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Encyclopedia > Severe combined immunodeficiency
Severe combined immunodeficiency
Classification & external resources
ICD-10 D81.0-D81.2
ICD-9 279.2
DiseasesDB 11978
eMedicine med/2214 
MeSH D016511

Severe combined immunodeficiency, or Boy in the Bubble Syndrome, is a genetic disorder in which both "arms" (B cells and T cells) of the adaptive immune system are crippled, due to a defect in one of several possible genes. SCID is a severe form of heritable immunodeficiency. It is also known as the "bubble boy" disease because its victims are extremely vulnerable to infectious diseases. The most famous case is the boy David Vetter. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ... T cells are a subset of lymphocytes that play a large role in the immune response. ... The immune system is the collection of organs and tissues involved in the adaptive defense of a body against foreign biological material. ... In medicine, immunodeficiency (or immune deficiency) is a state in which the immune systems ability to fight infectious disease is compromised or entirely absent. ... David Phillip Vetter (September 21, 1971 – February 22, 1984) was a boy from Shenandoah, Texas, United States who suffered from a rare genetic disease now known as severe combined immune deficiency syndrome (SCIDS). ...


Chronic diarrhea, 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. However, treatment options are much improved since David Vetter, and living in a bubble is no longer necessary. Pneumocystis carinii pneumonia (PCP) is a form of pneumonia which is caused by a microorganism called Pneumocystis carinii (It has been proposed that the organism be renamed Pneumocystis jiroveci). ...

Contents

Prevalence/incidence

Classical SCID has a reported incidence of about 1 in 69,000 live births in Australia. [1]


Recent studies indicate that one in every 2,500 children in the Navajo population inherit severe combined immunodeficiency. This condition is a significant cause of illness and death among Navajo children.[2] Ongoing research reveals a similar genetic pattern among the related Apache people.[3] For other uses, see Navajo (disambiguation). ... For other uses, see Apache (disambiguation). ...


Types

Common gamma chain (X-linked severe combined immunodeficiency)

Main article: X-SCID
See also: Common gamma chain

Most cases of SCID are due to mutations in the gene encoding the common gamma chainc), a protein that is shared by the receptors for interleukins IL-2, IL-4, IL-7, IL-9, IL-15 and IL-21. These interleukins and their receptors are involved in the development and differentiation of T and B cells. Because the common gamma chain is shared by many interleukin receptors, mutations that result in a non-functional common gamma chain cause widespread defects in interleukin signalling. The result is a near complete failure of the immune system to develop and function, with low or absent T cells and NK cells and non-functional B cells. X-linked Severe Combined Immunodeficiency (X-SCID) is a profound and severe immunodeficiency characterized by the complete absence of NK cells and T cells in the peripheral blood. ... Common gamma chain (γc) is a cytokine receptor subunit that is located on the surface of certain sub-sets of lymphocytes (white blood cells). ... For linguistic mutation, see Apophony. ... Common gamma chain (γc) is a cytokine receptor subunit that is located on the surface of certain sub-sets of lymphocytes (white blood cells). ... Interleukins are a group of cytokines that were first seen to be expressed by white blood cells (leukocytes, hence the -leukin) as a means of communication (inter-). The name is sort of a relic though; it has since been found that interleukins are produced by a wide variety of bodily... The abbreviation IL-2 can refer to: Interleukin-2, a cytokine responsible for stimulating the growth of T-lymphocytes. ... The Ilyushin Il-4 was a Soviet World War II bomber aircraft, widely used by the VVS although not well known. ... Interleukin 7 (IL7) is a hematopoietic growth factor secreted by the stromal cells of the red marrow and thymus capable of stimulating the proliferation of lymphoid progenitors. ... IL-9 can refer to: Interleukin 9 Illinois 9th congressional district Category: ... Interleukin 15 (IL-15) is a cytokine with structural similarity to IL-2 that is secreted by mononuclear phagocytes (and some other cells) following infection by virus(es). ... Interleukin 21 (IL-21), is a cytokine that has potent regulatory effects on cells of the immune system, including natural killer (NK) cells and cytotoxic T cells that can destroy virally infected or cancerous cells. ... T cells are a subset of lymphocytes that play a large role in the immune response. ... Natural killer cells (NK) are a type of lymphocyte (a white blood cell) and a component of nonspecific immune defense. ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ...


