Physical Fitness, Exercise Capacity and Activities of Daily Living in Primary Ciliary Dyskinesia: a Retrospective Study

Decreased pulmonary function, peripheral muscle strength, and exercise capacity were reported in primary ciliary dyskinesia (PCD) in recent studies. We aimed to investigate the data conducted between 10 July 2015 and 10 January 2015 of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL) in PCD and healthy counterparts retrospectively at the first stage of the study and the effects of inspiratory muscle training on pulmonary function, respiratory muscle strength, and exercise capacity in PCD patients with decreased inspiratory muscle strength from the database recorded between 10 July 2015 and 10 January 2015 retrospectively at the second stage of the study.

Study Overview

• Status: Completed
• Conditions: Primary Ciliary Dyskinesia; Kartagener Syndrome; Immotile Cilia Syndrome
• Intervention / Treatment: Other: Data Collection; Other: Data Collection

• Study Type: Observational
• Enrollment (Actual): 48

Contacts and Locations

Study Locations

• Turkey
  • Ankara, Turkey
    • 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

Patient group: PCD patients consulted from Hacettepe University, Faculty of Medicine, Department of Pediatric Chest Diseases, Child Health and Diseases/, Ankara, Turkey to our unit for evaluation in terms of respiratory physiotherapy were included in our study.

Healthy group: Healthy counterparts from our database of the previous studies were included in our study.

Description

Inclusion Criteria:

• Clinically stable
• Being able to cooperate with the assessments
• Not having any orthopedic, or neurological problems

Exclusion Criteria:

• Not being clinically stable
• Not being able to cooperate with the assessments
• Having any orthopedic, or neurological problems

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Retrospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Healthy Group; Data of 20 healthy subjects from the database recorded between 10 July 2015 and 10 January 2017.	Other: Data Collection; The first stage of the study: Retrospective data collection of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL). The second stage of the study -Retrospective data of pulmonary function, respiratory muscle function, functional capacity before and after supervised inspiratory muscle training with POWERbreathe K5 device using tapered flow resistive loading and airway clearance techniques performed for 6 weeks in PCD with decreased respiratory muscle strength.; Other: Data Collection; -Retrospective data collection of pulmonary function, respiratory muscle function, exercise capacity, physical fitness, and activities of daily living (ADL).
Primary Ciliary Dyskinesia (PCD); The first stage of the study: Data of 20 PCD patients from the database recorded between 10 July 2015 and 10 January 2017. The second stage of the study: -Data of 14 PCD patients who had lower inspiratory muscle strength from the same database underwent supervised inspiratory muscle training (IMT) with tapered flow resistive loading using Powerbreathe K5 device at our research unit and airway clearance techniques (ACT) as a home-based therapy for 6 weeks from the database recorded between 10 July 2015 and 10 January 2017.	Other: Data Collection; The first stage of the study: Retrospective data collection of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL). The second stage of the study -Retrospective data of pulmonary function, respiratory muscle function, functional capacity before and after supervised inspiratory muscle training with POWERbreathe K5 device using tapered flow resistive loading and airway clearance techniques performed for 6 weeks in PCD with decreased respiratory muscle strength.; Other: Data Collection; -Retrospective data collection of pulmonary function, respiratory muscle function, exercise capacity, physical fitness, and activities of daily living (ADL).

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
A primer outcome for the first stage of the study: Physical fitness	Munich Physical Fitness Test (MFT) was used to evaluate physical fitness. It is a test with six parameters including ball bounce, sandbag throwing, bending, vertical jumping, hanging and step test.	10 July 2015-10 January 2015-retrospectively
A primer outcome for the second stage of the study: Functional capacity	Functional capacity was evaluated with six minute walk test (6MWT).	10 July 2015-10 January 2015-retrospectively

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Demographic features	The age was recorded in years.	10 July 2015-10 January 2015-retrospectively
Demographic features	Weight was recorded in kilograms.	10 July 2015-10 January 2015-retrospectively
Demographic features	The length was saved in meters.	10 July 2015-10 January 2015-retrospectively
Demographic features	Body mass index was calculated by dividing the weight by the square of the height (kg/m^2) and recorded.	10 July 2015-10 January 2015-retrospectively
Pulmonary Function	Pulmonary function (FEV1 (forced expiratory volume in one second), FVC (forced vital capacity), FEV1/FVC, FEF25-75 (%) (forced expiratory volume 25-75 %) and PEF (peak expiratory flow)) assessed with a portable spirometer (Spirobank MIR, Italy).	10 July 2015-10 January 2015-retrospectively
Exercise Capacity	Exercise capacity was assessed with the modified shuttle walk test.	10 July 2015-10 January 2015-retrospectively
Activities of Daily Living	Glittre activities of daily living (ADL) test was used to evaluate daily life activity.	10 July 2015-10 January 2015-retrospectively
Respiratory muscle functions	Respiratory muscle strength (maximal inspiratory pressure (MIP);maximal expiratory pressure (MEP), respectively) was measured with a portable, electronic mouth pressure monitor (Micro Medical MicroMPM, UK) and recorded. For second stage of the study; average inspiratory flow, inspiratory volume, and inspiratory work was evaluated based on the outcomes of the Breathe-Link software using POWERbreathe K5 device before and after IMT with tapered flow resistive loading and recorded.	10 July 2015-10 January 2015-retrospectively

Collaborators and Investigators

• Sponsor: Hacettepe University
• Investigators: Study Chair: Deniz Inal Ince, Prof. Dr., Hacettepe University; Principal Investigator: Hazal Sonbahar Ulu, MSc, Hacettepe University; Study Director: Deniz Inal Ince, Prof. Dr., Hacettepe University

Study record dates

Study Major Dates

• Study Start (Actual): January 21, 2020
• Primary Completion (Actual): February 10, 2020
• Study Completion (Actual): February 10, 2020

Study Registration Dates

• First Submitted: August 24, 2021
• First Submitted That Met QC Criteria: September 3, 2021
• First Posted (Actual): September 10, 2021

Study Record Updates

• Last Update Posted (Actual): October 2, 2024
• Last Update Submitted That Met QC Criteria: September 30, 2024
• Last Verified: April 1, 2023

More Information

Terms related to this study

• Keywords: Exercise Capacity; Pulmonary Function; Physical Fitness; Primary Ciliary Dyskinesia; Immotile Cilia Syndrome; Inspiratory Muscle Strength Training; Respiratory Muscle Strength; Kartagener Syndrome
• Additional Relevant MeSH Terms: Pathologic Processes; Heart Diseases; Cardiovascular Diseases; Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Neurologic Manifestations; Disease; Congenital Abnormalities; Bronchial Diseases; Genetic Diseases, Inborn; Otorhinolaryngologic Diseases; Movement Disorders; Heart Defects, Congenital; Cardiovascular Abnormalities; Abnormalities, Multiple; Ciliopathies; Bronchiectasis; Respiratory System Abnormalities; Dextrocardia; Situs Inversus; Syndrome; Dyskinesias; Ciliary Motility Disorders; Kartagener Syndrome
• Other Study ID Numbers: GO 19/730

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
• product manufactured in and exported from the U.S.: No