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J Cyst Fibros. 2017 Mar;16(2):275-282. doi: 10.1016/j.jcf.2016.07.008. Epub 2016 Jul 29.

Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.

Author information

1
Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Department of Physics, Washington University in St. Louis, St. Louis, MO, United States.
2
Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
3
Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Division of Pulmonary Medicine, Cincinnati Children's Hospital, Cincinnati, OH, United States.
4
Division of Pulmonary Medicine, Cincinnati Children's Hospital, Cincinnati, OH, United States.
5
Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Department of Physics, Washington University in St. Louis, St. Louis, MO, United States; Division of Pulmonary Medicine, Cincinnati Children's Hospital, Cincinnati, OH, United States. Electronic address: Jason.woods@cchmc.org.

Abstract

BACKGROUND:

Cystic fibrosis (CF) is a genetic disease which carries high morbidity and mortality from lung-function decline. Monitoring disease progression and treatment response in young patients is desirable, but serial imaging via CT is often considered prohibitive, and detailed functional information cannot be obtained using conventional imaging techniques. Hyperpolarized 129Xe magnetic resonance imaging (MRI) can depict and quantify regional ventilation, but has not been investigated in pediatrics. We hypothesized that 129Xe MRI is feasible and would demonstrate ventilation defects in mild CF lung disease with greater sensitivity than FEV1.

METHODS:

11 healthy controls (age 6-16years) and 11 patients with mild CF (age 8-16years, Forced Expiratory Volume (FEV1) percent predicted >70%) were recruited for this study. Nine CF patients had an FEV1>85%. Each subject was imaged via hyperpolarized 129Xe MRI, and the ventilation defect percentage (VDP) was measured. FEV1 and VDP were compared between the groups.

RESULTS:

FEV1 for controls was 100.3%±8.5% (mean±sd) and for CF patients was 97.9%±16.0% (p=0.67). VDP was 6.4%±2.8% for controls and 18.3%±8.6% for CF (p<0.001). When considering the 9 CF patients with normal FEV1 (>85%), the mean FEV1 was 103.1%±12.3% (p=0.57 compared to controls) and VDP was 15.4%±6.3% (p=0.002).

CONCLUSIONS:

Hyperpolarized 129Xe MRI demonstrated ventilation defects in CF patients with normal FEV1 and more effectively discriminated CF from controls than FEV1. Thus 129Xe may be a useful outcome measure to detect mild CF lung disease, to investigate regional lung function in pediatric lung diseases, and to follow disease progression.

KEYWORDS:

Cystic fibrosis; Hyperpolarized; Mri; Pediatric

PMID:
27477942
PMCID:
PMC5274600
DOI:
10.1016/j.jcf.2016.07.008
[Indexed for MEDLINE]
Free PMC Article

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