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

NCT05999422 | Unknown

• 1 other identifier: Gazi University 55
• Study Type: observational
• Target: 52
• Locations: 1 country
• Sites: 1

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

Conditions

Primary Immunodeficiency Diseases

Keywords

Primary Immunodeficiency Diseases; Exercise capacity; Quality of life; Physical activity levels

Outcome Measures

Primary Outcomes (1)

• Maximal 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.

  through study completion, an average of 1 year

Secondary Outcomes (17)

• Functional exercise capacity

  through study completion, an average of 1 year

• Pulmonary function

  through study completion, an average of 1 year

• Pulmonary function (Peak flow rate (PEF))

  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

Study Arms (2)

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).

Eligibility Criteria

• Age: 6 Years - 18 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

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

You may qualify if:

• 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.

You may not qualify if:

• 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.

Sponsors & Collaborators

• Gazi University: lead

Study Sites (1)

Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit

Ankara, Çankaya, 06490, Turkey (Türkiye)

RECRUITING

Related Publications (14)

• Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland SM, Klein C, Morio T, Oksenhendler E, Picard C, Puel A, Puck J, Seppanen MRJ, Somech R, Su HC, Sullivan KE, Torgerson TR, Meyts I. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022 Oct;42(7):1473-1507. doi: 10.1007/s10875-022-01289-3. Epub 2022 Jun 24.

  PMID: 35748970 BACKGROUND

• Tangye SG, Al-Herz W, Bousfiha A, Chatila T, Cunningham-Rundles C, Etzioni A, Franco JL, Holland SM, Klein C, Morio T, Ochs HD, Oksenhendler E, Picard C, Puck J, Torgerson TR, Casanova JL, Sullivan KE. Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2020 Jan;40(1):24-64. doi: 10.1007/s10875-019-00737-x. Epub 2020 Jan 17.

  PMID: 31953710 BACKGROUND

• Kilic SS, Ozel M, Hafizoglu D, Karaca NE, Aksu G, Kutukculer N. The prevalences [correction] and patient characteristics of primary immunodeficiency diseases in Turkey--two centers study. J Clin Immunol. 2013 Jan;33(1):74-83. doi: 10.1007/s10875-012-9763-3. Epub 2012 Sep 15.

  PMID: 22983506 BACKGROUND

• Ameratunga R, Longhurst H, Lehnert K, Steele R, Edwards ESJ, Woon ST. Are All Primary Immunodeficiency Disorders Inborn Errors of Immunity? Front Immunol. 2021 Jul 21;12:706796. doi: 10.3389/fimmu.2021.706796. eCollection 2021. No abstract available.

  PMID: 34367167 BACKGROUND

• Hampson FA, Chandra A, Screaton NJ, Condliffe A, Kumararatne DS, Exley AR, Babar JL. Respiratory disease in common variable immunodeficiency and other primary immunodeficiency disorders. Clin Radiol. 2012 Jun;67(6):587-95. doi: 10.1016/j.crad.2011.10.028. Epub 2012 Jan 9.

  PMID: 22226567 BACKGROUND

• Verma N, Grimbacher B, Hurst JR. Lung disease in primary antibody deficiency. Lancet Respir Med. 2015 Aug;3(8):651-60. doi: 10.1016/S2213-2600(15)00202-7. Epub 2015 Jul 15.

  PMID: 26188881 BACKGROUND

• Modell V, Orange JS, Quinn J, Modell F. Global report on primary immunodeficiencies: 2018 update from the Jeffrey Modell Centers Network on disease classification, regional trends, treatment modalities, and physician reported outcomes. Immunol Res. 2018 Jun;66(3):367-380. doi: 10.1007/s12026-018-8996-5.

  PMID: 29744770 BACKGROUND

• Quinti I, Di Pietro C, Martini H, Pesce AM, Lombardi F, Baumghartner M, Colantuono S, Milito C, Tabolli S. Health related quality of life in common variable immunodeficiency. Yonsei Med J. 2012 May;53(3):603-10. doi: 10.3349/ymj.2012.53.3.603.

  PMID: 22477006 BACKGROUND

• Routes J, Costa-Carvalho BT, Grimbacher B, Paris K, Ochs HD, Filipovich A, Hintermeyer M, de Melo KM, Workman S, Ito D, Ye X, Bonnet P, Li-McLeod J. Health-Related Quality of Life and Health Resource Utilization in Patients with Primary Immunodeficiency Disease Prior to and Following 12 Months of Immunoglobulin G Treatment. J Clin Immunol. 2016 Jul;36(5):450-61. doi: 10.1007/s10875-016-0279-0. Epub 2016 Apr 18.

  PMID: 27091140 BACKGROUND

• Urschel S, Kayikci L, Wintergerst U, Notheis G, Jansson A, Belohradsky BH. Common variable immunodeficiency disorders in children: delayed diagnosis despite typical clinical presentation. J Pediatr. 2009 Jun;154(6):888-94. doi: 10.1016/j.jpeds.2008.12.020. Epub 2009 Feb 23.

  PMID: 19230900 BACKGROUND

• Jesenak M, Banovcin P, Jesenakova B, Babusikova E. Pulmonary manifestations of primary immunodeficiency disorders in children. Front Pediatr. 2014 Jul 25;2:77. doi: 10.3389/fped.2014.00077. eCollection 2014.

  PMID: 25121077 BACKGROUND

• Azizi G, Abolhassani H, Asgardoon MH, Alinia T, Yazdani R, Mohammadi J, Rezaei N, Ochs HD, Aghamohammadi A. Autoimmunity in common variable immunodeficiency: epidemiology, pathophysiology and management. Expert Rev Clin Immunol. 2017 Feb;13(2):101-115. doi: 10.1080/1744666X.2016.1224664. Epub 2016 Sep 16.

  PMID: 27636680 BACKGROUND

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  PMID: 9323243 BACKGROUND

• Sowers KL, Litwin BA, Lee ACW, Galantino MLA. Exercise Perception and Behaviors in Individuals Living with Primary Immunodeficiency Disease. J Clin Immunol. 2018 Feb;38(2):174-184. doi: 10.1007/s10875-017-0472-9. Epub 2018 Jan 6.

  PMID: 29307028 BACKGROUND

MeSH Terms

Conditions

Primary Immunodeficiency Diseases

Condition Hierarchy (Ancestors)

Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Immunologic Deficiency Syndromes; Immune System Diseases

Study Officials

• Meral Boşnak Güçlü, Prof.Dr.

  Gazi University

  STUDY DIRECTOR

• Riad Bejta, B.sc PT

  Gazi University

  STUDY CHAIR

• Caner Aytekin, Assoc. Prof

  MH. Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Meral Boşnak Güçlü, Prof.Dr.

CONTACT

+903122162647; meralbosnak@gazi.edu.tr

Riad Bejta, B.sc PT

CONTACT

+905466559427; riadbejta@gmail.com

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Study director, PT, PhD, Prof. Dr. Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Head of Cardiopulmonary Rehabilitation Clinic

Study Record Dates

• First Submitted: July 24, 2023
• First Posted: August 21, 2023
• Study Start: August 1, 2023
• Primary Completion: August 1, 2024
• Study Completion: October 1, 2024
• Last Updated: August 21, 2023

Record last verified: 2023-08

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)