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

Prince LS, Launspach JL, Geller DS, Lifton RP, Pratt JH, Zabner J, Welsh MJ
J Pediatr. 1999 135 (6): 786-9

PMID: 10586189 · DOI:10.1016/s0022-3476(99)70105-8

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.

MeSH Terms (10)

Absorption Amiloride Humans Infant, Newborn Male Membrane Potentials Nasal Mucosa Pseudohypoaldosteronism Renin Sodium Channels

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