Özet:

Objective: It was aimed to evaluate the demographic, clinical, laboratory and histopathological data at the time of diagnosis, treatment and response rates of pediatric patients with autoimmune hepatitis (AIH). Material and Methods: The data of patients diagnosed with AIH in our center in the last 10 years were analyzed retrospectively. Results: Of the 27 patients included in the study, 19 were female and 8 were male. The mean age at diagnosis was 10.89 ± 4.5 years, and 3 of them were Syrian ethnicity. Nine patients had extrahepatic diseases. The clinical presentation was most frequently acute hepatitis; 24 patients diagnosed with Type 1 AIH and 3 patients with seronegative AIH. The most common reason for admission was jaundice and fatigue. In physical examination, hepatomegaly and palmar erythema were observed most frequently. At the time of diagnosis, 23 patients' Ig G was more than 2 times higher and their ANA was positive. AMA in 2 patients and anti-SMA in 1 patient were positive. Liver biopsy revealed interface hepatitis in 23 patients and lymphoplasmacytic infiltration in 20 patients most frequently. Sclerosing cholangitis findings were observed in the magnetic resonance cholangiopancreatography of two patients. Complete clinical response was observed in 23 patients who were started on the first-line treatment regimen; There was partial response in 1 patient and no response in 3 patients. Treatment changes were made in seven patients due to treatment unresponsiveness and/or post-drug side effects. While corticosteroid monotherapy was preferred in two patients, tacrolimus was preferred in five patients. Conclusion: Due to the variability of the presentation type and duration of AIH in childhood, AIH should also be suspected in children presenting with liver disease findings. Studies with larger numbers of patients are needed on the diagnostic criteria, treatment regimens and second-line treatment agents of this rare disease.

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