scholarly journals Novel volumetric capnography indices measure ventilation inhomogeneity in cystic fibrosis

2022 ◽  
pp. 00440-2021
Author(s):  
Sotirios Fouzas ◽  
Anne-Christianne Kentgens ◽  
Olga Lagiou ◽  
Bettina Sarah Frauchiger ◽  
Florian Wyler ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Diagnostic Performance ◽  
Relative Difference ◽  
Lung Model ◽  
Discriminative Ability ◽  
Volumetric Capnography ◽  
Ventilation Inhomogeneity ◽  
Diagnostic Characteristics ◽  
Multiple Breath Washout ◽  
Better Than

BackgroundVolumetric capnography (VCap) is a simpler alternative of multiple-breath washout (MBW) to detect ventilation inhomogeneity (VI) in patients with cystic fibrosis (CF). However, its diagnostic performance is influenced by breathing dynamics. We introduce two novel VCap indices, the Capnographic Inhomogeneity Indices (CIIs) that may overcome this limitation and explore their diagnostic characteristics in a cohort of CF patients.MethodsWe analysed 320 N2-MBW trials from 50 CF patients and 65 controls (age 4-18 years) and calculated classical VCap indices, such as slope III (SIII) and the capnographic index (KPIv). We introduced novel CIIs based on a theoretical lung model, and assessed their diagnostic performance compared to classical VCap indices and the lung clearance index (LCI).ResultsBoth CIIs were significantly higher in CF patients compared with controls (mean±SD CII1 5.9±1.4% versus 5.1±1.0%, p=0.002; CII2 7.7±1.8% versus 6.8±1.4%, p=0.002) and presented strong correlation with LCI (CII1 R2=0.47 and CII2 R2=0.44 in CF patients). Classical VCap indices showed inferior discriminative ability (SIII 2.3±1.0%/L versus 1.9±0.7%/L, P=0.013; KPIv 3.9±1.3% versus 3.5±1.2%, P=0.071), while the correlation with LCI was weak (SIII R2=0.03; KPIv R2=0.08 in CF patients). CIIs showed lower intra-subject inter-trial variability, calculated as coefficient of variation for three and relative difference for two trials, than classical VCap indices, but higher than LCI (CII1 11.1±8.2% and CII2 11.0±8.0% versus SIII 16.3±13.5%; KPIv 15.9±12.8%; LCI 5.9%±4.2%).ConclusionCIIs detect VI better than classical VCap indices and correlate well with LCI. However, further studies on their diagnostic performance and clinical utility are required.

scholarly journals Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infection

2015 ◽  
Vol 46 (6) ◽  
pp. 1680-1690 ◽  
Author(s):  
Shannon J. Simpson ◽  
Sarath Ranganathan ◽  
Judy Park ◽  
Lidija Turkovic ◽  
Roy M. Robins-Browne ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Pulmonary Infection ◽  
Pulmonary Inflammation ◽  
First Year ◽  
Cross Sectional ◽  
Ventilation Inhomogeneity ◽  
Multiple Breath Washout ◽  
First Year Of Life ◽  
The Impact

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.

Multi-layer ventilation inhomogeneity in cystic fibrosis

2016 ◽  
Vol 233 ◽  
pp. 25-32 ◽  
Author(s):  
Sabine Krueger-Ziolek ◽  
Benjamin Schullcke ◽  
Zhanqi Zhao ◽  
Bo Gong ◽  
Susanne Naehrig ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Ventilation Inhomogeneity

A Therapeutic Trial of Fatty Acid Supplementation in Cystic Fibrosis

PEDIATRICS ◽  
1976 ◽  
Vol 57 (4) ◽  
pp. 474-479
Author(s):  
Robert B. Elliott
Keyword(s):  
Cystic Fibrosis ◽  
Fatty Acid ◽  
Clinical Course ◽  
Therapeutic Trial ◽  
Biochemical Abnormality ◽  
Fatty Acid Supplementation ◽  
Oil Emulsion ◽  
Soya Oil ◽  
One Year ◽  
Better Than

Seven children with cystic fibrosis (CF) have been treated for at least one year with intravenously administered soya oil emulsion. In all, an improvement of at least one biochemical abnormality in character with the disease appeared. The children's clinical course remains benign. This course is remarkably better than that of other children with CF treated without Intralipid in Auckland in the same period, though a placebo effect cannot be discounted. It is postulated that intravenous supplementation with essential fatty acid in CF may in turn partially correct an error of metabolism of prostaglandins present in the disease.

scholarly journals Patterns of regional lung physiology in cystic fibrosis using ventilation magnetic resonance imaging and multiple-breath washout

2018 ◽  
Vol 52 (5) ◽  
pp. 1800821 ◽  
Author(s):  
Laurie J. Smith ◽  
Guilhem J. Collier ◽  
Helen Marshall ◽  
Paul J.C. Hughes ◽  
Alberto M. Biancardi ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Total Lung Capacity ◽  
Resonance Imaging ◽  
Ventilation Distribution ◽  
Lung Clearance ◽  
Lung Capacity ◽  
Lung Clearance Index ◽  
Ventilation Heterogeneity ◽  
Multiple Breath Washout

Hyperpolarised helium-3 (3He) ventilation magnetic resonance imaging (MRI) and multiple-breath washout (MBW) are sensitive methods for detecting lung disease in cystic fibrosis (CF). We aimed to explore their relationship across a broad range of CF disease severity and patient age, as well as assess the effect of inhaled lung volume on ventilation distribution.32 children and adults with CF underwent MBW and 3He-MRI at a lung volume of end-inspiratory tidal volume (EIVT). In addition, 28 patients performed 3He-MRI at total lung capacity. 3He-MRI scans were quantitatively analysed for ventilation defect percentage (VDP), ventilation heterogeneity index (VHI) and the number and size of individual contiguous ventilation defects. From MBW, the lung clearance index, convection-dependent ventilation heterogeneity (Scond) and convection–diffusion-dependent ventilation heterogeneity (Sacin) were calculated.VDP and VHI at EIVT strongly correlated with lung clearance index (r=0.89 and r=0.88, respectively), Sacin (r=0.84 and r=0.82, respectively) and forced expiratory volume in 1 s (FEV1) (r=−0.79 and r=−0.78, respectively). Two distinct 3He-MRI patterns were highlighted: patients with abnormal FEV1 had significantly (p<0.001) larger, but fewer, contiguous defects than those with normal FEV1, who tended to have numerous small volume defects. These two MRI patterns were delineated by a VDP of ∼10%. At total lung capacity, when compared to EIVT, VDP and VHI reduced in all subjects (p<0.001), demonstrating improved ventilation distribution and regions of volume-reversible and nonreversible ventilation abnormalities.

scholarly journals CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis

2020 ◽  
Author(s):  
Nadine Bernasconi ◽  
Elisabeth Kieninger ◽  
Michelle Shaw ◽  
Johanna Kurz ◽  
Alexander Moeller ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Ventilation Inhomogeneity
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