Autoimmune hepatitis in children is a rare and severe inflammatory disease of unknown etiology, and progress to cirrhosis 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.
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