Reye syndrome after acute enteritis during the neonatal period

신생아기 장염에 의하여 발병된 Reye 증후군

  • Bang, Jun Suck (Department of Pediatrics, Chuncheon Sacred Heart Hospital, College of Medicine, Hallym University) ;
  • Nam, Sang Jung (Department of Pediatrics, Chuncheon Sacred Heart Hospital, College of Medicine, Hallym University) ;
  • Lee, Kyung Hwa (Department of Pediatrics, Chuncheon Sacred Heart Hospital, College of Medicine, Hallym University) ;
  • Bae, Eun Joo (Department of Pediatrics, Chuncheon Sacred Heart Hospital, College of Medicine, Hallym University) ;
  • Park, Won-Il (Department of Pediatrics, Chuncheon Sacred Heart Hospital, College of Medicine, Hallym University) ;
  • Lee, Hyun Sook (Department of Pediatrics, Sanggye Paik Hospital, College of Medicine, Inje University) ;
  • Son, Bae Young (Department of Pediatrics, Seoul Samsung Medical Center, College of Medicine, Sungkyunkwan University) ;
  • Choi, Hwan Suck (Department of Pediatrics, Wonju Christian Hospital, College of Medicine, Yonsei University) ;
  • Lee, Hong Jin (Department of Pediatrics, Chuncheon Sacred Heart Hospital, College of Medicine, Hallym University)
  • 방준석 (한림대학교 의과대학 춘천성심병원 소아과) ;
  • 남상정 (한림대학교 의과대학 춘천성심병원 소아과) ;
  • 이경화 (한림대학교 의과대학 춘천성심병원 소아과) ;
  • 배은주 (한림대학교 의과대학 춘천성심병원 소아과) ;
  • 박원일 (한림대학교 의과대학 춘천성심병원 소아과) ;
  • 이현숙 (인제대학교 의과대학 상계백병원 소아과) ;
  • 손배영 (성균관대학교 의과대학 삼성서울병원 소아과) ;
  • 최환석 (연세대학교 의과대학 원주기독병원 소아과) ;
  • 이홍진 (한림대학교 의과대학 춘천성심병원 소아과)
  • Received : 2005.09.05
  • Accepted : 2005.10.28
  • Published : 2006.03.15

Abstract

Purpose : The prevalence of Reye syndrome has decreased since late 1980's. But we report that recently there were concentrative attacks of Reye syndrome after acute enteritis during the neonatal period. Methods : Clinical symptoms and laboratory results(quantitative organic acid analysis, routine chemistry, arterial blood gas analysis, serum ammonia) of seven patients admitted at the Samsung Medical Center, Sanggye Paik Hospital, Wonju Christian Hospital and Chuncheon Sacred Heart Hospital, referred from Jan. 2005 to Apr. 2005, were analysed retrospectively. The major clinical symptoms were derived from the patients' clinical records sended with urine samples and quantification of organic acids were done with gas chromatography and mass spectrometry. Results : The mean age of seven cases is 18 days and the major preceding symptoms were gastrointestinal symptoms(vomiting, diarrhea, refusal to feeding). The major clinical symptoms were clouded conciousness, repiratory difficulty, vomiting, seizures, and diarrhea. One patient died; that patient's serum ammonia was twenty times higher than normal. Conclusion : The seven patients were neonates. Reye syndrome has been known to be closely related with upper respiratory infections as a preceding disease and to internal use of aspirin, but in our study, the major preceding disease of the seven cases was gastrointestinal infection and none of these used aspirin.

목 적 : Reye 증후군의 유병률은 1980년 후반 이후 급격히 감소하였으나 최근 Reye 증후군이 신생아 시기에 급성 장염 후에 집중적으로 발병되어 보고하는 바이다. 방 법 : 2005년 1월부터 2005년 4월까지 4개월간 삼성서울병원, 상계백병원, 원주기독병원, 춘천성심병원 등 4개 종합병원에서 본원 임상유전학 연구실로 분석이 의뢰되었던 환자 7례의 임상 소견, 검사 소견(유기산 대사분석, 일반 화학 검사, 동맥혈 가스검사, 혈청 암모니아)을 분석하였다. 임상 소견은 환자들의 증상 및 기본 검사 소견을 검체와 함께 보내온 환자 정보지를 분석하여 얻었고 유기산 분석은 gas chromatography와 mass spectrometry를 이용하여 정량 분석하였다. 결 과 : 7명의 환자들의 평균 연령은 18일이었고 이들의 주요 선행 증상은 위장관 증상(구토, 설사, 음식 거부)이였으며 임상증상으로는 의식 장애, 호흡 곤란, 구토, 경련, 설사 등이었다. 사망은 1례에서 발생하였고 사망 환자의 암모니아 수치는 정상 암모니아 수치의 20배 이상 높은 수치를 나타냈다. 결 론 : 7례 모두 신생아 시기의 환자들이었다. Reye 증후군은 선행 질환으로 상기도 감염과 아스피린 복용이 밀접한 관련이 있는 것으로 알려져 있으나 본 연구에서는 주요 선행 질환이 위장관 감염이었고 아스피린 복용력은 없었다.

Keywords

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