"Effects" of bronchiolitis on ventilation inhomogeneity (VI) at preschool age: comparison of Multiple Breath Washout (MBW) between post-bronchiolitis, cystic fibrosis and healthy children

2018 ◽  
Author(s):  
Giulia Cangiano ◽  
Riccardo Guarise ◽  
Antonella Borruso ◽  
Laura Menin ◽  
Gloria Tridello ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Preschool Age ◽  
Healthy Children ◽  
Ventilation Inhomogeneity ◽  
Multiple Breath Washout

scholarly journals The clinical utility of lung clearance index in early cystic fibrosis lung disease is not impacted by the number of multiple-breath washout trials

2018 ◽  
Vol 4 (1) ◽  
pp. 00094-2017 ◽  
Author(s):  
Rachel E. Foong ◽  
Alana J. Harper ◽  
Billy Skoric ◽  
Louise King ◽  
Lidija Turkovic ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Fluid Collection ◽  
Lavage Fluid ◽  
Sufficient Information ◽  
Healthy Children ◽  
Success Rates ◽  
Lung Clearance ◽  
Lung Clearance Index ◽  
Multiple Breath Washout

The lung clearance index (LCI) from the multiple-breath washout (MBW) test is a promising surveillance tool for pre-school children with cystic fibrosis (CF). Current guidelines for MBW testing recommend that three acceptable trials are required. However, success rates to achieve these criteria are low in children aged <7 years and feasibility may improve with modified pre-school criteria that accepts tests with two acceptable trials. This study aimed to determine if relationships between LCI and clinical outcomes of CF lung disease differ when only two acceptable MBW trials are assessed.Healthy children and children with CF aged 3–6 years were recruited for MBW testing. Children with CF also underwent bronchoalveolar lavage fluid collection and a chest computed tomography scan.MBW feasibility increased from 46% to 75% when tests with two trials were deemed acceptable compared with tests where three acceptable trials were required. Relationships between MBW outcomes and markers of pulmonary inflammation, infection and structural lung disease were not different between tests with three acceptable trials compared with tests with two acceptable trials.This study indicates that pre-school MBW data from two acceptable trials may provide sufficient information on ventilation distribution if three acceptable trials are not possible.

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.

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.

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

Selenium and Glutathione Peroxidase Levels in Cystic Fibrosis

PEDIATRICS ◽  
1980 ◽  
Vol 65 (5) ◽  
pp. 1010-1012
Author(s):  
John D. Lloyd-Still ◽  
Howard E. Ganther
Keyword(s):  
Cystic Fibrosis ◽  
New Zealand ◽  
Glutathione Peroxidase ◽  
Whole Blood ◽  
Selenium Concentration ◽  
Selenium Level ◽  
Infants And Children ◽  
Healthy Children ◽  
Normal Range

Whole blood selenium and glutathione peroxidase levels were measured in 20 infants and children (aged 6 months to 15 years) with cystic fibrosis. The whole blood selenium concentration in cystic fibrosis was 0.122 ± 0.025 µg/gm. Although the levels of selenium in cystic fibrosis children were below the levels found in a study of healthy children (0.223 ± 0.007 µg/gm), they are comparable to those found in children with phenylketonuria treated dietetically and exceed the blood selenium level of healthy children in New Zealand. Levels of the selenoenzyme glutathione peroxidase in children with cystic fibrosis (0.042 ± 0.007 units/mg Hb) were in the normal range (0.035 ± 0.003 units/mg of Hb). These results do not support the hypothesis that deficiency of selenium is responsible for cystic fibrosis.

Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients

2021 ◽  
Author(s):  
Dilara Bal Topcu ◽  
Gökcen Tugcu ◽  
Berrin Er ◽  
Sanem Eryilmaz Polat ◽  
Mina Hizal ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pediatric Patients ◽  
Adult Patients ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Children ◽  
Plasma Samples ◽  
Stable Period ◽  
Valuable Marker ◽  
Chitotriosidase Activity ◽  
Relevant Factors

Abstract Background We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Methods Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. Results YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with % FVC (r= -0.800, p = 0.014) and % FEV1 (r= -0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with % FVC (r= -0.697, p = 0.0082) and % FEV1 (r= -0.720, p = 0.006). Conclusions CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period of time compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology. Furthermore, as YKL-40 levels are correlated with FEV1 and FVC in patients, it may be useful for the monitoring of pulmonary function in CF patient.

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.

Lipid, nitrogen, water and energy content of a single stool sample in healthy children and children with cystic fibrosis

2003 ◽  
Vol 162 (11) ◽  
pp. 764-766 ◽  
Author(s):  
Anita Maria Van den Neucker ◽  
Pierre-Philippe Forget ◽  
Bernard van Kreel
Keyword(s):  
Cystic Fibrosis ◽  
Stool Sample ◽  
Energy Content ◽  
Healthy Children
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