Cystic fibrosis year in review 2020: Section 2 pulmonary disease, infections, and inflammation

Pediatr Pulmonol. 2022 Feb;57(2):347-360. doi: 10.1002/ppul.25459. Epub 2021 May 25.

Abstract

The outlook for those with cystic fibrosis (CF) has never been brighter with ever increasing life expectancy and the approval of the highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor. With that being said, the progressive pulmonary decline and importance of lung health, infection, and inflammation in CF remains. This review is the second part in a three-part CF Year in Review 2020. Part one focused on the literature related to CFTR modulators while part three will feature the multisystem effects related to CF. This review focuses on articles from Pediatric Pulmonology, including articles from other journals that are of particular interest to clinicians. Herein, we highlight studies published during 2020 related to CF pulmonary disease, infection, treatment, and diagnostics.

Keywords: clinical trials; cystic fibrosis; epidemiology; pulmonary function testing; respiratory technology.

Publication types

  • Review

MeSH terms

  • Aminophenols / therapeutic use
  • Benzodioxoles / therapeutic use
  • Child
  • Chloride Channel Agonists
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis* / drug therapy
  • Humans
  • Inflammation
  • Mutation

Substances

  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists
  • Cystic Fibrosis Transmembrane Conductance Regulator