Two Pediatric Cases of Type 1 Autoimmune Hepatitis with Normal Immunoglobulin G Levels Presenting as Fulminant Hepatitis.
- Author:
Ji Young HONG
1
;
Sun Hwan BAE
;
Wan Sup KIM
Author Information
1. Department of Pediatrics, School of Medicine, Eulji University, Seoul, Korea. bsh3209@eulji.or.kr
- Publication Type:Case Report
- Keywords:
Autoimmune hepatitis;
Children;
Normal immunoglobulin G;
Fulminant hepatitis
- MeSH:
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
- From:Journal of the Korean Pediatric Society
2002;45(10):1302-1307
- CountryRepublic of Korea
- Language:Korean
-
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.
