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Published in: Journal of Clinical Immunology 7/2013

01-10-2013 | Original Research

Non Invasive Assessment of Lung Disease in Ataxia Telangiectasia by High-Field Magnetic Resonance Imaging

Authors: Silvia Montella, Carmine Mollica, Andrea Finocchi, Andrea Pession, Maria Cristina Pietrogrande, Antonino Trizzino, Giusy Ranucci, Marco Maglione, Giuliana Giardino, Marco Salvatore, Francesca Santamaria, Claudio Pignata

Published in: Journal of Clinical Immunology | Issue 7/2013

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Abstract

Purpose

A sensitive imaging technique that assesses ataxia telangiectasia (AT) lung disease without ionizing radiation is highly desirable. We designed a study to evaluate lung changes using magnetic resonance imaging (MRI), and to investigate the relationships among severity and extent of pulmonary abnormalities and clinical, microbiological and functional data in children and young adults with AT.

Methods

Fifteen AT patients (age, 11.3 years; range, 6–31) underwent 3.0-T MRI, spirometry, and deep throat or sputum culture. Images were scored using a modified Helbich score.

Results

Although only 8 patients (53 %) had recurrent/chronic respiratory symptoms, MRI identified lung abnormalities in all. Bronchiectasis, peribronchial thickening, mucous plugging, and collapse/consolidation were present in 60 %, 87 %, 67 %, and 13 % of cases, respectively, with no difference between subjects with or without respiratory symptoms. No difference in changes of specific scores was found between the two groups, but the total MRI score was higher in patients with respiratory symptoms (6.5 versus 5, respectively; p = 0.02). Total or specific MRI scores were not associated with patients’ age. Of all scores, only mucous plugging subscore appeared significantly related to FEV1 (r = 0.7, p = 0.04) and FEF25–75% (r = 0.9, p = 0.001). MRI scores from patients with positive (n = 5) or negative (n = 10) sputum culture were not significantly different.

Conclusions

MRI is valuable in the assessment of extent and severity of pulmonary changes in children and adults with AT. It represents an helpful tool for the longitudinal evaluation of patients and may be also used as an outcome surrogate to track the effects of medications.
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Metadata
Title
Non Invasive Assessment of Lung Disease in Ataxia Telangiectasia by High-Field Magnetic Resonance Imaging
Authors
Silvia Montella
Carmine Mollica
Andrea Finocchi
Andrea Pession
Maria Cristina Pietrogrande
Antonino Trizzino
Giusy Ranucci
Marco Maglione
Giuliana Giardino
Marco Salvatore
Francesca Santamaria
Claudio Pignata
Publication date
01-10-2013
Publisher
Springer US
Published in
Journal of Clinical Immunology / Issue 7/2013
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-013-9933-y

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