الفهرس 
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Abstract
Hypergammaglobulinemia is frequently observed in patients with liver diseases of different causes. On the other hand, elevated levels of serum IgG are the best diagnostic marker for AIH. Absence of viral markers and negative screening for genetic disorders represent diagnostic difficulties that may lead to confusion with AIH, especially autoantibody-negative AIH (about 20% of patients with AIH). The problem arising in diagnosis makes the decision for clinical management difficult as corticosteroids, which constitute the treatment of choice in AIH are usually contraindicated in some disorders as they can induce exacerbation of liver disease such as cases of chronic viral hepatitis.
This study aimed to characterize the hypergammaglobulinemia in the different liver diseases in pediatrics. This study was carried out on 186 individuals (161 patients and 25 apparently normal individual). The 161 patients were classified as; HCV (34), WD (27), AIH (21), HAV (20), cholestasis (19), metabolic (15), CHF (12) and a group of heterogeneous etiologies ”Others group” (13). Patients and controls were age and sex matched.
The gained results showed that:
• he-globulin level in some of the studied groups group was significantly higher than that in control group.
• There was a significant positive correlation between -globulin level and (age, P.T and stage of fibrosis), while there was a significant negative correlation between -globulin level and (Alb., P.C%, Hb, WBCs and PLT).
• None of the diseased groups had positive ASMA and ANA at titer more than 1/20 except AIH group.
• There was statistical significant difference in -globulin levels of the diseased groups according to their different ASMA titers (p <0.01**), where the highest -globulin levels (5.06 ± 1.16) were in patients with ASMA titer 1/40 or more and the least levels were in patients with negative ASMA (less 1/20).
• There was statistical significant difference in -globulins levels of the diseased groups according their different ANA titers (p <0.05), where the highest -globulins levels (4.8 ± 0.81) were in patients with ANA titer 1/40 or more and the least levels (2.2 ± 1.11) were in patients with negative ANA (less 1/20).
• Nineteen percent of AIH group were of seronegative type (negative for routinely performed autoantibodies in our center; SMA, ANA, AMA, LKM-1, APCA), all were females and their mean age was 8.25 ± 2.98 years.
• The current study showed that the-globulin level tended to be higher in patients with more autoantibody combinations in type I AIH.
• The results of this study revealed that there was no statistical significant difference in -globulins levels in AIH group according their different ASMA titers p >0.05, yet it was still higher in those with higher ASMA and ANA titers.
• AIH patients with higher -globulin level (> 2 times ULN) had more advanced disease than those with -globulins level (< 2 times ULN), where age, PT and varices grade were significantly higher in patients with the higher -globulin level.
• There was hypergammaglobulinemia in Wilson group with mean level of -globulin was 2.3 g/dl ± 1. This hypergammaglobulinemia was next in extent to that in AIH group and there was no statistically significant difference in -globulin level between Wilson group with chronic liver disease presentation and those with neurological presentation.
• There was positive correlation between -globulins level and liver span and AST in WD group. Lower albumin level, lower platelets level and more prolonged PT were associated with higher -globulins level in WD group.
• There was hypergammaglobulinemia in HCV group with mean level of 1.95 ± 0.86. The largest proportion of patients was HCV (21%) with male predominance. This male predominance may be due to the higher risk factor for HCV transmission in Egypt (male circumcision).
• Older age, higher liver and spleen span, more grade of varices, higher bilirubin level, higher fibrosis scores, prolonged PT, lower Hb, lower WBCs, lower PLT were associated with higher -globulins level in HCV group.
• There was hypergammaglobulinemia in HAV group with mean level of-globulin was 1.8 ± 0.48. There was no significant difference in-globulin level between HAV group patients with fulminant presentation and those with non fulminant presentation, yet it was still higher in those with fulminant presentation.
• There was hypergammaglobulinemia in metabolic group but still lower than that of AIH group. Beta-globulins showed the highest level in the metabolic group with mean level of 1.1 ± 0.32.
• BA cases represented the majority of cholestasis group (36.36%).
• The mean-globulin level was not statistically significant different in the cholestasis group (1.35 gm/dl ± 0.66) compared to that of the control group (1.33 gm/dl ± 0.29). None of the cholestasis group had necroinflammatory activity in their liver biopsies and the majority (44.4%) had moderate degree of fibrosis.
• There were six cases in the ”Others” group (four cases were cryptogenic chronic hepatitis, one case was cryptogenic cirrhosis and another case was NAFLD) when applying the AIH score to these cases an assumed diagnosis to be AIH was considered.
• The cut-off point of -globulin level in diseased group Vs control group was 1.53 with specificity 76%, sensitivity 68.84%, NPV 27%, PPV 94.48% and AUROC was 76.6%.
• The Cut-off value of -globulins level in AIH group versus all was 2.83 g/dl with 85.7% sensitivity, 89.7% specificity, 51.42% PPV and 98% NPV. AUROC was 92.5% it was statistically significant p <0.05.
• The cut-off point of -globulin level in AIH group Vs Wilson group was 3.15 with specificity 74.1%, sensitivity 80.9%, NPV 83.3%, PPV 70.8% and AUROC was 82.7%.
In conclusions, although hypergammaglobulineamia and autoantibodies are still considered the key points in the diagnosis of AIH, they are being no more specific for it, as their occurrence, even with high level (>2 times ULN) for -globulins, were found in many different liver diseases especially WD implicating that an immunological basis was involved in the pathogenesis of these different liver diseases with variable levels. And the use of different -globulins cut-off points might be of help to differentiate, with acceptable significant performance, between different liver diseases in children.