immo-deficiency
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| what is Severe combined immunodeficiency disease (SCID)? | (1) a deficiency of the stem cell (2) abnormal differentiation of T cells due to abnormalities in the thymus gland.
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| SCID is also associated with which defunct gland | thymus - more spacifically, hypoplastic thymus.
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| what kind of deficiency is likely to develop graft-versus-host-disease (GVHD) | primary immune deficiency
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| what is another name for stem cell deficiency? | reticular dysgenesis
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| What contributes to the tendency of developing recurrent and severe infections? | primary immune deficiencies
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| When can a primary immune deficiency error occur? | at any point during the development of the immune system.
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| What results from failure in the development of an essential part of the immune system- congenital? | Primary immune deficiency
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| if one has a hypoplastic thymus, where will depletion occur? (PRIMARY IMMUNE DEFICIENCY) | few plasma cells, all classes of Ig depressed.
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| The infant affected from birth will exhibit failure to thrive, followed by severe infections under which condition? (PRIMARY IMMUNE DEFICIENCY) | SCID
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| what is the treatment for SCID? (PRIMARY IMMUNE DEFICIENCY) | Bone marrow transplantation (HLA compatible).
Gene therapy for SCID of ADA origin (adenosine deaminanse deficiency)
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| Deficiencies of antibodies are caused by what in (PRIMARY IMMUNE DEFICIENCY)? | 1. abnormal Ig synthesis
2. deficiency of immunoglobulin.
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| what causes a defect in the maturation of stem cells into B cells? (PRIMARY IMMUNE DEFICIENCY) | hypogammaglobulinemia (or agammaglobulinemia)
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| what causes an absence of B-cells for the blood? (PRIMARY IMMUNE DEFICIENCY) | hypogammaglobulinemia
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| with hypogammaglobulinemia, do major classes of Ig exist? (PRIMARY IMMUNE DEFICIENCY) | yes, low levels of major classes of Ig are found, but antigens are not cleared well from the body, and infants are susceptible to infection
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| Clinical manifestations in hypogammaglobulinemia include? (PRIMARY IMMUNE DEFICIENCY) | respiratory, sinus, and throat infections.
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| what part of the immune system remains competent with hypogammaglobulinemia? (PRIMARY IMMUNE DEFICIENCY) | cellular immune system
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| what is the treatment for hypogammaglobulinemia? (PRIMARY IMMUNE DEFICIENCY) | gamma globulin IgG with appropriate antibiotics.
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| how often does the pt. receive IgG preparations for hypogammaglobulinemia? (PRIMARY IMMUNE DEFICIENCY) | IgG preparations IM monthly at dosages determined by body weight or by circulating IgG levels.
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| what causes IgA deficiency? (PRIMARY IMMUNE DEFICIENCY) | hereditary, caused by failure in the synthesis and release of IgA.
B cell immaturity
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| IgA deficiency occurs in one out of how many ppl? (PRIMARY IMMUNE DEFICIENCY) | 700
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| IgA affect any other Ig serum levels? (PRIMARY IMMUNE DEFICIENCY) | no, these pt have normal serum levels of Ig
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| IgA deficiency affects the serum and ____? (PRIMARY IMMUNE DEFICIENCY) | external secretions
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| IgA deficiency will manifest as what type of reactions in the pt? (PRIMARY IMMUNE DEFICIENCY) | infection of sinus, respiratory, ulcerative colitis, pernicious anemia, malabsorption states, and atopic diseases.
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| IgA deficiency will also cause what disease? (PRIMARY IMMUNE DEFICIENCY) | autoimmune
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| Deficiency of Cell-Mediated Immunity is connected to what type of syndrome? (PRIMARY IMMUNE DEFICIENCY) | DiGeorge syndrome.
