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Endocrine > Adrenal Disorders - Part 1 - Exam 3 > Flashcards

Adrenal Disorders - Part 1 - Exam 3 Flashcards

1
Q

What are the 2 types of tissues in the adrenal glands? They are both enclosed by _____

A

Cortex and Medulla

enclosed by a capsule

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2
Q

The ____ of the adrenal glands has 3 zones. Name them

A

Cortex

Zona Glomerulosa
Zona Fasciculata
Zona Reticularis

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3
Q

____ is secreted from the Zona Glomerulosa (outer). What is the function?

A

mineralocorticoids primarily aldosterone

sodium retention
water retention
potassium excretion
increases blood pressure and blood volume

aka: kidneys retain Na, gets ride of K, when aldosterone is present, pee out K and keep Na and water follows so blood volume and pressure will increase

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4
Q

What is the function of aldosterone? **What is the effect on Renin when aldosterone is in excess and deficient?

A

-Na retention and water follows
-K+ excretion

**Aldosterone excess
Renin ↓
Aldosterone deficiency
Renin ↑ **

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5
Q

____ is secreted from the Zona Fasciculata (middle). What is the function?

A

glucocorticoids -> think cortisol

-gluconeogenesis in liver: utilizes (decreases) protein stores

-immune system suppression: ↓ eosinophil, lymphocytes and lymph tissue

-decreases inflammation

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6
Q

What is gluconeogenesis?

A

Gluconeogenesis refers to synthesis of new glucose from noncarbohydrate precursors, and provides glucose when dietary intake is insufficient or absent.

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7
Q

How much cortisol is released on a normal day? What happens when the body is stressed?

A

10-20 mg daily (basal level - w/o stress)

increased cortisol release during stress via ACTH stimulation (from the pituitary)

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8
Q

Describe the HPA axis and its function.

A

hypothalamus releases corticotropin-releasing hormone (CRH) -> CRH goes to Anterior Pituitary Gland and adrenocorticotropic hormone (ACTH) is released -> ACTH goes to adrenal cortex and cortisol is released -> cortisol exerts a negative feedback in the hypothalamus and shuts off the release of CRH and ACTH

controls glucocorticoid release

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9
Q

Describe the circadian rhythm of cortisol

A

highest in the morning around 8am and lowest around 8pm

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10
Q

____ is secreted from the Zona Reticularis (inner). What is the function?

A

secretes gonadocorticoids

primarily dehydroepiandrosterone (DHEA)
converts to sex steroids in gonads

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11
Q

The adrenal medulla is composed of ___ cells. What do they secrete?

A

chromaffin cells

secrete epinephrine and norepinephrine, which control the fight-or-flight response

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12
Q

_____ can result in a chronic increase in a patient’s ACTH and CRH levels.

A

Destruction of the adrenal glands

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13
Q

______ stimulates adrenal cells to begin steroid synthesis.
Adrenal cells require _____ to synthesize steroid hormones.

A

ACTH

cholesterol

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14
Q

All steroidogenic pathways require _____ import into the mitochondrion, a process initiated by the action of the ______, which shuttles _____ from the outer to the inner mitochondrial membrane.

A

cholesterol

steroidogenic acute regulatory (StAR) protein

cholesterol

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15
Q

______ are very important in the process that forms aldosterone, cortisone, androgens and DHEAS. What happens if they mess up?

A

Enzymes

major effects on adrenal gland disfunction, could result in excessive amounts of sex hormones

??Consider memorizing this picture if times allows??

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16
Q

What is primary adrenal insufficiency?

A

adrenal gland dysfunction
↓ cortisol/aldosterone

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17
Q

What is secondary adrenal insufficiency?

A

pituitary gland dysfunction
↓ ACTH
↓ cortisol

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18
Q

What is tertiary adrenal insufficiency?

A

hypothalamic dysfunction
↓ CRH
↓ ACTH
↓ cortisol

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19
Q

Why is aldosterone not affected in secondary or tertiary adrenal insufficiency?

A

aldosterone is controlled by the RAA system and not the HPA axis

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20
Q

____ is a destruction/dysfunction of the adrenal cortex resulting in insufficient production of glucocorticoids and mineralocorticoids

A

Addison’s disease aka primary adrenal insuffiency

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21
Q

What is the MC cause of Addison’s disease? What are the autoantigen’s involved? What age range?

