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EndoRepro 2 Exam 2 > Adrenal HYHO - Newman > Flashcards

Adrenal HYHO - Newman Flashcards

1
Q

what zone of the adrenal cortex produces mineralocorticoids?

A

glomerulosa

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2
Q

what zone of the adrenal cortex produces glucocorticoids?

A

fasciculata

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3
Q

what zone of the adrenal cortex produces sex steroids?

A

reticularis

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4
Q

what does the short term stress response involve?

A

stimulation of the adrenal medulla via preganglionic sympathetic fibers resulting in the release of catecholamines (Epi and NE)

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5
Q

what are the side effects of short term stress response?

A
  • increased HR
  • increased BP
  • glycogen conversion to glucose in the liver
  • dilaation of bronchioles
  • changes in blood flow patterns leading to decreased digestive system activity and reduced urine output
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6
Q

what does the long term stress response involve?

A

CRH stimulation of the anterior pituitary, stimulation of the adrenal cortex by ACTH, and release of mineralocorticoids and glucocorticoids

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7
Q

what are the effects of mineralocorticoid (aldosterone) release?

A
  • retention of Na and H2O by kidneys

- increased blood volume and blood pressure

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8
Q

what are the effects of glucocorticoids (cortisol) release?

A
  • protein and fat are converted to glucose or broken down for energy
  • increased blood glucose
  • suppression of immune system
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9
Q

what does the RAAS system regulate?

A

renal, cardiac and vascular physiology

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10
Q

what stimulates renin secretion?

A

decreased renal perfusion and/on increased sympathetic activity

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11
Q

what does renin do?

A

it converts Angiotensinogen -> Ang I

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12
Q

what does Ang II stimulate?

A

aldosterone secretion from the adrenal cortex

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13
Q

what is the MCC of ambiguous genitalia in a female?

A

congenital adrenal hyperplasia (causing virilization of the genitalia)
- CAH can be life threatening MUST be on the differential is ambiguous genitalia are found

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14
Q

what is the most common form of CAH?

A
  • *21-hydroxylase deficiency**

- results in deficiency of aldosterone and cortisol, increase in testosterone

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15
Q

signs/symptoms of what?

  • failure to thrive
  • recurrent vomiting
  • dehydration
  • hypotension
  • hyponatremia
  • hyperkalemia
  • shock
A

CAH

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16
Q

what are the levels of aldosterone, cortisol and androgens in CAH?

A
  • aldosterone = low
  • cortisol = low
  • androgens = high
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17
Q

what is the essential treatment of an infant in crisis due to CAH?

A
    • hydrocortisone (IV or IM)**
  • fluids/glucose
  • manage hyperkalemia
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18
Q

what is the purpose of mandatory newborn screening?

A

to detect potentially fatal or disabling conditions in newborns as early as possible, hopefully before serious illness develops

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19
Q

what are the three categories of adrenal gland defects responsible for primary adrenal insufficiency?

A
  • adrenal dysfunction (usually autoimmune)
  • adrenal dysgenesis
  • impaired steroidogenesis
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20
Q

what are the clinical signs/symptoms that nearly ALL patients complain of with primary adrenal insufficiency?

A
  • fatigue
  • reduced stamina
  • weakness
  • anorexia
  • weight loss
  • skin hyperpigmentation
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21
Q

what are the clinical signs/symptoms that most patients complain of with primary adrenal insufficiency?

A
  • abdominal pain/N/V
  • MSK pain
  • psychiatric sx (depression, anxiety, irritability)
  • HA, vomiting
  • salt craving
  • low BP (orthostatic)
22
Q
  • moderate neutropenia
  • low serum sodium
  • high serum potassium
  • fasting hypoglycemia
  • low MORNING plasma cortisol
A

lab findings of pt with primary adrenal insufficiency

23
Q

what are the signs/symptoms of acute adrenal crisis?

