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Absence of amiloride-sensitive sodium absorption in the airway of an infant with pseudohypoaldosteronism

Authors
Journal
The Journal of Pediatrics
0022-3476
Publisher
Elsevier
Publication Date
Volume
135
Issue
6
Identifiers
DOI: 10.1016/s0022-3476(99)70105-8
Disciplines
  • Medicine

Abstract

Abstract We report the measurement of transepithelial voltage across the nasal epithelium in a neonate with pseudohypoaldosteronism (PHA) type 1. A 5-day-old infant was seen with hyponatremia, hyperkalemia, and elevated plasma renin and aldosterone levels. Sweat Cl - concentration was also increased. Measurements of voltage showed a basal value of zero and the absence of an amiloride-sensitive voltage. However, voltage changed as expected for normal cyclic adenosine monophosphate–stimulated Cl - transport. These data demonstrate the absence of amiloride-sensitive Na + transport across airway epithelia in a neonate with PHA. The findings suggest that measurements of voltage could be of value in the diagnosis of PHA. (J Pediatr 1999;135:786-9)

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