Increased nasal potential difference and amiloride sensitivity in neonates with cystic fibrosis

C. W. Gowen, E. E. Lawson, J. Gingras-Leatherman, J. T. Gatzy, R. C. Boucher, M. R. Knowles

Research output: Contribution to journalArticle

Abstract

Patients with cystic fibrosis (CF) have an increased nasal transepithelial potential difference (PD) which reflects increased sodium absorption across epithelium relatively impermeable to chloride. To evaluate nasal epithelial function in neonates with CF, the PD was recorded and the voltage response to superfusion of 10-5M amiloride, an inhibitor of sodium transport, measured between a Ringer perfused bridge on the nasal mucosa and a reference electrode in the subcutaneous space. We studied three neonates with CF with meconium ileus and compared the results with those in 24 term healthy neonates, including one obligate heterozygote for CF, and 27 control neonates with disease. All three CF neonates had raised sweat chloride values (mean 100 mEq/L) at 2 months. The CF neonates had higher PDs (-64.0±8.4 mV) than those in normal (-24.4±2.0 mV) or control (-25.8±2.0 mV) neonates. Superfusion with amiloride induced a 72% reduction in PD in the CF neonates as compared with healthy (37.5±1.0%) and diseased (36.0±1.3%) neonates. The PD and amiloride response in CF neonates are similar to those in CF infants (2-24 months), older CF children (>6 years), and CF adults (-64.9±9.3 mV; 77.7±1.8%, n=51). These results suggest that (1) nasal epithelial dysfunction is present in patients with CF shortly after birth, and (2) the nasal PD may be a diagnostic adjunct to the sweat test in the early diagnosis of CF.

Original languageEnglish (US)
Pages (from-to)517-521
Number of pages5
JournalThe Journal of pediatrics
Volume108
Issue number4
DOIs
StatePublished - Apr 1986
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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