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severe combined immunodeficiency disease

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Noun1.severe combined immunodeficiency disease - a congenital disease affecting T cells that can result from a mutation in any one of several different genes; children with it are susceptible to infectious disease; if untreated it is lethal within the first year or two of life
immunodeficiency - immunological disorder in which some part of the body's immune system is inadequate and resistance to infectious diseases is reduced
monogenic disease, monogenic disorder - an inherited disease controlled by a single pair of genes
ADA-SCID - SCID resulting from mutation of a gene that codes for adenosine deaminase
X-linked SCID, X-SCID - SCID in male children resulting from mutation of a gene that codes for a protein on the surface of T cells that allows them to develop a growth factor receptor


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The strategy should work to treat hemophilia, thalassemia and severe combined immunodeficiency disease (the so-called bubble boy disease), Daley said.
Three products are currently marketed which utilize Enzon's technology: PEG-INTRON marketed by Schering-Plough for hepatitis C, ONCASPAR(R) for Acute Lymphoblastic Leukemia (ALL), and ADAGEN(R) a treatment for a form of Severe Combined Immunodeficiency Disease (SCID), commonly known as the "Bubble Boy Disease.
Both fetuses were found to have a condition known as severe combined immunodeficiency disease (SCID), in which the fetal immune system does not develop enough of certain infection-fighting cells known as T-cells, according to Evans.
 
 
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