Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Pheochromocytoma associated with cyanotic congenital heart disease

청색증형 선천성 심질환에 동반된 갈색세포종

  • Chung, Seung Joon (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Lee, Young Ah (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Shin, Choong Ho (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Yang, Sei Won (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Bae, Eun Jung (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Noh, Jung Il (Department of Pediatrics, College of Medicine, Seoul National University)
  • 정승준 (서울대학교 의과대학 소아과학교실) ;
  • 이영아 (서울대학교 의과대학 소아과학교실) ;
  • 신충호 (서울대학교 의과대학 소아과학교실) ;
  • 양세원 (서울대학교 의과대학 소아과학교실) ;
  • 배은정 (서울대학교 의과대학 소아과학교실) ;
  • 노정일 (서울대학교 의과대학 소아과학교실)
  • Received : 2007.09.03
  • Accepted : 2007.10.22
  • Published : 2008.01.15
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Abstract

Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition, or unusual symptoms such as diabetes mellitus develops in a cyanotic patient with congenital heart disease, pheochromocytoma must be ruled out. We report two patients presenting with cyanotic single-ventricle heart disease with pheochromocytoma.

갈색세포종은 부신 수질과 크롬친화성 조직에서 발생하는 소아기에는 드문 종양이다. 갈색세포종은 저산소증 상태와 연관이 있어, 단심실성 심장질환 수술 후에도 청색증이 지속되는 환자에서 병발하는 경우가 드물게 보고되고 있다. 갈색세포종의 증상이 애매할 뿐만 아니라 심장 질환 또는 치료 약제에 의하여 전형적인 증상들이 나타나지 않을 수 있다. 청색증형 선천성 심질환 환자에서 갑작스런 고혈압, 심장 상태의 악화, 또는 당뇨병 등을 포함한 애매한 증상들이 나타나는 경우에는 갈색세포종을 감별 진단하여야 한다. 저자들은 선천성 단심실성 심장질환과 갈색세포종이 동반된 2례를 보고한다.

Keywords

References

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