title: Recent advances in cystic fibrosis.
abstract: OBJECTIVE
  The field of cystic fibrosis (CF) continues to evolve at a fast pace thanks to novel observations that have enabled deeper understanding of the disease pathophysiology. Parallel groundbreaking developments in innovative therapies permit, for the first time, distinct disease modification.
  
  
  RESULTS
  This review highlights important discoveries in fluid homeostasis and mucus secretion in CF that further informs the pathophysiology of the airway disease that characterizes CF. In addition, current concepts and novel paradigms, such as 'theratypes' and 'CF transmembrane conductance regulator chaperome', which will be important for the continued development of disease modifying therapies, are reviewed.
  
  
  CONCLUSIONS
  The rate of progress in the field continues to accelerate with new knowledge informing the development of innovative therapies. This has already led to tangible substantial and unprecedented clinical benefit for selected subsets of the CF patient population. In the years ahead, further knowledge acquisition may motivate the extension of these benefits to the larger population of people with CF.
authors.affiliation: aThe Stanford Cystic Fibrosis Center, Palo Alto bCenter for Excellence in Pulmonary Biology, Stanford University School of Medicine, Stanford, California, USA.
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Milla
authors.forename: Richard B
authors.initials: RB
authors.lastname: Moss
mesh_terms: D000627:Aminophenols / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000235:genetics / Q000382:microbiology / Q000503:physiopathology / Q000628:therapy*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000187:drug effects* / Q000235:genetics; D006801:Humans; D058990:Molecular Targeted Therapy / Q000379:methods; D009154:Mutation; D019141:Respiratory System Agents / Q000627:therapeutic use*; D012141:Respiratory Tract Infections / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012462:Saline Solution, Hypertonic / Q000627:therapeutic use*
chemical_list: D000627:Aminophenols; D019141:Respiratory System Agents; D012462:Saline Solution, Hypertonic; D019005:Cystic Fibrosis Transmembrane Conductance Regulator