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37 Cards in this Set
- Front
- Back
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Chronic Granulomatous Disease
(defect, infection, test) |
NADPH Oxidase defect
opportunistic infections (-) nitroblue tetrazolium test |
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DiGeorge
(defect, infection) |
Failure of 3rd, 4th branchial pouches --> no thymus, parathyroids, low T
viral and fungal infections hypoCa |
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HLA DR7
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Steroid responsive nephrotic syndrome
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HLA DR5
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Pernicious anemia
Hashimoto |
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Ataxia-telangectasia
(defect, findings) |
DNA repair defect
low IgA, ataxia, spider angiomas hypoplastic thymus, ovaries |
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IL-12 deficiency
(infection) |
Recurrent mycobacterial infections
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HLA B8
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Graves
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HLA DR2
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MS
SLE Goodpasture's |
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HLA A3
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Hemochromotosis
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Selective Ig deficiency
(defect, most common sequellae) |
defect in isotype switching
usually no IgA --> lung infections, diarrhea, allergy to blood products with IgA |
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IL-5
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TH2 response
class witching to IgA eosinophil activation |
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Common Variable Immunodeficiency
(defect, succeptibility) |
nl B cells, low plasma cells, low Ig
increased autoimmune diseases and lymphoma |
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HLA B27
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Ankylosing Spondylitis
Reiter's Psoriasis IBD |
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hyper IgM
(defect, succeptibility) |
CD40L defect --> no class switching
defective macrophage activation recurrent pyogenic infections |
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Chediak Higashi
(genetics, defect, succeptibility, findings) |
AR
microtubule defect --> lysosomal abnormality recurrent pyogenic infections with staph and strep albinism peripheral neuropathy |
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MAC defect
(defect, succeptibility) |
C5-9 deficiency
recurrent Neisseria infection |
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Wiskott-Aldrich
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XR
no IgM response to capsular polysaccharides increased IgA, IgE, low IgM recurrent pyogenic infections deformed plts, eczema, TTP |
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IL-4
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TH2 response
stims IgE production |
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Chronic Mucocutaneous Candidiasis
(defect) |
T cell dysfn specifically against candida
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Bruton's
(genetics, defect, succeptibility) |
XR
tyr kin defect low B cells recurrent bacterial infections, autoimmune |
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IL-8
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secreted by M0
causes neutrophil chemotaxis |
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Job's
(defect, findings) |
no IFNg secretion by Th cells --> no PMN chemotaxis
coarse Facies, non-inflammed staph Abscesses, retained primary Teeth, high IgE, Dermatitis FATED |
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IL-10
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secreted by Treg
inhibits T cell activity |
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IL-7
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stims lymphocyte precursor proliferation
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HLA DR3
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Type 1 DM
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TGF B
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Causes class switch to IgA
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IFNg
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from TH1, NK
stimulates M0 |
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IL-1
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from M0, inflammatory cytokine
activates DCs, endothelium recruits leukocytes pyrogen |
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IL-2
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from T cells
growth of TH cells |
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TNF
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from M0, inflammatory
causes septic shock leukocyte recruitment DC activation |
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LAD
(defect, succeptibility, findings) |
LFA-1 defect
recurrent bacterial infections w neutrophilia no pus delayed umbilical separation |
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HLA DR4
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Rheumatoid Arthritis
Type 1 DM |
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IL-3
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from activated T cells
stims BM growth and differentiation |
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SCID
(genetics, defect, findings) |
XR
IL-2Rg deletion --> can't respond to cytokins or Adenosine deaminase deficiency (build up of toxic metabolites) or RAG mutations recurrent everything, no B or T |
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IL-12
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From B and M0
activates TH1, NK |
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Bare Lymphocyte Syndrome
(defect, findings) |
No MHC II
low CD4+ T cells |
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IL-6
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from TH, M0
production of acute phase reactants osteoclast activation (inhibited by estrogen) |
