scholarly journals Understanding FEV 1 for the purpose of cystic fibrosis registry comparisons: Does bias in annual review FEV 1 affect between‐centre comparison within the UK? An analysis of registry data

2019 ◽  
Vol 26 (1) ◽  
pp. 229-235
Author(s):  
Zhe Hui Hoo ◽  
Michael J. Campbell ◽  
Stephen J. Walters ◽  
Martin J. Wildman
Keyword(s):  
Cystic Fibrosis ◽  
Registry Data ◽  
Annual Review ◽  
The Uk

scholarly journals Development and external validation of 1- and 2-year mortality prediction models in cystic fibrosis

2019 ◽  
Vol 54 (3) ◽  
pp. 1900224 ◽  
Author(s):  
Sanja Stanojevic ◽  
Jenna Sykes ◽  
Anne L. Stephenson ◽  
Shawn D. Aaron ◽  
George A. Whitmore
Keyword(s):  
Cystic Fibrosis ◽  
Health Status ◽  
Prediction Models ◽  
Risk Model ◽  
External Validation ◽  
Registry Data ◽  
Receiver Operating Curve ◽  
Clinical Tool ◽  
Risk Of Death ◽  
The Uk

IntroductionWe aimed to develop a clinical tool for predicting 1- and 2-year risk of death for patients with cystic fibrosis (CF). The model considers patients' overall health status as well as risk of intermittent shock events in calculating the risk of death.MethodsCanadian CF Registry data from 1982 to 2015 were used to develop a predictive risk model using threshold regression. A 2-year risk of death estimated conditional probability of surviving the second year given survival for the first year. UK CF Registry data from 2007 to 2013 were used to externally validate the model.ResultsThe combined effect of CF chronic health status and CF intermittent shock risk provided a simple clinical scoring tool for assessing 1-year and 2-year risk of death for an individual CF patient. At a threshold risk of death of ≥20%, the 1-year model had a sensitivity of 74% and specificity of 96%. The area under the receiver operating curve (AUC) for the 2-year mortality model was significantly greater than the AUC for a model that predicted survival based on forced expiratory volume in 1 s <30% predicted (AUC 0.95 versus 0.68 respectively, p<0.001). The Canadian-derived model validated well with the UK data and correctly identified 79% of deaths and 95% of survivors in a single year in the UK.ConclusionsThe prediction models provide an accurate risk of death over a 1- and 2-year time horizon. The models performed equally well when validated in an independent UK CF population.

scholarly journals 241 Variation of cystic fibrosis annual review (AR) practices in the UK

2016 ◽  
Vol 15 ◽  
pp. S112
Author(s):  
C. Sellar ◽  
R.M. Kaminski ◽  
D. Nazareth
Keyword(s):  
Cystic Fibrosis ◽  
Annual Review ◽  
The Uk

scholarly journals WS10-6 The UK cystic fibrosis transplant pathway: from evaluation to post-transplant survival using Registry data

2019 ◽  
Vol 18 ◽  
pp. S19
Author(s):  
R.H. Keogh ◽  
D.K. Schluter ◽  
S. Carr ◽  
S.C. Charman ◽  
R. Cosgriff ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Registry Data ◽  
Transplant Survival ◽  
Post Transplant ◽  
The Uk

scholarly journals P085 Investigating time between annual review encounters in the UK Cystic Fibrosis Registry

2019 ◽  
Vol 18 ◽  
pp. S81-S82
Author(s):  
E. McClenaghan ◽  
R. Cosgriff ◽  
M. Yip ◽  
S.C. Charman ◽  
A. Lee ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Annual Review ◽  
The Uk

scholarly journals Comparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia

2005 ◽  
Vol 4 (2) ◽  
pp. 115-122 ◽  
Author(s):  
Jonathan McCormick ◽  
Erika J. Sims ◽  
Michael W. Green ◽  
Gita Mehta ◽  
Frank Culross ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Comparative Analysis ◽  
Registry Data ◽  
The Uk

scholarly journals The importance of data issues when comparing cystic fibrosis registry outcomes between countries: Are annual review FEV1 in the UK only collected when subjects are well?

2018 ◽  
Vol 24 (4) ◽  
pp. 745-751 ◽  
Author(s):  
Zhe Hui Hoo ◽  
Rachael Curley ◽  
Michael J. Campbell ◽  
Stephen J. Walters ◽  
Martin J. Wildman
Keyword(s):  
Cystic Fibrosis ◽  
Annual Review ◽  
The Uk

scholarly journals Cystic fibrosis

2019 ◽  
Vol 13 (1) ◽  
pp. 39-46
Author(s):  
Jen Standen
Keyword(s):  
Cystic Fibrosis ◽  
Genetic Testing ◽  
Neonatal Screening ◽  
Tertiary Care ◽  
Multidisciplinary Care ◽  
Shared Care ◽  
The Uk

In the UK over 10 000 people live with cystic fibrosis (CF), with 1-in-25 people being carriers of the disease. Multidisciplinary care is provided by tertiary care CF centres, with or without local secondary service shared care agreements. There are still, however, several reasons why CF sufferers or their families present to their GPs. This article aims to provide a brief overview of CF and its management. It also gives the information needed to guide patients about genetic testing and neonatal screening for the disease.

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