Disorders of the Adrenal Cortex Flashcards

1
Q

Cortex is split up into 3 zones =

A

Zona glomerulosa
Zona fasciculata
Zona reticularis

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2
Q

What hormones are made in the Zona glomerulosa

A

Mineralocorticoids - Aldosterone and corticosterone

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3
Q

What hormones are made in the Zona fasciculata

A

Glucocorticoids - cortisol and cortisone

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4
Q

What hormones are made in the Zona reticularis

A

Androgens - testosterone and estrogen

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5
Q

Adrenal hormones include: (4 S’s)

A

Aldosterone = SALT
Cortisol = SUGAR
Androgens = SEX
Epi, Norepi = STRESS

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6
Q

Adrenal Disorders are categorized as:

A

Hyper / Hypofunction

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7
Q

Hyperfunctional Adrenal gland results in

A

hormone excess (autoimmune disorders, neoplasms, exogenous administration)

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8
Q

Hypofunctional Adrenal gland results in

A

hormone deficiency (autoimmune, infection, surgery, inflammation, infarction, hemorrhage or tumor)

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9
Q

Adrenal disorders:
Cushing’s syndrome
Pheochromocytoma
Multiple endocrine neoplasia
Adrenal Adenoma
Adrenal Cancer

A

Hyperfunctioning

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10
Q

Adrenal disorders:
Addison’s Disease
Adrenal Deficiency

A

Hypofunctioning

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11
Q

Cushing syndrome - Pathophysiology

A

CUshing Syndrome = Cortisol Unchecked
Excess blood glucose production (excess gluconeogenesis; decreased Glycolysis for brain food)
increase in lipolysis
increase in protein catabolism
decrease insulin production and increased glucagon

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12
Q

Pathognomonic symptoms:
Central obesity
rounded face (moon facies)
Enlarged fat pad between shoulders (buffalo hump - dorsocervical area)
abdominal striae (purple stretch marks)
thinning skin
easy bruising

A

Cushing syndrome presentation

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13
Q

ACTH is released from where in the body

A

Pituitary

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14
Q

What stimulates ACTH to be released

A

CRH from the hypothalamus

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15
Q

Two types of Cushing’s Syndromes

A

iatrogenic - exogenous corticosteroid administration
ACTH dependent - problem in the pituitary
ACTH independent - problem in the adrenal

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16
Q

Cushing’s Syndrome workup (screening):

A

screening - elevated 24 hour urine free cortisol

elevated midnight plasma cortisol (normally time of lowest cortisol secretion)

Dexamethasone suppression test (should reduce steroid levels - positive is the cortisol levels have not reduced)

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17
Q

Cushing’s Syndrome workup (if screening is positive):

A

Plasma ACTH
determines if ACTH dependent or independent etiology
>15 pg/mL = ACTH dependent (problem is in the pituitary)
< 5 pg/mL = ACTH independent (problem is the adrenal gland)

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18
Q

Localizing the source for ACTH independent

A

CT of the adrenals
usually benign masses = think malignancy if > 4cm, usually atypically densities or contrast irregularities

19
Q

Localizing the source for ACTH dependent

A

MRI of the pituitary if no lesion there –> look for ectopic source =
CT scan of the chest, abdomen, thymus, pancreas and/or adrenals

20
Q

Treatment for Cushing’s Syndrome

A

Pituitary source - transsphenoidal resection of mass
Ectopic ACTH-secreting tumors - local surgical resection, if failed or not feasible - bilateral adrenalectomy and replacement hormones
Benign adrenal adenoma - laparoscopic resection (masses < 6cm)
Adrenal Carcinoma - surgical resection
symptomatic treatment if surgical treatment not feasible or declined

21
Q

What is a pheochromocytoma

A

sympathetic nervous system arising from the adrenal medulla
catecholamine secreting - epinephrine or norepinephrine

22
Q

Presentation:
HA
profuse sweating
palpitations and tachycardia
HTN, sustained or paroxysmal (new onset high HTN)
anxiety and panic attacks
(Variable presentation)

