Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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승모판 탈출증을 가진 소아에서 Pyridoxine 반응성 철적모구성 빈혈 1례

Pyridoxine responsive sideroblastic anemia in a boy with mitral valve prolapse

  • 성준승 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 김기환 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 한동균 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 김미정 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 조영국 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 정해율 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 백희조 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 마재숙 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 국훈 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과) ;
  • 황태주 (전남대학교 의과대학 소아과학교실, 화순전남대학교 병원 소아과)
  • Sung, June Seung (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Kim, Ki Hwan (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Han, Dong Gyun (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Kim, Mi Jeong (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Cho, Young Kook (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Chung, Hae Yul (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Baek, Hee Jo (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Ma, Jae Sook (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Kook, Hoon (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School) ;
  • Hwang, Tai Ju (Department of Pediatrics, Hwasun Chonnam National University Hospital Chonnam National University Medical School)
  • 투고 : 2006.08.16
  • 심사 : 2006.09.13
  • 발행 : 2006.11.15
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초록

철적모구성 빈혈은 heme 대사의 이상으로 혈청철의 증가와 소구성 저색소성 빈혈을 보이며, 골수 소견에서 적혈구계 과다형성과 함께 환상 철적모구가 많이 나타나는 것이 특징이다. 저자들은 2세 남아에서 드문 질환인 철적모구성 빈혈과 승모판 탈출증에 의한 승모판 역류가 동시에 있는 증례를 경험하였다. 환아는 철적모구성 빈혈에 의해 수혈에 따른 철분의 과잉 침착과 만성 빈혈에 의한 심계 항진에 의한 심근 기능 장애가 발생할 가능성이 있는데다, 동반된 승모판 탈출증으로 심기능의 장애의 위험이 증가할 것으로 사료된다. 현재 환아는 pyridoxine 투약 후 전신 상태와 혈색소 수치가 안정적으로 유지되고 있다.

Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of cardiac dysfunction in sideroblastic anemia due to transfusion-associated myocardial iron overload and chronic anemia, a coincidence of MVP will further increase the likelihood of the morbidity or mortality of th patient. in this patient. After response to pyridoxine, the patient remains in good condition with stable hemoglobin levels.

키워드

참고문헌

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