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Exacerbations in cystic fibrosis · 1: Epidemiology and pathogenesis


With the improving survival of patients with cystic fibrosis (CF), the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is an acute pulmonary exacerbation. Clinical and microbial epidemiology studies of CF pulmonary exacerbations continue to provide important insight into the course, prognosis and complications of the disease. This review provides a summary of the pathophysiology, clinical epidemiology and microbial epidemiology of a CF pulmonary exacerbation.

  • ARIC, Acute Respiratory Illness Checklist
  • CF, cystic fibrosis
  • CFTR, cystic fibrosis transmembrane regulator
  • FEV1, forced expiratory volume in 1 s
  • IL, interleukin
  • MCBT, multiple combination bactericidal testing
  • RSSQ, Respiratory and Systemic Symptoms Questionnaire
  • TNFα, tumour necrosis factor α

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