Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infection

Abstract

Measures of ventilation distribution are promising for monitoring early lung disease in cystic fibrosis (CF). This study describes the cross-sectional and longitudinal impacts of pulmonary inflammation and infection on ventilation homogeneity in infants with CF.Infants diagnosed with CF underwent multiple breath washout (MBW) testing and bronchoalveolar lavage at three time points during the first 2 years of life.Measures were obtained for 108 infants on 156 occasions. Infants with a significant pulmonary infection at the time of MBW showed increases in lung clearance index (LCI) of 0.400 units (95% CI 0.150-0.648; p=0.002). The impact was long lasting, with previous pulmonary infection leading to increased ventilation inhomogeneity over time compared to those who remained free of infection ( p<0.05). Infection with Haemophilus influenzae was particularly detrimental to the longitudinal lung function in young children with CF where LCI was increased by 1.069 units for each year of life (95% CI 0.484-1.612; p<0.001).Pulmonary infection during the first year of life is detrimental to later lung function. Therefore, strategies aimed at prevention, surveillance and eradication of pulmonary pathogens are paramount to preserve lung function in infants with CF. @ERSpublications Early life respiratory infections are detrimental to long-term lung function in children with cystic fibrosis

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

“…We found no associations between the presence of any other individual pathogens and FOT outcomes. A previous study by the AREST CF group showed that the presence of H. influenzae in the first 2 years of life was associated with persistently lower forced expiratory volume in 0.75 s and forced vital capacity up to school age [40] and increased LCI in infancy [41]. In addition, BRUMBACK et al [42] found that recent H. influenzae infection was associated with lower forced vital capacity in preschool children with CF.…”

Is Lung Clearance Index (LCI) Affected by the Severity of Lung Disease in CF?

“…During last decades, an increased interest for MBW method among patients suffering from CF has been noted [3,4,[33][34][58][59][60][61][62][63] MBW is a non-invasive method, feasible to be performed and without the risk of radiation exposure [64]. A lot of studies have suggested that LCI has the advantage over FEV 1 to detect CF lung disease in earlier stages [21,36,38,[64][65][66][67][68].…”

Early lung surveillance of cystic fibrosis: what have we learnt?

“…Despite the ability of spirometry to identify abnormal lung function in infants and young children with CF, the LCFC showed that LCI measured by MBW was more sensitive than spirometry for the detection of structural lung disease. AREST CF data show that infants who had a prior infection had a progressive increase in ventilation inhomogeneity compared to infants who were never infected [25]. While increased LCI was associated with infections, it was insensitive to detect structural abnormalities in infants [26].…”