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Lumacaftor–Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR
It's 5 years since Ramsey et al's landmark paper demonstrating Ivacaftors's ability to significantly change lung function in cystic fibrosis (CF) patients with the G551D mutation. The search is now on for a similar finding for the most common mutation—F508del. Although not as dramatic, Wainwright et al (N Engl J Med 2015;373:220–31) have shown that combining a CF transmembrane conductance regulator (CFTR) corrector—Lumacafor—and a CFTR potentiator—Ivacaftor—does produce a significant improvement in FEV1.
Secondhand smoke exposure and illness severity among children hospitalised with pneumonia
The ban on smoking in a car carrying children will become UK law in October 2015; it is already law in some US states. Ahn et al (J Paeds 2015; http://dx.doi.org/10.1016/j.jpeds.2015.06.049) add to the mounting evidence of the effect of secondhand smoke on children with their study of 2219 children admitted with pneumonia in Tennessee and Utah between 2010 and 2012. Children with ≥2 smokers in their household were more likely to go to an intensive …
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Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.