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Omenn's Disease

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Britnee Thompson

on 14 September 2012

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Transcript of Omenn's Disease

By Britnee Scutter Omenn's Syndrome Omenn’s syndrome is a rare autosomal recessive form of severe combined immunodeficiency.
Omenn’s syndrome is a result of cells in the immune system cancelling each other out and invading each other. In some patients these cells can also multiply to such extent that an invasion of the immune system occurs.
As a result the immune system shuts down, leaving the individual susceptible to disease and infection with no chance of immunity.
Omenn’s syndrome is fatal if untreated. Early recognition of this condition is important for genetic research and early treatment.
There is no known cure for Omenn's syndrome. Although being treated with bone marrow (haematopoietic stem cell) transplantation will dramatically prevent further developments of the disease. There are antibiotic and medication prescriptions that are available for diarrhoea and other symptoms of Omenn's Disease, though in some cases the antibiotics prove to be ineffective as T- cells continue to cancel out the antibodies. Treatment . Symptoms usually include...
shedding the outer layers of skin
chronic diarrhoea
widespread reddening of the skin
simultaneous enlargement of both the liver and the spleen
elevation of the white blood cell count
swelling of one or more lymph nodes
Persistent bacterial infections Symptoms Individuals with Omenn’s syndrome have a high level of circulating T cells (these play a vital role in cell immunity) in the blood and in the tissues of the skin, the intestine, the spleen, and the liver.
The thymus (important organ of the immune system), however, is almost completely rid of T cells.
These T cells in individuals with Omenn's syndrome exhibit a narrow T-cell antigen receptor, causing rashes and diarrhoea.
T cells in individuals with Omenn's syndrome respond poorly to antigens and to allogeneic cells which also explains why individuals are susceptible to opportunistic infections. Individuals with Omenn's syndrome usually lack circulating B cells which functions both as an antigen-presenting cell and as an effector cell in the humeral immune response.
This results in severe or decreased levels of circulating IgG (the most abundant antibody found in the circulation) in the body Omenn's Syndrome and the Effect on Cells in the Body Bone marrow transplantation is a relatively new medical procedure being used to treat diseases once thought incurable. Since its first successful use in 1968.
It has been used to treat patients diagnosed with leukemia, aplastic anemia, lymphomas such as Hodgkin's disease, multiple myeloma, immune deficiency disorders and some solid tumors such as breast and ovarian cancer.
70 percent of those needing marrow using donor marrow are unable to have one because a suitable bone marrow donor cannot be found. Bone Marrow Tranplantation Diffuse exfoliative
with hair loss.

Erythroderma in a baby
Omenn’s syndrome.

Erythroderma or"Exfoliative dermatitis," "Dermatitis exfoliativa,"
and "Red man syndrome" is an inflammatory skin
disease with erythema and scaling
that affects nearly the entire surface or the individuals skin,
usually 90% Erythroderma Skin and eye telangiectasias in girl
ataxia telangiectasia Normal compared to Omenn's Syndrome Murine models of Omenn syndrome
Serre-Yu Wong, David B. Roth
Published in Volume 117, Issue 5
J Clin Invest. 2007; 117(5):1213–1216
This article Copyright © 2007, The American
Society for Clinical Investigation Ataxia-telangiectasia is a rare childhood disease that affects the brain and other parts of the body.

Ataxia refers to uncoordinated movements, such as walking. Telangiectasias are enlarged blood vessels (capillaries) just below the surface of the skin. Telangiectasias appear as tiny, red, spider-like veins.

Ataxia-telangiectasia http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002369/
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