Microbiology – Immunodeficiency – Flashcards
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| common immunodeficiency characteristics |
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| recurrent skin rashes or abscesses chronic diarrhea abnormal growth hepatosplenomegaly thrombocytopenia endocrinopathies |
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| leukocyte adhesion deficiency |
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| the following scenario is descriptive of (blank) phagocytic cell dysfunction defective integrin (CD18) leukocytes can't move from blood to tissue; no diapedesis |
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| chronic granulomatous disease superoxide nitroblue tetrazolium dye |
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| the following describes (blank) disease can't make (blank) affects the ability of phagocytes to destroy ingested bacteria; think of oxidative respiratory burst inability to reduce (blank) is common lab test of this |
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| percentages of immunodeficiency diseases T cell Phagocytic cell complement? |
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| 50% B cell associated 30-40% T cell 15% Phagocytic cell 2-4% complement these values represent the percentages of (blank) diseases due to their respective cell types |
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| direct influences development |
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| (blank) influences - cause B and T cell immunodeficiencies are associated with blocks in (blank) of lymphocyte differentiation and function |
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| indirect influences regulation |
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| (blank) influences - cause B and T cell immunodeficiencies are associated with blocks in (blank) of lymphocyte differentiation and function |
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| antibody B cells SURFACE Ig POSITIVE CELLS staphylococcal protein A |
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| the following describes the sign posts of a deficiency in antibody-mediated (humoral) immunity: many (extra or intra cellular?) infections reduced serum (blank) levels reduced circulating (blank) cells; decreased percent of (blank) [(blank) cells should be at abt 20-30% of lymphocytes] reduced FUNCTIONAL (blank) cells; responsiveness to (blank) cell specific mitogens such as (blank) |
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| staphylococcal protein A mitosis |
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| (blank) is a B cell specific mitogen causes (blank) in a B cell |
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| intracellular CD3 CD4 CD8 PHYTOHEMAGGLUTININ (PHA |
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| the following describes how you can spot cell-mediated immunity immunodeficiency: frequent viral, fungal, and (blank) bacterial infections reduced circulating (blank) cells; such as those that express (blank) [all mature (blank) cells do!!], (blank) [which is characteristic of helpers], or (blank) [characteristic of killers] reduced FUNCTIONAL (blank) cells, which should respond to (blank), a mitogen |
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| Adenosine Deaminase (ADA) Deficiency Purine Nucleoside Phosphorylase (PNP) Deficiency |
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| 50% of autosomal SCIDs is caused by deficiency of (blank) or (blank) both these enzymes involved in nucleotide metabolism In SCIDs, progenitors of lymphocytes don't have working methods to get rid of toxic intermediaries and thus die... = basically no immune system |
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| SCIDs |
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| the following describe the 3 main autosomal origins of (blank) 1. defect in ADA or PNP (nucleotide metabolism) 2. Bare-leukocyte syndrome (no MHCs on APCs) 3. RAG deficiencies (can't recombinate to make antigen receptors!) |
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| X-linked |
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| the following describes (blank) type of SCID defective common gamma chain of cytokine receptors can't receive important cytokines IL-2, 4, 7, 9 and 15 IL-7 most important = used in lymphocyte development |
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| agammaglobulinemias X-linked congenital agammaglobulinemia and Common Variable Immunodeficiency (CVI) Selective IgA deficiency X-linked hyper-IgM syndrome Activation-induced cytidine deaminase (AID) Deficiency |
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| (blank) describes scenarios in which the patient displays deficiencies in antibody production it is the umbrella term for anything related to deficiency with B cell branch further divided into (blank) and (blank) (blank) can further be divided into 3 sub categories, (blank), (blank) and (blank) |
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| x-linked congenital agammaglobulinemia TYROSINE KINASE (btk) |
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| the following describes x-linked congenital agammaglobulinemia: B cells stuck at the (blank) stage DEFECTIVE (blank) (above blank) necessary for appropriate V to DJ joining [gene rearrangements] leading to proper antigen receptor expression primarily a disease of young males (x-linked!) |
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| puto |
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| efff you |
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| Selective IgA deficiency X-linked hyper-IgM syndrome Activation-induced cytidine deaminase (AID) Deficiency SELECTIVE IgA DEFICIENCY older equal plasma |
