Longitudinal SF6 multiple breath washout in infants and toddlers with cystic fibrosis

Impact of Reanalysis of Nitrogen Multiple-Breath Washout on its Relationship with Chest Magnetic Resonance Imaging Findings in Clinically Stable and Pulmonary Exacerbated Children with Cystic Fibrosis

“…Furthermore, the inter-individual variability of N 2 LCI cor , especially in CF patients during PEx, was smaller than of N 2 LCI pre ( Fig. 3 ), which was previously described by several groups reanalyzing MBW data 17 19 24 25 . As shown by other research groups, the reduced inter-individual variability of N 2 LCI cor provides a rather precise estimate on potentially reversible pulmonary complications in children with CF 17 25 .…”

Turning Lung Clearance Index on its head. Reference data for SF6 Multiple Breath Washout Derived Ventilation distribution efficiency (VDE)

“…decreasing values) has been confirmed in two sulfur hexafluoride (SF 6 ) MBW studies of healthy young children (aged 2 weeks to 19 years, and 3-60 months, respectively) using the gold standard respiratory mass spectrometer (RMS) based MBW system (13,14). Since 2016 the availability of a commercial SF 6 MBW system suitable for use in all age groups (Exhalyzer D®) has emerged (4)(5)(6)15,16), but applicability of the previously published RMS SF 6 MBW derived reference equations to this equipment (13,14) remains unclear (see Appendix for details of device differences).…”

“…Importantly, LCI has been demonstrated to be a sensitive marker of lung involvement in cystic fibrosis (CF) (3). Requiring only tidal breathing and passive cooperation, the MBW method is feasible across the entire paediatric age range, even in infancy (4)(5)(6), where the emergence of detectable impairment has been shown to occur (7,8). This utility in young children highlights the importance of robust age-appropriate reference data starting from infancy.…”

“…Triplet MBW recordings, using a mixture of 4.0% SF 6 in air for wash-in until equilibration, were performed with the EXHALYZER D® (ECO MEDICS AG, Duernten, Switzerland) using SPIROWARE® 3.2.1. Off-line breath by breath quality control analysis, as described in several previous publications (4,6,15,16), was performed using an in-house software developed in LabVIEW® (National Instruments, Austin, TX) based on identical algorithms used in the SPIROWARE® software. Group 1 performed MBW in the supine position, during quiet sleep (natural or after light sedation with intranasal Dexmedethomidine), with the EXHALYZER D® infant set-up, dead space reducer #1, and a putty sealed Rüsch face mask #1 or #2.…”

ERS International Congress 2021: highlights from the Paediatric Assembly

“…Longitudinal SF 6 MBW assessments by S andvik et al [ 31 ] in 2–45-month-olds with CF and healthy controls detected a higher LCI in the CF than the control group, with 64% of children with CF showing intermittent lung function impairment and a LCI increase of 0.22 units/year associated with an overall worsening. This highlights the importance of early therapeutic intervention and regular SF 6 MBW assessments during the early stages of CF.…”