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

Sponsor

Hacettepe University (Other)

Overall Status

Unknown status

CT.gov ID

NCT04259983

Collaborator

(none)

Enrollment

Location

Anticipated Duration (Months)

2.5

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

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.

Condition or Disease	Intervention/Treatment	Phase
Cystic Fibrosis	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 Design

Study Type:

Observational [Patient Registry]

Anticipated Enrollment :

60 participants

Observational Model:

Other

Time Perspective:

Cross-Sectional

Official Title:

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

Actual Study Start Date :

Jan 8, 2020

Anticipated Primary Completion Date :

Jan 8, 2022

Anticipated Study Completion Date :

Jan 8, 2022

Arms and Interventions

Arm	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-, 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-, 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-, 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.

Arm

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-, 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-, 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-, 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.

Outcome Measures

Primary Outcome Measures

Maximal exercise capacity [Maximal exercise test will be performed on the first day]
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.
Endothelial dysfunction [Endothelial dysfunction assessment will be performed on the first day]
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.
Arterial stiffness [Arterial stiffness assessment will be performed on the first day]
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.

Eligibility Criteria

Criteria

Ages Eligible for Study:

10 Years to 18 Years

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

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