Assessment of pulmonary structure-function relationships in young children and adolescents with cystic fibrosis by multivolume proton-MRI and CT

J Magn Reson Imaging. 2018 Aug;48(2):531-542. doi: 10.1002/jmri.25978. Epub 2018 Feb 19.


Background: Lung disease is the most frequent cause of morbidity and mortality in patients with cystic fibrosis (CF), and there is a shortage of sensitive biomarkers able to regionally monitor disease progression and to assess early responses to therapy.

Purpose: To determine the feasibility of noncontrast-enhanced multivolume MRI, which assesses intensity changes between expiratory and inspiratory breath-hold images, to detect and quantify regional ventilation abnormalities in CF lung disease, with a focus on the structure-function relationship.

Study type: Retrospective.

Population: Twenty-nine subjects, including healthy young children (n = 9, 7-37 months), healthy adolescents (n = 4, 14-22 years), young children with CF lung disease (n = 10, 7-47 months), and adolescents with CF lung disease (n = 6, 8-18 years) were studied.

Field strength/sequence: 3D spoiled gradient-recalled sequence at 1.5T.

Assessment: Subjects were scanned during breath-hold at functional residual capacity (FRC) and total lung capacity (TLC) through noncontrast-enhanced MRI and CT. Expiratory-inspiratory differences in MR signal-intensity (Δ1 H-MRI) and CT-density (ΔHU) were computed to estimate regional ventilation. MR and CT images were also evaluated using a CF-specific scoring system.

Statistical tests: Quadratic regression, Spearman's correlation, one-way analysis of variance (ANOVA).

Results: Δ1 H-MRI maps were sensitive to ventilation heterogeneity related to gravity dependence in healthy lung and to ventilation impairment in CF lung disease. A high correlation was found between MRI and CT ventilation maps (R2 = 0.79, P < 0.001). Globally, Δ1 H-MRI and ΔHU decrease with increasing morphological score (respectively, R2 = 0.56, P < 0.001 and R2 = 0.31, P < 0.001). Locally, Δ1 H-MRI was higher in healthy regions (median 15%) compared to regions with bronchiectasis, air trapping, consolidation, and to segments fed by airways with bronchial wall thickening (P < 0.001).

Data conclusion: Multivolume noncontrast-enhanced MRI, as a nonionizing imaging modality that can be used on nearly any MRI scanner without specialized equipment or gaseous tracers, may be particularly valuable in CF care, providing a new imaging biomarker to detect early alterations in regional lung structure-function.

Level of evidence: 3 Technical Efficacy: Stage 3 J. MAGN. RESON. IMAGING 2018;48:531-542.

Keywords: cystic fibrosis; lung; pediatrics.

MeSH terms

  • Adolescent
  • Biomarkers
  • Child
  • Child, Preschool
  • Cystic Fibrosis / diagnostic imaging*
  • Disease Progression
  • Female
  • Functional Residual Capacity
  • Humans
  • Infant
  • Lung / diagnostic imaging*
  • Magnetic Resonance Imaging*
  • Male
  • Protons
  • Respiration
  • Respiratory Function Tests
  • Retrospective Studies
  • Tomography, X-Ray Computed*
  • Total Lung Capacity
  • Young Adult


  • Biomarkers
  • Protons