الفهرس 
Introduction & Aim of the workOnly 14 pages are availabe for public view
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Abstract
In our study a large cohort of pediatric patients (73 cases) diagnosed with ALDs were included through the period of 2008 to 2013. The 73 cases were divided into 2 groups; 64 untreated cases and 9 treated cases that received treatment and underwent follow-up liver biopsy. The 64 untreated cases were further subdivided into; 24 cases of AIH, 8 cases of AIH/AIC overlap & 32 cases of possible AIH/overlap syndrome (not yet specified). All 64 untreated pediatric patients range in age from 2 to 17 years old with mean age 8.31 ± 3.44, including 35 female and 29 male. Female gender was prevalent in AIH and AIH-AIC compared to AIH/overlap patients (P = 0.017). Clinical presentation with jaundice, together with elevated serum levels of alkaline phosphatase and γ glutamyl transpeptidase were present in AIH/overlap and AIH-AIC compared to AIH patients. Elevated serum IgG levels and autoantibody positivety were present in patients with AIH but were also present in AIH-AIC and AIH/overlap patients. On histologic examination of liver biopsies, we evaluated the plasma cell density with its different grades (minimal, mild, moderate and marked) in different cases according to the number per high power field (HPF). It was noticed that the more density of plasma cell infiltrate, the higher the stage of fibrosis. Ductular changes and cholangiolitis were prevalent in cases of AIH/overlap and in AIH-AIC patients compared to AIH. Epithelioid granulomas were found in only 2 cases of AIH-AIC. We performed immunostains for IgG and IgM that were not compared with an external standard but were compared back-to-back on the same case and within the same portal tracts. We evaluated the number of the positive IgG and IgM plasma cell per HPF. The IgG/IgM ratio was then calculated for each case. A predominant pattern of IgG positive plasma cells/HPF was detected in AIH and in AIH/overlap group compaired to AIH-AIC group that showed predominance of IgM positive plasma cells. The ratio of the IgG/IgM plasma cells mean-counts was below 1 only in the AIH-AIC group. from a diagnostic standpoint, we believe that plasma cell-rich infiltrates seen in liver biopsies with IgG/IgM ratio ≤ 1 should alert the pathologist to the possibility of AIC, while an IgG/IgM ratio >1 renders a diagnosis of AIC significantly less likely. In the nine treated patients the degree of serum IgG elevation appeared to correlate with ALT and HAI, and thus IgG is not only a useful diagnostic marker for the detection of AIH, but is also a very helpful marker in the follow-up of AIH patients. Treatment should aim at normalizing both transaminase levels and IgG, as this indicates histological remission in almost all patients. When follow-up liver biopsies were examined comparing the number of plasma cell before and after therapy, we observed that patients with regression of fibrosis had a concomitant decrease in the number of plasma cell. In contrast, those without reduction of fibrosis, no decrease in the number of plasma cell was observed. Although it is only one case that showed progression of fibrosis, we observed increased number of plasma cell. Therefore, the number of plasma cell may serve as a prognostic marker in liver biopsy after therapy. This finding supports the idea that there is an intimate relationship between numbers of plasma cell and liver fibrosis in most cases of ALDs.