Literature DB >> 25300456

Nasal potential difference: Best or average result for CFTR function as diagnostic criteria for cystic fibrosis?

Katherine Keenan1, Julie Avolio2, Claudia Rueckes-Nilges3, Elizabeth Tullis4, Tanja Gonska5, Lutz Naehrlich6.   

Abstract

BACKGROUND: The current practice of averaging the nasal potential difference (NPD) results of right and left nostril measurements reduce inter-individual variability but may underestimate individual CFTR function.
METHODS: Best NPD response to Cl(-)-free and isoproterenol perfusion (=largest ΔPD(0Cl/Iso)) from the right and left nostril was compared to the average result in 13 cystic fibrosis (CF), 78 query-CF patients and 22 healthy controls from 2 cohorts.
RESULTS: Despite moderate to good correlation (p<0.001) between right and left measured ΔPD(0Cl/Iso), we observed large differences in some individuals. A comparison of average versus best ΔPD(0Cl/Iso) showed only moderate agreement (Giessen κ=0.538; Toronto κ=0.607). Averaging ΔPD(0Cl/Iso) showed a lower composite chloride response compared to best ΔPD(0Cl/Iso) and altered diagnostic NPD interpretation in 30 of 113 (27%) subjects.
CONCLUSIONS: The current practice of averaging the NPD results of right and left nostril measurements leads to an underestimation of the individual CFTR function and should be reconsidered.
Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Cystic fibrosis; Diagnosis; Nasal potential difference

Mesh:

Substances:

Year:  2014        PMID: 25300456     DOI: 10.1016/j.jcf.2014.09.006

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


  4 in total

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Authors:  Wenjun Di; Heather A Clark
Journal:  Anal Methods       Date:  2020-02-26       Impact factor: 2.896

2.  Using Drugs to Probe the Variability of Trans-Epithelial Airway Resistance.

Authors:  Kendra Tosoni; Diane Cassidy; Barry Kerr; Stephen C Land; Anil Mehta
Journal:  PLoS One       Date:  2016-02-29       Impact factor: 3.240

3.  What can the CF registry tell us about rare CFTR-mutations? A Belgian study.

Authors:  E De Wachter; M Thomas; S S Wanyama; S Seneca; A Malfroot
Journal:  Orphanet J Rare Dis       Date:  2017-08-22       Impact factor: 4.123

4.  Repeatability and Diagnostic Value of Nasal Potential Difference in a Genetically Admixed Population.

Authors:  Izabela Rocha Sad; Laurinda Yoko Shinzato Higa; Teresinha Leal; Raisa da Silva Martins; Ana Claudia de Almeida; Eloane Goncalves Ramos; Giselda Maria Kalil de Cabello; Maria Virginia Marques Peixoto
Journal:  J Clin Med Res       Date:  2015-12-03
  4 in total

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