Endocrine Emergencies 2

Question 1

Adrenal Medulla

1. Controlled by?
2. When stimulated it secretes?

2 Adrenal Cortex

1. Three zones: What are they?
2. What do they secrete?

Answer 1

Adrenal Medulla

1. Controlled by nervous system
2. When stimulated secretes epinephrine and norepinephrine into adrenal veins

Adrenal Cortex

1. Glomerulosa: mineralocorticoids… aldosterone
2. Fasciculata: glucocorticoids… cortisol
3. Reticularis: sex hormones

Question 2

Adrenocorticotropic Hormone ACTH

What does it do?

Primarily what?

Answer 2

Remember, -tropin or -tropic means to stimulate.

Therefore, adrenocorticotropic means “to stimulate the adrenal cortex”

Function: Stimulates synthesis and secretion of adrenocortical hormones (cortisol, androgens, and aldosterone)

ACTH primarily stimulates cortisol

Question 3

Describe the normal ACTH physiology

Answer 3

Question 4

What does cortisol stimulate? 4

Answer 4

1) Gluconeogenesis
2) Protein mobilization
3) Fat mobilization
4) Stabilizes lysosomes

Question 5

Adrenal Insufficiency vs Crisis

1. Adrenal Insufficiency is what?
2. What kind of disease is this?
3. Adrenal Crisis is what?
4. What kind of condition is this?

Answer 5

Adrenal Insufficiency

1. Failure of adrenal glands to produce essential BASAL secretion of steroids
2. Insidious wasting disease

Adrenal Crisis

3. Failure to RESPOND to the increased demands caused by stress or SUDDEN INABILITY to secrete essential steroids
4. Life-threatening condition

Question 6

Nova’s likely diagnosis:

Adrenal insufficiency, of which there are two forms:?

Answer 6

1. Primary adrenal insufficiency (Addison’s disease)
2. Secondary adrenal insufficiency

Question 7

Adrenal insufficiency?

1. Primary adrenal insufficiency (Addison’s disease) results from?
2. Secondary adrenal insufficiency results from?

Answer 7

Primary adrenal insufficiency (Addison’s disease)

1. Results from destruction or dysfunction of the adrenal cortex

Secondary adrenal insufficiency

2. Results from inadequate stimulation of adrenal cortex by ACTH (***By far the most common cause is chronic administration of exogenous steroids!)

Question 8

Describe how the adrenal pathway changes in primary adrenal insufficiency?

Answer 8

Question 9

What would happen to the adrenal pathway with exogenous administration of corticosteriods?

Answer 9

Question 10

Decreased blood pressure?

Decreased cortisol:

1. Cortisol up-regulates alpha1-adrenergic receptors on vascular smooth muscle (what does stimulation of the alpha1 receptors do again?) so a decreased amount would…? 3

Decreased aldosterone:

2. Aldosterone increases Na reabsorption by renal cells, leading to increases in extracellular fluid volume and blood volume, venous return, cardiac output, and arterial pressure. Therefore a decrease in aldosterone would…? 2

Answer 10

1.

• Cause vasodilation and
• decrease in peripheral vascular resistance
• and decreased BP

2.

• More NA+ to be excreted in the urine and also more water excretion.
• decreased blood pressure

Question 11

Adrenal Insuffieciency

Pulse rate increase?

Compensatory mechanisms as a result of: 4

Answer 11

1. Decreased volume
2. With decreased volume we have decreased arterial pressure
3. ➔ baroreceptor reflex
4. ➔ increased sympathetic outflow and therefore an increase in heart rate

Question 12

Decreased blood glucose?

Decreased cortisol:

One action of cortisol is to increase blood glucose concentration by promoting gluconeogenesis and decreasing glucose uptake by the tissues and therefore a decrease in cortisol would…?

Answer 12

Decrease blood glucose levels

Question 13

Hyperkalemia?

Decreased aldosterone:

In addition to stimulating Na reabsorption, aldosterone stimulates K secretion by the renal principal cells and therefore a decrease in aldosterone would…?

Answer 13

inhibit K secretion

Question 14

Late Distal Tubule/Collecting Duct Area

1. The principal cells reabsorb what? 2 And secrete what?
2. Aldosterone exerts its effects by increasing the function of the what?

Answer 14

Question 15

Hyponatremia?

Decreased aldosterone

Aldosterone increases Na reabsorption by renal principal cells. Therefore, with decreased aldosterone we would have…?

Answer 15

Increased Na and water excretion

Question 16

In contrast to indolent and progressive course of chronic adrenal insufficiency, ***adrenal crisis presents as a life-threatening emergency in which the primary manifestation is what??

