Immunodeficiencies

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Author:
mthoma98
ID:
86203
Filename:
Immunodeficiencies
Updated:
2011-06-10 20:59:44
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  1. Bruton's agammaglobulinemia
    • Boys, decreased CD19, decreased all Igs
    • Recurrent bacterial infections
    • Tx - IVIg
  2. Digeorge Syndrome
    • No CD3
    • No PTH
    • Recurrent viral/fungal infections
    • 22q11 deficiency
  3. SCID
    • Adenosine deaminase deficiency
    • Recurrent infec, chronic diarrhea, failure to thrive
    • NO LIVE VACCINES
  4. Chronic Mucocutaneous Candidiasis
    • Recurrent candida
    • T cell dysfxn
  5. Wiskott Aldrich
    • WAITER
    • immunodeficiency, thrombocytopenia (purpura), eczema, recurrent pyogenic infections
  6. Ataxia-telangiectasia
    • IgA deficiency
    • Cerebellar ataxia, poor smooth eye pursuit
    • Increased cancer (lymphoma, leukemia)
  7. IgA deficiency
    • asthma/allergies and sinus/lung infections
    • possible anaphylaxis to blood transfusion
  8. Chronic granulomatous disease
    • no NAPDPH oxidase activity
    • NBT dye does not change color
    • Infec with catalase positive: staph, e. coli, candida
    • Tx - TMP-SMX
  9. Chediak-Higashi Disease
    • defective lysosomal transport
    • giant cytoplasmic granules in PMNs
    • partial albinism
  10. Jobs syndrome
    • Hyperimmunoglobulin E syndrome
    • Increased IgE and eosinophils
    • eczema, staph aureus abscesses, course facial features
  11. Leukocyte adhesion deficiency syndrome
    • Phagocytes cannot exit circulation
    • Delayed separation of umbilicus

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