The common gamma chain is encoded by the gene IL-2 receptor gamma, or IL-2Rγ, which is located on the X-chromosome. Therefore, immunodeficiency caused by mutations in IL-2Rγ is known as X-linked severe combined immunodeficiency. The condition is inherited in an X-linked recessive pattern. All males (who have one X and one Y chromosome) who have an X chromosome with the defective gene will have SCID. Females (who have two X chromosomes) with one defective and normal chromosome will be healthy, as one normal IL-2Rγ gene is sufficient to have a normal immune system. However, these females are carriers. Their male children have a 50% chance of having X-linked SCID, and their female children have a 50% chance of being carriers. The Interleukin-2 Receptor (IL-2R) is heterotrimeric protein expressed on the surface of certain immune cells, such as lymphocytes, that binds and responds to a cytokine called interleukin 2. ... X-linked recessive is a mode of inheritance in which a mutation in a gene on the X chromosome causes the phenotype to be expressed in males (who are necessarily hemizygous for the gene mutation because they have only one X chromosome) and in females who are homozygous for the...


JAK3

Janus kinase-3 (JAK3) is an enzyme that mediates transduction downstream of the γc signal. Mutation of its gene also causes SCID.[4] Janus kinase (JAK) is a family of intracellular non-receptor tyrosine kinases, ranging from 120-140 kDa, that transduce cytokine-mediated signals via the JAK-STAT pathway. ...


V(D)J recombination

The manufacture of immunoglobulins requires recombinase enzymes derived from the recombination activating genes RAG-1 and RAG-2. These enzymes are involved in the first stage of V(D)J recombination, the process by which segments of a B cell or T cell's DNA are rearranged to create a new T cell receptor or B cell receptor (and, in the B cell's case, the template for antibodies). Schematic of antibody binding to an antigen An antibody is a protein complex used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ... The recombination activating genes encode enzymes that play an important role in the rearrangement and recombination of the genes of immunoglobulin and T cell receptor molecules during the process of VDJ recombination. ... The recombination activating genes encode enzymes that play an important role in the rearrangement and recombination of the genes of immunoglobulin and T cell receptor molecules during the process of VDJ recombination. ... V(D)J recombination is a mechanism of DNA recombination used by humans and other vertebrates for immunological protection against attacks by bacterial, viral, and parasitic invaders. ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ... T cells are a subset of lymphocytes that play a large role in the immune response. ...


Certain mutations of the RAG-1 or RAG-2 genes prevent V(D)J recombination, causing SCID.[5] V(D)J recombination is a mechanism of DNA recombination used by humans and other vertebrates for immunological protection against attacks by bacterial, viral, and parasitic invaders. ...


These genes are also associated with Omenn syndrome. Omenn syndrome is a rare immunodeficiency disease. ...


Adenosine deaminase

The second most common form of SCID after X_SCID is caused by a defective enzyme, adenosine deaminase (ADA), necessary for the breakdown of purines. Lack of ADA causes accumulation of dATP. This metabolite will inhibit the activity of ribonucleotide diphosphate reductase, the enzyme that reduces ribonucleotides to generate deoxyribonucleotides. The effectiveness of the immune system depends upon lymphocyte proliferation and hence dNTP synthesis. Without functional ribonucleotide reductase, lymphocyte proliferation is inhibited and the immune system is compromised. Adenosine deaminase deficiency, or ADA deficiency, is an inherited immunodeficiency syndrome accounting for about 25% of all cases of Severe combined immunodeficiency. ... Adenosine deaminase (sometimes known as ADA) is an enzyme in the purine metabolism. ... Purine (1) is a heterocyclic aromatic organic compound, consisting of a pyrimidine ring fused to an imidazole ring. ...


