Hyperpolarized Xenon MRI in Cystic Fibrosis Pulmonary Exacerbations

Status Completed

Clinical Trial Summary

The investigators aim to assess whether pulmonary MRI (hyperpolarised 129Xe ventilation imaging [Xe-MRI]) can detect changes in ventilation defects in patients with CF before and after treatment for a pulmonary exacerbation. The investigators will determine whether changes seen using pulmonary Xe-MRI are associated with changes in pulmonary function (spirometry, lung volumes, lung clearance index [LCI]) in patients with CF before and after pulmonary exacerbation.

Clinical Trial Description

Cystic fibrosis (CF) is one of the most common genetic diseases affecting children and young adults [1]. Lung disease is the primary cause of morbidity and mortality in these patients and sensitive markers of lung disease in CF are important for directing therapy in these patients.

LCI, measured by multiple breath washout (MBW), has been shown to be more sensitive than traditional pulmonary function tests (PFTs) for assessing the treatment effect of novel therapies in CF patients [2,3]. However, LCI provides no information regarding the spatial distribution of ventilation inhomogeneity within the lungs and improvements in mucus plugging of poorly ventilated regions can paradoxically worsen the LCI [4,5]. Thus an imaging technique that can capture regional changes in the distribution of ventilation might be better suited than LCI to detect treatment effects and could also help to better define the utility of LCI as a clinical tool.

Xe-MRI is a safe, non-ionizing modality for imaging the lungs, providing an accurate spatial representation of ventilation inhomogeneity [6]. Xe-MRI has been shown to be effective in imaging of adult patients with chronic obstructive pulmonary disease (COPD) and CF [6] however, there are no published studies using Xe-MRI in children.

The hypothesis of this study is that Xe-MRI and LCI will provide complimentary information when quantifying ventilation inhomogeneity in CF lung disease and that Xe-MRI will be able to define patients in whom LCI fails to capture positive effects of treatment. The ultimate goal is to develop more sensitive tools for longitudinal monitoring to direct the clinical care of CF patients in the future.

To accomplish this, the investigators will compare the ability of Xe-MRI and LCI to detect changes in ventilation inhomogeneity in patients with CF before and after treatment for a pulmonary exacerbation, a common pulmonary complication of CF. ;

Study Design

Related Conditions & MeSH terms

Clinical Trial Details

NCT number	NCT02606487
Study type	Observational
Source	The Hospital for Sick Children
Contact
Status	Completed
Phase
Start date	November 2015
Completion date	November 2017

View Details

See also
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Clinical trials are conducted in a series of steps, called phases - each phase is designed to answer a separate research question.

Phase 1: Researchers test a new drug or treatment in a small group of people for the first time to evaluate its safety, determine a safe dosage range, and identify side effects.
Phase 2: The drug or treatment is given to a larger group of people to see if it is effective and to further evaluate its safety.
Phase 3: The drug or treatment is given to large groups of people to confirm its effectiveness, monitor side effects, compare it to commonly used treatments, and collect information that will allow the drug or treatment to be used safely.
Phase 4: Studies are done after the drug or treatment has been marketed to gather information on the drug's effect in various populations and any side effects associated with long-term use.