Comparing Chest Images From MRI to CT in Patients With Cystic Fibrosis (CF)
Condition: Cystic Fibrosis
MeSH terms: Cystic Fibrosis; Fibrosis
Study type: Observational

Summary: 
      The purpose of this research is to learn more about the heart and blood vessels in the lungs
      of people with cystic fibrosis (CF). This study will include approximately 36 children and
      adults with CF and 12 children and adults without CF. The study will involve one magnetic
      resonance imaging (MRI) . The research also includes blood samples to look at inflammation
      and lung tests called spirometry and Lung Clearance Index (LCI) determined by Multiple Breath
      Washout test.

      For the CF subjects, enrollment will be timed to coincide with routine scheduled computerized
      tomography (CT) or the CF subject may choose to have a CT for research purposes. The MRI will
      be compared to CT images of the lungs. The goal is to develop better imaging that does not
      use radiation.
    

Detailed description: 
      Cystic Fibrosis (CF), the most common inherited disease in Caucasians, is characterized by
      chronic pulmonary inflammation and progressive loss of gas exchange units that eventually
      results in respiratory failure. There is strong evidence that in CF abnormally low vascular
      perfusion carries a high risk of death independent from the presence of pulmonary
      hypertension. However, the evolution of pulmonary vascular disease in CF and how it might
      contribute to the rate of decline in lung function is not known. Knowledge remains limited to
      the results of old observational studies which concluded that the major causes of pulmonary
      vascular remodeling and hypertension in CF are hypoxic respiratory failure and destruction of
      lung tissue. Recent data obtained by state-of-the-art Magnetic Resonance Imaging (MRI) of the
      pulmonary circulation, challenges the existing paradigm. Studies demonstrate that in the
      absence of hypoxia, significant changes in pulmonary perfusion and in surrogate measures of
      vascular resistance as well as in collateral blood flow begin early in the course of CF. The
      newly developed therapeutics in the last decade which altered dramatically the course of
      patients suffering from pulmonary vascular disease provide opportunities to understand the
      role of pulmonary vasculature in CF lung disease.

      This pilot study (Pilot 3) will assess the relationships between pulmonary perfusion, serum
      markers of pulmonary inflammation and vascular remodeling, and hemodynamic measures
      consistent with pulmonary hypertension. This study will establish MRI as a readily obtainable
      modality to be used in the CF population to obtain measurements of pulmonary perfusion;
      pulmonary arterial hemodynamic, dimension, and flow measures; and ventricular mass and
      function measures.

      The primary goal of this study is to improve the understanding of pulmonary vascular disease
      in CF by examining pulmonary perfusion abnormalities and their association with markers of
      pulmonary inflammation and pulmonary vascular remodeling, the severity of obstructive airways
      disease, and pulmonary hemodynamic abnormalities in CF.

      This study will include a single MRI of the pulmonary circulation on about 36 pediatric and
      adult patients with CF and about 12 non-CF controls. UTE (Ultrashort Echo Time) MRI will be
      performed without contrast. CF patients who are scheduled for clinical chest CT will be
      enrolled and the scheduling of the MRI will be coordinated with the clinical CT. CF subjects
      may also choose to enroll independent of clinical CT and choose to have a CT for research
      purposes only. Other study procedures include blood specimen for serum biomarkers and lung
      tests called spirometry and Lung Clearance Index (LCI) determined by Multiple Breath Washout
      test. Blood specimens will be analyzed for a panel of inflammatory markers.
    

Observational model: Case-Control
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        Cystic Fibrosis and Non-CF Controls
      
Biospecimen description: 
      serum
    
Keywords: Cystic Fibrosis; Pulmonary perfusion; Inflammatory markers; MRI; CT of chest; Lung Clearance Index