title: Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease.
abstract: BACKGROUND
  Studies demonstrating early structural lung damage in infants and preschool children with cystic fibrosis (CF) suggest that noninvasive monitoring will be important to identify patients who may benefit from early therapeutic intervention. Previous studies demonstrated that magnetic resonance imaging (MRI) detects structural and functional abnormalities in lungs from older patients with CF without radiation exposure.
  
  
  OBJECTIVE
  To evaluate the potential of MRI to detect abnormal lung structure and perfusion in infants and preschool children with CF, and to monitor the response to therapy for pulmonary exacerbation.
  
  
  METHODS
  MRI studies were performed in 50 children with CF (age, 3.1 ± 2.1 yr; range, 0-6 yr) in stable clinical condition (n = 40) or pulmonary exacerbation before and after antibiotic treatment (n = 10), and in 26 non-CF control subjects (age, 2.9 ± 1.9 yr). T1- and T2-weighted sequences before and after intravenous contrast and first-pass perfusion imaging were acquired, and assessed on the basis of a dedicated morphofunctional score.
  
  
  RESULTS
  MRI demonstrated bronchial wall thickening/bronchiectasis, mucus plugging, and perfusion deficits from the first year of life in most stable patients with CF (global score, 10.0 ± 4.0), but not in non-CF control subjects (score, 0.0 ± 0.0; P < 0.001). In patients with exacerbations, the global MRI score was increased to 18.0 ± 2.0 (P < 0.001), and was significantly reduced to 12.0 ± 3.0 (P < 0.05) after antibiotic therapy.
  
  
  CONCLUSIONS
  MRI detected abnormalities in lung structure and perfusion, and response to therapy for exacerbations in infants and preschool children with CF. These results support the development of MRI for noninvasive monitoring and as an end point in interventional trials for early CF lung disease. Clinical trial registered with www.clinicaltrials.gov (NCT00760071).
authors.affiliation: 1 Department of Diagnostic and Interventional Radiology.
authors.forename: Mark O
authors.initials: MO
authors.lastname: Wielpütz
authors.forename: Michael
authors.initials: M
authors.lastname: Puderbach
authors.forename: Annette
authors.initials: A
authors.lastname: Kopp-Schneider
authors.forename: Mirjam
authors.initials: M
authors.lastname: Stahl
authors.forename: Eva
authors.initials: E
authors.lastname: Fritzsching
authors.forename: Olaf
authors.initials: O
authors.lastname: Sommerburg
authors.forename: Sebastian
authors.initials: S
authors.lastname: Ley
authors.forename: Migle
authors.initials: M
authors.lastname: Sumkauskaite
authors.forename: Jürgen
authors.initials: J
authors.lastname: Biederer
authors.forename: Hans-Ulrich
authors.initials: HU
authors.lastname: Kauczor
authors.forename: Monika
authors.initials: M
authors.lastname: Eichinger
authors.forename: Marcus A
authors.initials: MA
authors.lastname: Mall
mesh_terms: D016022:Case-Control Studies; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D011237:Predictive Value of Tests; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index