Artemis/DCLRE1C

Mortan Cowan, MD, director of the Pediatric Bone Marrow Transplant Program at the University of California-San Francisco, noted that although researchers have identified about a dozen genes that cause SCID, the Navajo and Apache population has the most severe form of the disorder. This is due to the lack of a gene designated Artemis. Without the gene, children's bodies are unable to repair DNA or develop disease-fighting cells. [2][3] Berkeley Davis Irvine Los Angeles Merced Riverside San Diego Santa Barbara Santa Cruz UC Office of the President in Oakland The University of California (UC) is a public university system in the state of California. ... The structure of part of a DNA double helix Deoxyribonucleic acid, or DNA, is a nucleic acid molecule that contains the genetic instructions used in the development and functioning of all known living organisms. ...


Detection

Standard testing of SCID is not currently available for newborns due to the diversity of the genetic defect. Some SCID can be detected by sequencing fetal DNA if a known history of the disease exists. Otherwise, SCID is not detected until about six months of age, usually indicated by recurrent infections. The delay in detection is due to the fact that newborns carry their mother's antibodies for the first few weeks of life and SCID babies look normal. Each antibody binds to a specific antigen; an interaction similar to a lock and key. ...


Treatment

The most common treatment for SCID is bone marrow transplantation, which requires matched donors (a sibling is generally best).[6] David Vetter, the original "bubble boy", had one of the first transplantations, and finally died because of an unscreened virus, Epstein-Barr (tests were not available at the time), in his newly transplanted bone marrow from his sister. Today, transplants done in the first three months of life have a high success rate. Bone marrow transplantation or hematopoietic stem cell transplantation (HSCT) is a medical procedure in the field of hematology and oncology that involves transplantation of hematopoietic stem cells (HSC). ... David Phillip Vetter (September 21, 1971 – February 22, 1984) was a boy from Shenandoah, Texas, United States who suffered from a rare genetic disease now known as severe combined immune deficiency syndrome (SCIDS). ... The Epstein-Barr virus (EBV), also called Human herpesvirus 4 (HHV-4), is a virus of the herpes family (which includes Herpes simplex virus and Cytomegalovirus), and one of the most common viruses in humans. ...


More recently gene therapy has proved useful. Transduction of the missing gene to hematopoietic stem cells using viral vectors is being tested in ADA SCID and X-linked SCID. The first gene therapy trials were performed in 1990, with peripheral T cells. In 2000, the first gene therapy "success" resulted in SCID patients with a functional immune system. These trials were stopped when it was discovered that two of ten patients in one trial had developed leukemia resulting from the insertion of the gene-carrying retrovirus near an oncogene. In 2007, four of the ten patients have developed leukemias [7]. Work is now focusing on correcting the gene without triggering an oncogene. No leukemia cases have yet been seen in trials of ADA-SCID, which does not involve the gamma c gene that may be oncogenic when expressed by a retrovirus. Gene therapy is the insertion of genes into an individuals cells and tissues to treat a disease, and hereditary diseases in which a defective mutant allele is replaced with a functional one. ... The word transduction has several meanings: In developmental psychology, transduction is reasoning from specific cases to specific cases, typically employed by children. ... This article is about biological infectious particles. ... In epidemiology, a vector is an organism that does not cause disease itself but which spreads infection by conveying pathogens from one host to another. ... Leukemia or leukaemia(Greek leukos λευκός, “white”; aima αίμα, “blood”) (see spelling differences) is a cancer of the blood or bone marrow and is characterized by an abnormal proliferation (production by multiplication) of blood cells, usually white blood cells (leukocytes). ... An oncogene is a modified gene that increases the malignancy of a tumor cell. ... Genera Alpharetrovirus Betaretrovirus Gammaretrovirus Deltaretrovirus Epsilonretrovirus Lentivirus Spumavirus A retrovirus is any virus belonging to the viral family Retroviridae. ...


