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J Cyst Fibros. 2014 Jan;13(1):24-8. doi: 10.1016/j.jcf.2013.08.006. Epub 2013 Sep 7.

Nasal potential difference measurements in diagnosis of cystic fibrosis: an international survey.

Author information

1
Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany. Electronic address: lutz.naehrlich@paediat.med.uni-giessen.de.
2
Ruhr University Paediatric Clinic at St Josef Hospital, Bochum, Germany.
3
Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, United Kingdom; Respiratory Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, United Kingdom; Department of Gene Therapy, Imperial College London, United Kingdom.
4
CFTR Biomarker Centre and Translational CF Research Group Christiane Herzog Cystic Fibrosis Centre, Paediatric Pulmonology and Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany.
5
Hospital for Sick Children, Toronto, Canada.
6
Stockholm Cystic Fibrosis Center, Karolinska Institutet, Karolinska University Hospital Huddinge, Stockholm, Sweden.
7
CF Centre Cologne, Children's Hospital, University of Cologne, Germany.
8
Louvain Centre for Toxicology and Applied Pharmacology (LTAP), Université catholique de Louvain, Brussels, Belgium.
9
Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy.
10
Cystic Fibrosis Unit, Ludwig Engel Centre for Respiratory Research, Westmead Millennium Institute, University of Sydney at Westmead, Westmead, NSW, Australia.
11
Klinik für Pädiatrische Pneumologie, Allergologie und Neonatologie, OE 6710, Medizinische Hochschule Hannover, Hannover, Germany.
12
Cystic Fibrosis Reference Centre, University Hospital Gasthuisberg, Catholic University of Leuven, Leuven, Belgium.
13
Pediatric Gastroenterology, Hadassah University Hospital, Jerusalem, Israel.

Abstract

BACKGROUND:

The role of nasal potential difference (NPD) measurement as a diagnostic test for cystic fibrosis (CF) is a subject of global controversy because of the lack of validation studies, clear reference values, and standardized protocols for diagnostic NPD.

METHODS:

To determine diagnostic NPD frequency, protocols, interpretation, and rater agreement, we surveyed the 18 NPD centres of the European Cystic Fibrosis Society Diagnostic Network Working Group.

RESULTS:

Fifteen centres reported performing 373 diagnostic NPDs in 2012. Most use the CFF-TDN-SOP (67%) and the chloride-free + isoproterenol response of the side with the largest response (47%) as diagnostic criteria and use centre-specific reference ranges. Rater agreement for five NPD tracings - in general - was good, but poor in tracings with different responses between the two nostrils.

CONCLUSIONS:

NPD is frequently used as a diagnostic and research tool for CF. Performance is highly standardized, centre-specific reference ranges are established, and rater agreement - in general - is good. Centre-independent diagnostic criteria and reference ranges must be defined by multicentre validation studies to improve standardized interpretation for diagnostic use.

KEYWORDS:

Cystic fibrosis; Diagnosis; Nasal potential difference

PMID:
24022019
DOI:
10.1016/j.jcf.2013.08.006
[Indexed for MEDLINE]
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