Amyloidosis

Question 1

What is Amyloid

Answer 1

Misfolded protien- amorphous pink material

Question 2

Stain with congo red
look under light microscopy

Answer 2

Congo red- Stain with apple green birefiringince (under polarized light)

under EM- Beta plated sheets

Question 3

what are the 3 ways to get amyloydosis

Answer 3

1. Excess amounts ofnormal protein created by the body
2. Accumulation of protein the body cant get rid of
3. Mutations in proteins- misfolding protiens

Question 4

Amyloidosis most likely affected organs

Answer 4

1. kidneys- Renal amyloidosis
2. Heart - HFpEF&raquo_space; Restrictive cardiomyopathy&raquo_space;Thickened ventricles + Dilated LA

Question 5

most common cause of death from amyloidosis?

Answer 5

Heart issues

restricitve cardiomyopathy, HFpEF

Question 6

what are the 2 main causes of increased production amyloidosis , which problems each one will cause

Answer 6

1. Multiple myeloma- Serum Electrophoresis- AL (immunoglobulin light chains) * with (MM+) constipation = hypercalcemia
2. Chronic inflammation (RA act..)- Serum Amyloid A (AA) -> Renal deposits = Nephrotic syndrome secondary to amyloidosis

Question 7

amyloidosis Age raleted
name of protien accumulates, other name for this type

Answer 7

other name- Senile sys. amyloidosis&raquo_space; **Prealbumin (transtyretin) **» overtime process

Question 8

Which substance accumulate in CKD/ Dialyss related amyloidosis

Answer 8

Beta-2 microglobulins

accumulation and tissue deposition of amyloid fibrils consisting of beta2-microglobulin (beta2-m) in the bone, periarticular structures, and viscera of patients with end-stage kidney disease

Question 9

who can Down syn. cause amyloidosis

Answer 9

Accumulation of APP = plaques = Amyloid angiopathy = early onset dementia

מחלה של מבוגרים בד”כ אבל בדאון מופיעה בגיל צעיר. אלצהיימר בגיל צעיר.

Question 10

Parkinson like syndrome + multiple falls and visual halluciantion?

what the disease, whats bulid up in this disease

Answer 10

Lewy body dementia
accumulation of amyloid alpha synuclein

Question 11

Creutzfeldt Jakoc disease:
presentation + whats build up

Answer 11

young patients with rapidly progressive dementia + neuropatholgies

PrPsc protein build up

Question 12

Type II DM
whats bulid up and where

Answer 12

Amylin depositions in the pancreas

Question 13

Neck mass + amyloidosis

Answer 13

Thyroid cancer&raquo_space; MEN2

MEN 2- amyloidosis + interstitial nuromas ( MEN2B) -» Constipation

cutaneous lichen amyloidosis (CLA) during their lifetime