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14 Cards in this Set

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What are the Amyloidoses?
A group of diseases with extracellular accumulation of fibrillar protein deposits.
What causes the extracellular protein aggregates to form in amyloidosis?
Misfolding
Are the misfolded proteins mutated in amyloidosis?
Not necessarily; they can be.
What should happen to proteins normally when they're misfolded?
Proteolysis
Why aren't the misfolded proteins that aggregate and cause amyloidosis proteolysed?
Because they're either resistant to proteolysis, or the proteolytic enzyme may not achieve complete proteolysis.
What is the diagnostic test for amyloidosis?
Apple green birefringence of Congo red dye under polarized light.
What is Amyloid AA associated with?
Inflammation
What is Amyloid AL associated with? What is the protein precursor that accumulates?
Plasma cell dyscrasia
-Kappa or lambda light chains
What is Amyloid A-beta?
-Alzheimer's
-Congophilic angiopathy
What is Amyloid-Abeta2M?
Chronic dialysis induced aggregation of B2microglobulin
What do 89% of patients with AL amyloidosis have? What do 70% have?
Mprotein = 89%
Lambda = 70%
What do 5-15% of patients with myeloma have?
Coexistent amyloidosis
What are 5 complications of amyloid deposition?
-Nephrotic syndrome or renal insufficiency
-Congestive heart failure
-Conduction disturbances and arrythmias
-Peripheral neuropathy
-Carpal tunnel syndrome
What are 4 other complications that may be seen in amyloidosis?
-Hepatomegaly
-Splenomegaly
-Enlargment of the tongue
-Idiopathic malabsorption diarrhea or digestion problems