Trial treatments of SCID have been gene therapy's only success; since 1999, gene therapy has restored the immune systems of at least 17 children with two forms (ADA-SCID and X-SCID) of the disorder.


SCID in animals

Main article: Severe combined immunodeficiency (non-human)

SCID mice are used in disease, vaccine, and transplant research, especially as animal models for testing the safety of new vaccines or therapeutic agents in people with weakened immune systems.


An animal variation of the disease, an autosomal recessive gene with clinical signs similar to the human condition, also affects the Arabian horse. In horses, to date, the condition remains a fatal disease, as the animal inevitably succumbs to an opportunistic infection within the first four to six months of life.[8] However, carriers, who themselves are not affected by the disease, can be detected with a DNA test. Thus careful breeding practices can avoid the risk of an affected foal being produced.[9] An autosome is a non-sex chromosome. ... It has been suggested that this article or section be merged into Dominance relationship. ... The Arabian horse is a breed of horse with a reputation for intelligence, high spirit, and outstanding stamina. ... The structure of part of a DNA double helix Deoxyribonucleic acid, or DNA, is a nucleic acid molecule that contains the genetic instructions used in the development and functioning of all known living organisms. ... A foal is a young horse of either gender; a female foal is called a filly, while a male foal is called a colt. ...


Another animal with well-characterized SCID pathology is the dog. There are two known forms, an X-linked SCID in Basset Hounds that has similar ontology to X-SCID in humans[10], and an autosomal recessive form seen in one line of Jack Russell Terriers that is similar to SCID in Arabian horses and mice.[11] This article does not cite any references or sources. ... X-linked Severe Combined Immunodeficiency (X-SCID) is a profound and severe immunodeficiency characterized by the complete absence of NK cells and T cells in the peripheral blood. ... The Jack Russell Terrier is a type or landrace of small, principally white-bodied, smooth or rough-coated terrier that has its origins in fox hunting. ...


References

  1. ^ Yee A, De Ravin SS, Elliott E, Ziegler JB (2008). "Severe combined immunodeficiency: A national surveillance study". Pediatr Allergy Immunol. doi:10.1111/j.1399-3038.2007.00646.x. PMID 18221464.
  2. ^ a b News From Indian Country - A rare and once-baffling disease forces Navajo parents to cope. Retrieved on 2008-03-01.
  3. ^ a b Li L, Moshous D, Zhou Y, et al (2002). "A founder mutation in Artemis, an SNM1-like protein, causes SCID in Athabascan-speaking Native Americans". J. Immunol. 168 (12): 6323–9. PMID 12055248.
  4. ^ Pesu M, Candotti F, Husa M, Hofmann SR, Notarangelo LD, O'Shea JJ (2005). "Jak3, severe combined immunodeficiency, and a new class of immunosuppressive drugs". Immunol. Rev. 203: 127–42. doi:10.1111/j.0105-2896.2005.00220.x. PMID 15661026.
  5. ^ Haq IJ, Steinberg LJ, Hoenig M, et al (2007). "GvHD-associated cytokine polymorphisms do not associate with Omenn syndrome rather than T-B- SCID in patients with defects in RAG genes". Clin. Immunol. 124 (2): 165–9. doi:10.1016/j.clim.2007.04.013. PMID 17572155.
  6. ^ Severe Combined Immunodeficiency (SCID): Immunodeficiency Disorders: Merck Manual Professional. Retrieved on 2008-03-01.
  7. ^ Press release from the European Society of Gene Therapy
  8. ^ FOAL.org, an organization promoting research into genetic lethal diseases in horse
  9. ^ "The New DNA Test for Severe Combined Immunodeficiency (SCID) in Arabian Horses"
  10. ^ Henthorn PS, Somberg RL, Fimiani VM, Puck JM, Patterson DF, Felsburg PJ (1994). "IL-2R gamma gene microdeletion demonstrates that canine X-linked severe combined immunodeficiency is a homologue of the human disease" (abstract only). Genomics 23 (1): 69-74. PMID 7829104.
  11. ^ Perryman LE (2004). "Molecular pathology of severe combined immunodeficiency in mice, horses, and dogs". Vet. Pathol. 41 (2): 95–100. doi:10.1354/vp.41-2-95. PMID 15017021.

