Inflammation V

Question 1

What is the mechanism of cell damage in iron poisioning?

Answer 1

Cell death due to peroxidation of membrane lipids (p.217)

Question 2

What are the acute symptoms of iron poisioning?

Answer 2

Gastric bleeding (p.217)

Question 3

What are the chronic symptoms of iron poisioning?

Answer 3

Metabolic acidosis, scarring leading to GI obstruction (p.217)

Question 4

Describe the pathology of amyloidosis.

Answer 4

An abnormal aggregation of proteins or their fragments into b-pleated sheet structures, leading to cell damage and apoptosis (p.217)

Question 5

Describe the appearance of tissue affected by amyloidosis.

Answer 5

Affected tissue has a waxy appearance (p.217)

Question 6

Describe the appearance of amyloidosis in vasculature when stained.

Answer 6

Apple-green birefringence of amyloid deposits in an artery wall with a Congo Red stain (p.217)

Question 7

What stain is used to detect amyloidosis in vasculature?

Answer 7

Congo Red stain (p.217)

Question 8

What causes AL (primary) amyloidosis?

Answer 8

Caused by deposition of proteins from Ig Light chains. Can occur as a plasma cell disorder or is associated with multiple myeloma (p.217)

Question 9

What conditions are associated with primary AL amyloidosis?

Answer 9

Multiple Myeloma, plasma cell disorders (p.217)

Question 10

What six organs are impacted in primary AL amyloidosis?

Answer 10

Multi- organ system impact includes: renal (nephrotic syndrome), cardiac (heart failure, arrythmia), hematologic (easy bruising), hepatomeagly, and neuropathy (p.217)

Question 11

What disease states are associated with secondary AA amyloidosis?

Answer 11

Chronic diseases such as RA, IBD, spondyloarthropahty, chronic infections (p.217)

Question 12

Which organ systems are impacted in secondary AA amyloidosis?

Answer 12

Often multisystem (like primary AL amyloidosis) (p.217)

Question 13

What are the amyloid deposits of secondary AA composed of?

Answer 13

Fibrils composed of serum Amyloid A (p.217)

Question 14

What are the amyloid deposits of dialysis related amyloidosis composed of?

Answer 14

Fibrils composed of B2-microglobulin in patients with end stage renal disease and on long term dialysis (p.217)

Question 15

How does dialysis related amyloidosis most commonly present?

Answer 15

As carpal tunnel syndrome and other joint issues (p.217)

Question 16

What is heritable amyloidosis?

Answer 16

A heterogeneous group of disorders including ATTR neurologic/cardiac amyloidosis due to transthyretin (TTR or prealbumin) gene mutation (p.217)

Question 17

What genetic mutation causes ATTR neurologic/cardiac amyloidosis?

Answer 17

Mutation in transthyretin (TTR or prealbumin) gene mutation (p.217)

Question 18

Describe the amyloid deposition associated with age related (senile) systemic amyloidosis.

Answer 18

Deposition of normal (wild-type) TTR in myocardium and other sites (p.217)

Question 19

How does the progression of cardiac vs. AL amyloidosis compare in age-related (senile) systemic amyloidosis?

Answer 19

Slower progression of cardiac dysfunction vs. AL amyloidosis (p.217)

Question 20

What is organ specific amyloidosis?

Answer 20

Amyloid deposition localized in a single organ (p.217)

Question 21

What is the most common form of organ specific amyloidosis?

Answer 21

Alzheimers Disease (p.217)

Question 22

Describe the amyloid deposition in Alzheimer’s Disease.

Answer 22

Deposition of amyloid-b-protein cleaved from amyloid prescursor protein (APP) (p.217)