A

autoimmune destruction of adrenal enzymes (80% of cases in US) slow decrease over several years

**CYP21A2 (21-hydroxylase) - MC autoantigen
CYP11A1 (side-chain cleavage enzyme)
CYP17 (17-alpha-hydroxylase)

10-40 years old

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22
Q

What will a reduction/malfunction of CYP17A1 lead to?

A

excessive aldosterone and reduction in all hormones created by the adrenal gland

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23
Q

_______ genetic disorder resulting in accumulation of very long-chain fatty acids in the adrenal cortex. What does it inhibit?

A

Adrenoleukodystrophy

inhibiting the effects of ACTH on the adrenocortical cells

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24
Q

______ genetic mutation or absence of adrenal cortex

A

Congenital adrenal insufficiency/hyperplasia

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25
What is the MC cause of infectious Addison's disease?
TB but rare
26
Chronic Addison's dz can be caused by ORAL ketoconazole. Why?
inhibits the production of cortisol by blocking the CYP450 enzyme
27
phenytoin, barbiturates, rifampin all can ???? causing ????
accelerate the metabolism of cortisol causing Addison's disease aka the drugs break cortisol down faster before it can do its job
28
_____ is an adrenocorticolytic drug that diminishes cortisol synthesis by blocking steroid biosynthesis - used to treat adrenocortical carcinoma
mitotane
29
**_______ suppress the CRH or ACTH production.
glucocorticoids stopping steroid's abruptly can cause addison's dz
30
What are 2 causes of acute addison's dz?
Adrenal hemorrhage Adrenal “addisonian” Crisis
31
What can cause an adrenal hemorrhage?
results from sepsis, heparin-induced thrombocytopenia, anticoagulation, antiphospholipid antibody syndrome, trauma, surgery
32
**What is an adrenal crisis usually caused by?
insufficient cortisol caused by physical or emotional stress in an Addison's pt
33
Bronze pigmentation of skin (extensor surfaces, palmar creases, nail beds, mucosal membranes) changes in distribution of body hair GI disturbances: abdominal pain, N/V/D weakness, fatigue, decrease in stamina hypoglycemia postural hypotension weight loss, dehydration anorexia slow onset and nonspecific s/s vitiligo in 10% of pts generalized pain anxiety, irritability, depression gait disturbances, cognitive dysfunction amenorrhea (25% of female pts) What am I? **What are the first symptoms?
Chronic addison's dz presentation anorexia, weight loss, fatigue, ↓ stamina
34
profound fatigue dehydration vascular collapse decrease BP renal shut down decrease serum Na increase serum K What am I?
acute addison's dz
35
What is the function of mineralcorticoids?
increase Na retention increase water in the blood vessel increase blood volume and pressure K is eliminated into the urine
35
What is the function of glucocorticoids?
gluconeogensis in the liver decreases the immune response decreases protein stores aka muscle break down decreased inflammation
36
Why do patients with Addison’s disease present with hyperpigmentation?
ACTH attaches to melanotic receptors
37
What is vitiligo? When will you see it? What is it a result from?
patchy hypopigmentation result of autoimmune destruction of dermal melanocytes occurs in 10% of chronic addison's dz patients
38
sudden onset severe fever - 105℉ or higher severe abdominal pain, nausea, vomiting similar to an “acute abdomen¹” confusion hypotensive shock weakness, dizziness, syncope, hypotension, tachycardia
Addison's dz Adrenal crisis
39
**What is the key difference between an adrenal hemorrhage and an adrenal crisis?
adrenal hemorrhage will present like adrenal crisis without the fever
40
What will each lab value show of pt with addison's dz? CBC and CMP
CBC:eosinophilia, lymphocytosis CMP: hyponatremia, hyperkalemia (unless the pt has been vomiting/diarrhea may mask hyperkalemia) ↑ BUN/Cr - results from dehydration hypoglycemia if fasting or may occur spontaneously
41
**What additional tests would you want to order if you suspect Addison's dz? What results would you expect?
Plasma Cortisol at 8am! low cortisol (less than 3mcg) AND elevated ACTH low cortisol AND high ACTH ______ Plasma ACTH should be high in Addison's dz greater than > 200 pg/mL (7-63 pg/mL - normal value 7-10 AM)
42
How would you rule OUT addison's disease in am emergency setting?
Random cortisol > 25 mcg/dL usually rules out Addison’s
43
Describe the rapid ACTH stimulation test. When is it indicated? When is it used? What results would you expect with a pt with Addison's dz?
