Respiratory Function, Exercise Capacity and Peripheral Muscle Strength Among Patients With CF, PCD and Healthy Children

Comparison of Respiratory Function, Exercise Capacity and Peripheral Muscle Strength Among Patients With Cystic Fibrosis, Primary Ciliary Dyskinesia and Healthy Children

The aim of this study is to compare pulmonary function, respiratory muscle strength, exercise capacity and peripheral muscle strength of patients with CF, PCD and healthy childrens.

Study Overview

• Status: Completed
• Conditions: Cystic Fibrosis; Primary Ciliary Dyskinesia
• Intervention / Treatment: Other: Measurement of functional capacity; Other: pulmonary function test; Other: Functional capacity; Other: Peripheral muscle strength; Other: Respiratory muscle strength

Detailed Description

The impaired airway clearence and pulmonary functions, exercise intolerance, low physical activity level and decreased peripheral muscle strength make physiotherapy approaches important in the management of CF and PCD.

• Study Type: Observational
• Enrollment (Actual): 76

Contacts and Locations

Study Locations

• Turkey
  • Istanbul, Turkey
    • Bezmialem Vakıf University

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 18 years (ADULT, CHILD)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Children diagnosed with cystic fibrosis and primary ciliary dyskinesia and age matched healthy volunteers who do not have any diagnosed chronic diseases

Description

Inclusion Criteria:

• Diagnosis of cystic fibrosis or primary ciliary dyskinesia

Exclusion Criteria:

• Hospitalization history in past month
• Diagnosis of other chronic pediatric diseases which may impair exercise tolerance such as cerebral palsy or neuromuscular disease
• Candidates for lung transplantation or history of lung transplantation

Study Plan

How is the study designed?

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Cystic fibrosis; children with cystic fibrosis	Other: Measurement of functional capacity; Functional capacity of participants will be measured with six-minute walk test.; Other: pulmonary function test; It will be measured using basic spirometry and presented lung volume parameters such as FEV1,FVC,FEV1/FVC,PEF, FEF25-75; Other: Functional capacity; Functional capacity of participants will be measured with six-minute walk test and sit-to-stand test; Other: Peripheral muscle strength; Isometric M. Quadriceps strength will be measured using electronic hand held dynamometer in sitting position.; Other: Respiratory muscle strength; Inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures.
primary ciliary dyskinesia; children with primary ciliary dyskinesia	Other: Measurement of functional capacity; Functional capacity of participants will be measured with six-minute walk test.; Other: pulmonary function test; It will be measured using basic spirometry and presented lung volume parameters such as FEV1,FVC,FEV1/FVC,PEF, FEF25-75; Other: Functional capacity; Functional capacity of participants will be measured with six-minute walk test and sit-to-stand test; Other: Peripheral muscle strength; Isometric M. Quadriceps strength will be measured using electronic hand held dynamometer in sitting position.; Other: Respiratory muscle strength; Inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures.
healthy controls; Age-matched healthy volunteers	Other: Measurement of functional capacity; Functional capacity of participants will be measured with six-minute walk test.; Other: pulmonary function test; It will be measured using basic spirometry and presented lung volume parameters such as FEV1,FVC,FEV1/FVC,PEF, FEF25-75; Other: Functional capacity; Functional capacity of participants will be measured with six-minute walk test and sit-to-stand test; Other: Peripheral muscle strength; Isometric M. Quadriceps strength will be measured using electronic hand held dynamometer in sitting position.; Other: Respiratory muscle strength; Inspiratory and expiratory muscle strength has been assessed by maximal inspiratory and expiratory mouth pressures.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Six-minute walk test distance	Distance walked in six minutes will be recorded in meters. Test will be conducted according to the guideline of American Thoracic Society (ATS).	15 minutes

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Forced Vital Capacity (FVC)	FVC will be measured using basic spirometry and expressed as the percentage of the predicted value according to the guideline of European Respiratory Society (ERS)	5 minutes
Forced Expiratory Volume in 1 second (FEV1)	FEV1 will be measured using basic spirometry and expressed as the percentage of the predicted value according to the guideline of European Respiratory Society (ERS)	5 minutes
Peak Expiratory Flow (PEF)	PEF will be measured using basic spirometry and expressed as the percentage of the predicted value according to the guideline of European Respiratory Society (ERS)	5 minutes
M. Quadriceps strength	Isometric M. Quadriceps strength (kg) will be measured using electronic hand held dynamometer in sitting position.	5 minutes

Collaborators and Investigators

• Sponsor: Bezmialem Vakif University

Study record dates

Study Major Dates

• Study Start (ACTUAL): December 20, 2019
• Primary Completion (ACTUAL): February 24, 2020
• Study Completion (ACTUAL): March 12, 2020

Study Registration Dates

• First Submitted: November 8, 2019
• First Submitted That Met QC Criteria: November 11, 2019
• First Posted (ACTUAL): November 13, 2019

Study Record Updates

• Last Update Posted (ACTUAL): March 18, 2020
• Last Update Submitted That Met QC Criteria: March 17, 2020
• Last Verified: March 1, 2020

More Information

Terms related to this study

• Keywords: muscle strength; exercise capacity; respiratory functions
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Lung Diseases; Neurologic Manifestations; Congenital Abnormalities; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Otorhinolaryngologic Diseases; Movement Disorders; Pancreatic Diseases; Abnormalities, Multiple; Ciliopathies; Fibrosis; Cystic Fibrosis; Dyskinesias; Ciliary Motility Disorders
• Other Study ID Numbers: hdenizkulli2

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