Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Giant Coronary and Axillary Aneurysms in an Infant with Kawasaki Disease Associated with Thrombocytopenia

혈소판 감소증이 지속된 영아 가와사끼병 환아에서 발생한 거대관상동맥류와 액와동맥류

  • Seo, Sei Young (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Oh, Jin Hee (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Kim, Jong-Hyun (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Han, Ji-Whan (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) ;
  • Koh, Dae Kyun (Department of Pediatrics, College of Medicine, The Catholic University of Korea)
  • 서세영 (가톨릭대학교 의과대학 소아과학교실) ;
  • 오진희 (가톨릭대학교 의과대학 소아과학교실) ;
  • 김종현 (가톨릭대학교 의과대학 소아과학교실) ;
  • 한지환 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이경일 (가톨릭대학교 의과대학 소아과학교실) ;
  • 고대균 (가톨릭대학교 의과대학 소아과학교실)
  • Received : 2005.04.18
  • Accepted : 2005.06.01
  • Published : 2005.08.15
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Abstract

Kawasaki disease (KD) is a leading cause of acquired heart disease in children. Yet the etiology of KD is still unknown and diagnosis depends on the exclusion of other diseases and the clinical manifestations meeting the defined criteria. Young infants frequently show atypical clinical courses and are frequently complicated with coronary aneurysms. Some cases show thrombocytopenia, which is known as one of the risk factors for complications with coronary aneurysms. So, a high index of suspicion is the most important factor for the diagnosis of KD in very young infants or adolescents whose clinical courses are equivocal. We report herein on a case of KD in an 80-day-old female infant with fever and seizure with bloody stool; laboratory findings were those of sepsis with disseminated intravascular coagulopathy. In spite of aggressive treatments, fever and thrombocytopenia persisted for two weeks and huge coronary aneurysms developed at the third week in all three major coronary arteries; the diameter of the right one was as large as the aortic annulus. Three months later, huge pulsatile masses developed in both axillas; these were found to be huge axillary aneurysms defined very clearly on multi-detector CT scan. She has been under follow up with antiplatelets and anticoagulation therapy with poor regression of the aneurysms.

가와사끼병은 소아 연령에서 발병하는 급성 전신성 혈관염으로 현재 소아 후천성 심질환의 가장 흔한 원인이다. 아직 원인 불명이고 진단도 여전히 임상 증상에 의존하고 있는데, 최근 비호발 연령의 환자 및 비전형적 가와사끼병의 증례 보고와 이들에서 빈발하는 관상 동맥 합병증의 보고가 증가되고 있다. 관상동맥 합병증의 위험 인자 중 하나인 지속적 발열과 혈소판 감소증은 매우 어린 영아에서 간혹 초기 감별 진단을 어렵게 만든다. 저자들은 패혈증으로 전원된 3개월 여아에서 초기에 정맥글로불린과 스테로이드 등의 약제로 치료하였으나 빈혈, 혈소판 감소증과 발열이 지속되다 거대 관상 동맥류와 액와 동맥류를 합병한 1례를 보고하는 바이다.

Keywords

References

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