1 research outputs found
Autoimmune Hepatitis in Children: Experiences in a Tertiary Center
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