Cystic fibrosis. (May 2020)

• Record Type: Journal Article
• Authors: Ewence, Alexandra E.; Jones, Andrew L.
• Is Part Of: Medicine. Volume 48:Number 5(2020)
• Page Start: 344
• Page End: 348
• Publication Date: 2020-05
• Subjects: Bronchiectasis -- cystic fibrosis -- forced expiratory volume -- MRCP -- Orkambi® -- Pseudomonas; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Médecine factuelle -- Périodiques; Medicine; Periodicals; Electronic journals; Electronic journals; 610
• DOI: 10.1016/j.mpmed.2020.02.003
• Languages: English
• ISSNs: 1357-3039

Abstract

Abstract: Cystic fibrosis (CF) is the most common autosomal recessive condition to affect white individuals. Defects in the CFTR chloride channel result in progressive organ dysfunction, particularly affecting the respiratory system. Patients require life-long care from multidisciplinary teams in specialist centres. Over time, improvements in screening and understanding of pathogenic alleles have increased diagnosis, while advances in management have led to better survival. The recent emergence of CFTR-modulating agents offers the potential to stabilize and improve lung disease, nutrition and quality of life in many people with CF.