J Korean Pediatr Soc.  1996 Aug;39(8):1146-1150.

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

• Park KR
• Lee SY
• Kim SY
• Chang HS
• Back NS
• Kim CJ
• Kim JG
• Seo JK

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.

Keyword

Autoimmune hepatitis; Choledochal cyst; Pancreatitis

MeSH Terms

Abdominal Pain
Adrenal Cortex Hormones
Anemia, Hemolytic
Anemia, Hemolytic, Autoimmune
Antibodies, Antinuclear
Autoantibodies
Biopsy
Child
Choledochal Cyst*
Colitis, Ulcerative
Diagnosis
Female
Glomerulonephritis
Hepatitis, Autoimmune*
Humans
Hyperbilirubinemia
Hypergammaglobulinemia
Jaundice
Liver
Liver Failure
Lymphocytes
Male
Pallor
Pancreatitis*
Recurrence
Thyroid Gland
Thyroiditis
Transaminases
Adrenal Cortex Hormones
Antibodies, Antinuclear
Autoantibodies
Transaminases