Severe combined immunodeficiency is a rare group of genetic disorders characterized by the body's inability to create functional T cells and B cells. It is the most severe form of primary immunodeficiency, making those affected extremely vulnerable to infection due to their highly compromised immune system. There are at least seven different known genes in which mutations lead to a form of the disease, each with different clinical presentations.
Facts
Classification
Body SystemLymphatic or Immune System 1 ICD-10 ChapterIII: Diseases of the Blood and Blood-Forming Organs and Certain Disorders Involving the Immune Mechanism 1 Communicable or Non-communicable Transmission Route Causative Agent
Causative Agent (category) Connections
Treated By
Source Enzyme replacement therapy (Wikipedia)
Source Strimvelis (Wikipedia)
Sources
1. Wikipedia: ICD-10
WikipediaList of chapters, Chapter IIIQuote, List of chapters, Chapter III
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
2. Wikipedia: Non-communicable disease
WikipediaLead section, transmission-route statement (inferred from disease-category)
NCDs include Parkinson's disease, autoimmune diseases, strokes, heart diseases, cancers, diabetes, chronic kidney disease, osteoarthritis, osteoporosis, Alzheimer's disease, cataracts, and others.
Named examples section
NCDs include Parkinson's disease, autoimmune diseases, strokes, heart diseases, cancers, diabetes, chronic kidney disease, osteoarthritis, osteoporosis, Alzheimer's disease, cataracts, and others.
View the Source 3. Severe combined immunodeficiency (Wikipedia)
lead paragraphQuote, lead paragraph
Severe combined immunodeficiencies (SCIDs) are a rare group of genetic disorders characterized by the body's inability to create functional T cells and B cells.
Enzyme replacement therapy (Wikipedia)
Treated By: Enzyme Replacement Therapy, Lead: treats Severe Combined ImmunodeficiencyQuote, Treated By: Enzyme Replacement Therapy, Lead: treats Severe Combined Immunodeficiency
ERT has also been used to treat patients with severe combined immunodeficiency (SCID) resulting from an adenosine deaminase deficiency (ADA-SCID).
Strimvelis (Wikipedia)
Treated By: Strimvelis, Lead: treats Severe Combined ImmunodeficiencyQuote, Treated By: Strimvelis, Lead: treats Severe Combined Immunodeficiency
Autologous CD34+ enriched cell fraction that contains CD34+ cells transduced with retroviral vector that encodes for the human ADA cDNA sequence, sold under the brand name Strimvelis, is a medication used to treat severe combined immunodeficiency due to adenosine deaminase deficiency (ADA-SCID).
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