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immo-deficiency
Question | Answer |
---|---|
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. |
SCID is also associated with which defunct gland | thymus - more spacifically, hypoplastic thymus. |
what kind of deficiency is likely to develop graft-versus-host-disease (GVHD) | primary immune deficiency |
what is another name for stem cell deficiency? | reticular dysgenesis |
What contributes to the tendency of developing recurrent and severe infections? | primary immune deficiencies |
When can a primary immune deficiency error occur? | at any point during the development of the immune system. |
What results from failure in the development of an essential part of the immune system- congenital? | Primary immune deficiency |
if one has a hypoplastic thymus, where will depletion occur? (PRIMARY IMMUNE DEFICIENCY) | few plasma cells, all classes of Ig depressed. |
The infant affected from birth will exhibit failure to thrive, followed by severe infections under which condition? (PRIMARY IMMUNE DEFICIENCY) | SCID |
what is the treatment for SCID? (PRIMARY IMMUNE DEFICIENCY) | Bone marrow transplantation (HLA compatible). Gene therapy for SCID of ADA origin (adenosine deaminanse deficiency) |
Deficiencies of antibodies are caused by what in (PRIMARY IMMUNE DEFICIENCY)? | 1. abnormal Ig synthesis 2. deficiency of immunoglobulin. |
what causes a defect in the maturation of stem cells into B cells? (PRIMARY IMMUNE DEFICIENCY) | hypogammaglobulinemia (or agammaglobulinemia) |
what causes an absence of B-cells for the blood? (PRIMARY IMMUNE DEFICIENCY) | hypogammaglobulinemia |
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 |
Clinical manifestations in hypogammaglobulinemia include? (PRIMARY IMMUNE DEFICIENCY) | respiratory, sinus, and throat infections. |
what part of the immune system remains competent with hypogammaglobulinemia? (PRIMARY IMMUNE DEFICIENCY) | cellular immune system |
what is the treatment for hypogammaglobulinemia? (PRIMARY IMMUNE DEFICIENCY) | gamma globulin IgG with appropriate antibiotics. |
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. |
what causes IgA deficiency? (PRIMARY IMMUNE DEFICIENCY) | hereditary, caused by failure in the synthesis and release of IgA. B cell immaturity |
IgA deficiency occurs in one out of how many ppl? (PRIMARY IMMUNE DEFICIENCY) | 700 |
IgA affect any other Ig serum levels? (PRIMARY IMMUNE DEFICIENCY) | no, these pt have normal serum levels of Ig |
IgA deficiency affects the serum and ____? (PRIMARY IMMUNE DEFICIENCY) | external secretions |
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. |
IgA deficiency will also cause what disease? (PRIMARY IMMUNE DEFICIENCY) | autoimmune |
Deficiency of Cell-Mediated Immunity is connected to what type of syndrome? (PRIMARY IMMUNE DEFICIENCY) | DiGeorge syndrome. |
DiGeorge syndrome affects what structure, or lack there of? (PRIMARY IMMUNE DEFICIENCY) | absence of the thymus |
Neonate is extremely susceptible to what infections in DiGeorge syndrome? (PRIMARY IMMUNE DEFICIENCY) | fungal and viral infections |
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. |
physical manifestations in DiGeorge syndrome? (PRIMARY IMMUNE DEFICIENCY) | low-set ears, slanted eyes, hypoparathyroidism, congenital heart defects, and cell immunodeficiency. |
Treatment includes what for DiGeorge Syndrome (PRIMARY IMMUNE DEFICIENCY) | thymus transplant or bone marrow. |
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 |
secondary immune deficiency (SID) is brought on in what form? | acquired |
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. |
SID is related to what etiology? | stress and aging |
what decreases the action of the thymus through secretion of glucocorticoids? | stress |
what suppresses the inflammatory response? | glucocorticoids |
SID is also related to aging will cause what other problems? | autoimmune disease, antibody deficiency, impaired hypersensitivity response, and decreasing lymphocyte regeneration. |
SID will also include: | nutrition and co-existing infections |
Virally induced secondary immune state caused by exposure to retrovirus - aka | hiv |
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 |
why is making a vaccine for HIV difficult? | because of genetic variants of HIV vary during the progression of the disease (30 variants) |
Sero-antibody-positive in association with various clinical symptoms such as severe immunodeficiency and the inability to fight environmental antigens. | AIDS |
Antibody-positive form in HIV is also known as_______? | sero-antibody-positive. |
Detection of HIV antibodies can be determined by which screening blood product test? | ELISA test (enzyme-linked immunosorbent assay) |
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. |
what test is used to confirm positive antibody reaction to HIV. | Western Blot |
There is a variable period of time before an exposed individual develops the antibody also known as. | so a “window” |
what is the "window" in HIV? | exposed blood products may test negative for the antibody, but viral infection is present. |
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. |
what new form of testing narrows the window for confirmation of HIV/AIDS. | antigen P24 test - results in 20 minutes |
what antigen on the surface of helper T cells acts as primary receptor for HIV. | CD4 antigen |
AIDs causes a devastating infection of of helper T4 subset lymphocytes which includes ____ cells and ______. | T-helper, macrophages |
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. |
At the beginning of the infection there is a decrease of what cells? | T4 lymphocytes, T helper cells, macrophages. |
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.) |
One of the fastest growing routes of transmission of HIV is from pregnant women to _____? | fetus |
what two drugs are used with HIV/AIDS | AZT and HAART |