Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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장기간 치료받은 부신 피질 과형성증 환아에서 발생한 부신 피질 종양 1례

A case of adrenocortical adenoma following long-term treatment in a patient with congenital adrenal hyperplasia

  • 노승림 (가톨릭대학교 의과대학 소아과학교실) ;
  • 박소현 (가톨릭대학교 의과대학 소아과학교실) ;
  • 정민호 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이병철 (가톨릭대학교 의과대학 소아과학교실)
  • Lho, Seung Rim (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Park, So Hyun (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Jung, Min Ho (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Byung Churl (Department of Pediatrics, College of Medicine, The Catholic University of Korea)
  • 투고 : 2006.11.20
  • 심사 : 2007.02.15
  • 발행 : 2007.03.15
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초록

최근 해상도가 항진된 방사선학적 검사가 널리 사용되면서, 21-수산화효소 결핍증에 의한 선천성 부신 피질 과형성증 환아에서 부신피질종양이 발견되는 경우가 많아지고 있다. 일반적으로 적절한 스테로이드 호르몬 치료를 받은 21-수산화효소 결핍증 소아에서 부신피질 종양의 발생률는 매우 드물다. 저자들은 생후 초기부터 적절한 용량의 스테로이드 치료로 잘 조절되고 있는 염분소실형 21-수산화효소 결핍증을 가진 12세 여아에서 부신 종양이 우연히 발견된 1례를 보고하고자 한다.

As a result of the widespread use and enhanced quality of high-resolution radiological techniques, a recent report has revealed a relatively high prevalence of small adrenal tumors in patients with untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency. However, there are scarcely any pediatric cases of adrenocortical tumor following long-term treatment in patients suffering with congenital adrenal hyperplasia. We report here on a pediatric female case of adrenocortical adenoma despite adequate long-term treatment for the salt-losing type of congenital adrenal hyperplasia.

키워드

참고문헌

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