Ivacaftor in severe cystic fibrosis lung disease and a G551D mutation.

Ivacaftor is gene-specific oral therapy for patients with cystic fibrosis who have a cystic fibrosis transmembrane conductance regulator mutation, G551D. To date, limited information is available about the benefit in patients with severe CF related lung disease, as such patients were excluded from the phase III trials. We report the early results on clinical outcomes, sweat electrolytes and C-reactive protein in three adults with a G551D mutation and advanced lung disease.

The month of July: an early experience with pandemic influenza A (H1N1) in adults with cystic fibrosis.

Background: Pandemic Influenza A (H1N1) 2009 is a novel viral infection that emerged in March 2009. This is the first report addressing the clinical course of patients with cystic fibrosis (CF) and H1N1 infection.

Methods: All patients with an influenza-like illness (ILI) attending our adult centre during July 2009 were identified.

The changing epidemiology of Burkholderia species infection at an adult cystic fibrosis centre.

Background: This study reviews the impact of changing infection control practices at the Manchester Adult Cystic Fibrosis Centre (MACFC) upon the epidemiology of Burkholderia species infections.

Methods: We reviewed strain and genomovar typing of all available Burkholderia isolates at our centre between 1983-2006.

Results: The incidence/prevalence of infection with Burkholderia species between 1983-1990 was below 5%/9% each year.