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

被引:31
作者
Naehrlich, Lutz [1 ]
Ballmann, Manfred [2 ]
Davies, Jane [3 ,4 ,5 ]
Derichs, Nico [6 ,7 ]
Gonska, Tanja [8 ]
Hjelte, Lena [9 ]
van Konigsbruggen-Rietschel, Silke [10 ]
Leal, Teresinha [11 ]
Melotti, Paola [12 ]
Middleton, Peter [13 ]
Tuemmler, Burkhard [14 ]
Vermeulen, Francois [15 ]
Wilschanski, Michael [16 ]
机构
[1] Univ Giessen, Dept Pediat, D-35385 Giessen, Germany
[2] Ruhr Univ Bochum, St Josef Hosp, Paediat Clin, Bochum, Germany
[3] Royal Brompton & Harefield NHS Fdn Trust, Paediat Resp Med, London, England
[4] Royal Brompton & Harefield NHS Fdn Trust, Resp Biomed Res Unit, London, England
[5] Univ London Imperial Coll Sci Technol & Med, Dept Gene Therapy, London SW7 2AZ, England
[6] Charite, CFTR Biomarker Ctr, D-13353 Berlin, Germany
[7] Charite, Christiane Herzog Cyst Fibrosis Ctr, Translat CF Res Grp, D-13353 Berlin, Germany
[8] Hosp Sick Children, Toronto, ON M5G 1X8, Canada
[9] Karolinska Univ, Huddinge Hosp, Stockholm Cyst Fibrosis Ctr, Karolinska Inst, Stockholm, Sweden
[10] Univ Cologne, Childrens Hosp, CF Ctr Cologne, Cologne, Germany
[11] Catholic Univ Louvain, Louvain Ctr Toxicol & Appl Pharmacol LTAP, B-1200 Brussels, Belgium
[12] Azienda Osped Univ Integrata Verona, Cyst Fibrosis Ctr, Verona, Italy
[13] Univ Sydney, Ludwig Engel Ctr Resp Res, Westmead Millennium Inst, Cyst Fibrosis Unit, Westmead, NSW 2145, Australia
[14] Hannover Med Sch, Klin Padiatr Pneumol, Hannover, Germany
[15] Catholic Univ Louvain, Univ Hosp Gasthuisberi, Cyst Fibrosis Reference Ctr, B-3000 Louvain, Belgium
[16] Hadassah Univ Hosp, Pediat Gastroenterol, IL-91120 Jerusalem, Israel
关键词
Cystic fibrosis; Nasal potential difference; Diagnosis; SWEAT TEST; GUIDELINES; PROTOCOLS; CHLORIDE;
D O I
10.1016/j.jcf.2013.08.006
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
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-SCIP (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. (C) 2013 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:24 / 28
页数:5
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