Korean J Pediatr Gastroenterol Nutr.  2004 Sep;7(2):186-196.

Clinical Characteristics, Histology and Prognosis of Autoimmune Hepatitis in Korean Children

• Chung DL
• Seo JK
• Yang HR
• Ko JS
• Park SH

Abstract

PURPOSE: Autoimmune hepatitis is a chronic inflammatory liver disease with unknown cause that is characterized by liver histology, circulating autoantibodies and increased levels of immunoglobulin G. Only sporadic reports are available on autoimmune hepatitis in children. The aim of this study was to evaluate the clinical, biochemical, and histological features, and the long-term outcome of autoimmune hepatitis in Korean children.
METHODS
We reviewed the medical records of 14 children diagnosed as having autoimmune hepatitis at Seoul National University Children's Hospital from 1990 to 2004, and analyzed clinical, biochemical, and histological features, and clinical outcomes.
RESULTS
Mean age at diagnosis was 9 years and 11 of the 14 children were female. Six children presented with acute hepatitis-like manifestations. Jaundice and fatigue were the most common symptoms. Other autoimmune diseases accompanied in 6 children. Anti-nuclear antibody was detected in 13 patients and anti-smooth muscle antibody was positive in 8. All 14 patients were type 1 autoimmune hepatitis. The main histologic findings were interface hepatitis, rosette formation, and cirrhosis. Clinical and biochemical features were improved in six patients treated with ursodeoxycholic acid. Eight patients were treated with corticosteroid alone or in combination with azathioprine and five of them are in biochemical remission.
CONCLUSION
Autoimmune hepatitis is an inflammatory liver disease, which has a favorable long-term outcome if it is diagnosed and treated promptly. Therefore, autoimmune hepatitis should be suspected in children with chronic hepatitis of unknown etiology, especially in female patients who show hypergammaglobulinemia or some clinical features of autoimmune disease.

Keyword

Autoimmune hepatitis; Clinical characteristics; Histology; Prognosis; Children

MeSH Terms

Autoantibodies
Autoimmune Diseases
Azathioprine
Child*
Diagnosis
Fatigue
Female
Fibrosis
Hepatitis
Hepatitis, Autoimmune*
Hepatitis, Chronic
Humans
Hypergammaglobulinemia
Immunoglobulin G
Jaundice
Liver
Liver Diseases
Medical Records
Prognosis*
Rosette Formation
Seoul
Ursodeoxycholic Acid
Autoantibodies
Azathioprine
Immunoglobulin G
Ursodeoxycholic Acid