Original articleClinical application of transepithelial potential difference measurements in cystic fibrosis*
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Cited by (33)
Cystic fibrosis
2019, Emery and Rimoin's Principles and Practice of Medical Genetics and Genomics: Cardiovascular, Respiratory, and Gastrointestinal DisordersDevelopmental and Inherited Liver Disease
2018, MacSween's Pathology of the LiverMeasurement of ion fluxes across epithelia
2017, Progress in Biophysics and Molecular BiologyCitation Excerpt :The nasal potential value of healthy subjects is reported to be between −15 and −20 mV, while in CF patients, the nasal potential reaches much lower values −43 to −52 mV. The application of amiloride (apical ENaC sodium channel blocker) changes the nasal potential to the values 5 to −15 mV similar for both healthy and CF patients (Knowles et al., 1983; Gowen et al., 1986; Sauder et al., 1987; Middleton et al., 1993; Höfmann et al., 1997; Wilson et al., 1998; De Boeck et al., 2011; Sands, 2013). The mechanism of the nasal potential generation seems to involve both CFTR and ENaC channels present in the apical face of the nasal epithelium (see Fig. 1).
Nasal potential difference measurements in diagnosis of cystic fibrosis: An international survey
2014, Journal of Cystic FibrosisCitation Excerpt :Sodium conductance is assessed by perfusion with amiloride and CFTR function by perfusion with a chloride-free solution and isoproterenol [1]. NPD has been used as a diagnostic test for CF since the late 1980s [2–5] and was accepted as a diagnostic procedure equal to sweat chloride > 60 mmol/l and/or detection of two CF-causing mutations in 1998 in the Cystic Fibrosis Foundation (CFF) consensus statement [6]. This consensus statement highlighted the need for standardization, technical rigour, and own reference ranges, especially for diagnostic use [6].
Cystic Fibrosis
2013, Emery and Rimoin's Principles and Practice of Medical GeneticsGenetic and metabolic liver disease
2012, MacSween's Pathology of the Liver
- *
Supported by American Lung Association of Florida, Pediatric Pulmonary Center Grant MCJ-2013, and New Investigation Research Award HL-26952-03 from the National Heart, Lung, and Blood Institute.
in part at the Annual Meeting of the Cystic Fibrosis Club, Anaheim, Calif., 1984.