Endocrine Flashcards

1
Q

where is the drainage of the L adrenal vein?

A

left renal vein

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2
Q

where is the drainage of the R adrenal vein?

A

inferior vena cava

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3
Q

what is CRH?

A

corticotropin-releasing hormone

  • released from anterior hypothalamus
  • causes release of ACTH from anterior pituitary
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4
Q

what is ACTH?

A

adrenocorticotropic hormone

  • released normally by anterior pituitary
  • causes adrenal gland to release cortisol
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5
Q

what feeds back to inhibit ACTH secretion?

A

cortisol

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6
Q

what is Cushing’s syndrome?

A

excessive cortisol production

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7
Q

what is the most common cause of Cushing’s syndrome?

A

iatrogenic

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8
Q

what is the second most common cause of Cushing’s syndrome?

A

Cushing’s disease

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9
Q

what is Cushing’s disease?

A

Cushing’s syndrome caused by excess production of ACTH by anterior pituitary

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10
Q

what is an ectopic ACTH source?

A

tumor not found in the pituitary that secretes ACTH, which in turn causes adrenal gland to release cortisol without the normal negative feedback loop

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11
Q

what are the sign/symptoms of Cushing’s syndrome?

A

truncal obesity, hirsutism, ‘moon’ facies, acne, ‘buffalo hump’, purple striae, HTN, diabetes, weakness, depression, easy bruising, myopathy

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12
Q

how can cortisol levels be indirectly measured over a short duration?

A

urine cortisol or breakdown product of cortisol (17-OHCS)

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13
Q

what is a direct test of serum cortisol?

A

serum cortisol level

  • highest in the morning
  • lowest at night
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14
Q

what initial tests should be performed in Cushing’s syndrome?

A

electrolytes
serum cortisol
urine-free cortisol, urine 17-OHCS
low-dose dexamethasone-suppression test

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15
Q

what is the low-dose dexamethasone suppression test?

A

dexamethasone is a synthetic cortisol that results in negative feedback on ACTH secretion and subsequent cortisol secretion in healthy patients

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16
Q

what happens in the low-dose dexamethasone suppression test in patients with Cushing’s syndrome?

A

dexamethasone does not suppress their cortisol secretion

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17
Q

after the dexamethasone test in Cushing’s workup, what is next?

A

check ACTH levels

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18
Q

in ACTH-dependent Cushing’s syndrome, how do you differentiate between pituitary vs. ectopic ACTH source?

A

high-dose dexamethasone test

  • pituitary source: cortisol is suppressed
  • ectopic ACTH source: no cortisol suppression
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19
Q

Treatment of adrenal adenoma:

A

adrenalectomy

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20
Q

treatment of adrenal carcinoma

A

surgical excision

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21
Q

treatment of ectopic ACTH-producing tumor

A

surgical excision, if feasible

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22
Q

treatment of Cushing’s disease

A

transphenoidal adenomectomy

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23
Q

what is a complication of bilateral adrenalectomy?

A

Nelson’s syndrome

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24
Q

What is Nelson’s syndrome?

A

functional pituitary adenoma producing excessive ACTH and mass effect producing visual disturbances, hyperpigmentation, amenorrhea, with elevated ACTH levels

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25
Q

what is pheochromocytoma?

A

tumor of the adrenal medulla and sympathetic ganglion (from chromaffin cell lines)
- produces catecholamines (NE > epi)

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26
Q

what are the associated risk factors of pheochromocytoma?

A

MEN-II, fam hx, von Recklinghausen disease, von Hippel-Lindau disease

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27
Q

what are the signs/symptoms of pheochromocytoma?

A

classic triad:

  • hypertension
  • headache
  • episodic diaphoresis

also, HTN, pallor-> flushing, anxiety, weight loss, tachycardia, hyperglycemia

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28
Q

how can the pheochromocytoma symptoms triad be remembered?

A

PHEochromocytoma

  • Palpitations
  • Headache
  • Episodic diaphoresis
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29
Q

what diagnostic tests should be performed for suspected pheochromocytoma?