Answer 16

hypotension

Question 17

1. When you have a patient that presents with what always consider an adrenal crisis if there is not an immediately apparent cause?
2. ***The hypotension in adrenal crisis typically is

resistant to what? 2

3. If the missing _______ is not replaced, death will ensue!

Answer 17

1. severe hypotension
2. catecholamine and IV fluid administration!
3. cortisol

Question 18

Adrenal Crisis

Where is it seen? 4

Answer 18

1. In a previously undiagnosed patient with primary adrenal insufficiency who has been subjected to serious infection or other acute, major stress.
2. In a patient with known primary adrenal insufficiency who does not take more glucocorticoid during an acute infection (can occur during acute viral infections such as influenza) or other major illness, or has persistent vomiting caused by viral gastroenteritis or other gastrointestinal disorders.
3. After bilateral adrenal infarction or bilateral adrenal hemorrhage.
4. Rarely in patients with secondary adrenal insufficiency, can be seen in patients withdrawn abruptly from suppressive doses of corticosteroids

Question 19

Adrenal Crisis

1. Presentation? 2
2. Dx? 4
3. Tx? 2

Answer 19

1. Presentation
   - Marked hypotension
   - Abdominal and flank pain
2. Diagnosis
   - Clinically oriented for abrupt withdrawal from exogenous steroids
   - For primary adrenal insufficiency, a short cosyntropin test

(Cosyntropin [ACTH analogue] injected,

• plasma cortisol levels measured in 60 minutes,
• primary adrenal insufficiency excluded if poststimulation level > 550 nmol/L)
  3. Treatment
• If adrenal crisis suspected or diagnosed, IV glucocorticoids
• D5NS may be required to correct hypovolemia and hypoglycemia

Question 20

Pheochromocytoma

1. What is it?
2. The tumor releases what?
3. which causes? 5
4. reversible?
5. Where is the tumor located the majority of the time?

Answer 20

1. A tumor derived from neural crest cells of the sympathetic nervous system that is responsible for about 0.1-2% of all cases of hypertension.
2. The tumor releases catecholamines
3. Which cause episodic (this way most of the time) or sustained signs and symptoms, such as
   - palpitations,
   - sweating,
   - headaches,
   - fainting spells, and
   - hypertensive emergencies.
4. A surgically correctable form of HTN!
5. Majority of the time tumor located in adrenal medulla

Question 21

Catecholamines have two major effects, each mediated by a Beta1 receptor. What are they?

Answer 21

1. Increased heart rate
2. Increased contractility (or force of contraction)

Question 22

Symptoms can be described by the effects that epinephrine and norepinephrine have on the various organ systems:

Catecholamines have two major effects, each mediated by a Beta1 receptor: How does this affect the blood vessels? 2

Answer 22

1. Vasoconstriction of cutaneous blood vessels via Alpha1 receptor
2. Vasodilation of skeletal muscle blood vessels via Beta2 receptor

Question 23

Describe the 90% rule in pheochromocytoma

4

Answer 23

90% tumor (or 10% tumor):

1. 90% of the time they arise from the adrenal medulla (with the other 10% they can arise anywhere but the majority occur in the mediastinum or abdomen)
2. 90% of the time adrenal pheochromocytoma will be unilateral (otherwise it is bilateral and is likely a genetic syndrome)
3. 90% of the time it is not malignant
4. 90% of the time it occurs in adults

Question 24

Keys with pheochromocytoma:

1. History is exceptionally important! Symptoms? 4
2. Diagnosis is made by? 2
3. Once diagnosis is made: Hunt for the source how?

Answer 24

1. Pt usually has episodes of
   - hypertension,
   - headache,
   - palpitation, and
   - sweating
2. Demonstrating
   - elevated urinary excretion of catecholamines or their metabolites (metanephrines and vanillylmandelic acid)
   - DURING a period of hypertension
3. CT of abdomen with focus on adrenal glands

Question 25

You order a 24 hour urine measurement of what? The test is positive, a finding that provides nearly conclusive evidence of a pheochromocytoma A CT scan confirms a 3cm mass on Helen’s right adrenal gland… she needs an adrenalectomy

Answer 25

vanillylmandelic acid (VMA)

Question 26

Surgeons dislike performing surgery on someone with unstable blood pressure… and there is a natural inclination for the body to have a sympathetic response when surgery is performed leaving a patient with a pheochromocytoma to have a high morbidity and mortality unless blood pressure is controlled prior to surgery 1. Current recommendation is to initiate what before surgery? 2. Why do we do this? 3. Then start what 3-4 days after? 4. WHy?

Answer 26

1. initiate alpha blockade first to 2. - reduce afterload and - total peripheral resistance… 3. then to start a beta blocker about 3-4 days thereafter 4. to reduce the effects of the increased amount of catecholamines in the bloodstream

Question 27

1. Primary hypothyroidism usually has what kind of onset? 2. Typical labs include? 2 3. Emergency?

Answer 27

1. Primary hypothyroidism usually has insidious onset 2. Typical laboratory findings include - low T4 and - high TSH 3. Usually no urgency when suspecting primary hypothyroidism

Question 28

Describe the hypothyroidism feedback system

Answer 28

Question 29

Myxedema Coma 1. Occurs in individuals with waht? 2. How serious is this? 3. Correct diagnosis requires what? 4. Initiation of treatment is what? 5. Affected patients commonly in what population?

