Early cystic fibrosis lung disease: role of airway surface dehydration and lessons from preventive rehydration therapies in mice

MA Mall, SY Graeber, M Stahl… - The International Journal of …, 2014 - Elsevier
Cystic fibrosis (CF) lung disease starts in the first months of life and remains one of the most
common fatal hereditary diseases. Early therapeutic interventions may provide an …

Volumetric quantification of lung MR signal intensities using ultrashort TE as an automated score in cystic fibrosis

I Benlala, S Point, C Leung, P Berger, JC Woods… - European …, 2020 - Springer
Objectives The study aimed to validate automated quantification of high and low signal
intensity volumes using ultrashort echo-time MRI, with CT and pulmonary function test (PFT) …

A semiquantitative MRI-Score can predict loss of lung function in patients with cystic fibrosis: Preliminary results

JF Schaefer, A Hector, K Schmidt, M Teufel… - European …, 2018 - Springer
Objectives To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S)
as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease. Methods This …

Echo Time‐Dependent Observed Lung T1 in Patients With Chronic Obstructive Pulmonary Disease in Correlation With Quantitative Imaging and Clinical Indices

SMF Triphan, O Weinheimer… - Journal of Magnetic …, 2021 - Wiley Online Library
Background There is a clinical need for imaging‐derived biomarkers for the management of
chronic obstructive pulmonary disease (COPD). Observed pulmonary T1 (T1 (TE)) depends …

Within‐breath changes in respiratory system impedance in children with cystic fibrosis

E Zannin, S Nyilas, KA Ramsey, P Latzin… - Pediatric …, 2019 - Wiley Online Library
Background The aim of this study was to assess within‐breath respiratory system
impedance by the forced oscillation technique (FOT) in children with cystic fibrosis (CF) and …

Rapid 3D in vivo 1H human lung respiratory imaging at 1.5 T using ultra‐fast balanced steady‐state free precession

O Pusterla, G Bauman, MO Wielpuetz… - Magnetic resonance …, 2017 - Wiley Online Library
Purpose To introduce a reproducible, nonenhanced 1H MRI method for rapid in vivo
functional assessment of the whole lung at 1.5 Tesla (T). Methods At different respiratory …

[HTML][HTML] Reanalysis of N2-lung clearance index and the comparison to SF6-lung clearance index and magnetic resonance imaging

E Steinke, MO Wielpütz, C Joachim, MA Mall… - Journal of Cystic …, 2024 - Elsevier
Since the finding and correction of an error in previous spiroware software versions
commonly used with the Exhalyzer D for multiple-breath washout (MBW) analysis, there has …

[HTML][HTML] Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis

MM Willmering, DJ Roach, EL Kramer, LL Walkup… - Journal of Cystic …, 2021 - Elsevier
Background Two functional measurements (multiple breath washout [MBW] and
hyperpolarized 129 Xe ventilation magnetic resonance imaging [129 Xe MRI]) have been …

Multi-layer ventilation inhomogeneity in cystic fibrosis

S Krueger-Ziolek, B Schullcke, Z Zhao, B Gong… - Respiratory Physiology …, 2016 - Elsevier
Differences in regional lung function between the 3rd and 5th intercostal space (ICS) were
explored in 10 cystic fibrosis (CF) patients and compared to 10 lung-healthy controls by …

Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study

F Neemuchwala, M Ghadimi Mahani, Y Pang, E Lee… - Pediatric …, 2020 - Springer
Background Assessment tools for early cystic fibrosis (CF) lung disease are limited.
Detecting early pulmonary disease is crucial to increasing life expectancy by starting …