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Clin Med3: Endocrine > D/o of the adrenal cortex - McGowan > Flashcards

D/o of the adrenal cortex - McGowan Flashcards

1
Q

what hormones does the adrenal gland secrete

A

mineralocorticoids
glucocorticoids
androgens
catecholamines
peptides

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2
Q

what are the mineralocorticoids and where are they made

A

aldosterone and corticosterone
found within the Zona Glomerulosa in the adrenal gland

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3
Q

what are the glucocorticoids and where are they made

A

cortisol and cortisone
zona fasciculata in adrenal gland

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4
Q

what are the androgens and where are they made

A

estrogen and testosterone
zona reticularis in adrenal gland

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5
Q

what are the catecholamines and where are they made

A

epinephrine and norepinephrine
medulla of the adrenal gland

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6
Q

what are the peptides and where are they made

A

Somatostatin and Substance P
medulla of adrenal gland

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7
Q

what is the make up of the adrenal gland

A

cortex and medulla

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8
Q

what is the function/purpose of glucocorticoids and mineralocorticoids

A

physiologic stress response (long term)
blood pressure regulation and electrolyte homeostasis
aldosterone and cortisol

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9
Q

what is aldosterone responsible for

A

controls sodium/potassium/water balance
regulation of Blood volume
BP regulation (RAA system)

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10
Q

what is Cortisol responsible for

A

glucose production
BP regulation
anti-inflammatory

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11
Q

what are adrenal androgen precursors

A

DHEA (hehydroepiandrosterone)
converted to sex steroids in gonads or target tissues (estrogen and testosterone)

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12
Q

what are the actions of androgen hormones

A

male and female reproductive development
secondary sex characteristics
menstruation
muscle strenth/mass

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13
Q

what are the catecholamines responsible for

A

fight or flight response
regulates HR
regulates contractility
vasodilation/constriction
BP regulation
bronchodilation
glycogenesis

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14
Q

what are the 4 S’s of adrenal hromones

A

Aldosterone = Salt
Cortisol = sugar
Androgen = Sex
Epi, Norepi = Stress

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15
Q

what are hyperfunctioning adrenal disorders

A

Cushings syndrome
Pheochromocytoma
Multiple endocrine neoplasia
Adrenal adenoma
Adrenal Cancer

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16
Q

what are hypofunctioning adrenal disorders

A

Addisons disease
Adrenal deficiency

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17
Q

what can cause hypofunction

A

primarily due to glandular destruction: autoimmune, infection, surgery, inflammation, infarction, hemorrhage or tumor

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18
Q

what can cause hyperfunction

A

neoplasms (functional), autoimmune disorders, exogenous administration

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19
Q

What is Cushing Syndrome

A

secondary to glucocorticoid excess, F>M (4:1)
increased ACTH production (dependent vs independent)
pituitary adenomas
cortisol unchecked
excess blood glucose production
increase lipolysis
increase catabolism
decreased insulin production and increased glucagon

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20
Q

what is the presentation of Cushing syndrome

A

central obesity
rounded face (moon facies)
enlarged fat pad between shoulders (buffalo hump) abdominal striae (purple* stretch marks)

weight gain, Hirsutism, facial plethora (swelling/erythema)

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21
Q

what is the workup for Cushing syndrome

A

elevated 24 hour urine free cortisol
elevated midnight plasma cortisol
dexamethasone suppression test
Plasma ACTH
Localizing the source - ACTH dependent - MRI/CT; ACTH independent - CT of adrenals

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22
Q

what can impact 24 hour urine free cortisol tests

A

anti-epileptics, estrogen, testosterone and exogenous steroids

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23
Q

what is the overnight dexamatheasone suppression test

A

baseline plasma cortisol (high dose only)
give 1mg or 8mg dexamethasone at 11pm
check plasma cortisol at 8-9am

24
Q

what is the difference between ACTH dependent and ACTH independent

A

Dependent: problem upstream/outside of adrenals - pituitary
Independent: problem is in the adrenal gland

