Journalartikel

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


AutorenlisteKeenan, Katherine; Avolio, Julie; Rueckes-Nilges, Claudia; Tullis, Elizabeth; Gonska, Tanja; Naehrlich, Lutz

Jahr der Veröffentlichung2015

Seiten310-316

ZeitschriftJournal of Cystic Fibrosis

Bandnummer14

Heftnummer3

ISSN1569-1993

eISSN1873-5010

Open Access StatusBronze

DOI Linkhttps://doi.org/10.1016/j.jcf.2014.09.006

VerlagElsevier


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 isdproterenol perfusion (=largest Delta PD0Cl/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 Delta PD0Cl/Iso, we observed large differences in some individuals. A comparison of average versus best Delta PD0Cl/Iso showed only moderate agreement (Giessen kappa = 0.538; Toronto kappa = 0.607). Averaging Delta PD0Cl/Iso showed a lower composite chloride response compared to best Delta PD0Cl/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. (C) 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.




Zitierstile

Harvard-ZitierstilKeenan, K., Avolio, J., Rueckes-Nilges, C., Tullis, E., Gonska, T. and Naehrlich, L. (2015) Nasal potential difference: Best or average result for CFTR function as diagnostic criteria for cystic fibrosis?, Journal of Cystic Fibrosis, 14(3), pp. 310-316. https://doi.org/10.1016/j.jcf.2014.09.006

APA-ZitierstilKeenan, K., Avolio, J., Rueckes-Nilges, C., Tullis, E., Gonska, T., & Naehrlich, L. (2015). Nasal potential difference: Best or average result for CFTR function as diagnostic criteria for cystic fibrosis?. Journal of Cystic Fibrosis. 14(3), 310-316. https://doi.org/10.1016/j.jcf.2014.09.006



Schlagwörter


AIRWAY ION-TRANSPORTCHANNEL FUNCTIONCystic fibrosisGUIDELINESNasal potential differenceTRANSMEMBRANE CONDUCTANCE REGULATOR


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