Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Background and aim: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disorder of unknown etiology and different clinical presentations. The aim of our study is to determine the clinical, biochemical and histopathological characteristics, types of immunosuppressive therapy used and treatment outcomes of children with AIH. Methods: This observational study included all children presented to the Pediatric Hepatology Unit at Cairo University Pediatric Hospital, Egypt from January 2009 to December 2016 with established diagnosis of AIH. All data were retrieved from the patients' files. Results: Thirty-four AIH children were included. The mean ± SD age at disease onset was 7.2 ± 2.8 years. Sixty-one percent presented with a picture of chronic liver disease, meanwhile 27.8% presented with acute hepatitic illness and 11.1% with acute liver failure. Jaundice was the main presenting symptom in 79.4%. Transaminases were elevated in all patients on initial presentation. Synthetic dysfunction was present in half the patients.Twenty-six patients (76.4%) were type 1 AIH, while seven (20.5%) were type 2 AIH and one patient (2.9%) was autoantibody negative AIH. Liver biopsy findings included distorted hepatic architecture in 83.3% of the patients and piecemeal necrosis in 79%. The initial treatment was oral prednisolone in all patients. Azathioprine was added to prednisolone in the treatment of 88.2% of the patients. Biochemical remission was achieved in 79.8%

Issued also as CD