Diseases, Disorders information

Autoimmune disease in children. Well, it’s probably more common than we think. Typically we think of this as a disease affecting adolescent females but we have seen it in boys, we have seen it in little children, even as young as two or sometimes even less. Presentation, again, highly variable. They may turn up with chronic liver disease and all the characteristic usual associations; cirrhosis, portal hypertension. But where we sometimes see it, and it’s very bothersome when it occurs this way, as a cause of actual fulminant liver failure in sub-acute kind of presentation. Some of these little ones will need liver transplantation very rapidly.
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The diagnostic clue is that if you look at the proteins in the blood, the total serum protein is going to be high and the serum albumin is going to be low and the reason for that is that there is a hypergammaglobulinemia. So if you see a low serum albumin, a high total protein and you are beginning to think about chronic liver disease, it might make you think about autoimmune.
Then there is a whole array of diagnostic markers that I am not going to delineate for you; anti-smooth muscle, anti-mitochondrial antibodies, anti-liver-kidney microsomal. You can think about just about any antibody you want to and look for it as part of the way that you prove your diagnosis. Along, of course, with liver biopsy.
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The treatment, particularly if you are savvy and you make this diagnosis early, is immunosuppression. The mainstay is still steroids. You might add 6-MP, azathioprine or even some of the newer immunosuppressants to try and control the process. You have to remember that autoimmune diseases, no matter which one it is, are multi-system diseases so whether the presentation is in the liver or somewhere else, you should look at the joints, you look for funny rashes, you look for ulcerative colitis and Crohn’s disease, diabetes, thyroid problems. So don’t just stop at the liver. Make sure you have really carefully gone through all the other systems that could be involved.
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