Elsevier

The Journal of Pediatrics

Volume 135, Issue 6, December 1999, Pages 786-789
The Journal of Pediatrics

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

https://doi.org/10.1016/S0022-3476(99)70105-8Get rights and content
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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)

Abbreviations

CF
Cystic fibrosis
CFTR
Cystic fibrosis transmembrane regulator
ENaC
Epithelial Na+ channel
PHA
Pseudohypoaldosteronism
PRA
Plasma renin activity

Cited by (0)

Supported by the Clinical Core of a Cystic Fibrosis Foundation Research Development Program. Dr Prince is supported by the Cystic Fibrosis Foundation and an individual National Research Service Award. Dr Welsh is an investigator of the Howard Hughes Medical Institute.

☆☆

Reprint requests: Michael J. Welsh, MD, Howard Hughes Medical Institute, University of Iowa College of Medicine, 500 EMRB, Iowa City, IA 52242.

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