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

Naehrlich, Lutz;Wilschanski, Michael;Vermeulen, Francois;Tümmler, Burkhard;Ballmann, Manfred;et.al.
(2014) Journal of Cystic Fibrosis — Vol. 13, n° 1, p. 24-28 (2014)

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Authors
  • Naehrlich, LutzDepartment of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany
    Author
  • Wilschanski, MichaelPediatric Gastroenterology, Hadassah University Hospital, Jerusalem, Israel
    Author
  • Vermeulen, FrancoisCystic Fibrosis Reference Centre, University Hospital Gasthuisberg, Catholic University of Leuven, Leuven, Belgium
    Author
  • Tümmler, BurkhardKlinik für Pädiatrische Pneumologie, Allergologie und Neonatologie, Medizinische Hochschule Hannover, Germany
    Author
  • Leal, TeresinhaUCLouvain
    Author
  • Ballmann, ManfredRuhr University Paediatric Clinic at St Josef Hospital, Bochum, Germany
    Author
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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. © 2013 European Cystic Fibrosis Society.
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Naehrlich, L., Wilschanski, M., Vermeulen, F., Tümmler, B., Middleton, P., Melotti, P., Leal, T., van Konigsbruggen-Rietschel, S., Hjelte, L., Gonska, T., Derichs, N., Davies, J., & Ballmann, M. (2014). Nasal potential difference measurements in diagnosis of cystic fibrosis: An international survey. Journal of Cystic Fibrosis, 13(1), 24-28. https://doi.org/10.1016/j.jcf.2013.08.006 (Original work published 2014)