A
  • dehydration
  • dizziness/light headedness
  • rapid HR
  • rapid RR
  • confusion
  • N/V
  • fever
  • HA
  • abdominal pain/flank pain
24
Q
  • low serum cortisol
  • low blood sugar
  • low serum sodium
  • high serum potassium
  • metabolic acidosis
  • inadequate bump in cortisol level with ACTH stimulation test
A

lab findings of acute adrenal crisis

25
what are the main components of acute adrenal crisis treatment?
- **hydrocortisone** - fluids/glucose - fludrocortisone (after hydrocortosone) - treat hyperkalemia
26
what would lower than normal serum cortisol typically result in, if everything was working normally?
increased serum ACTH
27
what does a lack of pituitary secretion of ACTH in response to low serum cortisol indicate?
the problem is "higher" than the adrenal glands | - pituitary issue
28
what is low serum ACTH in the setting of low serum cortisol consistent with?
secondary adrenal insufficiency
29
what aer the signs/symptoms of Cushing disease?
- obesity/asymmetric weight gain (trunk/abd) - moon face - buffalo hump - hirsutism/acne - thirst, polyuria - HTN - dark purple striae - mental symptoms (difficulty concentrating) - impaired wound healing - susceptibility to opportunistic infections
30
what are elevated *midnight* cortisol levels indicative of?
Cushing syndrome
31
what is the best screening method for Cushing syndrome?
dexamethasone stimulation test - dexamethasone should suppress ACTH from pituitary - low dose given at night - serum cortisol is drawn in the morning - results are used in conjunction with serum ACTH levels drawn before dex was given
32
what does it mean if the serum cortisol is low after dexamethasone test?
Cushing syndrome can be excluded with certainty
33
what should be considered if ACTH is undetectable/low, and cortisol is not suppressed by high or low doses?
``` primary hypercortisolism (not driven by ACTH_ - possible adrenal tumor ```
34
what should be considered if ACTH is elevated in the hundreds, and cortisol is not suppressed by high or low doses?
ectopic ACTH syndrome | - **often associated with small cell carcinoma of the lung!**
35
what should be considered if ACTCH is normal to elevated, and cortisol is not suppressed by low doses, but suppressed by high doses?
Cushing disease
36
what are most patients with primary aldosterone (hyperaldosteronism) hypertensive?
excessive aldosterone production increases Na retention, which increases water retention -> increasing BP
37
what dx would you expect if patient has: - treatment-resistant HTN - severe HTN - early onset HTN - *low renin HTN* - HTN with family hx of early onset HTN or CVA - 1st degree relative with aldosteronism
primary aldosteronism
38
what does Newman want us to remember about primary aldosteronism?
1. low renin 2. hypokalemia 3. metabolic acidosis
39
what was initially used to describe the condition in which a unilateral aldosterone producing adrenal adenoma resulted in hypertension
Conn syndrome
40
what is Conn syndrome now used interchangeably with?
primary hyperaldosteronism, whether the pt has an adenoma or not
41
what is the primary location and secretion of a pheochromocytoma?
- location: adrenal medulla | - secretes: catecholamines (Epi and NE)
42
what is the primary location and secretion of a paraganglioma?
- location: outside the adrenal gland | - secretes: catecholamines (Epi and NE), OR can be NON-secreting
43
what are the symptoms of pheochromocytoma and paraganglioma?
disease of P's - paroxysmal - pressure elevated (HTN) - pounding pain (HA) - panic - palpitations - pallor
44
what is the most sensitive test for diagnosing secretory pheochromocytomas and paragangliomas?
plasma fractionated free metanephrines
45
what is the inheritance pattern of von Hippel-Lindau disease type 2
autosomal dominant | - 20% develop pheochromocytomas
46
- retinal capillary hemangiomas/hemangioblastomas (blood vessel tumors) - hemangioblastomas of CNS - increased risk for renal cysts that transform into renal cell carcinoma
von Hipple-Lindau diseae type 2
47
why do you always treat pheochromocytoma with an alpha-blocker prior to the use of beta-blockers?
unopposed alpha receptor stimulation can lead to further elevation of BP - after alpha blockage has been established, beta blockers can be started
48
what is the alpha blocker example that Newman gave for treating pheochromocytoma?
phenoxybenxamine
49
these tumors are usually found incidentally on abd CT/MRI - most are benign adrenal adenomas, but they can also be malignant tumors of several types - if greater than 4cm, and not obviously benign, cyst, or hemorrhage -> resect - if hx of malignancy exists in pt -> resect
adrenal gland incidentaloma
50
what do pts with adrenal incidentalomas require clinical assessment for? (3)
- Cushing syndrome - hyperaldosteronism - testing for pheochromocytoma (plasma fractionated free metanephrines)
51
what does Newman want us to recognize about MEN syndromes?
they are caused by gene mutations and therefore tend to run in families

EndoRepro 2 Exam 2 (10 decks)

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