The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype Julia C. Espel, Hannah L. Palac, Ankit.

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Presentation transcript:

The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype Julia C. Espel, Hannah L. Palac, Ankit Bharat, Joanne Cullina, Michelle Prickett, Marc Sala, Susanna A. McColley, Manu Jain Journal of Cystic Fibrosis Volume 17, Issue 1, Pages 34-42 (January 2018) DOI: 10.1016/j.jcf.2017.11.002 Copyright © 2017 European Cystic Fibrosis Society Terms and Conditions

Fig. 1 CONSORT diagram of subject population. Journal of Cystic Fibrosis 2018 17, 34-42DOI: (10.1016/j.jcf.2017.11.002) Copyright © 2017 European Cystic Fibrosis Society Terms and Conditions

Fig. 2 Sweat chloride distribution by most common genotype in each class. Panel A shows distribution of sweat chloride by individual genotype with box and whisker plots. The boxes show the 1st quartile, median and 3rd quartile. The error bars show the 5th and 95th percentile ranges. Panels B and C are histograms of sweat chloride values for the F508del homozygote and R117H/F508del genotypes with the percent of patients with sweat chloride values grouped by 10s. The F508del homozygote genotype had a normal distribution centered on a mean of 102mmol/L. The R117H/F508del genotype had a lower mean as well as a distribution skewed toward the lower sweat chloride values. Journal of Cystic Fibrosis 2018 17, 34-42DOI: (10.1016/j.jcf.2017.11.002) Copyright © 2017 European Cystic Fibrosis Society Terms and Conditions

Fig. 3 Receiver operating curves for selected genotypes. ROC analysis of sweat chloride as continuous variable shows for predicting mortality shows within an individual genotype. The area under the curve (AUC) shows good predictability for the R117H/F508del genotype and a more modest predictability for the 2789+5G→A/F508del genotype. There is less predictability in the G542X/F508del, F508del homozygote and G551D/F508del genotypes by ROC analysis. Journal of Cystic Fibrosis 2018 17, 34-42DOI: (10.1016/j.jcf.2017.11.002) Copyright © 2017 European Cystic Fibrosis Society Terms and Conditions

Fig. 4 Kaplan Meier curves for selected genotypes. Panels A–E show the Kaplan Meier curves for the G542X/F508del (A), F508del/F508del (B), G551D/F508del (C), and R117H/F508del (D) and 2789+5G>A/F508del genotypes with patients divided by tertiles for baseline sweat chloride values specific to each genotype. Journal of Cystic Fibrosis 2018 17, 34-42DOI: (10.1016/j.jcf.2017.11.002) Copyright © 2017 European Cystic Fibrosis Society Terms and Conditions