Physiological Responses to Exercise Tests in Primary Ciliary Dyskinesia Compared With Healthy Individuals

Physiological Responses to Exercise Tests in Primary Ciliary Dyskinesia Compared With Healthy Children and Investigation of the Relationship Between Exercise Capacity and Quality of Life in Primary Ciliary Dyskinesia

The individuals with primary ciliary dyskinesia (PCD) have lower aerobic fitness and anaerobic performance than healthy individuals. Cardiopulmonary exercise test (CPET) provides an integrated assessment of integrative exercise responses involving the pulmonary, cardiovascular, hematopoietic, neuropsychological, and skeletal muscle systems that are not adequately reflected by measurement of the function of organ systems. Maximum oxygen consumption is measured by performing gas exchange analysis with CPET, offering an objective measure of cardiorespiratory fitness. The six-minute walk test (6MWT) and the shuttle walk test (SWT) are field tests that produce oxygen consumption results similar to those during CPET. There is a need to determine the physiological responses to CPET, SWT and 6MWT in individuals with PCD. Additionally, the physiological responses of individuals with PCD to different exercise tests will be compared with the responses of healthy individuals. Low aerobic capacity in individuals with PCD may affect the physical, emotional, and social lives of individuals. This influence may cause a decrease in the quality of life of individuals with PCD. The relationship between exercise capacity and quality of life in individuals with PSD will be investigated with this study.

Study Overview

• Status: Completed
• Conditions: Primary Ciliary Dyskinesia
• Intervention / Treatment: Other: No intervention

• Study Type: Observational
• Enrollment (Actual): 46

Contacts and Locations

• Study Contact: Name: Aslihan Cakmak; Phone Number: 178 03123051576; Email: aslihancakmak90@gmail.com
• Study Contact Backup: Name: Deniz Inal-Ince; Phone Number: 03123051576

Study Locations

• Turkey
  • Ankara, Turkey, 06100
    • Hacettepe University

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 8 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Probability Sample

Study Population

The children with primary ciliary dyskinesia and healthy children

Description

Inclusion Criteria:

• Having been diagnosed with PCD in the Pediatrics Department, Pediatric Chest Diseases Unit of Hacettepe University Faculty of Medicine and being routinely referred to Hacettepe University, Faculty of Physical Therapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit for physiotherapy applications and pulmonary rehabilitation program,
• Being clinically stable,
• Being between 8-18 years old,
• Having a forced expiratory volume in the first second (FEV1) ≥ 40%,

The healthy group will be composed of individuals who do not have any known disease and volunteer to participate in the study. For healthy individuals, volunteers from acquaintances and/or relatives of the researchers will be included in the study.

Exclusion Criteria:

• Having unstable clinical condition
• Having severe neuromuscular and musculoskeletal problems,
• Having any congenital heart diseases other than situs inversus,
• Unable to cooperate

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Healthy controls; Healthy children	Other: No intervention; No intervention
The children with primary ciliary dyskinesia	Other: No intervention; No intervention

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Shuttle walk test distance	Shuttle walk test distance will be determined using shuttle walk test	up to 3 weeks
Quality of life score	Quality of life score will be determined using multidimensional measure to assess health-related quality of life in primary ciliary dyskinesia (QoL-PCD).	1st day
Maximal oxygen uptake	Maximal oxygen uptake will be determined using cardiopulmonary exercise test.	up to 3 weeks
6 minute walk test distance	6 minute walk test distance will be determined using 6 minute walk test	up to 3 weeks

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Pulmonary function test - Forced expiratory volume in one second	Pulmonary function test using a spirometer will be performed. Forced expiratory volume in one second will be recorded.	1st day
Pulmonary function test - Peak expiratory flow	Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded.	1st day
Pulmonary function test - forced vital capacity	Pulmonary function test using a spirometer will be performed. Forced vital capacity will be recorded.	1st day
Maximal inspiratory pressure	Maximal inspiratory pressure will be measured using a mouthpiece device.	1st day
Maximal expiratory pressure	Maximal expiratory pressure will be measured using a mouthpiece device.	1st day
Pulmonary function test - Forced mid-expiratory flow (FEF25-75)	Pulmonary function test using a spirometer will be performed. Peak expiratory flow will be recorded.	1stday

Collaborators and Investigators

• Sponsor: Hacettepe University
• Investigators: Study Chair: Deniz Inal-Ince, Hacettepe University; Principal Investigator: Ugur Ozcelik, Hacettepe University; Principal Investigator: Nagehan Emiralioglu, Hacettepe University; Principal Investigator: Melda Saglam, Hacettepe University; Principal Investigator: Naciye Vardar-Yagli, Hacettepe University; Principal Investigator: Ebru Calik-Kutukcu, Hacettepe University; Principal Investigator: Birce Sunman, Hacettepe University; Principal Investigator: Hazal Sonbahar-Ulu, Akdeniz University

Study record dates

Study Major Dates

• Study Start (Actual): October 7, 2020
• Primary Completion (Actual): October 7, 2023
• Study Completion (Actual): October 7, 2023

Study Registration Dates

• First Submitted: January 26, 2023
• First Submitted That Met QC Criteria: January 26, 2023
• First Posted (Actual): February 3, 2023

Study Record Updates

• Last Update Posted (Actual): January 18, 2024
• Last Update Submitted That Met QC Criteria: January 17, 2024
• Last Verified: January 1, 2024

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Neurologic Manifestations; Congenital Abnormalities; Genetic Diseases, Inborn; Otorhinolaryngologic Diseases; Movement Disorders; Abnormalities, Multiple; Ciliopathies; Dyskinesias; Ciliary Motility Disorders
• Other Study ID Numbers: GO 20/647

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