Journal of The Korean Society of Inherited Metabolic disease (대한유전성대사질환학회지)

The Korea Society of Inherited Metabolic Disease (대한유전성대사질환학회)
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Iatrogenic Hemocromatosis Case in Propionic Acidemia

프로피온산 혈증 환아에서 경험한 의원성 헤모크로마토시스 I례

  • Kim, Sook Za (Korea Genetics Research Center/KSZ Children's Hospital) ;
  • Jeon, Young Mi (Korea Genetics Research Center/KSZ Children's Hospital) ;
  • Song, Woong Ju (Korea Genetics Research Center/KSZ Children's Hospital)
  • 김숙자 (한국유전학연구소/김숙자소아청소년병원) ;
  • 송웅주 (한국유전학연구소/김숙자소아청소년병원) ;
  • 전영미 (한국유전학연구소/김숙자소아청소년병원)
  • Published : 2013.06.30
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Abstract

Propionic acidemia is an inherited organic acid metabolic disorder. During chronic recurrent metabolic crisis, multiple blood transfusions can cause secondary hemochromatosis. We report a patient with propionic acidemia who had iron overload that resulted in liver dysfunction, cardiomyopathy and diabetes. When multiple blood transfusions are unavoidable, use of chelating agents for iron can prevent complications such as diabetes and hemochromatosis.

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