Gene therapy for cystic fibrosis lung disease: overcoming the barriers to translation to the clinic

M Donnelley, DW Parsons - Frontiers in pharmacology, 2018 - frontiersin.org
Cystic fibrosis (CF) is a progressive, chronic and debilitating genetic disease caused by
mutations in the CF Transmembrane-Conductance Regulator (CFTR) gene. Unrelenting …

Experimental methods for flow and aerosol measurements in human airways and their replicas

F Lizal, J Jedelsky, K Morgan, K Bauer, J Llop… - European Journal of …, 2018 - Elsevier
Recent developments in the prediction of local aerosol deposition in human lungs are driven
by the fast development of computational simulations. Although such simulations provide …

Synchrotron-based dynamic computed tomography of tissue motion for regional lung function measurement

S Dubsky, SB Hooper, KKW Siu… - Journal of The Royal …, 2012 - royalsocietypublishing.org
During breathing, lung inflation is a dynamic process involving a balance of mechanical
factors, including trans-pulmonary pressure gradients, tissue compliance and airway …

Quantification of heterogeneity in lung disease with image-based pulmonary function testing

CS Stahr, CR Samarage, M Donnelley, N Farrow… - Scientific reports, 2016 - nature.com
Computed tomography (CT) and spirometry are the mainstays of clinical pulmonary
assessment. Spirometry is effort dependent and only provides a single global measure that …

Functional imaging for assessing regional lung ventilation in preclinical and clinical research

D Karmali, M Sowho, S Bose, J Pearce… - Frontiers in …, 2023 - frontiersin.org
Dynamic heterogeneity in lung ventilation is an important measure of pulmonary function
and may be characteristic of early pulmonary disease. While standard indices like …

Inhaled antibiotics: dry or wet?

HAWM Tiddens, AC Bos, JW Mouton… - European …, 2014 - Eur Respiratory Soc
Dry powder inhalers (DPIs) delivering antibiotics for the suppressive treatment of
Pseudomonas aeruginosa in cystic fibrosis patients were developed recently and are now …

[PDF][PDF] Methods for dynamic synchrotron X-ray respiratory imaging in live animals

KS Morgan, D Parsons, P Cmielewski… - Journal of …, 2020 - journals.iucr.org
Small-animal physiology studies are typically complicated, but the level of complexity is
greatly increased when performing live-animal X-ray imaging studies at synchrotron and …

[PDF][PDF] Dose optimization approach to fast X-ray microtomography of the lung alveoli

G Lovric, SF Barré, JC Schittny… - Journal of applied …, 2013 - journals.iucr.org
A basic prerequisite for in vivo X-ray imaging of the lung is the exact determination of
radiation dose. Achieving resolutions of the order of micrometres may become particularly …

Real-time in vivo imaging of regional lung function in a mouse model of cystic fibrosis on a laboratory X-ray source

RP Murrie, F Werdiger, M Donnelley, Y Lin… - Scientific reports, 2020 - nature.com
Most measures of lung health independently characterise either global lung function or
regional lung structure. The ability to measure airflow and lung function regionally would …

Quantifying ventilation by X-ray velocimetry in healthy adults

T Siddharthan, K Grealis, JP Kirkness, T Ötvös… - Respiratory …, 2023 - Springer
Rationale X-ray velocimetry (XV) has been utilized in preclinical models to assess lung
motion and regional ventilation, though no studies have compared XV-derived physiologic …