Introduction: autoimmune hepatitis (AIH) is one of the most common disorder resulting in end stage liver disease (ESLD) among children. Scarce data is available in this regard from Pakistan. In this study we have analyzed clinical and biochemical parameters of children suffering from this disorder.

Methods: it was a cross sectional study conducted in the Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation (SIUT) Karachi from January 2005 to June 2016. Patients aged up-to 18 years were included. AIH was diagnosed by using International Autoimmune hepatitis group (IAIHG) pre-treatment and simplified AIH score. Patients with both probable and definite score were included. Biochemical, serological, sonographic and demographics were recorded at the time of diagnosis, liver biopsy was also performed in most of the cases. Data was analyzed by using SPSS ver.20 and p-value of < 0.05 was considered significant.

Results: total 51 patients were enrolled most of them were females (68.6%). Mean age of presentation was around 10 years. Males had statistically significant earlier age of presentation, p-value = 0.007. The most common presenting complain was jaundice. Hypergammaglobulinemia is seen in almost all patients. Type I AIH was the most common entity while Type II AIH was statistically more significant in males p-value = 0.019. Raised GGT was also seen in male patient specifically in Type II AIH, p-value = 0.001.

Conclusion: autoimmune hepatitis predominantly affects female children who have late age of presentation as compare to the males. Type I AIH was the most common while Type II AIH was more common in males and they also had raised GGT.