All issues > Volume 37(1); 1994

Case Report

J Korean Pediatr Soc. 1994;37(1):122-128. Published online January 15, 1994.

Two Case of Psedohypoaldosteronism Type 1

Ji Eun JE Lee¹, Jung Wan JW Seo¹, Seung Joo SJ Lee¹

¹Department of Pediatrics, College of Medicine, Ewah Womans University Hospital, Seoul, Korea

Abstract

Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous an exogenou aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances. We experienced 2 cases of PHA type 1 in 38-day and 450day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of Psedohypoaldosteronism Type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients.

Keywords :Psedohypoaldosteronism Type 1, Hyponatremia, Renal salt wasting