when serum cortisol and serum ACTH are non-diagnostic Pre-test: hold steroids 24 hours before test Hospital setting Step 1: Measure serum cortisol Step 2: Administer Cosyntropin (synthetic ACTH) 0.25 mg given IM Step 3: Measure serum cortisol 45 minutes post injection ------------ Normal - rise in serum cortisol ≥ 20 mcg/dL a rise could also indicated a Addison’s Disease - rise in serum cortisol less < 20 mcg/dL normal should rise more than 20mcg, Addison's there is a minor to no increase
44
When in the plasma renin test used? What would you expect to see? What is the drawback?
on a pt with Addison's dz to determine if they need mineralcorticoid replacement increased in Addison’s disease due to diminished aldosterone resulting in depleted intravascular volume has MANY interfering factors!!
45
What imaging would you order for a pt with Addison's dz? Why?
CXR: if you suspect TB or pneu CT of the abdomen to scan the adrenal gland
46
What are some common finds on a CT of the abdomen on a Addison's pt? What does it mean?
small without calcifications - autoimmune enlarged - TB, fungal, adrenal hemorrhage, metastatic calcifications - TB, fungal, adrenal hemorrhage, pheochromocytoma, melanoma
47
What is the treatment for chronic addison's dz? What is the dosing? What is the alt?
Hydrocortisone 15-30 mg daily: ⅔ dose in morning and ⅓ dose in late afternoon/early evening Prednisone or methylprednisolone alternative glucocorticoids AND Fludrocortisone 0.05–0.3 mg daily or every other day
48
What is the treatment for Addison's dz if the pt is stressed?
increase dose of glucocorticoid by up to 50% at higher stress doses of glucocorticoids (> 100 mg/d of hydrocortisone) Fludrocortisone is not needed
49
How do you monitor fludrocortisone? When do you need to increase the dose?
monitor with PRA - if increased, fludrocortisone dose needs increased orthostatic hypotension, hyponatremia, hyperkalemia
50
What is the treatment for an acute addison's dz?
1st: order serum cortisol and ACTH but DO NOT WAIT on results to start treatment 1st: IV hydrocortisone then switch to oral hydrocortisone once the pt can tolerate oral intake 2nd: broad spectrum abx and treat all electrolyte abnormalities
51
What is the addison's dz management and monitoring?
monitor for Cushing Syndrome WBC w/ diff to monitor for electrolytes and renal function DEXA scan: screen for osteoporosis
52
What are some pt education points to know about Addison's dz?
medical alert bracelet **all infections must be treated immediately: aka the abx threshold is much lower** educate the pts on how to do injectable hydrocortisone
53
______ a condition that results from an excessive amount of systemic cortisol. What is the MC cause? Is it ACTH dependent or independent?
Cushing's SYNDROME aka too many prescribed steroids Independent
54
_____ manifestations of excessive corticosteroids due to hypersecretion of ACTH. What is the MC cause? What gender? Is it ACTH dependent or independent?
Cushing DISEASE benign anterior pituitary adenoma females > male dependent
55
What are 4 causes of Cushing DISEASE?
pituitary hypersecretion of ACTH Ectopic secretion of ACTH Ectopic secretion of CRH factitious Cushing's syndrome: due to administration of exogenous ACTH – rare < 1 %
56
Ectopic secretion of ACTH is caused by ??? Name some common locations
non-pituitary tumor - MC locations lungs, thymus, pancreas
57
Ectopic secretion of CRH are caused by ???
non-hypothalamic tumors causing pituitary hypersecretion of ACTH - rare < 1 %
58
What are the effects of cortisol?
gluconeogensis decreased protein stores suppresses the immune system decreased inflammation
59
How does ACTH affect aldosterone?
ACTH can stimulate the adrenal cells of the zona glomerulosa to release aldosterone outside of the RAAS
60
Fatigue reduced physical endurance weight gain: central obesity with protuberant abdomen with thin extremities mood face supraclavicular fat pads buffalo hump skin atrophy with large purple striae easy bruisability proximal muscle weakness: especially shoulders and hips immune system suppression: frequent infections menstrual irregularities hyperpigmentation elevated BP hirsutism, male pattern hair loss What am I?
Cushing disease
61
What would a CBC and CMP look like on a pt with Cushing's syndrome?
CBC: leukocytosis with neutrophilia¹, lymphocytopenia, ↓ eosinophils CMP: elevated glucose +/- hypernatremia, hypokalemia
62
When would you expect to find abnormal Na and K levels?
Cushing's disease increase in ACTH stimulates aldosterone Zona glomerulosa is secreting too much aldosterone
