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Cystic Fibrosis

Cystic Fibrosis (CF) is an inherited autosomal recessive disease resulting from mutations in the CF Transmembrane conductance Regulator (CFTR) gene. CF patients progressively develop a pronounced respiratory phenotype, as the absence of CFTR function in the lung is associated with the thickening of secretions as well as the inability to properly excrete or clear them. This leads to bacteria accumulation and eventually bronchiectasis, allergic bronchopulmonary aspergillosis, and even respiratory failure.

Experimental research in this area aims to increase knowledge of the underlying pathophysiological mechanisms associated with disease manifestation and progression while attempting to address the challenges of therapeutic intervention and early disease detection.

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Relevant, translational and reproducible outcomes

As a result of thickened mucus accumulation that forms in cystic fibrotic lungs, airways become impeded and lung function is hindered. This also progressively leads to airflow obstruction from mucus plaques and plugs, and typically leads to infection and inflammation.

In humans, CF is typically diagnosed using sweat tests, computed tomography (CT) scans and pulmonary function tests. The ability of the flexiVent system to synchronize with micro-CT scanners, to provide static and dynamic measurements of respiratory mechanics, as well as to capture information on specific lung volumes or flows make it an extremely valuable tool to investigate CF at the preclinical level. As an example, changes in static compliance, acquired using the sensitive pressure-volume curves, allowed researchers to distinguish between degrees of disease severity or therapeutic efficacy. 

In addition to measuring key CF-relevant pulmonary parameters such as static compliance and tissue mechanics, the flexiVent has the capacity to study clinically translational expiration measurements such as airflow (FEV) and lung capacity (FVC) which are sensitive indicators of the progression of lung remodelling as a result of cystic fibrosis. 

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Repeated ventilatory parameters

The induced changes in lung parenchyma observed in preclinical models of cystic  fibrosis are likely to result in impaired breathing patterns, which can be captured repeatedly in conscious spontaneously breathing subjects using one of the various plethysmography techniques (e.g. whole body plethysmography, double-chamber plethysmography, head-out plethysmography).

Additional insight

The lung parenchyma, like the airway smooth muscle, possesses contractile properties and can also be studied in vitro in isolated tissue baths. In CF, the accumulation of mucus plugs or plaques can potentially alter the contractility of the lung parenchyma. Studying this tissue in the absence of external influences can offer additional insights when exploring pathophysiological mechanisms, characterizing preclinical disease models, or assessing the efficacy of a potential therapeutic treatment. In response to a constricting agent, increased contractility has been consistently observed in lung parenchymal from a fibrotic model relative to a corresponding control group.

There is also mounting evidence that CFTR plays a direct role in the airway smooth muscle. Measuring the reactivity of isolated tracheal rings or strips ex vivo, in tissue baths, allows for a functional assessment in absence of external influences.  This approach was taken to study the effect of CFTR function on human airway smooth muscle and to confirm its role in bronchorelaxation.

References & Publications


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