Investigation of Relationship Between Vascular Functions, Exercise Capacity, and Respiratory Functions in CF

Investigation of the Relationship Between Micro and Macrovascular Functions and Exercise Capacity and Respiratory Functions in Children With Cystic Fibrosis of Different Obstruction Severities

In the literature, there is no study that evaluates the endothelial function and arterial stiffness together and investigates its relationship with exercise capacity and respiratory functions in children with CF of different obstruction severities. Therefore, the aim of this study is to investigate and compare microvascular (endothelium), macrovascular (arterial stiffness) functions in children with CF of different obstruction severities, and to investigate the relationship between micro and macrovascular functions and exercise capacity and respiratory functions in children with CF of different obstruction severities.

Study Overview

• Status: Completed
• Conditions: Cystic Fibrosis
• Intervention / Treatment: Other: Maximal exercise test; Other: Endothelial dysfunction assessment; Other: Arterial stiffness assessment

Detailed Description

Cystic fibrosis (CF) is a rare disease with congenital deterioration in mucociliary clearance that causes recurrent or chronic rhinosinusitis, airway infection and bronchiectasis in pediatric age. There are pulmonary genetic disorders associated with inflammation. Children with CF have been shown to have vascular endothelial dysfunction compared to healthy children. Very young children with CF have been reported to have increased arterial stiffness and some decrease in right-left ventricular function. In another study on the subject, it was found that stiffness of the great arteries increased in children with CF; The change in arterial compliancy has been shown to be significant in childhood. The increase in arterial stiffness is associated with systemic inflammation, regardless of blood pressure or diabetes. Hemodynamic changes have been reported in the presence of systemic inflammation in children with CF. Exercise peak workload in children with CF is known to be associated with endothelial dysfunction. It is not known how exercise capacity is affected by endothelial dysfunction and arterial stiffness in CF patients of varying severity. In the literature, there are no studies comparing endothelial function, arterial stiffness and its effect on exercise capacity and pulmonary functions in CF children; this subject is open to research. The aim of this study was to investigate and compare microvascular (peripheral endothelial), macrovascular (arterial stiffness) functions in children with CF and to investigate the relationship of micro and macrovascular functions with exercise capacity and pulmonary functions in children with CF of different obstruction severities.

• Study Type: Observational
• Enrollment (Actual): 60

Contacts and Locations

Study Locations

• Turkey
  • Ankara, Turkey
    • Hacettepe University, Faculty of Physical Therapy and Rehabilitation

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 10 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

Children who are diagnosed with Cystic Fibrosis with different obstruction severity at Hacettepe University Faculty of Medicine, Department of Pediatric Chest Diseases and who reffered to the Cardiopulmonary Rehabilitation Unit for Physiotherapy also who are willing to participate in the study will be include in the study.

Description

Inclusion Criteria:

• Being clinically stable
• To be cooperated in evaluations to be made
• Forced expiratory volume in the first second (FEV1)> 40%
• No exacerbation in the past three months
• Regular medication for the past 12 months
• No medication changes for at least three weeks

Exclusion Criteria:

• Cardiovascular disease
• Hypertension
• Diabetes
• Pulmonary hypertension
• Smoking
• Resting oxygen saturation (SpO2) <92%
• Using vasoactive drugs (nitrates, b-blockers, angiotensin-converting enzyme inhibitors, etc.)
• Using oral steroids
• Undergoing lung surgery
• Being a highly orthopedic, neurological disease
• Injury to the lower extremity such as injury, sprain, or fracture in the past six months

Study Plan

How is the study designed?

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Patients with CF with normal obstruction severity; In CF children with normal obstruction severity will be subjected to the same maximal exercise test, endothelial dysfunction and arterial stiffness assessments.	Other: Maximal exercise test; The maximal exercise test will be performed on an electronically braked bicycle ergometer.; Other: Endothelial dysfunction assessment; Endothelial dysfunction will be determined by blood samples. Blood samples will be collected and analyzed for the responses of ICAM-1, VCAM-1, E-Selectin, VEGF, ET-1.; Other: Arterial stiffness assessment; Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.
Patients with CF with mild obstruction severity; In CF children with mild obstruction severity will be subjected to the same maximal exercise test, endothelial dysfunction and arterial stiffness assessments.	Other: Maximal exercise test; The maximal exercise test will be performed on an electronically braked bicycle ergometer.; Other: Endothelial dysfunction assessment; Endothelial dysfunction will be determined by blood samples. Blood samples will be collected and analyzed for the responses of ICAM-1, VCAM-1, E-Selectin, VEGF, ET-1.; Other: Arterial stiffness assessment; Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.
Patients with CF with moderate obstruction severity; In CF children with moderate obstruction severity will be subjected to the same maximal exercise test, endothelial dysfunction and arterial stiffness assessments.	Other: Maximal exercise test; The maximal exercise test will be performed on an electronically braked bicycle ergometer.; Other: Endothelial dysfunction assessment; Endothelial dysfunction will be determined by blood samples. Blood samples will be collected and analyzed for the responses of ICAM-1, VCAM-1, E-Selectin, VEGF, ET-1.; Other: Arterial stiffness assessment; Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Maximal exercise capacity	The maximal exercise test will be performed on an electronically braked bicycle ergometer using the Godfrey protocol. The peak work capacity (Wzirve) measurement will be expressed as a percentage of normal values.	Maximal exercise test will be performed on the first day
Endothelial dysfunction	Endothelial dysfunction will be determined by intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), E-selectin, Vascular endothelial growth factor (VEGF), Endothelin-1 (ET-1). Blood samples will be collected.	Endothelial dysfunction assessment will be performed on the first day
Arterial stiffness	Arterial stiffness, oscillometric pulse wave velocity (PWV) (m s - 1) and augmentation index (AI@75) (%) will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.	Arterial stiffness assessment will be performed on the first day

Collaborators and Investigators

• Sponsor: Hacettepe University
• Investigators: Principal Investigator: Aslihan Cakmak, MSc, Hacettepe University; Principal Investigator: Senem Simsek, MSc, Hacettepe University; Study Director: Deniz Inal-Ince, PhD, Hacettepe University; Principal Investigator: Elmas Ebru Gunes-Yalcin, PhD, Hacettepe University; Principal Investigator: Murat Timur Budak, PhD, Hacettepe University; Principal Investigator: Nagehan Emiralioglu, PhD, Hacettepe University; Principal Investigator: Yasemin Kartal, MSc, Hacettepe University; Principal Investigator: Suna Sabuncuoğlu, PhD, Hacettepe University; Principal Investigator: Meltem Akgül Erdal, MSc, Hacettepe University

Study record dates

Study Major Dates

• Study Start (Actual): April 11, 2022
• Primary Completion (Actual): December 8, 2022
• Study Completion (Actual): May 2, 2023

Study Registration Dates

• First Submitted: January 28, 2020
• First Submitted That Met QC Criteria: February 4, 2020
• First Posted (Actual): February 7, 2020

Study Record Updates

• Last Update Posted (Estimated): December 6, 2023
• Last Update Submitted That Met QC Criteria: November 30, 2023
• Last Verified: November 1, 2023

More Information

Terms related to this study

• Keywords: Children; Exercise Capacity; Arterial Stiffness; Endothelial Dysfunction; Respiratory Functions
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Pancreatic Diseases; Fibrosis; Cystic Fibrosis
• Other Study ID Numbers: GO19/1156

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No