Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis

궤양성 대장염에 동반된 자가면역성 간염-원발성 경화성 담관염의 중복 증후군 1예

  • Hong, Jeana (Departments of Pediatrics, Seoul National University College of Medicine) ;
  • Song, Mi Kyoung (Departments of Pediatrics, Seoul National University College of Medicine) ;
  • Ko, Jae Sung (Departments of Pediatrics, Seoul National University College of Medicine) ;
  • Kang, Gyeong Hoon (Departments of Pathology, Seoul National University College of Medicine) ;
  • Kim, Woo Sun (Departments of Radiology, Seoul National University College of Medicine) ;
  • Seo, Jeong Kee (Departments of Pediatrics, Seoul National University College of Medicine)
  • 홍지나 (서울대학교 의과대학 소아과학교실) ;
  • 송미경 (서울대학교 의과대학 소아과학교실) ;
  • 고재성 (서울대학교 의과대학 소아과학교실) ;
  • 강경훈 (서울대학교 의과대학 병리학교실) ;
  • 김우선 (서울대학교 의과대학 영상의학과교실) ;
  • 서정기 (서울대학교 의과대학 소아과학교실)
  • Received : 2008.09.05
  • Accepted : 2008.11.05
  • Published : 2009.04.15
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Abstract

Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of AIH and PSC have been reported, especially in children. Moreover, PSC is known to be the most frequent liver disorder associated with inflammatory bowel diseases such as ulcerative colitis. We report one case of AIH-PSC overlap syndrome in a child who was diagnosed as having ulcerative colitis.

자가면역성 간염, 원발성 담관성 간경화증, 원발성 경화성 담관염은 대표적인 자가 면역성 간질환이다. 이 각 질환의 임상적, 조직학적, 혈청학적 특징이 혼재되어 어느 한 질환으로 진단하기 어려운 경우를 중복증후군(overlap syndrome)이라 하고, 최근 소아에게서도 드물게 보고되고 있다. 한편 경화성 담관염은, 염증성 장질환에서 가장 흔히 동반되는 간담도계질환으로 알려져 있다. 저자들은 궤양성 대장염으로 진단된 환아에게서 간조직 검사, 혈청 검사, 방사선 검사 등을 통해 자가면역성 간염-원발성 경화성 담관염의 중복증후군을 진단하였기에 보고하는 바이다. 염증성 장질환 환아에게서 자가 면역성 간질환 또는 중복증후군 동반여부를 염두에 두어 임상적, 조직학적, 혈청학적 검사를 통해 적절한 진단과 치료가 필요하겠다.

Keywords

References

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