Cystic Fibrosis

An Overview

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Abstract

Cystic fibrosis (CF) is an inherited disease affecting multiple systems, which can result in progressive lung disease, pancreatic insufficiency, malnutrition, distal intestinal obstruction syndrome, liver dysfunction, and other disorders. In 1938, all children with cystic fibrosis died in infancy, but today the average life expectancy is between 28 and 47.7 years. To optimize short- and long-term outcomes, early diagnosis and treatment by multidisciplinary teams are paramount. Traditional supportive therapies are still crucial in preventing or treating damage to every organ system, and also lately, the introduction of highly effective modulator therapies targeting specific CF transmembrane conductance regulator protein defects resulting from individual genetic mutations has considerably improved the life span and quality and prognosis of CF patients. We hope that this chapter will provide healthcare professionals with an up-to-date assessment of the CF’s pathophysiology, diagnosis, clinical features, and evidence-based new treatments of CF.

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Yalçın, E., Özçelik, U. (2023). Cystic Fibrosis. In: Cingi, C., Yorgancıoğlu, A., Bayar Muluk, N., Cruz, A.A. (eds) Airway diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-22483-6_109-1

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