Abstract
PurposeTo test quantitative functional lung MRI techniques in young adults with cystic fibrosis (CF) compared to healthy volunteers and to monitor immediate treatment effects of a single inhalation of hypertonic saline in comparison to clinical routine pulmonary function tests.Materials and methodsSixteen clinically stable CF patients and 12 healthy volunteers prospectively underwent two functional lung MRI scans and pulmonary function tests before and 2h after a single treatment of inhaled hypertonic saline or without any treatment. MRI-derived oxygen enhanced T1 relaxation measurements, fractional ventilation, first-pass perfusion parameters and a morpho-functional CF-MRI score were acquired.ResultsCompared to healthy controls functional lung MRI detected and quantified significantly increased ventilation heterogeneity in CF patients. Regional functional lung MRI measures of ventilation and perfusion as well as the CF-MRI score and pulmonary function tests could not detect a significant treatment effect two hours after a single treatment with hypertonic saline in young adults with CF (p>0.05).ConclusionThis study shows the feasibility of functional lung MRI as a non-invasive, radiation-free tool for monitoring patients with CF.
Highlights
Cystic fibrosis (CF) is an autosomal recessive, monogenetic multi-organ disorder
This study shows the feasibility of functional lung magnetic resonance imaging (MRI) as a non-invasive, radiation-free tool for monitoring patients with CF
The datasets of the pre-treatment scan of all CF patients of the treatment group (n = 10) and first scan of the CF patients of the control group, who had not participated in the treatment group (n = 2) were used for the comparison of CF patients with healthy volunteers
Summary
Cystic fibrosis (CF) is an autosomal recessive, monogenetic multi-organ disorder. Airway disease determines morbidity and prognosis in most patients [1]. Therapeutic interventions to improve mucus clearance are a cornerstone of treatment in CF [4]. Such interventions include regular chest physiotherapy, mucolytics, and aerosolized hypertonic saline (HTS; 3% to 7% NaCl) [5]. An adequate technology to address this issue could be functional lung magnetic resonance imaging (MRI), which has recently been shown to be feasible and sensitive to regional lung function changes in CF patients [10,11,12]. In recent years efforts have been made to assess regional pulmonary function using lung MRI: Oxygen enhanced functional lung MRI exploits changes of the T1 times under normoxic and hyperoxic conditions related to a combination of ventilation, perfusion and diffusion capacity of the lung[13]. A 4D dataset of T1 weighed images with a high temporal resolution is acquired after injection of i.v. contrast medium, allowing calculation of different hemodynamic parameters including pulmonary parenchymal blood flow[18,19,20]
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