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

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

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

Conditions
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Cystic Fibrosis

Intervention / Treatment
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* Other: Maximal exercise test
* Other: Endothelial dysfunction assessment
* Other: Arterial stiffness assessment


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 10 Years
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients with CF with normal obstruction severity<br>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<br>* The maximal exercise test will be performed on an electronically braked bicycle ergometer.<br>Other: Endothelial dysfunction assessment<br>* 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.<br>Other: Arterial stiffness assessment<br>* Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.<br>|
| Patients with CF with mild obstruction severity<br>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<br>* The maximal exercise test will be performed on an electronically braked bicycle ergometer.<br>Other: Endothelial dysfunction assessment<br>* 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.<br>Other: Arterial stiffness assessment<br>* Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.<br>|
| Patients with CF with moderate obstruction severity<br>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<br>* The maximal exercise test will be performed on an electronically braked bicycle ergometer.<br>Other: Endothelial dysfunction assessment<br>* 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.<br>Other: Arterial stiffness assessment<br>* Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.<br>|

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Endothelial Dysfunction, Arterial Stiffness, Exercise Capacity, Respiratory Functions, Children