Cystic Fibrosis
Conditions
Keywords
Endothelial Dysfunction, Arterial Stiffness, Exercise Capacity, Respiratory Functions, Children
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.
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.
Interventions
The maximal exercise test will be performed on an electronically braked bicycle ergometer.
OTHEREndothelial 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.
Arterial stiffness, oscillometric pulse wave velocity (PWV) and augmentation index will be evaluated by recorded brachial pulse waves with automatically with the oscillometric device.
Sponsors
Hacettepe University
Study design
Observational model
OTHER
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
10 Years to 18 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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. |
Countries
Turkey (Türkiye)
Outcome results
None listed