Exercise Capacity, Physical Activity Level and Quality of Life in Children With Primary Immunodeficiency

August 13, 2023 updated by: Meral Boşnak Güçlü, Gazi University

Comparison of Exercise Capacity, Physical Activity Level and Quality of Life of Children With Primary Immunodeficiency With Healthy Children

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

Study Overview

Status

Recruiting

Conditions

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.

Study Type

Observational

Enrollment (Estimated)

52

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

  • Turkey
    • Çankaya
      • Ankara, Çankaya, Turkey, 06490
        • Recruiting
        • Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
        • Contact:
        • Contact:

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Child
  • Adult

Accepts Healthy Volunteers

Yes

Sampling Method

Non-Probability Sample

Study Population

26 patients diagnosed with primary immune deficiency will be recruited, together with 26 healthy individuals.

Description

Inclusion Criteria:

Patients:

  • 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:

  • 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.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
Patients with primary immunodeficiency
Maximal exercise capacity measured with Cardiopulmonary exercise testing, functional exercise capacity with six minute walk test, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, muscle oxygenation using Moxy monitor, respiratory muscle endurance using incremental threshold loading test, life quality using "Pediatric Quality of Life Inventory™ 4.0 (PedsQL™ 4.0)" (Turkish version).
Healthy control
Maximal exercise capacity measured with Cardiopulmonary exercise testing, functional exercise capacity with six minute walk test, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, muscle oxygenation using Moxy monitor, respiratory muscle endurance using incremental threshold loading test, life quality using "Pediatric Quality of Life Inventory™ 4.0 (PedsQL™ 4.0)" (Turkish version).

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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.
through study completion, an average of 1 year

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Functional exercise capacity
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Pulmonary function
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Pulmonary function (Peak flow rate (PEF))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Respiratory muscle endurance
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Respiratory muscle strength
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Peripheral muscle strength
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Muscle Oxygenation (Local oxygen saturation (SmO2))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Muscle Oxygenation (Total haemoglobin (THb))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical activity (Total energy expenditure)
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical activity (Active energy expenditure (joule / day))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical Activity (Physical activity time (min / day))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical Activity (Average metabolic equivalent (MET / day))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical Activity (Number of steps (steps / day))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical Activity (Time spent lying down (min / day))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Physical Activity (Sleep time (min / day))
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year
Pediatric Quality of Life Inventory™ 4.0 (Quality of Life Questionnaire)
Time Frame: through study completion, an average of 1 year
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.
through study completion, an average of 1 year

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Gazi University

Investigators

  • Study Director: Meral Boşnak Güçlü, Prof.Dr., Gazi University
  • Study Chair: Riad Bejta, B.sc PT, Gazi University
  • Principal Investigator: Caner Aytekin, Assoc. Prof, MH. Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

August 1, 2023

Primary Completion (Estimated)

August 1, 2024

Study Completion (Estimated)

October 1, 2024

Study Registration Dates

First Submitted

July 24, 2023

First Submitted That Met QC Criteria

August 13, 2023

First Posted (Actual)

August 21, 2023

Study Record Updates

Last Update Posted (Actual)

August 21, 2023

Last Update Submitted That Met QC Criteria

August 13, 2023

Last Verified

August 1, 2023

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • Gazi University 55

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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