Table 1

 Potential adverse effects of hypoxia in CF

Adverse effect
Pulmonary circulationIncreased pulmonary artery pressures related to levels of sleep and exercise hypoxaemia17
Lung inflammationUp-regulated cytokine expression19 with increased neutrophil chemoattraction, neutrophilic inflammation, and resultant parenchymal lung damage
Bacterial profile in the CF lungEnhanced P aeruginosa growth in biofilms20 with increased antibiotic resistance28
Possible increased S aureus virulence30
Exercise abilityLimited exercise ability due to effects on pulmonary vasculature and also lung inflammation and bacterial colonisation. Reduced exercise ability sets up “vicious circle” (fig 1)
Muscle strengthReduced skeletal muscle mass29
Quality of lifePotential adverse effects on quality of life, including maintenance of school or work attendance7
Poor sleep quality18
EndotheliumEndothelial dysfunction31
(CFTR trafficking)Reduced CFTR protein trafficking shown in vitro under hypoxic conditions in a non-CF cell line32
Effects not documented in a CF cell line