A

urine screen: VMA, metanephrine, and normetanephrine

urine/serum epinephrine/norepinephrine levels

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30
Q

what are other common lab findings in pheochromocytoma?

A
hyperglycemia
- epinephrine -> increase glucose
- norepinephrine -> decrease insulin
polycythemia
- resulting from intravascular volume depletion
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31
Q

what is the classic pheochromocytoma ‘rule of 10’s’?

A
10% malignant
10% bilateral
10% in children
10% multiple tumors
10% extra-adrenal
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32
Q

what is the preoperative/medical treatment of pheochromocytoma?

A

increase intravascular volume

  • α-blockade: phenoxygenzamine or prazosin
  • to allow reduction in catecholamine-induced vasoconstriction and resulting volume depletion
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33
Q

in the patient with pheochromocytoma, what must be ruled out?

A

MEN-II

- almost all cases are bilateral

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34
Q

what is the organ of Zuckerkandl?

A

body of embryonic chromaffin cells around the abdominal aorta (heart he IMA)
- normally atrophies during childhood, but is the most common site of extra-adrenal pheochromocytoma

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35
Q

what is Conn’s syndrome?

A

primary hyperaldosteronism

- due to high aldosterone production

36
Q

what are the common sources of Conn’s syndrome?

A

adrenal adenoma or adrenal hyperplasia

37
Q

what is the normal physiology for aldosterone secretion

A

low BP in renal afferent arteriole & hyponatremia/hyperkalemia -> renin secretion from juxtaglomerular cells -> angiotensinogen -> angiotensin I -> ACE from the lung -> angiotensin II -> adrenal glomerulosa cells secrete aldosterone

38
Q

what is the normal physiologic effect of aldosterone?

A

sodium retention for exchange of potassium in kidney

  • fluid retention
  • increased BP
39
Q

what are the sign/symptoms of Conn’s syndrome?

A

hypertension, headache, polyuria, weakness

40
Q

what are the two classic clues of Conn’s syndrome?

A

hypertension

hypokalemia

41
Q

what is Addison’s disease?

A

acute adrenal insufficiency

42
Q

what are the renin levels in patients with primary hyperaldosteronism?

A

normal or low

43
Q

what are the electrolyte findings in Addison’s disease?

A

hyperkalemia

hyponatremia

44
Q

what is an insulinoma?

A

insulin-producing tumor arising from ß-cells

45
Q

what are the associated risks of an insulinoma?

A

MEN-I syndrome

  • pituitary
  • pancreas
  • parathyroid tumors
46
Q

what are the signs/symptoms of an insulinoma?

A

sympathetic nervous system symptoms resulting from hypoglycemia
- palpitations, diaphoresis, tremulousness, irritability, weakness

47
Q

what are the neurological symptoms of an insulinoma?

A

personality changes, confusion, obtundation, seizures, coma

48
Q

what is Whipple’s triad?

A
  • hypoglycemic symptoms produced by fasting
  • blood glucose <50 during symptomatic attack
  • relief of symptoms by administration of glucose
49
Q

what lab tests should be performed in suspected insulinoma?

A

fasting hypoglycemia with inappropriately high levels of insulin
- 72hr fast, then check glucose & insulin q 6hrs

50
Q

what is the medical treatment of an insulinoma?

A

diazoxide

51
Q

what is a glucagonoma?

A

glucagon-producing tumor

52
Q

where is a glucagonoma located?

A

pancreas (usually in the tail)

53
Q

what are the symptoms of a glucagonoma?

A

necrotizing migratory erythema (usually below the waist), glossitis, stomatitis, diabetes

54
Q

what are the skin findings in a glucagonoma?

A

necrotizing migratory erythema

  • red, often psoriatic-appearing rash
  • serpiginous borders
  • over the trunk and limbs
55
Q

what stimulation test is used for a glucagonoma?

A

tolbutamide stimulation test

- IV tolbutamide -> elevated glucagon levels

56
Q

what test is used for localization of a glucagonoma?

A

CT scan

57
Q

what is the medical treatment of necrotizing migratory erythema?

A

somatostatin

IV amino acids

58
Q

what is the treatment of a glucagonoma?

