Cystic fibrosis papers of the year 2017

Paediatr Respir Rev. 2018 Jun:27:2-5. doi: 10.1016/j.prrv.2018.03.001. Epub 2018 Mar 15.

Abstract

The number of published articles on Cystic Fibrosis (CF) continues to increase year on year. The evidence base for small molecule therapies in CF has continued to expand, with evidence for lumacaftor/ivacaftor in younger patients and longer-term evidence in adults, and pivotal studies on tezacaftor/ivacaftor. There were reports on emerging CFTR mutation agnostic therapies, and new evidence for long standing therapies.

Keywords: Cystic fibrosis; Doxycycline; Ivacaftor; Lumacaftor; MRSA; Tezacaftor.

Publication types

  • Review

MeSH terms

  • Adult
  • Aminophenols / pharmacology*
  • Aminopyridines / pharmacology*
  • Benzodioxoles / pharmacology*
  • Child
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Cystic Fibrosis* / genetics
  • Cystic Fibrosis* / physiopathology
  • Cystic Fibrosis* / therapy
  • Disease Management
  • Drug Combinations
  • Humans
  • Lung / physiopathology
  • Membrane Transport Modulators / pharmacology
  • Mutation
  • Quinolones / pharmacology*
  • Respiratory Function Tests
  • Zeit

Substances

  • Aminophenols
  • Aminopyridines
  • Benzodioxoles
  • Drug Combinations
  • Membrane Transport Modulators
  • Quinolones
  • lumacaftor, ivacaftor drug combination
  • Cystic Fibrosis Transmembrane Conductance Regulator