Document Detail


Nasal potential difference: Best or average result for CFTR function as diagnostic criteria for cystic fibrosis?
MedLine Citation:
PMID:  25300456     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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 ΔPD0Cl/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 ΔPD0Cl/Iso, we observed large differences in some individuals. A comparison of average versus best ΔPD0Cl/Iso showed only moderate agreement (Giessen κ=0.538; Toronto κ=0.607). Averaging ΔPD0Cl/Iso showed a lower composite chloride response compared to best ΔPD0Cl/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.
Authors:
Katherine Keenan; Julie Avolio; Claudia Rueckes-Nilges; Elizabeth Tullis; Tanja Gonska; Lutz Naehrlich
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-10-6
Journal Detail:
Title:  Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society     Volume:  -     ISSN:  1873-5010     ISO Abbreviation:  J. Cyst. Fibros.     Publication Date:  2014 Oct 
Date Detail:
Created Date:  2014-10-10     Completed Date:  -     Revised Date:  2014-10-11    
Medline Journal Info:
Nlm Unique ID:  101128966     Medline TA:  J Cyst Fibros     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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