Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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양수과다증 산전력이 있는 미숙아의 가성저알도스테론혈증 1예

Pseudohypoaldosteronism in a premature neonate with severe polyhydramnios in utero

  • 안소윤 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 신손문 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 김경아 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 이연경 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 고선영 (관동대학교 의과대학 제일병원 소아청소년과)
  • Ahn, So Yoon (Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center Kwandong University College of Medicine) ;
  • Shin, Son Moon (Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center Kwandong University College of Medicine) ;
  • Kim, Kyung Ah (Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center Kwandong University College of Medicine) ;
  • Lee, Yeon Kyung (Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center Kwandong University College of Medicine) ;
  • Ko, Sun Young (Department of Pediatrics, Cheil General Hospital & Womens Healthcare Center Kwandong University College of Medicine)
  • 투고 : 2008.08.25
  • 심사 : 2008.10.30
  • 발행 : 2009.03.15
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초록

가성 저알도스테론혈증은 신장 세뇨관의 알도스테론 수용체의 이상으로 알도스테론의 분비는 증가되지만 반응성은 감소되어 저알도스테론혈증의 증상을 보이는 질병이다. 가성 저알도스테론혈증은 다뇨증, 과도한 소변 내 염분소실, 저나트륨혈증, 고칼륨혈증, 대사성 산증을 특징으로 하며, 산전부터 다뇨 증상이 시작되면 양수과다증을 초래할 수 있다. 미숙아에서는 생후 초기 불감 수분 손실량이 많으며, 폐 기능과 신장 기능이 미숙하여 전해질 이상을 흔히 동반하는데, 몸무게의 소실에도 불구하고 지속적인 다뇨와 저나트륨혈증, 고칼륨혈증의 증상이 있다면 전해질 불균형을 초래하는 기저질환을 확인하여야 한다. 저자들은 양수 과다증의 산전력이 있는 산모에게서 출생한 재태주령 32주 미숙아에서 지속적인 몸무게 감소에도 불구하고 계속되는 다뇨와 저나트륨혈증, 고칼륨혈증이 있어 17수산화프로게스테론을 조기에 선별 검사하여 선천성 부신 과형성증의 가능성을 배제함으로써 불필요한 스테로이드 치료를 피하고, 지속적인 전해질 보충으로 점차 전해질 이상의 호전을 보인 가성 저알도스테론혈증 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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.

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참고문헌

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피인용 문헌

  1. A case of pseudohypoaldosteronism type 1 with a mutation in the mineralocorticoid receptor gene vol.54, pp.2, 2011, https://doi.org/10.3345/kjp.2011.54.2.90
  2. Pseudohypoaldosteronism in a newborn male with functional polymorphisms in the mineralocorticoid receptor genes vol.20, pp.4, 2009, https://doi.org/10.6065/apem.2015.20.4.230