Physiology and pathophysiology of human airway mucus
The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …
Cystic fibrosis and Pseudomonas aeruginosa: the host-microbe interface
In human pathophysiology, the clash between microbial infection and host immunity
contributes to multiple diseases. Cystic fibrosis (CF) is a classical example of this …
contributes to multiple diseases. Cystic fibrosis (CF) is a classical example of this …
Lung clearance index and structural lung disease on computed tomography in early cystic fibrosis
KA Ramsey, T Rosenow, L Turkovic… - American journal of …, 2016 - atsjournals.org
Rationale: The lung clearance index is a measure of ventilation distribution derived from the
multiple-breath washout technique. It has been suggested as a surrogate for chest …
multiple-breath washout technique. It has been suggested as a surrogate for chest …
Magnetic resonance imaging detects progression of lung disease and impact of newborn screening in preschool children with cystic fibrosis
M Stahl, E Steinke, SY Graeber, C Joachim… - American journal of …, 2021 - atsjournals.org
Rationale: Previous cross-sectional studies have demonstrated that chest magnetic
resonance imaging (MRI) is sensitive to detect early lung disease in infants and preschool …
resonance imaging (MRI) is sensitive to detect early lung disease in infants and preschool …
Neonatal pulmonary magnetic resonance imaging of bronchopulmonary dysplasia predicts short-term clinical outcomes
Rationale: Bronchopulmonary dysplasia (BPD) is a serious neonatal pulmonary condition
associated with premature birth, but the underlying parenchymal disease and trajectory are …
associated with premature birth, but the underlying parenchymal disease and trajectory are …
The effect of inhaled hypertonic saline on lung structure in children aged 3–6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled …
HAWM Tiddens, Y Chen, ER Andrinopoulou… - The lancet Respiratory …, 2022 - thelancet.com
Summary Background In the Saline Hypertonic in Preschoolers (SHIP) study, inhaled 7%
hypertonic saline improved the lung clearance index in children aged 3–6 years with cystic …
hypertonic saline improved the lung clearance index in children aged 3–6 years with cystic …
Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution
G Dournes, F Menut, J Macey, M Fayon, JF Chateil… - European …, 2016 - Springer
Objectives We hypothesized that non-contrast-enhanced PETRA (pointwise encoding time
reduction with radial acquisition) MR (magnetic resonance) sequencing could be an …
reduction with radial acquisition) MR (magnetic resonance) sequencing could be an …
Elastase exocytosis by airway neutrophils is associated with early lung damage in children with cystic fibrosis
C Margaroli, LW Garratt, H Horati… - American Journal of …, 2019 - atsjournals.org
Rationale: Neutrophils are recruited to the airways of individuals with cystic fibrosis (CF). In
adolescents and adults with CF, airway neutrophils actively exocytose the primary granule …
adolescents and adults with CF, airway neutrophils actively exocytose the primary granule …
Early lung disease in infants and preschool children with cystic fibrosis. What have we learned and what should we do about it?
The past decade has seen significant advances in understanding of the pathogenesis and
progression of lung disease in cystic fibrosis (CF). Pulmonary inflammation, infection, and …
progression of lung disease in cystic fibrosis (CF). Pulmonary inflammation, infection, and …
Improvement in lung clearance index and chest computed tomography scores with elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis aged 12 …
Rationale: Clinical trials have shown that use of elexacaftor/tezacaftor/ivacaftor (ETI) is
associated with improvements in sweat chloride, pulmonary function, nutrition, and quality of …
associated with improvements in sweat chloride, pulmonary function, nutrition, and quality of …