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| the following describes common variable immunodeficiencies (CVI): [remember this represents a subset of agammaglobulinemias apart from X-linked congenital agammaglobulinemia!] CVI can further be divided into 3 subdivisions (blank), (blank), and (blank) selective isotype deficiencies of any isotype can be observed (ex: no IgE or no IgD) most common of these isotype deficiencies is (blank) affects what general age group of patient population? (blank) frequencies of affected males and females are (blank) B cells (but no (blank) cells) often present mechanisms for these syndromes are complex and unclear |
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| selective IgA deficiency |
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| most common immunodeficiency (1 in 400) vast majority remain asymptomatic |
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| CD40 ligand isotype switching IgM |
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| the following describes X-linked hyper-IgM syndrome: defective (blank) on activated T cells; which is used to activate B cells thus, cannot stimulate B cells to differentiate to IgG = no (blank) means lots and lots of (blank) |
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| Activation-induced cytidine deaminase (AID) Deficiency |
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| (blank) is a type of condition of CVI [agammaglobulinemia] in which the patient demonstrates inability to isotype class switch and B cells cannot reach affinity maturation |
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| DiGeorge's Syndrome thymic aplasia thymus no calcium, high phosphorus, muscle tetany |
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| (blank) - developmental defects affecting all tissues in the upper chest cavity, striking set of facial features congenital (blank) (blank) [(blank) deformed or absent] uneducated pre-T cells present no (blank), high (blank), muscle (blank) |
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| mucocutaneous candidiasis yeast |
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| the following describes (blank) (blank) T cells of cell-mediated immunity branch don't work (CTLs, TH1 and TH17); caused by (blank) infection simple (blank) infections can disseminate systemically and become life-threatening |
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| acquired immunodeficiency syndrome (AIDS) |
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| (blank) is transmitted by HIV virus know modes of transmission and certain behaviors that are high risk |
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| CD4+ Gp120 |
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| what does HIV infect cells of immune system, especially (blank) cells (blank) of HIV binds to CD4+ chemokine receptors CCR5 (Ts and Macs) and CXCR4 (only Ts) are used as "co-receptors" for the virus |
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| CCR5 and CXCR4 |
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| chemokine receptors (Ts and macs for CCR5 and Ts only for CXCR4) used as co-receptors to facilitate HIV entry into immune cells |
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| Kaposi's Sarcoma |
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| cancer that increases in incidence among HIV patients |
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| tools for monitoring course of HIV infection |
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| screening for HIV seropositivity (ELISA assay) -danger of false positives (confirm with WB) -danger of false negatives (early diagnosis difficult) tests for presence of HIV genome using PCR tests for circulating CD4+ cells (immunofluorescence with monoclonal antibodies) |
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| what is normal level of CD4 T cells what about in asymptomatic HIV patients full blown AIDS |
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| 1200/micro liter 800/micro liter less than 200/micro liter |
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| describe chain reaction that happens once CD4+ cells are destroyed in an HIV patient's immune system |
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| CD4+ T lymphocytes consist of TH1 and TH2 cells and are responsible for activating inflammatory/cell-mediated (TH1) and humoral (TH2) responses. TH1 subset (IL-2, IFN-gamma, TNF-alpha) TH2 subset (IL-4, IL-5, IL-10) |
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| bare-leukocyte syndrome class I or class II MHC molecules cell-mediated immunity humoral immunity TAP genes CD8+ |
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| the following describes (blank), one of three autosomal origins of SCIDs: a family of disorders involving deficient expression or function of (blank) or (blank) (blank) molecules Direct interference with (blank)-(blank) (blank) Indirect interference with (blank) (blank) one variation involved mutated (blank) genes that code for protein that pumps protein fragments into ER; resulting in impaired antigen presentation to (blank) T cells |
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| RAG deficiency syndrome Recombination Activation Genes RAG1/RAG2 |
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| the following describes (blank), one of three autosomal origins of SCIDs: it involves the impaired expression of (blank) genes these genes code for proteins/enzymes called (blank), which are involved in genetic rearrangements in B and T cells without these proteins, antigen receptors cannot be produced - nonfunctional immune system |
