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A Journal on Diseases of the Respiratory System

Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index




Minerva Pneumologica 2013 June-September;52(2-3):89-110


language: English

Update on the management of cystic fibrosis

Castro M. C. S., Firmida M. C., Taulois M. M., Lopes A. J.

Pulmonology Department State University of Rio de Janeiro, Rio de Janeiro, Brazil


Cystic fibrosis (CF) is the most common autosomal-recessive genetic disease among Caucasian populations. CF exhibits a particular affinity for the respiratory and digestive systems, and its clinical manifestations are characterised by chronic and suppurative pulmonary obstruction and gastrointestinal malabsorptive disorders. Although CF is classically known as a lethal disease, with patients not surviving until adulthood, several advances over recent decades have dramatically changed that outlook. Treatment is based on measures to combat airway obstruction, inflammation, and infection, in addition to ensuring satisfactory nutrition. Intensive treatment following a multidisciplinary approach and judicious use of the wide scope of available therapeutic resources have contributed to delayed CF progression and afford timely treatment of complications, resulting in a substantial increase in patient survival and quality of life. Measures to improve mucociliary clearance and lung ventilation, including respiratory physical therapy, are among the treatment pillars. Antibiotics are primarily used under the following three circumstances: upon initial identification of a microorganism (eradication), maintenance treatment, and exacerbations. In addition to antibiotics, other therapeutic possibilities are being developed to fight microorganisms, including hyperimmune gamma-globulin G and a recombinant human antibody. Drugs that are correctors and potentiators of the activity of CF transmembrane conductance regulator are currently being developed and tested. Presently, lung transplantation is a relevant tool to improve patient life expectancy and quality of life in cases with severe pulmonary involvement.

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