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12 Cards in this Set
- Front
- Back
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Focal three-layered necrotizing inflammation of arteries only:
What three Ag's can be seen in polyarteritis nodosa? How is it different from other inflammatory vessel diseases? |
polyarteritis nodosa
Hep B, Hep B, cryoglobulins does not involve the lungs |
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abnormal IGA deposition in skin and kidney usually seen in children:
Affects arteries, veins, lungs, anti-myeloperoxidase, p-ANCA, Ab's against neutrophils: |
Henoch-Schonlein purpura
microscopic polyangitis, small vessel polyarteritis |
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Patients usually have eye, ear, nose, throat symptoms, Ab's against proteinase 3:
What vessels/organs are usually affected? Vasculitis with markedly increased eosinophils, +ANCA: |
Wegener's granulomatosis
necrosis/inflammation of vessels in kidney, lungs, spleen Churg-Strauss |
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Ulcers in mouth and on genitals, inflammation of iris/ciliary body:
What is the immune defect? What is a positive pathergy test? It is a cause of blindness in what country? |
Behcet's disease
T-cells respond to heat shock proteins stick pt's arm with sterile needle, look for blisters in 24-48 hrs. Japan |
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"Amyloid" means what?
What is the general pathology of amyloidosis? Alzheimer's is what amyloid? Amylin in islets of Langerhans is characteristic of what disease? |
starch-like
abnormal B-pleated, homogeneous, insoluble proteins accumulate, push out normal tissue Beta/A4 amyloid Type II DM |
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Amyloid has apple-green birefringence with what dye?
The liver making abnormal AAP is what type of amyloidosis? Bedsores and chronic infections (TB, syphilis, CF) are associated with which amyloidosis? |
Congo red
Amyloidosis AA Amyloidosis AA |
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Abnormal transthyretin (prealbumin), usually only significant if the heart is involved:
B-2 microglobulin, HLA light chain involved, seen with chronic renal failure: |
Amyloidosis AF
Amyloidosis H |
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What happens to the following organs in amyloidosis?
Kidney: Spleen: Heart: GI: Adrenal: |
Kidney - leaky gloms, nephrotic syndrome (major COD)
Spleen - "tapioca" - white pulp; "lardaceous" - red pulp Heart - restrictive cardiomyopathy (#1 cause) GI - stiff tongue, malabsorption, abnormal peristalsis Adrenal - Addisonism |
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How is the lung affected by amyloid? (3)
What drug interferes with polymerization in amyloidosis? |
nodules
diffuse interstitial infiltration (dyspnea) tracheobronchial amyloid (airway obstruction) Eprodisate |
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Small lymph nodes, no germinal centers, BTK mutation:
most common immunodeficiency, increased risk of Giardia infections: 22q11 deletion, thymic dysembryogenesis, facial abnormalities: |
Bruton's (XL Agammaglobuline
IGA deficiency DiGeorge syndrome |
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50% of AR SCID is what disorder?
David Veter, the "bubble boy", had what deficiency? |
ADA deficiency
XL SCID |
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WASP gene defect, immunodeficiency, thrombocytopenia, eczema:
Hereditary angioedema is what defect? Which complement defect is most common? |
Wiskott-Aldrich
C1 esterase complement deficiency C2 most common |
