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amyloidosis

amyloidosis

introduction

  • a variety of conditions in which amyloid proteins are deposited in tissues.
  • a protein is described as being amyloid if, due to an alteration in its secondary structure, it takes on a particular aggregated insoluble form similar to the beta-pleated sheet.

classification

  • the modern classification of amyloid disease tends to use an abbreviation of the protein that makes up the majority of deposits, prefixed with the letter A.
  • see wikipedia for details

pathologic features

  • most clinical effects are via parenchymal ischaemia ⇒ atrophy due to blood vessel compression, but also cause impaired function such as in glomeruli, cardiac muscle, tongue, etc;
  • spleen: sago spleen as confined to Malpighian corpuscles
  • liver: in space of Disse in liver ⇒ atrophy may cause portal HT, jaundice, liver failure;
  • kidneys - in glomeruli, arterioles, interstitial tissue b/n tubules ⇒ nephrotic synd, small scarred, type II renal tubular acidosis (RTA)
  • heart - enlarged, rigid, firm as b/n muscle fibres & in walls small blood vessels
    • ⇒ constrictive pericarditis-like effect;, HB, arrhythmias;
  • GIT ⇒ tongue ⇒ dysphagia, speech probs; obstruction/ulceration/malabsorption/diarrhoea;
  • lungs ⇒ nodules may obstruct; infiltrative ⇒ haemoptysis, restrictive failure;
amyloidosis.txt · Last modified: 2016/04/23 13:21 (external edit)