63
What is the workup goals for Cushing disease?
Is it due to excessive steroid use? Is there hypercortisolism? then determine the cause
64
What are the 3 first line tests for Cushings? When can you dx Cushings?
Dexamethasone Suppression Test 24 Urine Free Cortisol (2 separate measurements) Late night salivary cortisol (2 separate measurements) **need TWO positive tests for diagnosis
65
What are the guidelines when performing a Low-dose Dexamethasone Suppression Test? What can cause the test to be "off'?
give dexamethasone 1 mg PO at 11 PM then serum cortisol next morning at 8 AM Results - < 5 mcg/dL likely excludes Cushing’s syndrome 3% false-negative rate: antiseizure drugs, rifampin and estrogens diminish suppressibility 20-30% false-positive rate: psychiatric disorders, emotional/physical stress
66
What are the guidelines for 24 hour urine free cortisol test? What is considered a positive test?
day 1: first morning void in the toilet, collect all urine throughout the day day 2: first morning void in the jug test complete 3x upper limit of normal on both occasions (around 150mcg)
67
What are the guidelines for the late night salivary cortisol test?
Place swab in mouth and leave for 90 seconds. Allow saliva to saturate. Do not chew swab collected by patient between 11 pm and midnight. Normal: less than 100 lots of interfering factors!: steroid use, brushed teeth, oral intake, inadequate collection must be performed on 2 separate occasions!! and both must be elevated to be positive
68
What are some conditions in which a late night salivary test might be falsely elevated.
erratic sleep schedules or shift work pregnancy exogenous steroid/estrogen use, anticonvulsants mental illness, chronic alcohol use acute stress (hospitalization/surgery)
69
what are the guidelines for Cushing dz test interpretation.
2 first line test with negative results if low index of suspicion - no further workup - monitor pt if high index of suspicion - refer to endocrinologist 1 out of 2 are positive: repeat test at random intervals consider: cyclic Cushing Disease as dx refer to endo 2/2 are positive refer to endo
70
What is the MC cause of cyclic Cushing Disease?
ACTH-secreting pituitary adenoma
71
_____ is a good test to order when determining the cause for hypercortisolism. Why? **How do you interpret the results?
Serum ACTH will differentiate ACTH-dependent vs. ACTH-independent etiology **interpretation < 20 pg/dL order adrenal CT; > 20 pg/dL order pituitary MRI
72
**What are the three red flags for malignant adrenal gland CT?
greater than > 4 cm growth of nodule (requires previous CT for comparison) density of lesion is > 10 Hounsfield units (HU)
73
What is the best way to view the pituitary gland? What is the parameter for treatment if you find a lesion?
MRI with contrast lesion greater than 5mm begin treatment no lesion or lesion less than 5mm do an inferior petrosal sinus sampling
74
the inferior petrosal sinus sampling comes back elevated, what does this mean? normal?
elevated ACTH levels - pituitary Cushing’s disease normal ACTH level - look for ectopic source of ACTH, order CT scan of chest/abdomen. If negative, perform whole body PET scan
75
When stopping glucocorticoid/ACTH therapy, what is the protocol?
prolonged therapy can suppress the HPA axis; **rapid withdrawal can result in acute adrenal insufficiency** HPA axis is usually fully recovered within 6-12 months
76
What is the management for Cushing dz caused by a adenoma/ACTH-secreting tumor?
surgery: post surgical adrenal insufficiency will require lifelong glucocorticoid replacement radiation
77
While waiting for sx or sx not an option, how do you treat Cushings Dz?
**11β-hydroxylase inhibitors - blocking cortisol steroidogenesis
78
What is the medical management for a pituitary ACTH tumor?
pasireotide (Signifor) - somatostatin analog - inhibits ACTH secretion
79
What is the medical management for an adrenocortical carcinoma?
mitotane - blocks cortisol secretion
80
What is the medical management of mineralcorticoid HTN?
1st: K+ sparing diuretic: spironolactone, eplerenone 2nd: ACEI
81
_____ is given to women for hyperandrogenism. Its MOA inhibits androgen uptake and/or inhibits binding of androgen in target tissues
flutamide
82
What are some other conditions that commonly arise along side of Cushings dz?
HTN, DM, increased risk of infection, complications from osteoporosis, nephrolithiasis, psychosis
83

Endocrine (14 decks)

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