Respiratory System Disease

Pediatr Clin North Am. 2016 Aug;63(4):637-59. doi: 10.1016/j.pcl.2016.04.007.

Abstract

Respiratory system involvement in cystic fibrosis is the leading cause of morbidity and mortality. Defects in the cystic fibrosis transmembrane regulator (CFTR) gene throughout the sinopulmonary tract result in recurrent infections with a variety of organisms including Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and nontuberculous mycobacteria. Lung disease occurs earlier in life than once thought and ideal methods of monitoring lung function, decline, or improvement with therapy are debated. Treatment of sinopulmonary disease may include physiotherapy, mucus-modifying and antiinflammatory agents, antimicrobials, and surgery. In the new era of personalized medicine, CFTR correctors and potentiators may change the course of disease.

Keywords: Bronchiectasis; CFTR; Cystic fibrosis; Lung disease; Lung function; Pulmonary exacerbation.

Publication types

  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / therapy
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Humans
  • Infant
  • Respiratory Tract Diseases / diagnosis
  • Respiratory Tract Diseases / etiology*
  • Respiratory Tract Diseases / therapy

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator