Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

Abstract

Background: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiplebreath inert gas washout (MBW) technique, has been shown to detect abnormal lung function more readily than spirometry in preschool children with cystic fibrosis, but whether this holds true during infancy is unknown. Objectives: To compare the extent to which parameters derived from the MBW and the raised lung volume rapid thoraco-abdominal compression (RVRTC) techniques identify diminished airway function in infants with cystic fibrosis when compared with healthy controls. Methods: Measurements were performed during quiet sleep, with the tidal breathing MBW technique being performed before the forced expiratory manoeuvres. Results: Measurements were obtained in 39 infants with cystic fibrosis (mean (SD) age 41.4 (22.0) weeks) and 21 controls (37.0 (15.1) weeks). Infants with cystic fibrosis had a significantly higher respiratory rate (38 (10) vs 32 (5) bpm) and LCI (8.4 (1.5) vs 7.2 (0.3)), and significantly lower values for all forced expiratory flow-volume parameters compared with controls. Girls with cystic fibrosis had significantly lower forced expiratory volume ) than boys (mean (95% CI girls-boys): -1.2 (-2.1 to 20.3) for FEV 0.5 Z score; FEF 25-75 : -1.2 (-2.2 to 20.15)). When using both the MBW and RVRTC techniques, abnormalities were detected in 72% of the infants with cystic fibrosis, with abnormalities detected in 41% using both techniques and a further 15% by each of the two tests performed. Conclusions: These findings support the view that inflammatory and/or structural changes in the airways of children with cystic fibrosis start early in life, and have important implications regarding early detection and interventions. Monitoring of early lung disease and functional status in infants and young children with cystic fibrosis may be enhanced by using both MBW and the RVRTC.

Growth of Lung Parenchyma in Infants and Toddlers with Chronic Lung Disease of Infancy

“…This finding suggests marked ventilation inhomogeneity was not present in our CLDI group, which is consistent with data presented by Hü lskamp and colleagues, who reported a small effect on lung clearance index (LCI) with neonatal oxygen exposure; 100 days of oxygen exposure during the neonatal period correlated with an increase in LCI from approximately 7.0 to 7.3 (18). This small increase in LCI in infants with CLDI contrasts to a much higher LCI observed in clinically stable outpatient infants with cystic fibrosis (CF), who have an LCI of 8.4 (19). The much higher LCI of infants with CF compared with those with CLDI is consistent with CF being primarily an airway disease with marked ventilation inhomogeneity, whereas CLDI or ''new BPD'' may be primarily a parenchymal disease.…”

Nature and severity of lung function abnormalities in extremely pre-term children at 11 years of age

“…*: p,0.05; **: p,0.01; ***: p,0.0001. in persons with cystic fibrosis [30] it has not been found to be particularly discriminative when assessing BPD or prematurity either during infancy [31,35] or in the current study.…”

Volumetric capnography as a tool to detect early peripheric lung obstruction in cystic fibrosis patients

“…No entanto, está cada vez mais difícil utilizar o VEF 1 para documentar e interpretar as alterações precoces e acompanhar a progressão da doença pulmonar, principalmente em crianças pré-escolares e escolares [1][2][3][4][5][6][7][8][9][10][11][12] .…”

“…Esses resultados podem ser determinados precocemente por meio da técnica de eliminação de gases chamada multiple-breath washout [(MBW), lavagem por múltiplas respirações] com gases como hélio (He), nitrogê-nio (N 2 ) e hexafluoreto de enxofre (SF 6 ). O lung clearance index (LCI, índice de depuração pulmonar), proveniente dos achados da técnica de MBW, é considerado um marcador altamente sensível do comprometimento das pequenas vias aéreas na FC [2][3][4][5][6][7][8][9][10][11][12][13] .…”