A

Pheochromocytoma

23
Q

Classic Triad of PHEochromocytoma

A

Palpitations
HA
Episodic sweating

Diagnosis likely if all three + HTN

24
Q

Catecholamine pulses are released but with no ________

A

correlated cause

this is why it can be misdiagnosed as an anxiety attack

25
Q

Pheochromocytoma can result in

A

catastrophic HTN crisis
fatal arrhythmias
pulmonary edema or HF
ARDS and multisystem failure

26
Q

Pheochromocytoma workup (labs)

A

lab testing - plasma fractionated free metanephrines (most sensitive)
specimen obtained after sitting quietly for 15 mins
if positive - retest after lying supine in a quiet room 30-90 min
confirm with 24 hr urine for fractionated metanephrines and creatine

27
Q

Pheochromocytoma workup (imaging)

A

non-contrast abdominal CT
MRI with or without gadolinium contrast

28
Q

Pheochromocytoma treatment

A

surgical resection (partial or total - laparoscopic preferred)
alpha blockers or CCB for HTN
beta blockers for arrhythmia
caution - treatment resistant hypotension post-op
if malignant - add chemo, recheck BP and plasma free metanephrines, follow with serum chromogranin A (CgA)

29
Q

Four distinct types of Multiple Endocrine Neoplasia (MEN)

A

MEN 1
MEN 2 (2A)
MEN 3 (2B)
MEN 4

30
Q

MEN 1 presentation

A

classic triad of tumors - parathyroids, pancreatic islets, anterior pituitary
hyperparathyroidism is the initial presenting sx

31
Q

MEN 2 (2A) presentation

A

classic triad - medullary thyroid carcinoma, pheochromocytomas, parathyroid tumors

32
Q

MEN 3 (2B) presentation

A

characterized by mucosal neuromas
marphan-like habitus
delayed puberty, skeletal abnormalities
pheochromocytoma - 60%

33
Q

MEN 4 presentation

A

most rare of the MEN subsets
often pituitary, parathyroid, pancreatic tumors

34
Q

MEN workup

A

primary diagnostic tool is genetic testing
screening for patients with known FH
screening for neuroendocrine tumors with targeted tests/ imaging (MRI for pituitary and adrenal tumors)

35
Q

MEN treatment

A

Parathyroidectomy if sx
prophylactic thymectomy if getting parathyroidectomy
high dose PPIs and control hypercalcemia if gastrinomas
+/- chemo

36
Q

Classical presentation of Adrenal Insufficiency

A

fatigue
reduced stamina
weakness
anorexia/ wt loss

37
Q

Adrenal Insufficiency is

A

deficiency of the adrenal cortex hormones
primary and secondary causes

38
Q

Primary adrenal disease is aka

A

addison’s disease (adrenal deficiency)
Primarily autoimmune in origin

39
Q

Acute adrenal crisis is

A

life threatening
more common with primary insufficiency
N/V, fever, dehydration, profound hypotension
progression to shock unresponsive to fluids or vasopressors

40
Q

Adrenal Insufficiency workup labs

A

plasma cortisol < 3 mcg/mL is diagnostic
often low serum DHEA levels but non-specific
conformation with cosyntropin stimulation test (synthetic ACTH) - must hold exogenous steroid meds

41
Q

Differentiating between primary and secondary adrenal insufficiency

A

Plasma ACTH differentiates between primary and secondary insufficiency
High > 200 pg/mol = primary insufficiency
Low or normal = secondary insufficiency

42
Q

Adrenal insufficiency workup imaging

A

MRI of hypothalamus and pituitary to look for masses
CT of the adrenals for cases of primary insufficiency that are not dx with antibody testing - small noncalcified adrenals in autoimmune, enlarged adrenals in metastatic or granulomatous disease

43
Q

Adrenal insufficiency treatment

A

Glucocorticoid replacement therapy
15-30 mg hydrocortisone daily 2-3 doses
alternatives prednisone or methylprednisolone
increased doses when under stress
Mineralocorticoid replacement therapy
+DHEA supplementation for women

44
Q

Treatment for adrenal crisis

A

loading dose of hydrocortisone 100-300 mg IV
Re-hydration with saline solution
Hydrocortisone 50-100 mg q6h IV, titrate down and to PO as able
broad spectrum abx until infection is ruled out as cause