Determinants of respiratory pump function in patients with cystic fibrosis

Paediatr Respir Rev. 2015 Jan;16(1):75-9. doi: 10.1016/j.prrv.2014.01.001. Epub 2014 Jan 28.


Respiratory failure constitutes the major cause of morbidity and mortality in patients with Cystic Fibrosis (CF). Respiratory failure could either be due to lung parenchyma damage or to insufficiency of the respiratory pump which consists of the respiratory muscles, the rib cage and the neuromuscular transmission pathways. Airway obstruction, hyperinflation and malnutrition have been historically recognised as the major determinants of respiratory pump dysfunction in CF. Recent research has identified chronic infection, genetic predisposition, dietary and pharmaceutical interventions as possible additional determinants of this impairment. Furthermore, new methodological approaches in assessing respiratory pump function have led to a better understanding of the pathogenesis of respiratory pump failure in CF. Finally, respiratory muscle function could be partially preserved in CF patients with structured interventions such as aerobic exercise, inspiratory muscle training and non-invasive ventilation and CF patients could consequently be relatively protected from respiratory fatigue and respiratory failure.

Keywords: CF; Cystic Fibrosis; Respiratory failure; Respiratory muscles.

Publication types

  • Review

MeSH terms

  • Animals
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / physiopathology*
  • Humans
  • Respiration
  • Respiratory Insufficiency / etiology
  • Respiratory Muscles / physiopathology*