| A Case Report of Autoimmune Hepatitis Associated with Choledochal Cyst and Pancreatitis |
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Kyung Ran Park1, Sun Young Lee1, Soon Young Kim1, Hyoung Shim Chang1, Nam Su Back1, Chong Jai Kim2, Joong Gon Kim1, Jeong Kee Seo1 |
1Department of Pediatrics, College of medicine, Seoul National University, Seoul, Korea
2Department of Pathology, College of medicine, Seoul National University, Seoul, Korea |
| 총담관낭과 췌장염을 동반한 자가면역성 간염 1례 |
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박경란1, 이선영1, 김순영1, 장형심1, 백남선1, 김종재2, 김중곤1, 서정기1 |
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학교실 |
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| Abstract |
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Autoimmune hepatitis in children is a rare and severe inflammatory disease of unknown
etiology, and progress to cirrohosis and liver failure, generally is responsive to immunosuppressive
therapy. It is more prevalent in women than men, and characterized by the presence of
circulating autoantibodies, a high serum globulin. Extrahepatic manifestations such as thyroiditis,
ulcerative colitis, glomerulonephritis and autoimmune hemolytic anemia, are associated.
We report, to our knowledge, the first case of autoimmune hepatitis in conjunction with
choledochal cyst and pancreatitis in 11-year-old female patient.
At the time of diagnosis, she suffered from acute upper abdominal pain, jaundice, and pallor.
Laboratory findings showed Cooms positive hemolytic anemia, hypergammaglobulinemia,
hyperbilirubinemia, and high serum transaminases. Antinuclear antibody was of homogeneous
type. In liver biopsy, cellular infiltrates largely lymphocytes were noted. Treatment with
corticosteroids induced clinical, biochemical remission, but subsequent withdrawal leaded to
relapse. Incidentally choledochal cyst were found and then acute pancreatitis developed. After
management for acute pancreatitis, surgical resection of cyst with hepatojejunostomy was
performed. |
| Key Words:
Autoimmune hepatitis, Choledochal cyst, Pancreatitis |
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