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• W86595887 abstract "Reas HW. J Pediatr 1963;62:31-5. Fifty years after this article by Herman Reas, cystic fibrosis (CF) remains a life-shortening disease with morbidity caused primarily by airway obstruction with viscous mucus and recurrent episodes of infection. However, the mean survival of patients with CF in 2010 is 48 years (in Canada), which is much greater than in the 1960s, when most patients with CF died in the first decade of life.1Cystic Fibrosis Canada. http://www.cff.org/research/ResearchMilestones. Accessed August 15, 2012.Google Scholar This remarkable increase in survival is the result of several factors, including interventions aimed at enhancing the nutritional status of these patients, reducing mucus viscosity and improving anti-infection therapies. Although there have been several clinical trials of N-acetylcysteine, including the intervention studied by Reas in the aforementioned paper, there is no clear evidence that this specific treatment leads to an improvement in airway health.2Nash E.F. Stephenson A. Ratjen F. Tullis E. Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis.Cochrane Database Syst Rev. 2009; 1: CD007168PubMed Google Scholar Discovery of the CF gene in 1989 led to our current understanding of the essential role of its protein product: the cystic fibrosis transmembrane conductance regulator (CFTR) chloride and bicarbonate channel in regulating the hydration, pH, and viscosity of airway secretions.3Rommens J.M. Iannuzzi M.C. Kerem B. Drumm M.L. Melmer G. Dean M. et al.Identification of the cystic fibrosis gene: chromosome walking and jumping.Science. 1989; 245: 1059-1065Crossref PubMed Scopus (2517) Google Scholar Now, we know that tracheobronchial secretions are viscous in patients with CF because of the absence or the dysfunction of mutant CFTR channels on the surface of respiratory epithelial cells. A new therapeutic strategy has emerged with the goal of “repairing” the basic defects in the CFTR protein caused by CFTR gene mutations. This strategy uses CFTR protein modulators that act to restore proper channel activity to poorly functional CFTR mutants (such as G551D-CFTR).4Van Goor F. Hadida S. Grootenhuis P.D. Burton B. Cao D. Neuberger T. et al.Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.Proc Natl Acad Sci USA. 2009; 106: 18825-18830Crossref PubMed Scopus (886) Google Scholar The potentiator, Ivacaftor (or VX-770), was approved by the Food and Drug Administration for use in patients with the G551D mutation in 2012. Early intervention with protein-modulatory drugs promises to prevent the development of viscous mucus and the resulting airway disease. The development of drugs such as Ivacaftor provides optimism that CF will be considered a manageable disease in the not-too-distant future." @default.
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• W86595887 date "2013-01-01" @default.
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• W86595887 title "50 Years Ago in The Journal of Pediatrics" @default.
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• W86595887 doi "https://doi.org/10.1016/j.jpeds.2012.08.006" @default.
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