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16 Cards in this Set
- Front
- Back
Low levels of all Ig Tyrosine kinase mutation Normal CMI |
X-linked agammaglobulinemia Bruton's agammaglobulinemia *treat with pooled gamma globulin |
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Failure of isotype switching Recurrent bacterial sinus and lung infections |
Selective IgA Deficiency *do not treat with gamma globulin |
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Defect in B cell maturation to plasma cells Most common form of severe antibody deficiency |
Common variable immunodeficiency |
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X-linked: defect in IL-2 receptors in T cells Autosomal: ADA deficiency Bubble baby |
Severe combined ID |
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Specific T-cell deficiency for Candida Ablicans Recurrent candidiasis |
Chronic mucocutaneous candidiasis |
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Profound deficits of T cell Failure of development of thymus and parathyroid Defect in 3rd and 4th pharyngeal pouches Normal humoral immunity |
Di George syndrome SSx > tetany due to hypocalcemia Tx > transplabt of fetal thymus (<14wks to prevent graft vs host disease) CATCH 22 - TOF, abnormal facies, thymic aplasia, cleft palate, hypocalcemia, 22q11.2 chromosomal deletion |
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X-linked Inability to mount IgM response Mutation in WASP genr for actin-filament assembly |
Wiskott-Aldrich Syndrome > thrombocytpenia + infections + eczema + WASP |
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Autosomal recessive Mutations in DNA repair enzymes IgA deficiency |
Ataxia telangiectasia |
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Lack of NADPH oxidase activity Failure of oxidase activity |
Chronic granulomatous disease > A. fumigatus, widespread granulomas |
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phagolysosomal fusion Failure of phagolysosomal fusionAutosomal recessive Autosomal recessive e |
Chediak-Higashi |
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Mutation in integrin Autosomal recessive disease Defective LFA-1 proteins in the surface of phagocytes |
Leukocyte Adhesion Deficiency Lazy Leukocyte Disease |
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Most common complement deficiency |
C2 deficiency |
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Specific deficiency at C5-C9 Inability to form membrane attack complexes |
Terminal complement deficiency |
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Decreased supply of AA Decreased synthesis of IgG and complement |
Malnutrition |
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Loss of cell-mediated immunity Attacks CD4+ helper T-cells |
AIDS |
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Most common form of severe antibody deficiency affecting both children and adults |
Common variable immunodeficiency disease (CVID) |