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Longitudinal magnetic resonance imaging of changes in lung morphology and perfusion in children with cystic fibrosis from infancy through adolescence

Rationale: The progression of lung changes in cystic fibrosis (CF) from infancy through adolescence remains poorly understood as a result of limited longitudinal imaging data. - - Objectives: To assess changes in lung morphology and perfusion in children with CF through the pediatric age range by l...

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Main Authors: Wielpütz, Mark Oliver (Author) , Stahl, Mirjam (Author) , Triphan, Simon M. F. (Author) , Wucherpfennig, Lena (Author) , Leutz-Schmidt, Patricia (Author) , Gestewitz, Sonja (Author) , Steinke, Eva (Author) , Graeber, Simon Y. (Author) , Kauczor, Hans-Ulrich (Author) , Eichinger, Monika (Author) , Puderbach, Michael (Author) , Alrajab, Abdulsattar (Author) , Schenk, Jens-Peter (Author) , Sommerburg, Olaf (Author) , Mall, Marcus A. (Author)
Format: Article (Journal)
Language:English
Published: 2025
In: Annals of the American Thoracic Society
Year: 2025, Volume: 22, Issue: 1, Pages: 93-103
ISSN:2325-6621
DOI:10.1513/AnnalsATS.202404-396OC
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1513/AnnalsATS.202404-396OC
Verlag, lizenzpflichtig, Volltext: https://www.atsjournals.org/doi/10.1513/AnnalsATS.202404-396OC
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Author Notes:Mark O. Wielpütz, Mirjam Stahl, Simon M.F. Triphan, Lena Wucherpfennig, Patricia Leutz-Schmidt, Sonja Gestewitz, Eva Steinke, Simon Y. Graeber, Hans-Ulrich Kauczor, Monika Eichinger, Michael U. Puderbach, Abdulsattar Alrajab, Jens-Peter Schenk, Olaf Sommerburg, Marcus A. Mall
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Summary:Rationale: The progression of lung changes in cystic fibrosis (CF) from infancy through adolescence remains poorly understood as a result of limited longitudinal imaging data. - - Objectives: To assess changes in lung morphology and perfusion in children with CF through the pediatric age range by longitudinal chest magnetic resonance imaging (MRI). - - Methods: 1,112 annual chest MRI scans were performed in 226 patients with CF aged 0-18 years. MRI was assessed using a validated MRI scoring system. - - Results: The MRI global score continuously increased from 5.5 ± 4.6 at infancy (0 yr) to 17.9 ± 8.5 at adolescence (12-18 yr), and the MRI morphology score increased from 5.0 ± 3.9 to 12.3 ± 6.1 (P < 0.001). Bronchiectasis/wall thickening prevalence increased from 89.1% at infancy to approximately 100% at preschool age (1-5 yr), and the subscore increased from 3.1 ± 1.9 at infancy to 6.6 ± 2.1 at adolescence (P < 0.001). Mucus plugging prevalence increased from 55.4% at infancy to 83.0% at adolescence, and the subscore increased from 1.2 ± 1.6 to 3.7 ± 2.6 in the same period (P < 0.001). Perfusion abnormalities were found in 44.4% at infancy, and increased to approximately 90% at preschool age (P < 0.001). The MRI perfusion score increased from 1.1 ± 1.6 at infancy to 5.6 ± 3.0 at adolescence (P < 0.001). Chronic Pseudomonas aeruginosa infection was associated with higher MRI scores at school age (6-11 yr; P < 0.05-0.001). - - Conclusions: This is the first study to assess longitudinal changes in lung morphology and perfusion in CF throughout the pediatric age range, providing percentiles as age-specific references for lung disease severity. Our data may facilitate the use of MRI as an endpoint in clinical trials in children with CF. - - Clinical trial registered with www.clinicaltrials.gov (NCT 00760071 and NCT 02270476).
Item Description:Gesehen am 31.05.2025
Physical Description:Online Resource
ISSN:2325-6621
DOI:10.1513/AnnalsATS.202404-396OC

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