Amyloidosis

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Summary1)First Aid for the Basic Sciences: Organ Systems by Tao Le

Amyloidosis is the deposition of amyloid in vessel walls.

Pathophysiology2)Amyloidosis (Wikipedia)

The fragments or actual proteins are at risk of misfolding as they are synthesized, to make a poorly functioning protein but are destroyed via proteolysis. The problem occurs when the proteins do not dissolve in proteolysis. This happens because the misfolded proteins sometimes become robust enough that they are not dissolved by normal proteolysis. When the fragments do not dissolve, they get spit out of proteolysis and they aggregate to form oligomers which can aggregate together and further stabilize to make amyloid fibrils. Both the oligomers and amyloid fibrils are toxic to cells and can interfere with proper organ function.

Histology – Amyloid displacing tissues (PathPedia)

Causes3)Amyloidosis (Wikipedia)

  • Accumulation of misfolded proteins

Symptoms 4)Fundamentals of Pathology by Hussain Sattar

  • Nephrotic syndrome
  • Restrictive cardiomyopathy or arrhythmia
  • Tongue enlargement, malabsorption and hepatosplenomegaly

Diagnosis

  • Tissue Biopsy – amyloid deposition

Gross Pathology – Waxy and enlarged appearance due to amyloid deposits (Clinical Science Blog)

Treatment 5)Fundamentals of Pathology by Hussain Sattar

  • Transplant of damaged organs

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