteria:

-Patients aged 6-18 years with primary immunodeficiency

Exclusion Criteria:

* Acute pulmonary exacerbation, acute upper or lower respiratory tract infection
* Serious neurological, neuromuscular, orthopedic and other systemic diseases or other diseases affecting physical functions
* Participating in a planned exercise program in the past three months
* Cognitive impairment, which may cause difficulty understanding and following exercise test instructions

Primary outcomes:
measure: Oxygen Consumption
timeFrame: First Day
Secondary outcomes:
measure: Pulmonary function (Forced vital capacity (FVC))
timeFrame: Second Day
measure: Pulmonary function (Forced expiratory volume in the first second (FEV1))
timeFrame: Second Day
measure: Pulmonary function (FEV1 / FVC)
timeFrame: Second Day
measure: Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
timeFrame: Second Day
measure: Pulmonary function (Peak flow rate (PEF))
timeFrame: Second Day
measure: Respiratory Muscle Strength
timeFrame: Second Day
measure: Respiratory Muscle Endurance
timeFrame: Second Day
measure: Peripheral Muscle Strength
timeFrame: Second Day
measure: Functional exercise capacity
timeFrame: Second Day
measure: Quality of Life (For Children)
timeFrame: First Day
measure: Dyspnea
timeFrame: First and Second Day
measure: Fatigue
timeFrame: First and Second Day
measure: Muscle oxygenation
timeFrame: First and Second Day
measure: Physical Activity Level (Total energy expenditure)
timeFrame: Second Day
measure: Physical activity (Active energy expenditure (joule / day))
timeFrame: Second Day
measure: Physical activity (Physical activity time (min / day))
timeFrame: Second Day
measure: Physical activity (Average metabolic equivalent (MET / day))
timeFrame: Second Day
measure: Physical activity (Number of steps (steps / day))
timeFrame: Second Day
measure: Physical activity (Time spent lying down (min / day) days))
timeFrame: Second Day
measure: Physical activity (Sleep time (min / day))
timeFrame: Second Day
Other outcomes:
Locations:
city: Ankara
country: Turkey
facility: Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
state: Çankaya
Study officials:
affiliation: Gazi University
name: Betül Yoleri, Pt. MsC
role: PRINCIPAL_INVESTIGATOR
affiliation: MH. Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital
name: Caner Aytekin, Assoc. Prof.
role: PRINCIPAL_INVESTIGATOR
affiliation: Gazi University
name: Meral Boşnak Güçlü, Prof. Dr.
role: STUDY_DIRECTOR
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