severe combined immunodeficiency

severe combined immunodeficiency
severe combined immunodeficiency n a rare congenital disorder of the immune system that is characterized by inability to produce a normal complement of antibodies and T cells and that results usu. in early death abbr. SCID called also severe combined immune deficiency, severe combined immunodeficiency disease see ADENOSINE DEAMINASE

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(SCID) a group of rare congenital disorders characterized by gross impairment of both humoral and cell-mediated immunity and absence of T lymphocytes; some forms are also characterized by a lack of B lymphocytes. In most cases all classes of immunoglobulins are nearly or completely absent, and there is marked lymphocytopenia. Persistent diarrhea, chronic mucocutaneous candidiasis, and failure to thrive occur in infancy. Blood transfusions can result in graft-versus-host disease and routine vaccinations in fatal infection. Unless immune function is restored by a histocompatible bone marrow or fetal tissue transplant or the patient is kept in gnotobiotic isolation, death from opportunistic infection usually occurs before the first birthday. In approximately 50 per cent of cases, the disorder is X-linked and due to a defect in the γ chain of the receptor for IL-2 and other interleukins. B lymphocyte numbers are usually normal. The remaining 50 per cent of cases are of autosomal recessive inheritance and have varying etiologies, including defects in activity of adenosine deaminase, purine-nucleoside phosphorylase, or Janus kinase 3 (JAK3). A rare type of the autosomal recessive form is called reticular dysgenesis.

Medical dictionary. 2011.

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  • Severe combined immunodeficiency — Infobox Disease Name = Severe combined immunodeficiency Caption = DiseasesDB = 11978 ICD10 = ICD10|D|81|0|d|80 ICD10|D|81|2|d|80 ICD9 = ICD9|279.2 ICDO = OMIM = MedlinePlus = eMedicineSubj = med eMedicineTopic = 2214 MeshID = D016511 Severe… …   Wikipedia

  • Severe Combined Immunodeficiency — Klassifikation nach ICD 10 D81.0 SCID mit retikulärer Dysgenesie D81.1 SCID mit niedriger T und B Zellenzahl D81.2 …   Deutsch Wikipedia

  • severe combined immunodeficiency — noun 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 • Syn:… …   Useful english dictionary

  • Severe combined immunodeficiency (non-human) — Severe Combined Immunodeficiency (SCID) is a severe immunodeficiency genetic disorder that is characterised by the complete inability of the adaptive immune system mount, coordinate, and sustain an appropriate immune response, usually due to… …   Wikipedia

  • severe combined immunodeficiency syndrome — noun A severe form of congenital immunological deficiency in which both T and B lymphocytes are absent (abbrev SCID) • • • Main Entry: ↑severe …   Useful english dictionary

  • Severe Combined Immunodeficiency Disorder — Klassifikation nach ICD 10 D81.1 Schwerer kombinierter Immundefekt [SCID] mit niedriger T und B Zellen Zahl …   Deutsch Wikipedia

  • severe combined immunodeficiency disease — noun 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 • Syn:… …   Useful english dictionary

  • severe combined immunodeficiency — noun Date: 1973 a rare congenital disorder of the immune system that is characterized by inability to produce a normal complement of antibodies and T cells and that usually results in early death called also severe combined immune deficiency …   New Collegiate Dictionary

  • severe combined immunodeficiency disease — (SCID) see under immunodeficiency …   Medical dictionary

  • severe combined immunodeficiency — noun → SCID …  

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