Pediatric Gastroenterology, Hepatology & Nutrition
- Volume 7 Issue 2
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- Pages.186-196
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- 2004
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- 2234-8646(pISSN)
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- 2234-8840(eISSN)
Clinical Characteristics, Histology and Prognosis of Autoimmune Hepatitis in Korean Children
소아 자가면역성 간염: 임상적 특성, 조직 소견 및 예후
- Chung, Dae-Lim (Department of Pediatrics, Seoul National University College of Medicine) ;
- Seo, Jeong Kee (Department of Pediatrics, Seoul National University College of Medicine) ;
- Yang, Hye Ran (Department of Pediatrics, Seoul National University College of Medicine) ;
- Ko, Jae Sung (Department of Pediatrics, Seoul National University College of Medicine) ;
- Park, Sung Hye (Department of Pathology, Seoul National University College of Medicine)
- 정대림 (서울대학교 의과대학 소아과학교실) ;
- 서정기 (서울대학교 의과대학 소아과학교실) ;
- 양혜란 (서울대학교 의과대학 소아과학교실) ;
- 고재성 (서울대학교 의과대학 소아과학교실) ;
- 박성혜 (서울대학교 의과대학 병리학교실)
- Received : 2004.08.19
- Accepted : 2004.09.20
- Published : 2004.09.01
Abstract
Purpose: Autoimmune hepatitis is a chronic inflammatory liver disease with unknown cause that is characterized by liver histology, circulating autoantibodies and increased levels of immunoglobulin G. Only sporadic reports are available on autoimmune hepatitis in children. The aim of this study was to evaluate the clinical, biochemical, and histological features, and the long-term outcome of autoimmune hepatitis in Korean children. Methods: We reviewed the medical records of 14 children diagnosed as having autoimmune hepatitis at Seoul National University Children's Hospital from 1990 to 2004, and analyzed clinical, biochemical, and histological features, and clinical outcomes. Results: Mean age at diagnosis was 9 years and 11 of the 14 children were female. Six children presented with acute hepatitis-like manifestations. Jaundice and fatigue were the most common symptoms. Other autoimmune diseases accompanied in 6 children. Anti-nuclear antibody was detected in 13 patients and anti-smooth muscle antibody was positive in 8. All 14 patients were type 1 autoimmune hepatitis. The main histologic findings were interface hepatitis, rosette formation, and cirrhosis. Clinical and biochemical features were improved in six patients treated with ursodeoxycholic acid. Eight patients were treated with corticosteroid alone or in combination with azathioprine and five of them are in biochemical remission. Conclusion: Autoimmune hepatitis is an inflammatory liver disease, which has a favorable long-term outcome if it is diagnosed and treated promptly. Therefore, autoimmune hepatitis should be suspected in children with chronic hepatitis of unknown etiology, especially in female patients who show hypergammaglobulinemia or some clinical features of autoimmune disease.
목적: 자가면역성 간염은 원인 불명의 진행성 염증성 간질환으로 혈중 자가항체의 출현과 고감마글로불린혈증, 그리고 조직학적으로 문맥주위 염증을 특징으로 한다. 소아에서는 산발적인 증례 보고가 있었으나 국내에서는 아직까지 체계적인 연구가 없었다. 이에 저자들은 소아에서 자가면역성 간염의 임상적, 조직학적 소견과 치료 경과 및 예후에 대해 알아보고자 본 연구를 시행하였다. 방법: 1990년부터 2004년까지 서울대학교 어린이병원에서 자가면역성 간염으로 진단되었던 14명의 환자들의 임상적 특징, 혈액 검사 소견 및 간 조직 검사 소견을 분석하였다. 또한 이들 환자들에게 시행된 치료 약제 및 치료에의 반응, 그리고 재발 여부 등 치료 경과를 조사하였다. 결과: 대상 환자 14명의 진단시 연령은 평균