Pulmonology: CFTR modulators for cystic fibrosis
E Bertoncini, D Colomb-Lippa - JAAPA, 2013 - journals.lww.com
While many children have simplified the name of their disease to 65 roses, cystic fibrosis
(CF) is not at all simple to manage. 1 The pulmonary component of CF is responsible for the
associated mortality and predicted survival of only 38.3 years. Research has led to a
thorough understanding of the underlying pathophysiology, promoting development of new
medications to modify the disease process. 2 While the respiratory aspect of CF is
responsible for deleterious lung infections, unremitting inflammation, and ultimately fatality, it …
(CF) is not at all simple to manage. 1 The pulmonary component of CF is responsible for the
associated mortality and predicted survival of only 38.3 years. Research has led to a
thorough understanding of the underlying pathophysiology, promoting development of new
medications to modify the disease process. 2 While the respiratory aspect of CF is
responsible for deleterious lung infections, unremitting inflammation, and ultimately fatality, it …
