title: Non invasive assessment of lung disease in ataxia telangiectasia by high-field magnetic resonance imaging.
abstract: OBJECTIVE
  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.
authors: Department of Translational Medical Sciences, "Federico II" University, Via Pansini 5, 80131, Naples, Italy.
authors: Silvia
authors: S
authors: Montella
authors: Carmine
authors: C
authors: Mollica
authors: Andrea
authors: A
authors: Finocchi
authors: Andrea
authors: A
authors: Pession
authors: Maria Cristina
authors: MC
authors: Pietrogrande
authors: Antonino
authors: A
authors: Trizzino
authors: Giusy
authors: G
authors: Ranucci
authors: Marco
authors: M
authors: Maglione
authors: Giuliana
authors: G
authors: Giardino
authors: Marco
authors: M
authors: Salvatore
authors: Francesca
authors: F
authors: Santamaria
authors: Claudio
authors: C
authors: Pignata
mesh_terms: D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000175:diagnosis* / Q000503:physiopathology; D001987:Bronchiectasis / Q000175:diagnosis* / Q000503:physiopathology; D002648:Child; D018450:Disease Progression; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009093:Mucus / Q000378:metabolism; D011877:Radionuclide Imaging; D012129:Respiratory Function Tests; D055815:Young Adult
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