الفهرس 
Autoimmune HepatitisOnly 14 pages are availabe for public view
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Abstract
This study was aiming to investigate children with autoimmune liver disease to evaluate the appearance of HLA –DRB1 as a risk factor, its relation to treatment failure and assess if MELD score can predict this outcome through assessment of HLADRB1.
The study was conducted on a group of 25 patients with AIH attending the Hepatology Clinic of Children’s Hospital, Ain Shams University and Prof. Yassin Abd El-Ghaffar charity center for Liver Diseases and Research. They were 17 patients type I AIH (68%), 5 patients type II AIH (20%) and 3 patients (12%) were sero negative.
A historical cohort of 548 normal healthy people were subjected to HLA typing was included as a control group.
They were subjected to detailed history taking, Clinical examination and Investigation in the form of (CBC, ESR, Liver Function, auto antibodies, Gamma globulin, liver biopsy and HLADRB1 by SSOP).
Data was collected, tabulated and statistically analyzed.
The results of this study revealed that:
It was found 17 patients (68%) were females with AIH more predominant than male (32%).
The most common presenting symptoms were symptoms of cholestasis in the form of (jaundice, dark urine, clay stool and pruritis) in (76%) of patients followed with fever in (40%) of patients.
The most common sign was jaundice in 19 patients (76%) followed with hepatomegaly in 17 patients (68%) then Splenomegaly in 9 patients (36%) and finally ascites in 10 patients (40%).
20 patients (80%) of AIH patients presented with acute hepatitis while 5 patients (20%) presented with chronic hepatitis.
Six patients (24%) preceded with hepatitis A infection and they were the most severe cases as as 2 of them were presented with portal hypertension, 2 of them were presented with hepatic encephalopathy and 2 of them were partial responders to treatment.
γ globulin of the studied ranged from 2.02 to 5.9 gm/dL with a mean of 3.3 ± 1.8 gm/dL.
Antinuclear antibodies (ANA) were positive in (36%) of patients. Smooth muscle antibodies (SMA) were positive in (68%) of patients and Liver-kidney microsomal (LKM) antibody was positive in 5 patients (20%) of patients. Both ANA and ASM were positive in 9 patients (36%).
Liver biopsy was done for 12 (48%) patients. It was found that all of them had fibrosis while 4 (34%) of patients had cirrhosis, 7 patients (58.3%) had piecemeal necrosis, 11patients (91.7%) had inflammatory cells and7patients (58.3%) had interface hepatitis. For these 12 patients HAI- scoring –biopsy had been calculated for 8 patients, It was ranged from 4 to 12 with a mean of 8.5 ± 2.3.
In this study show that the most common allele found in the studied group was HLA-DRB1*13 as it was found in18 alleles (36%) then HLA-DRB1*04 in 9 alleles (18%), HLA-DRB1*03 in 7 alleles.
On comparison between cases and control groups as regards HLA-DRB1,there was highly statistically significant difference more in the studied cases than control group as regards HLA-DRB1*03 and HLA-DRB1*13, but there was no statistically significant difference between AIH patients and the control group as regards other alleles.