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curry college

immo-deficiency

QuestionAnswer
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
Created by: nurd-sing