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J Cyst Fibros. 2016 Sep;15(5):579-82. doi: 10.1016/j.jcf.2016.06.009. Epub 2016 Jul 15.

Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis.

Author information

1
Cystic Fibrosis Centre, Azienda Ospedaliera Universitaria Integrata Verona, Italy.
2
Cystic Fibrosis Centre, Azienda Ospedaliera Universitaria Integrata Verona, Italy; Department of Pathology and Diagnostics Medicine, Cystic Fibrosis Translational Research Laboratory "Daniele Lissandrini", University of Verona, 37134 Verona, Italy.
3
Cystic Fibrosis Centre, Azienda Ospedaliera Universitaria Integrata Verona, Italy. Electronic address: paola.melotti@ospedaleuniverona.it.

Abstract

BACKGROUND:

When cystic fibrosis (CF) is suspected Nasal Potential Difference (NPD) measurements are proposed to support controversial diagnosis: we investigated appropriate outcomes at the CF Centre of Verona.

SUBJECTS/METHODS:

NPD were measured in 196 subjects: 50 non-CF, 65 classical CF (the reference group) and 81 with uncertain CF (case group). Discriminating power was determined by comparison between several outcomes from the CF reference group versus non-CF: basal, amiloride, 0Cl, isoproterenol, ATP, Delta-amiloride, Delta-0Cl, Delta-isoproterenol, Delta-ATP, Delta-isoproterenol+Delta-0Cl, Wilschanski Index (WI) and Sermet score (SS). The most appropriate cut-off values for variables with the best discriminating power were then applied to the case group. Descriptive statistics, logistic regression models and ROC curve analysis were applied.

RESULTS:

WI and SS were the most powerful in discriminating CF from non-CF subjects. In the reference group sensitivity of the 0.82 WI cut-off was 98%, specificity 96%; both sensitivity and specificity of the -0.44 SS cut-off value were 100%. For the case group, WI and SS were, respectively, consistent with CF diagnosis in 94% and 92% of the cases.

CONCLUSIONS:

Formulae have the highest discriminating power and can support the diagnosis in uncertain cases; they should be utilized for standardized interpretation of NPD for diagnosis and possibly for clinical research.

KEYWORDS:

CFTR genetic test; CFTR related disorders; Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Diagnosis; Nasal potential difference measurements; Sweat test

PMID:
27423539
DOI:
10.1016/j.jcf.2016.06.009
[Indexed for MEDLINE]
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