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immuno deficiencies

WVSOM -- Immuno/Micro -- Immunodeficiency Diseases

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