Investigation of Relationship Between Vascular Functions, Exercise Capacity, and Respiratory Functions in CF
Condition: Cystic Fibrosis
MeSH terms: Cystic Fibrosis; Fibrosis
Study type: Observational [Patient Registry]

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.
    

Intervention name(s): Maximal exercise test; Endothelial dysfunction assessment; Arterial stiffness assessment
Intervention type(s): Other; Other; Other
Intervention description(s): The maximal exercise test will be performed on an electronically braked bicycle ergometer.; 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.
Observational model: Other
Time perspective: Cross-Sectional
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.
      
Biospecimen description: 
      Blood sample
    
Keywords: Endothelial Dysfunction; Arterial Stiffness; Exercise Capacity; Respiratory Functions; Children