Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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A Case of Alagille Syndrome with Atresia of the Hepatic Duct

간관 폐쇄증이 동반된 알라질 증후군 (Alagille Syndrome) 1예

  • Kim, Hyo-Sun (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Koh, Hong (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Chung, Ki-Sup (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Oh, Jung-Tak (Department of Surgery, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Park, Young-Nyun (Department of Pathology, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Kim, Myeung-Jun (Department of Radiology, Yonsei University College of Medicine, Severance Children's Hospital)
  • 김효선 (연세대학교 의과대학 소아과학교실) ;
  • 고홍 (연세대학교 의과대학 소아과학교실) ;
  • 정기섭 (연세대학교 의과대학 소아과학교실) ;
  • 오정탁 (연세대학교 의과대학 소아외과학교실) ;
  • 박영년 (연세대학교 의과대학 진단병리과학교실) ;
  • 김명준 (연세대학교 의과대학 영상의학과학교실)
  • Received : 2008.01.31
  • Accepted : 2008.02.29
  • Published : 2008.03.30
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Abstract

A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.

담도 폐쇄증은 수술적 치료가 필요하며 알라질 증후군은 대증적 치료가 가능하기 때문에 이를 감별하는 것이 중요하다. 저자들은 신생아기에 선천성 심장질환과 함께 담즙 정체 소견이 있는 환아에게서 원인 질환을 진단하는 과정에서 수술적 담관조영술 통해 제2형 담도 폐쇄증인 간관 폐쇄를 동반한 알라질 증후군 1예를 경험하였기에 보고하는 바이다.

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