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NCT Number: NCT05816876

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

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.

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Key information

Conditions

Primary Ciliary Dyskinesia Abnormalities, Multiple Behavior Ciliary Motility Disorders Ciliopathies Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Motor Activity Otorhinolaryngologic Diseases Respiratory Tract Diseases

Age range

6 year–18 year

Sex eligibility

All sexes

Study type

Observational

Primary location

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

Ankara, Çankaya, 06490, Turkey (Türkiye)

About this study

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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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.

Treatment and study plan

Primary outcomes

  1. Functional exercise capacity

    Time frame: First Day

    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.

Secondary outcomes

  1. Physical activity (Total energy expenditure)

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Total energy expenditure (joule / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  2. Physical activity (Active energy expenditure (joule / day))

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Active energy expenditure (joule / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  3. Physical activity (Physical activity time (min / day))

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Physical activity time (min / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  4. Physical activity (Average metabolic equivalent (MET / day))

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Average metabolic equivalent (MET / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  5. Physical activity (Number of steps (steps / day))

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Number of steps (steps / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  6. Physical activity (Time spent lying down (min / day) days))

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Time spent lying down (min / day) days) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  7. Physical activity (Sleep time (min / day))

    Time frame: Second Day

    Physical activity was evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient wore the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient was informed about removing the device while taking a bath. Sleep time (min / day) was measured with the multi-sensor physical activity monitor. The parameters measured over two days were averaged and analyzed with the "SenseWear® 7.0 Software" program.

  8. Pulmonary function (Forced vital capacity (FVC))

    Time frame: First Day

    Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced vital capacity (FVC) was evaluated.

  9. Pulmonary function (Forced expiratory volume in the first second (FEV1))

    Time frame: First Day

    Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced expiratory volume in the first second (FEV1) was evaluated.

  10. Pulmonary function (FEV1 / FVC)

    Time frame: First Day

    Pulmonary function was evaluated with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, FEV1 / FVC was evaluated.

  11. Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))

    Time frame: First Day

    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%) was evaluated.

  12. Pulmonary function (Peak flow rate (PEF))

    Time frame: First Day

    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) was evaluated.

  13. Respiratory muscle strength

    Time frame: First Day

    Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength were measured with a portable mouth pressure measuring device according to ATS and ERS criteria.

  14. Peripheral muscle strength

    Time frame: Second day

    Isometric peripheral muscle strength was measured with a portable hand dynamometer (JTECH Commander, USA). Measurements were repeated on the shoulder abductors and knee extensors three times on the right and left.

  15. Respiratory muscle endurance

    Time frame: Second Day

    Respiratory muscle endurance was assessed by the POWERbreathe Wellness (POWERbreathe, Inspiratory Muscle Training (IMT) Technologies Ltd., Birmingham, UK) device and the respiratory muscle endurance test at increased threshold load. The test was started with 20% of the maximal inspiratory pressure and the pressure was increased to 40%, 60%, 80% and 100% every two minutes. Patients were asked to continue breathing through the device during the test. During the test, the number of breaths delivered and the maximal time reached during each 2-minute period were recorded. If the individual could not breathe 3 consecutive times, the test was 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 was multiplied. The value found was recorded as the respiratory muscle endurance value.

  16. Life quality

    Time frame: Second Day

    It was evaluated with the Turkish version of the disease-specific Primary Ciliary Dyskinesia Quality of Life scale. The questions were about physical function, emotional function, social function, respiratory symptoms, treatment burden, ear and hearing, sinus symptoms, role function, health perceptions, school functioning, eating and weight problems.

Sponsors and collaborators

Lead sponsor

Gazi University

Other

Registry information

Official study title

Comparison of Respiratory and Peripheral Muscle Functions, Exercise Capacity and Physical Activity Level in Primary Ciliary Dyskinesia and Kartagener Syndrome

Important dates

Study start
2016
Primary completion
2023
Study completion
2023
First posted
Apr 18, 2023
Registry last updated
Apr 18, 2023

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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