amyloidosis

Question 1

what are the side effects associated with diflunisal

Answer 1

related to NSAID properties: bleeding, fluid retention, renal insufficiency, hypertension

Question 2

what are the side effects associated with tafamidis

Answer 2

diarrhea, stomachache, vaginal infection, UTI

Question 3

what are the side effects associated with patisiran

Answer 3

vitamin A deficiency, infection site rxn

Question 4

what are the side effects associated with inotersen

Answer 4

vitamin A deficiency, injection site rxn, fever, thrombocytopenia, glomerulonephritis

Question 5

what is patisiran FDA approved for

Answer 5

hATTR, neuropathy

Question 6

what is inotersen FDA approved for

Answer 6

hATTR, neuropathy

Question 7

what is tafamidis FDA approved for

Answer 7

cardiomyopathy, neuropathy

Question 8

what is diflunisal approved for

Answer 8

it is not FDA approved for these indications, off label use for cardiomyopathy and neuropathy

Question 9

what is the dose for patisiran

Answer 9

0.3 mg/kg (up to 30 mg) IV every 3 weeks

Question 10

what is the dose for inotersen

Answer 10

284 mg sq every week

Question 11

what is the dose for tafamidis

Answer 11

20, 61, 80 mg po

Question 12

what is the dose for diflunisal

Answer 12

250 mg po bid

Question 13

what organs does AL amyloidosis affect

Answer 13

any organ: heart, kidney, GI, tongue. nerves, liver, soft tissue

Question 14

what organs does wtATTR affect

Answer 14

heart and carpal tunnel syndrome

Question 15

what organs does hATTR affect

Answer 15

heart and nerves and carpal tunnel syndrome

Question 16

does AL or ATTR have more severe left ventricular hypertrophy

Answer 16

ATTR

Question 17

AL is contributed to the misfolding of what protein

Answer 17

immunoglobulin light chain

Question 18

ATTR is contributed to the misfolding of what protein

Answer 18

transthyretin

Question 19

what test diagnoses AL

Answer 19

haematologic tests (serum free light chain)

Question 20

what test diagnoses ATTR

Answer 20

PYP scintigraphy w/ SPECT

Question 21

cardiac signs of amyloidosis

Answer 21

dyspnea, fatigue, edema, conduction abnormalities, LV/RV/atrial wall thickening, persistent low-level troponinemia

Question 22

neuro signs of amyloidosis

Answer 22

orthostatic hypotension, paresthesia, weakness

Question 23

ortho signs of amyloidosis

Answer 23

carpal tunnel syndrome, lumbar spinal stenosis, hip and knee arthroplasty

Question 24

“other” weird signs of amyloidosis

Answer 24

weight loss, foamy urine