Definitions for severe combined immunodeficiency
se·vere com·bined im·mun·od·e·fi·cien·cy

Princeton's WordNetRate this definition:0.0 / 0 votes

1. severe combined immunodeficiency, severe combined immunodeficiency disease, SCIDnoun

   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

WikipediaRate this definition:0.0 / 0 votes

1. Severe combined immunodeficiency

   Severe combined immunodeficiency (SCID), also known as Swiss-type agammaglobulinemia, is a rare genetic disorder characterized by the disturbed development of functional T cells and B cells caused by numerous genetic mutations that result in differing clinical presentations. SCID involves defective antibody response due to either direct involvement with B lymphocytes or through improper B lymphocyte activation due to non-functional T-helper cells. Consequently, both "arms" (B cells and T cells) of the adaptive immune system are impaired due to a defect in one of several possible genes. SCID is the most severe form of primary immunodeficiencies, and there are now at least nine different known genes in which mutations lead to a form of SCID. It is also known as the bubble boy disease and bubble baby disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, have become famous for living in a sterile environment. SCID is the result of an immune system so highly compromised that it is considered almost absent. SCID patients are usually affected by severe bacterial, viral, or fungal infections early in life and often present with interstitial lung disease, chronic diarrhea, and failure to thrive. Ear infections, recurrent Pneumocystis jirovecii (previously carinii) pneumonia, and profuse oral candidiasis commonly occur. These babies, if untreated, usually die within one year due to severe, recurrent infections unless they have undergone successful hematopoietic stem cell transplantation or gene therapy in clinical trials.

WikidataRate this definition:0.0 / 0 votes

1. Severe combined immunodeficiency

   Severe combined immunodeficiency, is a genetic disorder characterized by the absence of functional T-lymphocytes, which results in a defective antibody response due to either direct involvement with B lymphocytes or through improper B lymphocyte activation due to non-functional T-helper cells. Consequently, both "arms" of the adaptive immune system are impaired due to a defect in one of several possible genes. SCID is the most severe form of primary immunodeficiencies, and there are now at least nine different known genes in which mutations lead to a form of SCID. It is also known as the bubble boy disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, become famous for living in a sterile environment. SCID is the result of an immune system so highly compromised that it is considered almost absent. SCID patients are usually affected by severe bacterial, viral, or fungal infections early in life and often present with interstitial lung disease, chronic diarrhea, and failure to thrive. Ear infections, recurrent Pneumocystis jirovecii pneumonia, and profuse oral candidiasis commonly occur. These babies, if untreated, usually die within 1 year due to severe, recurrent infections unless they have undergone successful Hematopoietic stem cell transplantation.

U.S. National Library of MedicineRate this definition:0.0 / 0 votes

1. Severe Combined Immunodeficiency

   Group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. It is inherited as an X-linked or autosomal recessive defect. Mutations occurring in many different genes cause human Severe Combined Immunodeficiency (SCID).

Matched Categories

1. • Immunodeficiency
   • Monogenic Disorder

How to pronounce severe combined immunodeficiency?

1. Alex

   US English

   David

   US English

   Mark

   US English

   Daniel

   British

   Libby

   British

   Mia

   British

   Karen

   Australian

   Hayley

   Australian

   Natasha

   Australian

   Veena

   Indian

   Priya

   Indian

   Neerja

   Indian

   Zira

   US English

   Oliver

   British

   Wendy

   British

   Fred

   US English

   Tessa

   South African

How to say severe combined immunodeficiency in sign language?

Numerology

1. Chaldean Numerology

   The numerical value of severe combined immunodeficiency in Chaldean Numerology is: 3

2. Pythagorean Numerology

   The numerical value of severe combined immunodeficiency in Pythagorean Numerology is: 1

References

1. ^ Princeton's WordNet
   http://wordnetweb.princeton.edu/perl/webwn?s=severe combined immunodeficiency
2. ^ Wikipedia
   https://en.wikipedia.org/wiki/Severe_Combined_Immunodeficiency
3. ^ Wikidata
   https://www.wikidata.org/w/index.php?search=severe combined immunodeficiency