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@ARTICLE{Wielptz:128536,
author = {M. O. Wielpütz$^*$ and M. Puderbach and A.
Kopp-Schneider$^*$ and M. Stahl and E. Fritzsching and O.
Sommerburg and S. Ley and M. Sumkauskaite and J.
Biederer$^*$ and H.-U. Kauczor and M. Eichinger and M. A.
Mall},
title = {{M}agnetic resonance imaging detects changes in structure
and perfusion, and response to therapy in early cystic
fibrosis lung disease.},
journal = {American journal of respiratory and critical care medicine},
volume = {189},
number = {8},
issn = {1535-4970},
address = {New York, NY},
publisher = {American Thoracic Society},
reportid = {DKFZ-2017-04552},
pages = {956 - 965},
year = {2014},
abstract = {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).},
cin = {E010 / C060},
ddc = {610},
cid = {I:(DE-He78)E010-20160331 / I:(DE-He78)C060-20160331},
pnm = {315 - Imaging and radiooncology (POF3-315)},
pid = {G:(DE-HGF)POF3-315},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:24564281},
doi = {10.1164/rccm.201309-1659OC},
url = {https://inrepo02.dkfz.de/record/128536},
}