All issues > Volume 39(8); 1996

Case Report

J Korean Pediatr Soc. 1996;39(8):1146-1150. Published online August 15, 1996.

A Case Report of Autoimmune Hepatitis Associated with Choledochal Cyst and Pancreatitis

Kyung Ran KR Park¹, Sun Young SY Lee¹, Soon Young SY Kim¹, Hyoung Shim HS Chang¹, Nam Su NS Back¹, Chong Jai CJ Kim², Joong Gon JG Kim¹, Jeong Kee JK Seo¹

¹Department of Pediatrics, College of medicine, Seoul National University, Seoul, Korea
²Department of Pathology, College of medicine, Seoul National University, Seoul, Korea

Abstract

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.

Keywords :Autoimmune hepatitis, Choledochal cyst, Pancreatitis