Pediatric Infection and Vaccine

  • 제17권2호
  • /
  • Pages.177-181
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  • 2010
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  • 2384-1079(pISSN)
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  • 2384-1087(eISSN)
대한소아감염학회 (The Korean Society of Pediatric Infectious Diseases)
권호 표지

치료불응 가와사끼병의 임상양상을 보인 대식세포활성 증후군

Macrophage Activation Syndrome as the Extreme Form of Kawasaki Disease

  • 박현진 (가톨릭대학교 의과대학 소아과학교실) ;
  • 조윤정 (가톨릭대학교 의과대학 소아과학교실) ;
  • 배이영 (가톨릭대학교 의과대학 소아과학교실) ;
  • 최의윤 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이수영 (가톨릭대학교 의과대학 소아과학교실) ;
  • 정대철 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이경일 (가톨릭대학교 의과대학 소아과학교실) ;
  • 강진한 (가톨릭대학교 의과대학 소아과학교실)
  • Park, Hyoun-Jin (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Cho, Yoon-Jeong (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Bae, E-Young (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Choi, Ui-Yoon (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Soo-Young (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Jeong, Dae Chul (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Kyung Yil (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Kang, Jin Han (Department of Pediatrics, College of Medicine, The Catholic University of Korea)
  • 투고 : 2010.09.16
  • 심사 : 2010.10.18
  • 발행 : 2010.12.25
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초록

KD의 급성 열성기에 드물지만 MAS가 발생할 수 있다. 저자들은 19개월 여아의 KD 치료 중에 발생된 MAS를 경험하였다. 환아는 IVIG과 steroid 치료를 받았지만, 발열과 간비종대는 지속되었고 골수생검에서 혈구탐식구증이 관찰되었다. KD에 동반된 MAS를 인지하여 적기에 치료하기 위해서는 임상양상과 치료반응을 토대로, MAS의 가능성을 고려하는 것이 필요하다.

Few cases of macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) during the acute febrile phase of Kawasaki disease (KD) have been reported. We report on a case of a 19 month-old girl with MAS or reactive HLH during the course of KD. Despite immunoglobulin and steroid therapy, she showed persistent fever with hepatosplenomegaly and evidence of hemophagocytosis in the bone marrow. A high index of suspicion for clinical features associated with MAS is necessary for KD patients in order to provide appropriate treatment.

키워드

참고문헌

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