Elsevier

The Journal of Pediatrics

Volume 111, Issue 3, September 1987, Pages 353-358
The Journal of Pediatrics

Original article
Clinical application of transepithelial potential difference measurements in cystic fibrosis*

https://doi.org/10.1016/S0022-3476(87)80453-5Get rights and content

We studied transepithelial potential difference (PD) in normal persons, patients with chronic disease, and patients with cystic fibrosis (CF), using the technique described by knowles and co-workers. A maximal PD value (PDmax) and an average PD value (PDmean) were determined for each study of the nasal respiratory epithelium. The volfage response to superfusion of 10−4 mol/L amiloride onto nasal mucosa was noted. The PD of the palm, wrist, and between two fingertips was also measured. Nasal PDmax and PDmean of the CF group were more negative than the control (P<0.01) and chronic disease groups (P<0.01). After application of amiloride, the voltage change in nasal PD was greater in the CF group than in the non-CF control groups (P<0.01). There were no clinically significant differences in the PD of the palm, wrist, or fingertips of the three groups. These data confirm the observation that patients with CF have hyperpolarized nasal epithelia that demonstrate greater change in response to amiloride than that in non-CF controls. These results indicate a possible role for the use of in vivo nasal PD measurements as a diagnostic test for cystic fibrosis.

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Cited by (33)

  • Cystic fibrosis

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  • Measurement of ion fluxes across epithelia

    2017, Progress in Biophysics and Molecular Biology
    Citation 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 Fibrosis
    Citation 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 Genetics
  • Genetic and metabolic liver disease

    2012, MacSween's Pathology of the Liver
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*

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.

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