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gm1147
on FreezingBlue Flashcards.
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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
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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
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Xlinked agammaglobulinemia: aka, presentation, dx, tx
- Bruton
- male kids, sinupulmonary infections
- Dec BN cells, dec lymphoid tissue, normal T cells
- Abx and regular IVIG
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SCID: presentation, dx, tx
- sinopulmonary, PCP, varicella, candida
- Dec B and T cells
- Bone marrow transplant
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IgA deficiency: presentation, tx
- sinopulmonary, atopic diseases, anaphylaxis to blood transfusion, fat malabsorption, other autoimmune
- Tx infections
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Hyper IgE syndrome
recurrent staph skin infections
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Wiskott-Aldrich syndrome
- Immunodeficiency + thrombocytopenia + eczema
- Deficient T cells
- Bone marrow transplant
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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
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