166 CF liver disease and lung function

Pseudomonas aeruginosa, cyanide accumulation and lung function in CF and non-CF bronchiectasis patients.

In patients with cystic fibrosis (CF) and non-CF bronchiectasis, Pseudomonas aeruginosa is the most important respiratory pathogen. It is able to synthesise hydrogen cyanide, a potent inhibitor of cellular respiration. The present study investigated whether cyanide is present in the sputum of CF and non-CF bronchiectasis patients infected with P. aeruginosa, and whether the detection of cyanide...

NETs and CF Lung Disease: Current Status and Future Prospects

Cystic Fibrosis (CF) is the most common fatal monogenic disease among Caucasians. While CF affects multiple organ systems, the principle morbidity arises from progressive destruction of lung architecture due to chronic bacterial infection and inflammation. It is characterized by an innate immune defect that results in colonization of the airways with bacteria such as Staphylococcus aureus and P...

MRI follow-up of CF-related infectious lung disease

Lung clearance index in CF: a sensitive marker of lung disease severity.

Is Lung Clearance Index (LCI) Affected by the Severity of Lung Disease in CF?

Background: LCI has been proven an effective tool in the detection of lung disease in CF. Objectives: a) To assess the correlation of ventilation inhomogeneity indices with structural damages of the lung, among different groups of disease severity and b) To compare the associations among MBW parameters with spirometry and CT. Method: Forty-four children and adolescents with CF participated in t...