Abstract
In cystic fibrosis (CF) patients airways mucus shows an increased viscoelasticity due to the concentration of high molecular weight components. Such mucus thickening eventually leads to bacterial overgrowth and prevents mucus clearance. The altered rheological behavior of mucus results in chronic lung infection and inflammation, which causes most of the cases of morbidity and mortality, although the cystic fibrosis complications affect other organs as well. Here, we present a quantitative study on the correlation between cystic fibrosis mucus viscoelasticity and patients clinical status. In particular, a new diagnostic parameter based on the correlation between CF sputum viscoelastic properties and the severity of the disease, expressed in terms of FEV1 and bacterial colonization, was developed. By using principal component analysis, we show that the types of colonization and FEV1 classes are significantly correlated to the elastic modulus, and that the latter can be used for CF severity classification with a high predictive efficiency (88%). The data presented here show that the elastic modulus of airways mucus, given the high predictive efficiency, could be used as a new clinical parameter in the prognostic evaluation of cystic fibrosis.
Highlights
Cystic fibrosis (CF) is the most frequent life-limiting genetic disease in Caucasian populations, among whom it occurs in approximately 1 in 3000 births [1]
We investigate the correlation between cystic fibrosis (CF) sputum viscoelastic properties and disease severity, in terms of Forced Expiratory Volume in 1 second (FEV1)% and bacterial colonization
That B. cepacia and S. matophilia lungs colonization increases the possibility of morbidity and mortality, being associated with a rapid decline in pulmonary function, more than P. aeruginosa and S. aureus [33,34,35]
Summary
Cystic fibrosis (CF) is the most frequent life-limiting genetic disease in Caucasian populations, among whom it occurs in approximately 1 in 3000 births [1]. From the clinical point of view, the altered rheological behavior of mucus results in lung chronic infection and inflammation, which causes most of the cases of morbidity and mortality, despite the CF complications affect other organs as well [13]. In light of such pathological relevance, the rheological characterization of body fluids such as blood [14,15,16,17], amniotic fluid, synovial fluid [18] and mucus [10,19] have been the subject of a number of studies. Two main approaches have been followed so far: i) microrheology, that is based on a magnetic microrheometer, an elegant technique for measuring rheological properties [20] of small volume of mucus [21,22,23], as well as multiple particle tracking [12] and dynamic light scattering [24]; the drawbacks of these techniques are related to mucus heterogeneity [12]; ii) macrorheology, where classical
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