Muscle Function, Exercise Capacity and Physical Activity Level in Primary Ciliary Dyskinesia and Kartagener Syndrome

NCT ID: NCT05816876

Last Updated: 2023-04-18

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 65 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2016-11-10
• Study Completion Date: 2023-02-01

Brief Summary

Studies evaluating respiratory and peripheral muscle functions in PCD patients and comparing them with healthy children are limited in the literature. There is no study investigating pulmonary and extrapulmonary effects in Kartagener syndrome, which is a form of PCD. The aim of our study is to compare respiratory functions, respiratory muscle strength and endurance, exercise capacity, peripheral muscle strength, physical activity level and quality of life in patients with PCD, Kartagener syndrome and healthy children.

Detailed Description

Primary ciliary dyskinesia (PCD) is an autosomal recessive disease characterized by abnormal ciliary movement and impaired mucociliary clearance. Kartagener syndrome is an autosomal recessive primary ciliary dyskinesia syndrome characterized by situs inversus, bronchiectasis and chronic sinusitis and is the most serious form of PCD, accounting for 50% of all PCD cases. In these syndromes, there is both movement and structural defect in the cilia, and mucociliary clearance is impaired. Symptoms usually appear in childhood. Organ anomalies can also be seen in Kartagener syndrome. Primary ciliary dyskinesia is a disease that threatens lung functions starting from preschool age. One of the main causes of respiratory muscle weakness in chronic lung diseases is worsening of lung functions. Evaluation of exercise capacity in patients with PCD has prognostic importance. Decreased exercise capacity, respiratory function, and cardiopulmonary function cause physical inactivity. In chronic lung diseases, sedentary lifestyle, airway obstruction, malnutrition and decreased exercise capacity cause muscle weakness. Muscle strength and function are important for performing activities of daily living. There is no study in the literature comparing respiratory and physical functions in children with PCD and in children with Kartagener's syndrome, which is the most serious form of PCD. The aim of our study is to compare respiratory functions, respiratory muscle strength and endurance, exercise capacity, peripheral muscle strength, physical activity level and quality of life in patients with PCD, Kartagener syndrome and healthy children.

The study was planned as a cross-sectional, retrospective. Individuals aged 6-18 years, who were diagnosed with PCD and Kartagener syndrome, and received standard medical treatment, were included in the study. For the healthy group, individuals aged 6-18 years without a known chronic disease were included. Individuals' exercise capacity, respiratory functions, physical activity levels, peripheral and respiratory muscle strength, respiratory muscle endurance and quality of life will be evaluated. Exercise capacity evaluated using six-minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand-held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version). The assessments will be completed in two days.

Conditions

Primary Ciliary Dyskinesia

Study Design

• Observational Model Type: OTHER
• Study Time Perspective: RETROSPECTIVE

Study Groups

Patients with primary ciliary dyskinesia

Exercise capacity assessed using six minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version).

No interventions assigned to this group

Patients with Kartagener Syndrome

Exercise capacity assessed using six minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version).

No interventions assigned to this group

Healthy controls

Exercise capacity assessed using six minute walk test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using "The Primary Ciliary Dyskinesia Quality of Life scale" (Turkish version).

No interventions assigned to this group

Eligibility Criteria

Inclusion Criteria

Patients;

• Individuals aged 6-18 years, who were diagnosed with PCD and Kartagener syndrome, and received standard medical treatment, were 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 functional 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 functional capacity will not be included.

• Minimum Eligible Age: 6 Years
• Maximum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: Yes

Sponsors

Gazi University

OTHER

Sponsor Role: lead

Responsible Party

Meral Boşnak Güçlü

Prof. Dr.

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

Meral Boşnak Güçlü, Prof. Dr.

Role: STUDY_DIRECTOR

Gazi University

Merve Fırat, MD

Role: STUDY_CHAIR

Hacettepe University

Şeyma Mutlu, MD

Role: PRINCIPAL_INVESTIGATOR

Baskent University

Betül Yoleri, MD

Role: PRINCIPAL_INVESTIGATOR

Gazi University

Locations

Gazi University Facutly of Health Sciences Department of Physiotheraphy and Rehabilitation, Cardiopulmonary Rehabilitation Unit

Ankara, Çankaya, Turkey (Türkiye)

Countries

Turkey (Türkiye)

References

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Other Identifiers

Gazi University 32

Identifier Type: -

Identifier Source: org_study_id