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A Case of Pseudohypoaldosteronism

Journal of the Korean Pediatric Society 1992;35(7):984-988.
Published online July 15, 1992.
A Case of Pseudohypoaldosteronism
Yong Soon Kwon, Hyo Gyoung Shin, Mi Soo Ahn, Hong Bae Kim
Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea
가성 저알테스테론증 1례
권용순, 신효경, 안미수, 김홍배
왈레스 기념 침례회 병원 소아과
Abstract
Pseudohypoaldosteronism(PHA) is rare salt losing disease which is characterized by mineralcorticoid unresponsiveness of the end organ. Severe hyponatremia and hyperkalemia are present despite high plasma aldosterone. We experienced a case of PHA in a 40-days old male infant who was presented with anorexia, vomiting and lethargy for several days. Labortory data showed hyponatremia, hyperkalemia and metabolic acidosis. Renal function was normal except initial transient elevation of BUN and creatinine, normalized after correction of dehydration. Adrenal function was normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the impression of pseudohypoaldosteronism, we tried oral NaCl supplementation. Thereafter the clinical state of the patients was improved. We report a case of PHA with brief review of related literatures.
Key Words: Pseudohypoaldosteronism, Mineralcorticoid unresponsiveness


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