25
What is the treatment of Cushing syndrome
piruitary source (dependent): trans-sphenoidal resection of mass Ectopic ACTH-secreting tumors: local surgical resection benign adrenal adenoma: laparoscopic resection adrenal carcinomas: surgical resection
26
what is required for longterm treatment of cushing syndrome
long-term glucocorticoid replacement younger = more likely to fully recover
27
what are negative prognostic indicators for Cushings
older age at diagnosis higher pre-op ACTH longer disease presence
28
what is a sympathetic nervous system tumor arising from the adrenal medulla
pheochromocytoma avg. age at dx is 40 yo cause of death in 1/3 of pts prior to dx
29
what is secreted from pheochromocytomas
catechomaines (epi and norepi)
30
what are the rule of 10 for pheo
~10% bilateral ~10% exra-adrenal ~10% malignant
31
what is the presentation for variable presentation
'the great masquerader'- variable presentation HA, Profuse sweating, palpitation and tachycardia, HTN (sustained or paroxysmal), Anxiety/panic attacks
32
What is the classic triad of PHEo
Palpitations Headache Episodic sweating diagnosis likely if all three + HTN
33
what can pheo result in
catastrophic HTN crisis, fatal arrhythmias, pulmonary edema or HF may see ARDS and mutisystem failure
34
what is the workup for Pheo
plasma fractionated free metanephrines (most sensitive) other lab tests: elevated plasma catecholamines, 24 hour urine catecholamines, serum chromogranin A (CgA), clonidine suppression test Non-contrast abdominal CT MRI with or without Gadolinium contrast
35
what are the treatment options for pheo
tx of choice: surgical resection must medically optimize HTN and tachycarrhythmias preop caution: tx resistant hypotesion post-op => AKI, MI
36
what is the treatment for malignant pheo
add chemo recheck BP and plasma free metanephinres follow up for serum chromogranin a (CgA)
37
what is multiple endocrine neoplasia (MEN)
inherited tumor syndromes - autosomal dominant (genetic testing) tumor in 2+ endocrine glands 4 distinct types
38
what is the presentation of MEN1
90% have mutation in Menin gene abnormalities can be deltected around 14-18 years old clinical symptoms appear in 20s-30s mean life expectancy: 55 years classic triad: parathyroids, pancreatic islets, anterior pituitary
39
What is the presentation of MEN2 (2A) and Men 3 (2B)
ret protooncogene mutation difference is clinical presentation Classic triad: meduallary thyroid carcinoma (MTC), pheochromocytomas, parathyroid tumors
40
what gene is mutated in MEN4
CDKN1B
41
what is the presentation of MEN2
classic triad: Medullary thyroid carcinoma (MTC), pheochromocytomas, parathryoid tumors
42
What is the presentation of MEN3
characterized by mucosal neuromas marfan like habitus medullary thyroid carcinomas
43
what is the presentaiton of MEN4
most rare of MEN subsets often pituitary, parathyroid and pancreatic tumors
44
how do you work up MEN
genetic testing screen patients with known FH screen for neuroendocrine tumors with targeted tests/imaging
45
what is the treatment of MEN1
parathyroidectomy if symptomatic prophylactic thymectomy if getting parathyroidectomy high does PPIs and control of hyeprcalcemia if gastrinomas
46
what is the treatment for MEN 2 and MEN3
if known FH and + genetic screening total thyroidectomy : by age 6 for MEN2, by 6 months for Men 6 no GLP-1 inhibitors for DM screening for pheo and thyroid cancer
47
what can cause adrenal insufficiency
primary of secondary causes primary - adrenal gland dysfunction (cortisol and aldosterone) secondary - ACTH deficiency due to hypothalamus or pituitary dysfunction
48
What is a primary adrenal disease
Addison's Disease (Adrenal Deficiency) primarily autoimmune in origin
49
what is the classic presentation of adrenal insufficiency
fatigue, reduced stamina, weakness, anorexia and weight loss
50
what are the later presenting facors for adrenal insufficiency
N/V, abdominal pain +/- diarrhea pain: arthralgia, myalgias, CP, abdominal pain, back/leg pain, HA Psych: irritability, depression, anxiety hyperpigmentation
51
what is acute adrenal crisis
more common with primary insufficiency life threatening N/V/Fever, dehydration, profound hypotension progression to shock, unresponsive to fluids or vasopressors
52
what is the workup for adrenal insufficiency
plasma cortisol low serum DHEA levels confirmation with cosynotropin stimulation test (synthetic ACTH)
53
what is the treatment for adrenal insufficiency
glucocortioid replacement therapy (15-30mg hydrocortisone daily in 2-3 doses) mineralocorticoid replacement therapy (fludrocortisone acetate) +/- DHEA supplementation for women
54
what is the treatment of adrenal crises
loading dose of hydrocortisone 100-300mg IV rehydration with saline solution (generous resuscitation, plus dextrose) then, hydrocortisone 50-100mg q6h IV, titrate down to PO as able broad spectrum ABX if infection ruled out as cause
55
how do you prevent adrenal criese
patient education of times of increased risk give extra doses and plenty of refills stress dose steroids dose adjustment for hot weather, vigorous exercise anti-emetics PRN to prevent N/V consider other routes of administration if unable to take PO due to N/V

Clin Med3: Endocrine (7 decks)

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