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Multisystem entities

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amyloidosis


rare, usually systemic disease
  • extracellular deposition of insoluble B-pleated sheet configured protein that stains with Congo red

  • systemic (80-90%)
    • primary
      • M > F
      • 55-60y
      • often presents with renal insufficiency or CHF
    • secondary
  • localized (10-20%)

manifestations

  • chest
    • tracheal nodules/narrowing
    • pulmonary nodules
    • diffuse parenchymal pattern
  • heart: enlargement (Tc-99m pyrophosphate positive)
  • kidneys
    • large kidneys
    • small kidneys
    • focal masses
    • calcifications
  • bladder/ureters: focal or diffuse infiltration
  • bones: pain/weakness associated with lytic lesions +/- calcium
  • joints (shoulder, elbow, wrist, hip)
    • ST enlargement
    • subchondral cysts
  • gastrointestinal tract


Brian Funaki, MD  -  14 February 1995
Last updated:  1 October 2013


 

Related CHORUS documents:
amyloidosis: gastrointestinal manifestations
phakomatoses
Beckwith-Wiedemann syndrome
blue rubber bleb nevus syndrome
Carney syndrome
adenoma sebaceum

 

Copyright © 2013, Charles E. Kahn, Jr.