A digital object identifier (or DOI) is a standard for persistently identifying a piece of intellectual property on a digital network and associating it with related data, the metadata, in a structured extensible way. ... 2008 (MMVIII) is the current year, a leap year that started on Tuesday of the Anno Domini (or common era), in accordance to the Gregorian calendar. ... is the 60th day of the year (61st in leap years) in the Gregorian calendar. ... A digital object identifier (or DOI) is a standard for persistently identifying a piece of intellectual property on a digital network and associating it with related data, the metadata, in a structured extensible way. ... A digital object identifier (or DOI) is a standard for persistently identifying a piece of intellectual property on a digital network and associating it with related data, the metadata, in a structured extensible way. ... 2008 (MMVIII) is the current year, a leap year that started on Tuesday of the Anno Domini (or common era), in accordance to the Gregorian calendar. ... is the 60th day of the year (61st in leap years) in the Gregorian calendar. ... A digital object identifier (or DOI) is a standard for persistently identifying a piece of intellectual property on a digital network and associating it with related data, the metadata, in a structured extensible way. ...

External links

  • Learning About Severe Combined Immunodeficiency (SCID) NIH
  • Buckley RH (2004). "Molecular defects in human severe combined immunodeficiency and approaches to immune reconstitution". Annu Rev Immunol 22: 625-55. PMID 15032591.
  • Chinen J, Puck JM (2004). "Successes and risks of gene therapy in primary immunodeficiencies". J Allergy Clin Immunol 113 (4): 595-603; quiz 604. PMID 15100660.
  • Church AC (2002). "X-linked severe combined immunodeficiency". Hosp Med 63 (11): 676-80. PMID 12474613.
  • Gennery AR, Cant AJ (2001). "Diagnosis of severe combined immunodeficiency". J Clin Pathol 54 (3): 191-5. PMID 11253129.
A renal cell carcinoma (chromophobe type) viewed on a hematoxylin & eosin stained slide Pathologist redirects here. ... In medicine, immunodeficiency (or immune deficiency) is a state in which the immune systems ability to fight infectious disease is compromised or entirely absent. ... Primary immunodeficiencies are disorders in which part of the bodys immune system is missing or does not function properly. ... Each antibody binds to a specific antigen; an interaction similar to a lock and key. ... Humoral immunity is the aspect of immunity that is mediated by secreted antibodies, produced in the cells of the B lymphocyte lineage (B cell). ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ... Hypogammaglobulinemia is a type of immune deficiency. ... X-linked agammaglobulinemia (also called X-linked hypogammaglobulinemia, XLA, Bruton type agammaglobulinemia) is a rare X-linked genetic disorder that affects the bodys ability to fight infection (origin of the name: A=no, gammaglobulin=Antibody). ... Selective immunoglobulin A (IgA) deficiency is a relatively mild genetic immunodeficiency. ... Leukocyte adhesion deficiency (abbreviated LAD), is a rare autosomal recessive disorder characterized by immunodeficiency resulting in recurrent infections. ... Cell-mediated immunity is an immune response that does not involve antibodies but rather involves the activation of macrophages and NK-cells, the production of antigen-specific cytotoxic T-lymphocytes, and the release of various cytokines in response to an antigen. ... T cells are a subset of lymphocytes that play a large role in the immune response. ... 22q11. ... Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive disease characterized by eczema, thrombocytopenia (low platelet counts), immune deficiency, and bloody diarrhea (due to the low platelet counts). ... Nezelof syndrome (a form of thymic dysplasia) is a congenital immunodeficiency condition due to underdevelopment of the thymus. ... Adenosine deaminase deficiency, or ADA deficiency, is an inherited immunodeficiency syndrome accounting for about 25% of all cases of Severe combined immunodeficiency. ... Omenn syndrome is a rare immunodeficiency disease. ... X-linked Severe Combined Immunodeficiency (X-SCID) is a profound and severe immunodeficiency characterized by the complete absence of NK cells and T cells in the peripheral blood. ... Purine nucleoside phosphorylase deficiency is a rare congenital immunodeficiency of purine nucleoside phosphorylase. ... Bare lymphocyte syndrome is a condition caused by deficiencies in major histocompatibility complex: Type 1: MHC class I Type 2: MHC class II The bare lymphocyte syndrome, type II (BLS II) is a rare recessive genetic condition in which a group of genes called major histocompatibility complex class II (MHC... Complement deficiency is a condition of absent or suboptimal functioning of one of the complement system proteins. ... Angioedema (BE: angiooedema), also known by its eponym Quinckes edema, is the rapid swelling (edema) of the skin, mucosa and submucosal tissues. ... Chédiak-Higashi syndrome is a rare childhood autosomal recessive disorder that affects multiple systems of the body, which arises from a mutation that causes silvery hair. ... Chronic granulomatous disease (CGD) is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly, the superoxide radical) used to kill certain ingested pathogens. ... Hyper IgE syndrome is a heterogeneous group of disorders characterized by recurrent staphylococcal infections, unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles (balloon-like lesions that may be filled with air or pus or scar tissue) and very high concentrations of serum IgE. Some patients have... In medicine, immunodeficiency, centromere instability and facial anomalies syndrome (ICF syndrome) is a very rare recessive autosomal disorder. ... Immunoproliferative disorders (or immunoproliferative diseases, or immunoproliferative neoplasms) are a group of conditions affecting the immune system where there is overproduction of immunoglobulins or pluripotential hemopoietic stem cell or by abnormal proliferation of primary cells. ... Gamma globulins, or Igs, are a class of proteins in the blood, identified by their position after serum protein electrophoresis. ... Paraproteinemia (or Paraproteinaemia) is the presence of a monoclonal gammopathy in the blood. ... Cryoglobulinemia is the presence of abnormal proteins in the bloodstream which thicken or gel on exposure to cold. ... POEMS syndrome is a rare syndrome, the main features of which are polyneuropathy (peripheral nerve damage), organomegaly (enlargement of liver or spleen), endocrinopathy (damage to hormone-producing glands)/edema, M-protein (an abnormal antibody) and skin changes. ... Monoclonal gammopathy of undetermined significance (MGUS, unknown or uncertain may be substituted for undertermined) is a condition in which a low or non-quantifiable level of a monoclonal paraprotein is detected in the blood by means of protein electrophoresis. ... Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Hypersensitivity refers to undesirable (damaging, discomfort-producing and sometimes fatal) reactions produced by the normal immune system. ...

  Results from FactBites:
 
Severe combined immunodeficiency - Wikipedia, the free encyclopedia (725 words)
Severe Combined Immunodeficiency, or SCID, is a genetic disorder in which both "arms" (B cells and T cells) of the adaptive immune system are crippled, due to a defect in one of several possible genes.
SCID is a severe form of heritable immunodeficiency.
David Vetter, the original "bubble boy," endured several failed transplantations, and finally passed away because of an unscreened virus, Epstein-Barr, in his newly-transplanted bone marrow from his sister.
  More results at FactBites »

 
 

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