Immuno step2

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  1. Hereditary angioedema: cause, dx, tx
    • From C1 esterase inhibitor deficiency
    • Can be assoc with minor trauma
    • Dec levels of C2 and C4 and the enzyme
    • Tx with airway, FFP, long term androgens (danazol, stanazol) Does not respond to steroids
  2. CVID presentation. dx, tx
    • Adult, recurrent sinopulmonary infections, malabsorption, giardiasis, other autoimmune diseases, inc risk of lymphoma
    • All Ig subtypes are decreased and dec response to antigen stimulation of B cells (normal B cell # and normal lympoid tissue)
    • Abx, regular IVIG
  3. Xlinked agammaglobulinemia: aka, presentation, dx, tx
    • Bruton
    • male kids, sinupulmonary infections
    • Dec BN cells, dec lymphoid tissue, normal T cells
    • Abx and regular IVIG
  4. SCID: presentation, dx, tx
    • sinopulmonary, PCP, varicella, candida
    • Dec B and T cells
    • Bone marrow transplant
  5. IgA deficiency: presentation, tx
    • sinopulmonary, atopic diseases, anaphylaxis to blood transfusion, fat malabsorption, other autoimmune
    • Tx infections
  6. Hyper IgE syndrome
    recurrent staph skin infections
  7. Wiskott-Aldrich syndrome
    • Immunodeficiency + thrombocytopenia + eczema
    • Deficient T cells
    • Bone marrow transplant
  8. Chronic granulomatous disease: presentation, bugs, dx
    • LN with purulent material leaking, aphthous ulcers, nare inflammation, obstrucion of GI or urinary tracts
    • Staph, burkholderia, nocardia, aspergillus
    • Nitroblue tetrazolium test for dec resp burst/NADPH/hydrogen peroxide
Card Set
Immuno step2
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