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Pseudohypoaldosteronism in a premature neonate with severe polyhydramnios in utero

Korean Journal of Pediatrics 2009;52(3):376-379.
Published online March 15, 2009.
Pseudohypoaldosteronism in a premature neonate with severe polyhydramnios in utero
So Yoon Ahn, Son Moon Shin, Kyung Ah Kim, Yeon Kyung Lee, Sun Young Ko
Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center, Kwandong University College of Medicine, Seoul, Korea
양수과다증 산전력이 있는 미숙아의 가성저알도스테론혈증 1예
안소윤, 신손문, 김경아, 이연경, 고선영
관동대학교 의과대학 제일병원 소아청소년과
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.
Key Words: Pseudohypoaldosteronism, Polyhydramnios

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