Two Pediatric Cases of Type 1 Autoimmune Hepatitis with Normal Immunoglobulin G Levels Presenting as Fulminant Hepatitis
Journal of the Korean Pediatric Society
; : 1302-1307, 2002.
Article
in Ko
| WPRIM
| ID: wpr-77169
Responsible library:
WPRO
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Autoantibodies
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Splenectomy
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Ursodeoxycholic Acid
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Biopsy
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Immunoglobulin G
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Immunoglobulins
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Prednisolone
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Cholecystectomy
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Gallstones
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Cholangitis
Limits:
Adolescent
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Child
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Female
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Humans
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Male
Language:
Ko
Journal:
Journal of the Korean Pediatric Society
Year:
2002
Type:
Article