Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Vincristine 투여로 호전된 미만성 신생아 혈관종증

Successful Management with Vincristine after Failure of Prednisolone Therapy for Diffuse Neonatal Hemangiomatosis

  • 이학성 (대구파티마병원 소아과) ;
  • 허순영 (대구파티마병원 소아과) ;
  • 김원덕 (대구파티마병원 소아과)
  • 투고 : 2005.06.23
  • 심사 : 2005.08.01
  • 발행 : 2005.09.15
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초록

혈관종은 영아에서 가장 흔한 양성 종양으로 이 중 15-30%는 다발성 혈관종을 보인다. 미만성 신생아 혈관종증은 피부와 다수의 내부 장기를 침범하는 질환으로 치료하지 않는 경우 치명적일 수 있기 때문에 신속하고 적극적인 치료가 요구된다. 치료는 부신피질 호르몬이 1차 선택 약제로 사용되며 이에 반응하지 않으면 인터페론, 절재술, 전색술, 방사선 치료 등을 이용할 수 있다. Interferon alfa-2a는 매우 효과적이나 강직성 양마비(spastic diplegia)와 같은 심각한 합병증이 보고되고 있다. 저자들은 출생 직후 호흡곤란과 전신의 피부 혈관종을 주소로 입원하여 Kasabach-Merritt 증후군을 동반한 미만성 신생아 혈관종증을 진단 받았던 1례에서 스테로이드 치료에 대해 혈소판 감소증 및 소모성 응고장애의 호전은 있었으나 혈관종의 수 및 크기의 호전이 없어 vincristine을 투여하여 치료에 성공하였기에 문헌 고찰과 함께 보고하는 바이다.

Hemangiomas are the most common benign tumors of infancy. Fifteen to 30% of these patients have multiple hemangiomas. Diffuse neonatal hemangiomatosis (DNH) is a disease that often has a fatal outcome if left untreated, and is characterized by multiple cutaneous and visceral hemangiomas. Corticosteroids are the commonly accepted first line treatment, but if no effect is seen, further treatment is required such as interferon, surgical excision, embolization and radiotherapy. Interferon is effective, but the neurologic sequela including spastic diplegia can be a complication. We experienced a case of DHN in a neonate. In this case, the baby presented with multiple cutaneous and visceral hemangiomas with Kasabach-Merritt syndrome (KMS) that included thrombocytopenia and consumptive coagulophthy. The baby was successfully treated with vincristine after the failure of steroid therapy.

키워드

참고문헌

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