Today in Chest was published Comprehensive review: The Evolution of Cystic Fibrosis Care.
Cystic
fibrosis (CF) is the most common life-limiting inherited illness of
whites. Most of the morbidity and mortality in CF stems from impaired
mucociliary clearance leading to chronic, progressive airways
obstruction and damage. Significant progress has been made in the care
of patients with CF, with advances focused on improving mucociliary
clearance, minimizing inflammatory damage, and managing infections;
these advances include new antimicrobial therapies, mucolytic and
osmotic agents, and antiinflammatory treatments.
More recently,
researchers have targeted disease-causing mutations using therapies to
promote gene transcription and improve channel function, which has led
to impressive physiologic changes in some patients. As we develop more
advanced, allele-directed therapies for the management of CF, it will
become increasingly important to understand the specific genetic and
environmental interactions that cause the significant heterogeneity of
lung disease seen in the CF population. This understanding of CF
endotypes will allow for more targeted, personalized therapies for
future patients. This article reviews the genetic and molecular basis of
CF lung disease, the treatments currently available, and novel
therapies that are in development.
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