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New severity assessment in cystic fibrosis: signal intensity and lung volume compared to LCI and FEV1: preliminary results

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Abstract

Objectives

Magnetic resonance imaging (MRI) aids diagnosis in cystic fibrosis (CF) but its use in quantitative severity assessment is under research. This study aims to assess changes in signal intensity (SI) and lung volumes (Vol) during functional MRI and their use as a severity assessment tool in CF patients.

Methods

The CF intra-hospital standard chest 1.5 T MRI protocol comprises of very short echo-time sequences in submaximal in- and expiration for functional information. Quantitative measurements (Vol/SI at in- and expiration, relative differences (Vol_delta/SI_delta), and cumulative histograms for normalized SI values across the expiratory lung volume) were assessed for correlation to pulmonary function: lung clearance index (LCI) and forced expiratory volume in 1 s (FEV1).

Results

In 49 patients (26 male, mean age 17 ± 7 years) significant correlation of Vol_delta and SI_delta (R = 0.86; p < 0.0001) during respiration was observed. Individual cumulated histograms enabled severity disease differentiation (mild, severe) to be visualized (defined by functional parameter: LCI > 10). The expiratory volume at a relative SI of 100% correlated significantly to LCI (R = 0.676 and 0.627; p < 0.0001) and FEV1 (R = − 0.847 and − 0.807; p < 0.0001). Clustering patients according to Vol_SI_100 showed that an amount of ≤ 4% was related to normal, while an amount of > 4% was associated with pathological pulmonary function values.

Conclusion

Functional pulmonary MRI provides a radiation-free severity assessment tool and can contribute to early detection of lung impairment in CF. Lung volume with SI below 100% of the inspiratory volume represents overinflated tissue; an amount of 4% of the expiratory lung volume was a relevant turning point.

Key Points

• Signal intensity and lung volumes are used as potential metric parameters for lung impairment.

• Quantification of trapped air impacts on therapy management.

• Functional pulmonary MRI can contribute to early detection of lung impairment.

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Abbreviations

CF:

Cystic fibrosis

CT:

Computer tomography

FEV1 :

Forced expiratory volume in 1 s

LCI:

Lung clearance index

MRI:

Magnetic resonance imaging

PFT:

Pulmonary function test

SI:

Signal intensity

SI/Vol_delta:

Signal intensity/volume difference between in- and expiration

SI/Vol_in/ex:

In-/expiratory signal intensity

Vol:

Lung volumetry

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Acknowledgments

We would like to separately acknowledge with gratitude the substantial work of the late Dr. Riethmueller toward this project and his meaningful close collaboration. We would also like to thank Dr. Andrew Dickinson for his help editing this manuscript.

Funding

The authors state that this work has not received any funding.

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Author notes

  1. Sabrina Fleischer and Mareen Sarah Kraus contributed equally to this work.

Authors and Affiliations

  1. Department of Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076, Tuebingen, Germany

    Sabrina Fleischer, Mareen Sarah Kraus, Sergios Gatidis, Ilias Tsiflikas & Juergen Frank Schaefer

  2. Department of Pediatrics, University Hospital Tuebingen, Hoppe-Seyler-Strasse 1, 72076, Tuebingen, Germany

    Winfried Baden, Andreas Hector & Dominik Hartl

Authors
  1. Sabrina Fleischer
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Corresponding author

Correspondence to Mareen Sarah Kraus.

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Guarantor

The scientific guarantor of this publication is Prof. JF Schäfer.

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The authors declare that they have no conflict of interest.

Statistics and biometry

One of the authors has significant statistical expertise and no complex statistical methods were necessary for this paper.

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Written informed consent was obtained from all patients in this study.

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Institutional Review Board approval was obtained.

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Fleischer, S., Kraus, M.S., Gatidis, S. et al. New severity assessment in cystic fibrosis: signal intensity and lung volume compared to LCI and FEV1: preliminary results. Eur Radiol 30, 1350–1358 (2020). https://doi.org/10.1007/s00330-019-06462-8

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  • DOI: https://doi.org/10.1007/s00330-019-06462-8

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