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Saturday, October 24, 2009

What is Autoimmune Enteropathy?

The diagnosis they have been tagging Hannah's lab's is autoimmune enteropathy. What is that? here is an article I found...

Advances in the understanding and management of autoimmune enteropathy


There have been real advances in understanding the pathogenesis of autoimmune enteropathy, including determination of specific autoantigens. The most important clinical association is with IPEX (X-linked immune polyendocrinopathy) syndrome, which is due to mutation in the Foxp3 transcription factor, a molecule critical in generation of regulatory T cells. Association of non-IPEX autoimmune enteropathy with T cell activation defects further point to impairment of T cell tolerance mechanisms as the primary underlying cause of autoimmune enteropathy. This also explains the frequency of other autoimmune manifestations. The centrality of T cell responses in autoimmune enteropathy, rather than B cell autoantibody production, as previously thought, is further suggested by the finding of late-onset gut autoimmunity in APS-1 (autoimmune polyglandular syndrome-1), a condition where negative selection of T cells within the thymus is disrupted due to mutation in the Aire (autoimmune regulator) gene. However, this form of autoimmune enteropathy is milder because the immune target is within entero-endocrine cells rather than absorptive enterocytes. There have also been important changes in management, with introduction of more potent immunoregulatory therapy, and more recently the use of bone marrow transplantation, which may theoretically offer hope of a cure in what frequently used to be a fatal condition.

Keywords: Autoimmune enteropathy, IPEX syndrome, Foxp3, APS-1, Aire, Bone marrow transplantation

Warwick Medical School, Clinical Sciences Research Institute, Clifford Bridge Road, Coventry CV2 2DX, UK

PII: S0957-5839(06)00085-6


© 2006 Elsevier Ltd. All rights reserved.

Thursday, October 15, 2009

The Climb

Thursday, October 08, 2009

Cincinnati has a treatment plan...

After Spending almost the whole month of september in the hospital, I am overwhelmed with the news that Cincinnati has finally given word of a plan! a little nervous and nauseated, but relieved at the same time.
Monday Hannah will start taking Tacrolimus a drug that comes with some serious side effects, and strict precautions. Alot like getting chemo. Hannah's health is so fragile right now... I think now more than ever I will need all the help and support I can get. This is going to make her very sick.
Why are they starting this med? Tacrolimus is an anti rejection drug given to people who have had an organ transplant. Basically they think Hannah's body is rejecting her own organs.
Omaha is cordinating all her care through Cincinnati's guidance and then we will go back to Cincinnati in 6 months.
I agree with this plan, I think it is a long time due to have a plan! I pray this plan does something good for Hannah. But I am also very intimidated. And it takes alot to do that to me! But it time to buck up and take this head on!