Autoimmune Hepatitis in Children: Experiences in a Tertiary Center

AUTHORS

seyedMohsen Dehghani 1 , * , Mahmood Haghighat 2 , Mohammad-Hadi Imanieh 3 , Nasser Honar 4 , Amir-Masoud Negarestani 5 , Abdorrasoul Malekpour 6 , Mehran Hakimzadeh 7 , Naghi Dara 8

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How to Cite: Dehghani S, Haghighat M, Imanieh M, Honar N, Negarestani A, et al. Autoimmune Hepatitis in Children: Experiences in a Tertiary Center, Iran J Pediatr. 2013 ; 23(3):302-308.

ARTICLE INFORMATION

Iranian Journal of Pediatrics: 23 (3); 302-308
Published Online: May 02, 2013
Article Type: Research Article
Received: December 28, 2012
Accepted: March 29, 2013

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Abstract

Objective: Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology that occurs in the children of all ages. The present study aimed to evaluate the clinical and paraclinical presentations, including pattern of autoantibodies, response to treatment, mortality, and liver transplantation outcome in the Iranian children with AIH.
Methods: The medical records of 87 children (56 girls and 31 boy) diagnosed with AIH between 2001 and 2010 were retrospectively analyzed for clinical and paraclinical profiles and also treatment outcome.
Findings: The mean age of the patients was 10.1±4.5 years (64.4% females). The most common clinical findings were jaundice (70.1%), splenomegaly (67.8%), and hepatomegaly (51.7%). Antinuclear, anti-smooth muscle, and anti LKM antibodies were positive in 14/62, 22/53 and 6/40 patients, respectively (36 patients had type 1 AIH, 6 patients had type 2 AIH, 26 patients were seronegative, and autoantibodies were not available in 19 cases). The most common histological finding in the liver biopsies was chronic hepatitis with interface activity that was seen in 65 (74.7%) patients. The complete response was seen in 52 (59.8%) patients and 24 (27.6%) patients underwent liver transplantation. One-year and five-year survival rates were 87.5% and 80% in the transplanted patients.
Conclusion: AIH should be kept in mind in the differential diagnosis of both acute and chronic liver diseases in the children and treatment with combination of corticosteroids and azathioprine is a good treatment option. In the patients with end stage liver cirrhosis that did not respond to medical therapy, liver transplantation is the treatment of choice.

 

Keywords

Autoimmune Hepatitis Autoantibody Liver Function Tests Liver Transplantation

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