A

surgical resection

59
Q

what is a somatostatinoma?

A

pancreatic tumor that secretes somatostatin

60
Q

what is the diagnostic triad of a somatostatinoma?

A

DDD

  • diabetes
  • diarrhea (steatorrhea)
  • dilation of the gallbladder with gallstones
61
Q

what is used to make the diagnosis of a somatostatinoma?

A

CT scan and somatostatin level

62
Q

what is the treatment of a somatostatinoma?

A

resection

63
Q

what is Zollinger-Ellison syndrome?

A

gastrinoma

  • non-ß islet cell tumor of the pancreas
  • produces gastrin -> gastric hyper secretion of HCl -> GI ulcers
64
Q

what is the associated syndrome of Zollinger-Ellison syndrome?

A

MEN-I

  • pituitary
  • pancreas
  • parathyroid
65
Q

with gastrinoma, what lab tests should be ordered to scream for MEN-I?

A

calcium level

PTH level

66
Q

what are the signs/symptoms of Zollinger-Ellison syndrome?

A

peptic ulcers, diarrhea, weight loss, abdominal pain

67
Q

what causes the diarrhea in Zollinger-Ellison syndrome?

A

massive acid hyper secretion and destruction of digestive enzymes

68
Q

what are the signs of Zollinger-Ellison syndrome?

A

peptic ulcer disease
- epigastric pain, hematemesis, melena, hematochezia
GERD, diarrhea, recurrent ulcers, ulcers in unusual locations

69
Q

what is the secretin-stimulation test?

A

IV secretin is administered and gastrin level is determined

- patients with Z-E syndrome will have a paradoxic increase in gastrin

70
Q

what are the classic secretin stimulation results?

A

NL - decreased gastrin

Z-E syndrome - increased gastrin

71
Q

define Passaro’s triangle

A

aka ‘gastrinoma triangle’

  • cystic duct/CBD junction
  • junction of the second and third portions of the duodenum
  • neck of the pancreas
72
Q

what is the medical treatment for Zollinger-Ellison syndrome?

A

H2 blockers, omeprazole, somatostatin

73
Q

what is multiple endocrine neoplasia also known as?

A

MEN syndrome

74
Q

what is MEN?

A

inherited condition of propensity to develop multiple endocrine tumors

75
Q

how is MEN inherited?

A

AD with significant degree of variation in penetrance

76
Q

which patients should be screened for MEN?

A

all family members of patients diagnosed with MEN

77
Q

what is the gene defect in MEN-I?

A

chromosome 11

78
Q

what are the most common tumors of MEN-I?

A
parathyroid hyperplasia
pancreatic islet cell tumors
- gastrinoma: ZE syndrome
- insulinoma
pituitary tumors
79
Q

what are the highest-yield screening lab tests for MEN-I?

A

calcium
PTH
gastrin
prolactin

80
Q

what is the genetic defect in MEN-IIa?

A

RET

81
Q

what are the most common tumors of MEN-IIa?

A

MPH

  • medullary thyroid carcinoma (calcitonin secreted)
  • pheochromocytoma (catecholamine excess)
  • hyperparathyroidism (hypercalcemia)
82
Q

what are the best screening lab tests for MEN-II?

A
calcitonin level
calcium levels
PTH
catecholamines and metabolites
RET gene testing
83
Q

what are the most common abnormalities in MEN-IIb?

A

MMMP

  • mucosal neuromas
  • medullary thyroid carcinoma
  • marfanoid habitus
  • pheochromocytoma
84
Q

what is the anatomic distribution of medullary thyroid carcinoma in MEN-II?

A

almost always bilateral

85
Q

what are the physical findings/signs of MEN-IIb?

A

mucosal neuromas
marfinoid habitus
pes cavus/planum
constipation

86
Q

what is the major difference between MEN-IIa and MEN-IIb?

A
MEN-IIa = parathyroid hyperplasia
MEN-IIb = NO parathyroid hyperplasia
87
Q

what type of parathyroid disease is associated with MEN-I and MEN-IIa?

A

hyperplasia