Document Details
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Document Type |
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Article In Journal |
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Document Title |
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Long-term outcome of autoimmune hepatitis in children Long-term outcome of autoimmune hepatitis in children |
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Subject |
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autoimmune hepatitis |
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Document Language |
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English |
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Abstract |
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Background and Aim: Autoimmune hepatitis (AIH) is a chronic disease of unknown etiology, which usually progresses to cirrhosis if not diagnosed and treated promptly. Data on long-term follow up in
children with AIH are scant. The aim of this study is to assess the long-term outcome of autoimmune hepatitis in children with respect to clinical and laboratory features at presentation. Methods: Data were extracted from the medical records of patients presenting over a 28-year period (1972–2000) to the Royal Children’s Hospital, Melbourne, Australia. Additional information was obtained by interviewing patients, and their current physicians. Of the 30 patients (22 females, mean age 9 years) identified, 18 had type I, three had type II, four had autoimmune–polyendocrinopathy syndrome type 1, one had infantile giant-cell hepatitis associated with Coomb’s-positive hemolytic anemia, and four were seronegative (antinuclear antibody (ANA), smooth muscle antibody (SMA) and liver–kidney microsomal antibody (LKM)). Results: Clinical features at presentation included hepatomegaly (86%), jaundice (66%) and splenomegaly (50%). Initial investigations revealed a median serum bilirubin level of 55 mmol/L (range 6–425), median aspartate aminotransferase level of 678 IU (range 70–2548), and abnormal clotting in 33% of patients. Liver biopsies were performed on all patients at presentation and 11 showed cirrhosis (36%). The mean follow-up period was 10.0 ± 7.8 years with 43% being followed for > 10 years. Only two patients died and one required transplantation. Fourteen (50%) patients continue to be on low dose prednisolone with azathioprine, two (7%) are on prednisolone alone, and six (21%) are on no therapy. When the cirrhotic and non-cirrhotic patients were compared, the albumin level at presentation was significantly lower in the cirrhotic group (P = 0.01). Of the patients who were cirrhotic at presentation,
six (54%) remain compensated with a mean follow-up period of 8 years. All 24 patients currently under follow up are engaged in age-appropriate activities including school, part- or full-time work.
Conclusion: Autoimmune hepatitis has a favorable long-term outcome with a transplant-free survival rate of 90% over a mean period of 10.0 ± 7.8 years (range: 0.5–23), and a normal or near normal lifestyle irrespective of presenting clinical, laboratory or histological features.. |
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ISSN |
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0954-691X |
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Journal Name |
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Journal of Gastroenterology and Hepatology |
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Volume |
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16 |
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Issue Number |
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11 |
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Publishing Year |
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1422 AH
2001 AD |
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Article Type |
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Article |
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Added Date |
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Thursday, November 18, 2010 |
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Researchers
| عمر سعادة | Saadah, Omar I | Investigator | Doctorate | saadaho@hotmail.com |
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