All issues > Volume 52(3); 2009

Case Report

Korean J Pediatr. 2009;52(3):376-379. Published online March 15, 2009.

Pseudohypoaldosteronism in a premature neonate with severe polyhydramnios in utero

So Yoon SY Ahn¹, Son Moon SM Shin¹, Kyung Ah KA Kim¹, Yeon Kyung YK Lee¹, Sun Young SY Ko¹

¹Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center, Kwandong University College of Medicine, Seoul, Korea

Abstract

We report a case of a premature newborn baby who presented with hyponatremia, hyperkalemia, and metabolic acidosis accompanied by severe polyhydramnios in utero. The baby was diagnosed with pseudohypoaldosteronism on the basis of normal 17-hydroxyprogesterone levels, elevated aldosterone, and clinical symptoms. His serum electrolyte levels were corrected with sodium chloride supplementation. Sodium supplementation was reduced gradually and discontinued at 5 months of age. At 5 months, the child was able to maintain normal serum electrolyte levels without oral sodium chloride supplementation, and showed normal physical and neurological development. This case illustrates that pseudohypoaldosteronism must be considered if a newborn infant with an antenatal history of severe polyhydramnios shows excessive salt loss with normal levels of 17-hydroxyprogesterone.

Keywords :Pseudohypoaldosteronism, Polyhydramnios