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| DiGeorge syndrome affects what structure, or lack there of? (PRIMARY IMMUNE DEFICIENCY) | absence of the thymus
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| Neonate is extremely susceptible to what infections in DiGeorge syndrome? (PRIMARY IMMUNE DEFICIENCY) | fungal and viral infections
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| DiGeorge syndrome may be caused by? (PRIMARY IMMUNE DEFICIENCY) | Since there is no contributing abnormal family history, the defect is presumed to be caused by an environmental agent or drug exposure during twelfth week of gestation.
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| physical manifestations in DiGeorge syndrome? (PRIMARY IMMUNE DEFICIENCY) | low-set ears, slanted eyes, hypoparathyroidism, congenital heart defects, and cell immunodeficiency.
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| Treatment includes what for DiGeorge Syndrome (PRIMARY IMMUNE DEFICIENCY) | thymus transplant or bone marrow.
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| with DiGeorge Syndrome, if the defect is not complete by the age 5 years, what happens? (PRIMARY IMMUNE DEFICIENCY) | T cell function may repair itself
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| secondary immune deficiency (SID) is brought on in what form? | acquired
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| what is SID? | Loss of a previously competent immune system; there is loss of immunoglobulins, inadequate synthesis of Ig, loss of specific lymphocytes responsible for cell-mediated immunity, loss of phagocytic inflammatory cells, or a combination of these.
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| SID is related to what etiology? | stress and aging
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| what decreases the action of the thymus through secretion of glucocorticoids? | stress
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| what suppresses the inflammatory response? | glucocorticoids
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| SID is also related to aging will cause what other problems? | autoimmune disease, antibody deficiency, impaired hypersensitivity response, and decreasing lymphocyte regeneration.
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| SID will also include: | nutrition and co-existing infections
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| Virally induced secondary immune state caused by exposure to retrovirus - aka | hiv
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| HIV is a RNA virus that infects cells by binding to the surface of _______ cells through ______ and inserting RNA into the target cell. | target, receptor
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| why is making a vaccine for HIV difficult? | because of genetic variants of HIV vary during the progression of the disease (30 variants)
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| Sero-antibody-positive in association with various clinical symptoms such as severe immunodeficiency and the inability to fight environmental antigens. | AIDS
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| Antibody-positive form in HIV is also known as_______? | sero-antibody-positive.
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| Detection of HIV antibodies can be determined by which screening blood product test? | ELISA test (enzyme-linked immunosorbent assay)
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| A positive ELISA test confirms what? | means that the person has been exposed to the virus but does not necessarily mean that the person has active infection.
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| what test is used to confirm positive antibody reaction to HIV. | Western Blot
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| There is a variable period of time before an exposed individual develops the antibody also known as. | so a “window”
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| what is the "window" in HIV? | exposed blood products may test negative for the antibody, but viral infection is present.
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| during the "Window period" where an individual may not have produced sufficient antibody, but have the virus growing.... are precautions to be taken? | YES, the virus levels can be high and infectious to others. take precautions.
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| what new form of testing narrows the window for confirmation of HIV/AIDS. | antigen P24 test - results in 20 minutes
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| what antigen on the surface of helper T cells acts as primary receptor for HIV. | CD4 antigen
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| AIDs causes a devastating infection of of helper T4 subset lymphocytes which includes ____ cells and ______. | T-helper, macrophages
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| In healthy individuals, the ratio of helper to suppresser cells is 2:1, but in AIDS this changes to what ratio? | 1:2 as helper cells are depleted.
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| At the beginning of the infection there is a decrease of what cells? | T4 lymphocytes,
T helper cells,
macrophages.
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| Since 1993, the AIDS diagnosis applies to individuals who are HIV positive and whose count of infection fighting WBCs (CD4+ antigen on surface of T-helper cells, primary receptor for HIV virus) is below | 200/cu. ml. (Normal: 800-1000 cu. ml.)
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| One of the fastest growing routes of transmission of HIV is from pregnant women to _____? | fetus
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| what two drugs are used with HIV/AIDS | AZT and HAART
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