Cystic Fibrosis: Microbiology and Host Response

Pediatr Clin North Am. 2016 Aug;63(4):617-36. doi: 10.1016/j.pcl.2016.04.003.

Abstract

The earliest descriptions of lung disease in people with cystic fibrosis (CF) showed the involvement of 3 interacting pathophysiologic elements in CF airways: mucus obstruction, inflammation, and infection. Over the past 7 decades, our understanding of CF respiratory microbiology and inflammation has evolved with the introduction of new treatments, increased longevity, and increasingly sophisticated laboratory techniques. This article reviews the current understanding of infection and inflammation and their roles in CF lung disease. It also discusses how this constantly evolving information is used to inform current therapeutic strategies, measures and predictors of disease severity, and research priorities.

Keywords: Biomarker; Cystic fibrosis; Infection; Inflammation; Microbiology; Neutrophil.

Publication types

  • Review

MeSH terms

  • Anti-Bacterial Agents / therapeutic use
  • Cystic Fibrosis / microbiology*
  • Cystic Fibrosis / physiopathology
  • Humans
  • Inflammation / physiopathology*
  • Lung Diseases / etiology
  • Lung Diseases / microbiology*
  • Respiratory Tract Infections / etiology
  • Respiratory Tract Infections / microbiology*

Substances

  • Anti-Bacterial Agents