SECRETS Endo - MEN, FHH

Question 1

MEN I

Answer 1

pituitary adenoma
pancreatic neuroendocrine tumor (Zollinger-Ellison)
hyperparathyroidism

menin tumor suppressor gene

Question 2

MEN IIa

Answer 2

medullary thyroid carcinoma
pheochromocytoma
hyperparathyroidism

Cutaneous lichen amyloidosis

RET proto-oncogene

Question 3

MEN IIb

Answer 3

medullary thyroid carcinoma
pheochromocytoma
mucosal neuromas
intestinal ganglioneuromas
marfanoid habitus

RET protooncogene

Question 4

Zollinger Ellison

Answer 4

excessive gastrin –> excessive HCl –> PUD

gastrinoma in duodenum or pancreas

if in pancreas, think MEN I

Question 5

hypercalcemia causes

Answer 5

primary hyperparathyroidism (MEN)
hypercalcemia of malignancy
calcium antacids
drugs (thiazide diuretics, lithium)
paraneoplastic syndromes
FHH
excessive vitD

Question 6

FHH

Answer 6

deficient renal calcium excretion –> hypercalcemia

AD
mut in CASR gene

modest elevation in PTH, rare kidney stones

mild hypercalcemia
hypocalcuria

Question 7

CASR gene

Answer 7

in parathyroid glands
mediates feedback inhibition of PTH secretion

mut (FHH) = disinhibits PTH
higher than normal serum Ca level required to inhibit PTH

Question 8

PTH

Answer 8

from chief cells in parathyroid gland

responds to low serum Ca

raises serum Ca

stimulates 1a-hydroxylase in kidney –> more active VitD

Question 9

Vit D

Answer 9

from diet & synthesis through cholestrol with UV light

raise serum Ca

Question 10

Calcitonin

Answer 10

from parafollicular diets in thyroid

responds to high serum Ca

lowers serum Ca

Question 11

Vit D activation

Answer 11

inactive D3 from skin/diet

25-hydroxylase in liver = 25(OH)D3

1a-hydroxylase in kidney = 1,25(OH)D3 – active

PTH stimulates 1a-hydroxylase