Cystic fibrosis disease
Cystic fibrosis (CF) causes constitutive inflammation in many organs
and it particularly affects the lung. Chronic airway inflammation with
chronic bacterial infections becomes established in most patients and
it may lead to lung damage, destruction and eventually death.
The bacterial factors and the molecular mechanisms which provoke
inflammation in the respiratory epithelium of cystic fibrosis patients
remain unclear. This topic is tackled using a coordinated and
interdisciplinary approach by joining efforts by 16 European Countries.
Competencies range from chemistry to microbiology and medicine.
This multi- and inter-disciplinary approach is aimed at enabling the
development of novel antibacterial inhibitors for anti-inflammatory
therapy in cystic fibrosis patients.
Reasons for the Action
The major reasons for launching this Action are to rapidly develop
novel therapies for CF and non-CF patient groups at risk for lung
infections with opportunistic Gram-positive and Gram-negative bacterial
pathogens. This aim will be achieved by the coordination of researchers
into a European network of the already funded national research.
Networking will also avoid repetition or duplication of scientific work
and thus spare valuable resources of prior isolated research groups in
Europe. We will create a defined panel of clinically relevant bacterial
pathogens as an open user resource. This COST Action will enable
researchers to develop novel inhibitors of bacterial cell wall synthesis
with the aim to reduce lung inflammation in CF airways and to eradicate
the pathogens with novel treatment strategies. This will increase the
life expectancy of the CF patients. The pathogens that will be studied
are also known to affect other patient groups including patients with
immunodeficiencies such as burns patients and ventilated patients in
critical care units. Also in these large patient groups, mortality due
to respiratory infections is high. Improvement in knowledge of bacterial
pathogenesis would have important implications for these patient groups,
the quality of life of affected individual, and with the potential to
reduce the inappropriate use of currently administered antibiotics,
which increase the number of resistant pathogens in the European
community.