Background: MRI has been suggested as a radiation-free imaging modality to investigate early structural alterations and regional functional impairment in cystic fibrosis (CF) lung disease.
Purpose/hypothesis: To compare functional and morphological MRI changes over the course of the disease to changes in spirometry.
Study Type: Longitudinal retrospective study.
Population: Twenty patients with CF lung disease (at baseline, age = 16.5 (13.3-20.6) years, forced expiratory volume in 1 second (as % of predicted [%pred]) FEV = 71 (59-87) %pred, forced expiratory flow at 25-75% of forced vital capacity FEF = 39 (25-63) %pred.
Field Strength/sequence: 1.5T / T -weighted HASTE; T -weighted TSE-PROPELLER; T -weighted bSSFP; T -weighted 3D GRE.
Assessment: Nonenhanced chest MRI and spirometry were retrospectively collected over a 3-year period from the initial recruitment visit. Images acquired at end-inspiration and end-expiration were registered by software using the optical flow method to measure expiratory-inspiratory differences in MR signal-intensity (Δ H-MRI). Measures of CF functional impairment were defined from Δ H-MRI: Δ H-MRI median, Δ H-MRI quartile coefficient of variation (QCV), and percent low-signal-variation volume (LVV). MR images were also evaluated by three readers using a CF-specific scoring system.
Statistical Tests: Spearman correlation analysis, Spearman rank correlation analysis, linear mixed-effect model analysis, intraclass correlation coefficient.
Results: Functional imaging parameters and total morphological score correlated with all spirometric measures, as did subscores of bronchial wall thickening/bronchiectasis, mucus plugging, and consolidation. Overall, the percent change of Δ1H-MRI median correlated with the percent change of FEV (ΔFEV , r = 0.41, P < 0.01) and the percent change of FEF (ΔFEF25-75%, r = 0.38, P < 0.01). The percent change of LVV correlated with ΔFEV (r = -0.47, P < 0.001) and ΔFEF (r = -0.50, P < 0.001).
Data Conclusion: These preliminary results suggest that nonenhanced multivolume MRI may provide a feasible tool to regionally map early pulmonary alterations for longitudinal evaluation of CF lung disease, without exposing the patients to ionizing radiation.
Level Of Evidence: 3T TECHNICAL EFFICACY STAGE: 5.
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http://dx.doi.org/10.1002/jmri.27461 | DOI Listing |
Hum Gene Ther
January 2025
Department of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
Cystic fibrosis (CF) is caused by mutations in the (). While gene therapy holds promise as a cure, the cell-type-specific heterogeneity of expression in the lung presents significant challenges. Current CF ferret models closely replicate the human disease phenotype but have limitations in studying functional complementation through cell-type-specific CFTR restoration.
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Division of Otolaryngology - Head & Neck Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
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BMC Pediatr
January 2025
Department of Pediatrics II (Neonatology), Medical University of Innsbruck, Innsbruck, Austria.
Preterm infants are at high risk of developing respiratory distress syndrome (RDS). Mutations in the genes encoding for surfactant proteins B and C or the ATP-binding cassette transporter A3 (ABCA3) are rare but known to be associated with severe RDS and interstitial lung diseases. The exact prevalence of these mutations in the general population is difficult to determine, as they are usually studied in connection with clinical symptoms.
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January 2025
Infectious Diseases Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.
Infectious complications significantly impact morbidity and mortality following lung transplantation (LuTx), with over 25% of post-transplant deaths attributed to infections. Antibiotic prophylaxis during the surgical procedure is crucial for reducing early infections, though the current use of wide-spectrum antibiotics, especially in cases of multidrug-resistant organisms (MDROs), is contentious and varies widely across centre. This practice raises concerns about antimicrobial resistance (AMR), particularly in immunosuppressed patients requiring lifelong healthcare access.
View Article and Find Full Text PDFJ Med Internet Res
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Department of Pediatric Pulmonology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, Netherlands.
Background: Home spirometers have been widely implemented in the treatment of people with cystic fibrosis (CF). Frequent spirometry measurements at home could lead to earlier detection of exacerbations. However, previous research indicates that the long-term use of home spirometry is not well maintained by people with CF.
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