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

NCT05040607 | Completed

• 1 other identifier: GO 19/730
• Study Type: observational
• Target: 48
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Primary Ciliary Dyskinesia; Kartagener Syndrome; Immotile Cilia Syndrome

Keywords

Primary Ciliary Dyskinesia; Kartagener Syndrome; Immotile Cilia Syndrome; Pulmonary Function; Respiratory Muscle Strength; Exercise Capacity; Physical Fitness; Inspiratory Muscle Strength Training

Outcome Measures

Primary Outcomes (2)

• 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 Outcomes (8)

• Demographic features

  10 July 2015-10 January 2015-retrospectively

• Demographic features

  10 July 2015-10 January 2015-retrospectively

• Demographic features

  10 July 2015-10 January 2015-retrospectively

• Demographic features

  10 July 2015-10 January 2015-retrospectively

• Pulmonary Function

  10 July 2015-10 January 2015-retrospectively

Study Arms (2)

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

Healthy Group

Data of 20 healthy subjects from the database recorded between 10 July 2015 and 10 January 2017.

Other: Data Collection

Interventions

Data Collection OTHER

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.

Primary Ciliary Dyskinesia (PCD)

Eligibility Criteria

• Age: 8 Years - 18 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• 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.

You may qualify if:

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

You may not qualify if:

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

Sponsors & Collaborators

• Hacettepe University: lead

Study Sites (1)

Hacettepe University

Ankara, Turkey (Türkiye)

Location

MeSH Terms

Conditions

Ciliary Motility Disorders; Kartagener Syndrome

Interventions

Data Collection

Condition Hierarchy (Ancestors)

Respiratory Tract Diseases; Otorhinolaryngologic Diseases; Ciliopathies; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Bronchiectasis; Bronchial Diseases; Respiratory System Abnormalities; Dextrocardia; Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Situs Inversus

Intervention Hierarchy (Ancestors)

Epidemiologic Methods; Investigative Techniques; Health Care Evaluation Mechanisms; Quality of Health Care; Health Care Quality, Access, and Evaluation; Public Health; Environment and Public Health

Study Officials

• Hazal Sonbahar Ulu, MSc

  Hacettepe University

  PRINCIPAL INVESTIGATOR

• Deniz Inal Ince, Prof. Dr.

  Hacettepe University

  STUDY CHAIR

• Deniz Inal Ince, Prof. Dr.

  Hacettepe University

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: MSc, PT

Study Record Dates

• First Submitted: August 24, 2021
• First Posted: September 10, 2021
• Study Start: January 21, 2020
• Primary Completion: February 10, 2020
• Study Completion: February 10, 2020
• Last Updated: October 2, 2024

Record last verified: 2023-04

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)