Cystic fibrosis (CF) is the most common inherited disease among the Caucasian population with
considerable morbidity and reduced life expectancy.
Excessive oxidants released by activated inflammatory cells and persisting infections are
considered the main mechanism of damage of respiratory epithelium in CF.Glutathione (GSH)
represents the first-line defence of the lung against oxidative stress-induced cell injury;
however, a depletion of its levels has been observed in the airways of patients affected by
CF. In vitro studies have showed that CFTR protein plays a pivotal role in transmembrane
glutathione transport. Therapeutic approaches with inhaled GSH could improve the reduced lung
antioxidant capacity in order to counterbalance the oxidant stress linked to the chronic
airway inflammation and bacterial infection.
Primary objective of the study is to investigate whether a 12 months treatment with inhaled
GSH can improve airway obstruction in CF patients. Secondary objectives include the effects
of GSH therapy on exercise capacity, body mass index (BMI), respiratory symptoms, quality of
life, frequency of pulmonary exacerbations, hospital admissions, and antibiotic
administration. Moreover the study will evaluate the effect of GSH therapy on markers of
oxidative stress in exhaled breath condensate (EBC) and in serum, and on inflammatory markers
on brushed nasal epithelial cells.