Literature DB >> 26996268

Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations.

F Vermeulen1, C Le Camus2, J C Davies3, D Bilton4, D Milenković5, K De Boeck6.   

Abstract

INTRODUCTION: Sweat chloride concentration, a biomarker of CFTR function, is an appropriate outcome parameter in clinical trials aimed at correcting the basic CF defect. Although there is consensus on a cut-off value to diagnose CF, we have only limited information on the within subject variability of sweat chloride over time. Such information would be useful for sample size calculations in clinical trials. Therefore, we retrospectively analyzed repeated sweat chloride values obtained in patients with G551D mutation(s) assigned to placebo in an ivacaftor interventional trial.
METHODS: In subjects with G551D at least 12years of age, a pilocarpine sweat test using Macroduct collector was taken on both arms at 8 time points over 48weeks. We explored 1062 pilocarpine sweat test values obtained in 78 placebo patients of the VX08-770-102 trial.
RESULTS: Mean overall sweat chloride value (all patients, all tests, n=1062) was 100.8mmol/L (SD 12.7mmol/L). Using a multilevel mixed model, the between-subject standard deviation (SD) for sweat chloride was 8.9mmol/L (95% CI 7.4-10.6) and within-subject SD was 8.1mmol/L (95% CI 7.5-8.7). Limits of repeatability for repeat measurements were -19.7 to +21.6mmol/L using values from one arm, and -13.3 to 11.8mmol/L using mean of values obtained at 4 test occasions. Sample size calculations showed that the minimal treatment effect on sweat chloride concentration that can be demonstrated for a group of 5 patients is around 15mmol/L, using a cross-over design and combinations of 4 tests for each phase of the trial.
CONCLUSION: Although the sweat test is considered a robust measure, sweat chloride measurements in patients with CF and a G551D mutation had an inherent biological variability that is higher than commonly considered. Further analyses of placebo group data are crucial to learn more about the natural variability of this outcome parameter.
Copyright © 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Cystic fibrosis; Endpoint; G551D; Sweat chloride; Variability

Mesh:

Substances:

Year:  2016        PMID: 26996268     DOI: 10.1016/j.jcf.2016.02.015

Source DB:  PubMed          Journal:  J Cyst Fibros        ISSN: 1569-1993            Impact factor:   5.482


  7 in total

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2.  Analytical and biological variation in repeated sweat chloride concentrations in clinical trials for CFTR modulator therapy.

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Journal:  J Cyst Fibros       Date:  2017-07-22       Impact factor: 5.482

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Journal:  Genes (Basel)       Date:  2021-07-29       Impact factor: 4.096

4.  Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.

Authors:  Edith T Zemanick; Michael W Konstan; Donald R VanDevanter; Steven M Rowe; J P Clancy; Katherine Odem-Davis; Michelle Skalland; Nicole Mayer-Hamblett
Journal:  J Cyst Fibros       Date:  2021-02-08       Impact factor: 5.482

5.  Sweat chloride quantification using capillary electrophoresis.

Authors:  Patricia Dubot; Jing Liang; Jacobé Dubs; Yohann Missiak; Cédric Sarazin; François Couderc; Elizabeth Caussé
Journal:  Pract Lab Med       Date:  2018-12-07

6.  Out-of-clinic measurement of sweat chloride using a wearable sensor during low-intensity exercise.

Authors:  Dong-Hoon Choi; Grant B Kitchen; Mark T Jennings; Garry R Cutting; Peter C Searson
Journal:  NPJ Digit Med       Date:  2020-03-27

7.  Soft, skin-interfaced sweat stickers for cystic fibrosis diagnosis and management.

Authors:  Tyler R Ray; Maja Ivanovic; Paul M Curtis; Daniel Franklin; Kerem Guventurk; William J Jeang; Joseph Chafetz; Hannah Gaertner; Grace Young; Steve Rebollo; Jeffrey B Model; Stephen P Lee; John Ciraldo; Jonathan T Reeder; Aurélie Hourlier-Fargette; Amay J Bandodkar; Jungil Choi; Alexander J Aranyosi; Roozbeh Ghaffari; Susanna A McColley; Shannon Haymond; John A Rogers
Journal:  Sci Transl Med       Date:  2021-03-31       Impact factor: 17.956

  7 in total

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