J Korean Pediatr Soc.  2002 Oct;45(10):1302-1307.

Two Pediatric Cases of Type 1 Autoimmune Hepatitis with Normal Immunoglobulin G Levels Presenting as Fulminant Hepatitis

• Hong JY
• Bae SH
• Kim WS

Abstract

Autoimmune hepatitis is a chronic necroinflammatory liver disorder of unknown cause associated with circulating autoantibodies and hypergammaglobulinemia. We report two pediatric cases of type 1 autoimmune hepatitis with normal serum immunoglobulin G levels who presented with fulminant hepatitis. The cases were associated with hereditary spherocytosis or atrioventricular septal defect. The first case was a 13-year-old boy with hereditary spherocytosis and gallstones. Acute cholecystitis and cholangitis developed during his clinical course and cholecystectomy, splenectomy and liver wedge biopsy were performed. The second case was an 8-year-old girl with repaired atrioventricular septal defect. In both cases, antinuclear antibody tests were positive and immunoglobulin G levels were normal. Interface hepatitis with moderate lobular inflammation were noted on their liver histology. They responded to the treatment of prednisolone and ursodeoxycholic acid.

Keyword

Autoimmune hepatitis; Children; Normal immunoglobulin G; Fulminant hepatitis

MeSH Terms

Adolescent
Antibodies, Antinuclear
Autoantibodies
Biopsy
Child
Cholangitis
Cholecystectomy
Cholecystitis, Acute
Female
Gallstones
Hepatitis*
Hepatitis, Autoimmune*
Humans
Hypergammaglobulinemia
Immunoglobulin G*
Immunoglobulins*
Inflammation
Liver
Male
Prednisolone
Splenectomy
Ursodeoxycholic Acid
Antibodies, Antinuclear
Autoantibodies
Immunoglobulin G
Immunoglobulins
Prednisolone
Ursodeoxycholic Acid