WVSOM -- Immuno/Micro -- Immunodeficiency Diseases
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| What are the primary immuno deficiencies? | b-cell defects; t cell defects; combined t and b cell defects; phagocyte defects; complement defects
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| What are teh antibody deficiencies? (B-Cell) | IgA deficiency; X-linked agammaglobulinaemia; IgG subclass deficiency; immunodeficiency with increased IgM; common variable immunodeficiency
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| How does Tcell help for B-cell differentiation? | B cell CD40 binds to T cell CD 40 ligand (CD154)
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| Explain antibody immunodeficiency with increased IgM | low igG, IgA adn increaed IgM; defect in T-cell help so there isn't differentiation.
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| What is common variable immunodeficiency | CVID; occurs in 2/3 decades; no plasma cells; recurrent sinopulmonary infections
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| Whoat is CVID treated? | IVIg antibodies
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| What is primary immunodeficiency | due to intrinsic (congenital or acquired) defects in cells or factors of the immune system
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| What is secondary immunodeficiency? | results from extrinsic factors, such as drugs, irradiation, malnutrition or infection
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| What is most common cause of secondary immunodeficiency? | malnutrition
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| What is seen more, primary or secondary immunodeficiency? | secondary
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| What is hereditary angioedema? | C-1 inhibitor is deficient. recurrent edema of subcutaneous and submucoseal tissue.
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| What does C1 inhibitor do? | activation of the clotting, kinin, plasmin, and complement systems.
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| How do you diagnose an immunodeficiency? | recurrent infections; persistent or severe infections; unusual infections; paucity of lymphoid tissue; failure to thrive in infatns; skin lesions; oral and perineal candidiasis; diarrhea and malabsorption
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| What is the laboratory evaluation of immune competence? | serum Ig quantitation; cbc and differential; lymphocyte counts; phagocytic index; complement activity; response to vaccination
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| What is the major immunologic deficit in X linked agammaglobulinemia? | no B cells. affets male children
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| what is the treatment for x-linked agammaglobulinemia? | IVIg
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| What is the most commmon immunoglobulin deficiency? | IgA
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| What symptoms are seen with IgA deficiency? | asymptomatic or recurrent bacterial infections
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| What is severe combined immunodeficiency? | SCID; combined t and B cell defect. recurrent serious infections. fatal within 2 years w/o treatment.
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| What is wiskott-aldrich syndrome | Immunodeficiency with eczeme, thrombocytopenia and recurrent infections; defects in Ig and T cells
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| What are the 2 T adn B cell defects | SCID adn Wiskott-Aldrich syndrome
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| What is deficient in SCID? | ADA and PNP
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| Infants with SCID are prone to what types of infections? | oral candidiasis and opprotunistic diseases
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| Waht treatments are available for victims of SCID | bone marrow transplant; bubbles; gene therapy
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| How woudl deficiencies in MHC genes affect teh production of fucntional T cells | MCH will decide if a T cell will live or apoptose in teh thymus; deficiencies will not allow bad T cells to apoptose
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| What is DiGeorge's syndrome? | thymus and parathyroid fail to devleop.
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| What is the triad of DiGeorge? | hypercalcemia; dec T cells; congential heart disease
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| What is the treatment for DiGeorge? | thymic tissue graft or fetal thymus transplantation
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| What are extrinsic disorders of phagocytes? | defects in some other aspect of the immune or inflammatory response that diminish phagocytosis
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| What are intrinsic disorders of phagocytes? | involve defects in teh intracellular killing machinery
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| What is an example of an intrinsic phagocyte disorder? | chronic granulomatous disease
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| What is chronic granulomatous disease? | defects in intracellular killing; granuloma formation; age of onsite is 2 years; x-linked;
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| What is deficient adn defective in granulomatous disesase? | IFN-gamma and IL-12 defects and Rac2 deficiency; LEUKOCYTE ADHESION DEFICIENCY TYPES I and II; myeloperoxicase deficiency
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| What is the primary defect in leukocyte adhesion deficiency? | B2 integrin defect
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| What is seen in LAD? | High Blood PMN count; servere periodontitis, recurrent infections, early tooth decay
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