Titlebook: Cystic Fibrosis in Primary Care; An Essential Guide t Douglas Lewis, MD, FAAFP Book 2020 Springer Nature Switzerland AG 2020 cystic fibrosi

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Clara Terzaghi,Cecilia Pasquali,Elia Paiusco long arm of chromosome 7 (Knowlton et al. Nature 318:380–2, 1985). This mutation can result in absent, abnormal, or reduced function of the CFTR protein which is a cAMP-regulated ion channel that controls transport of chloride, sodium, and bicarbonate across various cell membranes. Defective CFTR p

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Yoshiharu Shimozono,John G. Kennedysregulation of inflammatory mediators. The inflammatory component of cystic fibrosis begins early in life and is sustained and intense. This inflammation leads to airway damage in the form of bronchiectasis which is the main cause of morbidity and mortality for the cystic fibrosis patient. There are