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severe combined immunodeficiency
(redirected from X-linked severe combined immunodeficiency)

   Also found in: Medical, Acronyms, Wikipedia 0.01 sec.
severe combined immunodeficiency
n. Abbr. SCID
A usually fatal congenital disorder of the immune system in which the body is unable to produce enough B cells and T cells to resist infection.
ThesaurusLegend:  Synonyms Related Words Antonyms
Noun1.severe combined immunodeficiency - 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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Children with X-linked severe combined immunodeficiency, a rare condition that affects only males and is sometimes referred to as "baby in the bubble syndrome", die within their first year without treatment.
A US government health panel has recommended that 2 US gene therapy trials involving children with X-linked severe combined immunodeficiency (X-SCID) be allowed to resume but only on a limited basis.
FDA initiated the action after learning that two children who received a retrovirus in a French gene therapy study for X-linked Severe Combined Immunodeficiency (SCID), commonly known as "bubble baby syndrome" were diagnosed with leukemia.
 
 
 
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