Publisher: Cystic Fibrosis News Today


Levels of Neutrophil Elastase May Help Predict Disease Severity in CF

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Levels of neutrophil elastase, a pro-inflammatory enzyme normally released by immune cells known as neutrophils, plus a greater diversity and amount of microbes could drive disease severity in cystic fibrosis (CF), a study in two siblings suggests. The study, Two Siblings Homozygous for F508del-CFTR Have Varied Disease Phenotypes and Protein Biomarkers, was published in the… Read More


Potential CF Mutation, F1099L, Identified in 2 Minority Children in US Mentions UTHSC

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A rare and disease-causing mutation for cystic fibrosis (CF), called the F1099L mutation and characterized by a protein maturation defect, was identified in a recent study. Treatment with lumacaftor was also seen to effectively rescue function of the defective CFTR protein that results from this mutation, found in two infants — one Black, one mixed… Read More