**1. Introduction**

Cystic fibrosis (CF) is the most prevalent life-shortening autosomal recessive disorder in Caucasian populations [1]. Occurring in 1 out of every 3,500 newborns in the United States,

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and 1 out of every 2,000–3,000 in the European Union, CF affects more than 70,000 individuals worldwide [2]. Chronic lung disease is the major factor contributing to morbidity and mortality among CF patients, as abnormal airway secretions and chronic endobronchial infection lead to progressive airway obstruction. In addition to the respiratory tract, the disease may also affect the pancreas, liver, kidneys, intestine, and reproductive system [3].

Disease severity varies greatly among those with CF, depending largely upon the degree to which the lungs are affected. However, eventual deterioration of the lungs leading to airway obstruction and death is inevitable, and for many years the average CF patient was not expected to reach adulthood [2]. Over the course of the past three decades, advancements in modern medicine have allowed physicians to postpone debilitating changes to the lungs, slowing the progression of disease and allowing many individuals with CF to live well into their 50s or 60s. Despite these advances in current therapy, the median age of survival remains only 33.4 years [2], emphasizing the need for novel therapeutic approaches to further improve patient outcomes in CF.
