Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators

2021 ◽  
Author(s):  
Cassidy Du Berry ◽  
Nicole Westrupp ◽  
Shivanthan Shanthikumar ◽  
Liam Welsh
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake ◽  
Cftr Modulators

scholarly journals Airway Inflammation and Host Responses in the Era of CFTR Modulators

2020 ◽  
Vol 21 (17) ◽  
pp. 6379
Author(s):  
Karen Keown ◽  
Ryan Brown ◽  
Declan F. Doherty ◽  
Claire Houston ◽  
Michael C. McKelvey ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Lung Disease ◽  
Airway Inflammation ◽  
Host Responses ◽  
Current Evidence ◽  
Future Research ◽  
Clinical Endpoints ◽  
New Class ◽  
Cftr Modulators

The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in disease management, as these small molecules directly target the upstream underlying protein defect. Further advancements in the development and scope of these genotype-specific therapies have been transformative for an increasing number of people with CF (PWCF). Despite clear improvements in CFTR function and clinical endpoints such as lung function, body mass index (BMI), and frequency of pulmonary exacerbations, current evidence suggests that CFTR modulators do not prevent continued decline in lung function, halt disease progression, or ameliorate pathogenic organisms in those with established lung disease. Furthermore, it remains unknown whether their restorative effects extend to dysfunctional CFTR expressed in phagocytes and other immune cells, which could modulate airway inflammation. In this review, we explore the effects of CFTR modulators on airway inflammation, infection, and their influence on the impaired pulmonary host defences associated with CF lung disease. We also consider the role of inflammation-directed therapies in light of the widespread clinical use of CFTR modulators and identify key areas for future research.

Frühgeborene mit bronchopulmonaler Dysplasie: Geeignete Therapien im Kindheitsverlauf entwickeln

2020 ◽  
Vol 8 (2) ◽  
pp. 86-88
Author(s):  
David Brandt
Keyword(s):  
Physical Activity ◽  
Lung Function ◽  
Oxygen Uptake ◽  
Sedentary Behaviour ◽  
Linear Model ◽  
Exercise Capacity ◽  
General Linear Model ◽  
Peak Oxygen Uptake ◽  
Exercise Intolerance ◽  
General Linear

Background: Extreme prematurity has been associated with exercise intolerance and reduced physical activity. We hypothesized that children with bronchopulmonary dysplasia (BPD) would be especially affected based on longterm lung function impairments. Therefore, the objective of this study was to compare exercise capacity and habitual physical activity between children born very and extremely preterm with and without BPD and term-born children. Methods: Twenty-two school-aged children (aged 8 to 12 years) born with a gestational age < 32 weeks and a birthweight < 1500 g (9 with moderate or severe BPD (=BPD), 13 without BPD (=No-BPD)) and 15 healthy termborn children (=CONTROL) were included in the study. Physical activity was measured by accelerometry, lung function by spirometry and exercise capacity by an incremental cardiopulmonary exercise test. Results: Peak oxygen uptake was reduced in the BPD-group (83 ± 11%predicted) compared to the No-BPD group (91 ± 8%predicted) and the CONTROL group (94 ± 9%predicted). In a general linear model, variance of peak oxygen uptake was significantly explained by BPD status and height but not by prematurity (p < 0.001). Compared to CONTROL, all children born preterm spent significantly more time in sedentary behaviour (BPD 478 ± 50 min, No-BPD 450 ± 52 min, CONTROL 398 ± 56 min, p < 0.05) and less time in moderate-to-vigorous-physical activity (BPD 13 ± 8 min, No-BPD 16 ± 8 min, CONTROL 33 ± 16 min, p < 0.001). Prematurity but not BPD contributed significantly to explained variance in a general linear model of sedentary behaviour and likewise moderate-tovigorous-physical activity (p < 0.05 and p < 0.001 respectively). Conclusion: In our cohort, BPD but not prematurity was associated with a reduced exercise capacity at school-age. However, prematurity regardless of BPD was related to less engagement in physical activity and more time spent in sedentary behaviour. Thus, our findings suggest diverging effects of prematurity and BPD on exercise capacity and physical activity.

Relationship between FEV1 and peak oxygen uptake in children with cystic fibrosis

2005 ◽  
Vol 40 (4) ◽  
pp. 324-329 ◽  
Author(s):  
Paul Pianosi ◽  
John LeBlanc ◽  
Anthony Almudevar
Keyword(s):  
Cystic Fibrosis ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake

Late Breaking Abstract - The association between dynamic lung function and peak oxygen uptake in a healthy general population. The HUNT Study

2017 ◽  
Author(s):  
Øystein Rasch-Halvorsen ◽  
Sigurd Steinshamn ◽  
Erlend Hassel ◽  
Ben Michael Brumpton ◽  
Arnulf Langhammer
Keyword(s):  
Lung Function ◽  
General Population ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake ◽  
Hunt Study

The association between lung function and peak oxygen uptake in coronary artery disease

2018 ◽  
Author(s):  
Øystein Rasch-Halvorsen ◽  
Erlend Hassel ◽  
Ben M Brumpton ◽  
Haldor Jenssen ◽  
Martijn A Spruit ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Lung Function ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake ◽  
Artery Disease

PREDICTORS OF PEAK OXYGEN UPTAKE IN PATIENTS WITH CYSTIC FIBROSIS

1992 ◽  
Vol 24 (Supplement) ◽  
pp. S161
Author(s):  
P. Nixon ◽  
D. Orenstein ◽  
S. Kelsay
Keyword(s):  
Cystic Fibrosis ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake

Estimating peak oxygen uptake in adolescents with cystic fibrosis

2013 ◽  
Vol 99 (1) ◽  
pp. 21-25 ◽  
Author(s):  
M. S. Werkman ◽  
E. H. J. Hulzebos ◽  
P. J. M. Helders ◽  
B. G. M. Arets ◽  
T. Takken
Keyword(s):  
Cystic Fibrosis ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake

Supramaximal Verification of Peak Oxygen Uptake in Adolescents With Cystic Fibrosis

2011 ◽  
Vol 23 (1) ◽  
pp. 15-21 ◽  
Author(s):  
Maarten S. Werkman ◽  
Hendrik J. Hulzebos ◽  
Pauline B. van de Weert-van Leeuwen ◽  
Hubertus G.M. Arets ◽  
Paul J.M. Helders ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Oxygen Uptake ◽  
Peak Oxygen Uptake

scholarly journals Peak Oxygen Uptake and Mortality in Cystic Fibrosis: Systematic Review and Meta-Analysis

2018 ◽  
Vol 64 (1) ◽  
pp. 91-98 ◽  
Author(s):  
Fernanda Maria Vendrusculo ◽  
João Paulo Heinzmann-Filho ◽  
Juliana Severo da Silva ◽  
Margarita Perez Ruiz ◽  
Márcio Vinícius Fagundes Donadio
Keyword(s):  
Systematic Review ◽  
Cystic Fibrosis ◽  
Oxygen Uptake ◽  
Meta Analysis ◽  
Peak Oxygen Uptake
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