TY  - JOUR
AU  - Wielpütz, Mark Oliver
AU  - Puderbach, Michael
AU  - Kopp-Schneider, Annette
AU  - Stahl, Mirjam
AU  - Fritzsching, Eva
AU  - Sommerburg, Olaf
AU  - Ley, Sebastian
AU  - Sumkauskaite, Migle
AU  - Biederer, Jürgen
AU  - Kauczor, Hans-Ulrich
AU  - Eichinger, Monika
AU  - Mall, Marcus A
TI  - Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease.
JO  - American journal of respiratory and critical care medicine
VL  - 189
IS  - 8
SN  - 1535-4970
CY  - New York, NY
PB  - American Thoracic Society
M1  - DKFZ-2017-04552
SP  - 956 - 965
PY  - 2014
AB  - 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.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.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.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.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).
LB  - PUB:(DE-HGF)16
C6  - pmid:24564281
DO  - DOI:10.1164/rccm.201309-1659OC
UR  - https://inrepo02.dkfz.de/record/128536
ER  -