Amyloid CIS Flashcards
pathophysiology of amino acid amyloidosis
inflammation triggers hepatic production of SAA protein
inadequate breakdown and/or excessive accumulation results
this causes accumulation of AA amyloid
amyloidosis causes
alzheimer dementia
sometimes you notice the amyloid first
how SAA becomes AA protein
chronic inflammation leads to macrophage activation
this releases IL 1 and 6 to the liver cells which makes SAA
limited proteolysis leads to AA proteins being made.
How to diagnose AZD?
renal biopsy after presentation with kidney dysfunction
amyloids form (AA)
beta pleated sheets and oligomerize and become ultra stable
because of this, the fibril cannot break down easily, so it continues to accumulate
Amyloids can accumulate even with a good genetic protein breakdown system, but if someone has acquired genetic/heritable mutations, the protein degradation system can (AL)
be impaired!
protein can still be broken down, but less efficiently
beta pleated sheets are going to accumulate like normal with an intrinsic inability to degrade proteins efficiently
There is also a non-degradable amyloid protein that is mutated
person has a normal degradation system, but the amyloid is (ATTR)
totally unable to be broken down
Why was a renal biopsy done?
renal issues show up as peripheral edema typically dependent (sinks down to the ankles)
why does PE happen?
protein is excreted in urine too much, and blood fluid can be pushed through the vessels and cause edema
why do i think amyloid is present on the slide?
pink deposits, need to prove it
confirmatory testing for amyloid
congo red or electron microscopy
congo red
binds between fibrils of amyloid and looks red but then use polarized light and shows apple green birefringence
confirms amyloid presence
electorn microscopy
can identify fibrils directly
what kind of amyloid is it?
AA amyloid stain is positive (brown is positive)
diagnosis is
AA amyloidosis based on case study in class
peripheral edema, proteinuria, biopsy, green stain, brown stain–> diagnosis!