Answer 29

1. long-standing preexisting hypothyroidism presents with life-threatening decompensation 2. \*\*Rare, potentially life-threatening 3. high degree of suspicion 4. empirical decision 5. geriatric

Question 30

Myxedema Coma Presentation Severe decline in metabolic function? 8

Answer 30

1. Alteration in mental status 2. Hypothermia 3. Bradycardia 4. Hypotension 5. Hypoventilation (increased CO2 retention) 6. Cardiovascular collapse 7. Decreased drug clearance 8. Usually patient has history of primary hypothyroidism or previous thyroid surgery

Question 31

What precipitates Myxedema coma? 4

Answer 31

1. Medication noncompliance 2. Cold exposure 3. Severe infection 4. Addition of new medications

Question 32

Myxedema Coma ## Footnote 1. Laboratory findings may reveal? 9 2. EKG may demonstrate what? 3

Answer 32

1. - Anemia, - hyponatremia, - hypoglycemia, - elevated transaminases, - elevated CPK, - elevated lactate dehydrogenase, - hypercholesterolemia, - decreased PO2, - increased PCO2 2. EKG may demonstrate - sinus bradycardia, - prolongation of QT interval, and - low voltage with flattening or inversion of T waves

Question 33

Myxedema coma tx? 5

Answer 33

Treatment 1. Stabilization (patient may require intubation and mechanical ventilation for airway protection and correction of hypoventilation, hypercapnia, and hypoxia) 2. Correction of hypothermia with cautious use of gentle passive external rewarming 3. IV levothyroxine initial bolus followed by reduced daily dose until orals tolerated 4. Routine administration of glucocorticoid recommended 5. Gentle fluid restriction to correct dilutional hyponatremia

Question 34

1. Classic symptoms in patients with hyperthryoidism include? 6 2. If symptoms are mild, you have time for outpatient evaluation. High clinical suspicion may warrant what?

Answer 34

1. Classic symptoms in patients with hyperthryoidism include: - Heat intolerance, - palpitations, - weight loss, - sweating, - tremor, nervousness, - weakness, and fatigue 2. If symptoms are mild, you have time for outpatient evaluation. High clinical suspicion may warrant empiric symptomatic therapy (e.g. beta-blockers)

Question 35

How is thyroid storm different than hyperthyroidism: Usually precipitated in a previously unrecognized or poorly treated hyperthyroidism patient by? 9

Answer 35

1. Infection, 2. trauma, 3. DKA, 4. MI, 5. CVA, 6. Thromboembolic disease, 7. surgery, 8. withdrawal of thyroid meds, 9. iodine administration Rare, but a medical emergency Treatment initiated empirically

Question 36

How is thyroid storm different than hyperthyroidism: Symptoms that may be seen in thyroid storm NOT necessarily seen with hyperthyroidism? 4

Answer 36

1. Fever 2. Arrhythmia 3. Congestive heart failure 4. CNS dysfunction: Agitation, confusion, delirium, stupor, coma, seizure

Question 37

Thyroid Storm tx? 4

Answer 37

1. Stabilization, airway protection, oxygenation, IV fluids, and monitoring 2. Beta-blockers for severe adrenergic symptoms 3. Antithyroid agents such as PTU or Methimazole 4. Administration of iodine to decrease preformed thyroid hormone

Question 38

Some Important Points Thyrotoxicosis is not the same thing as thyroid storm 1. The term thyrotoxicosis is used to refer to what? 2. How will the hyroid levels change?

Answer 38

1. accelerated thyroid state 2. Most studies have not demonstrated differences in thyroid hormone levels in patients with symptomatic, uncomplicated hyperthyroidism, and thyroid storm

Question 39

1. What is the single most important initial step in the treatment of DKA? 2. Don’t let a normal what in the patient with DKA fool you!? 3. Remember the big three for therapy with DKA includes what? 3 4. Those at greatest risk for HHS is what?

Answer 39

1. \*\*\*Rapid fluid administration 2. K (or hyperkalemic) They likely have profound total-body potassium deficits 3. - volume repletion (the fundamental one), - insulin therapy, and - potassium replacement (with this one the key is frequent monitoring and replacement initiation once K is in normal range!) 4. poorly controlled diabetic elderly patients without free access to water

Question 40

1. The most common cause of acute adrenal insufficiency is what? 2. Remember, only with what will you have hyperpigmentation as a result of increased ACTH? 3. In contrast to indolent and progressive course of chronic adrenal insufficiency, \*\*\*adrenal crisis presents what? 4. \*\*\*The hypotension in adrenal crisis typically is resistant to what? What do you have to do?

Answer 40

1. sudden withdrawal of exogenous steroids 2. Addison’s disease (antibodies directed against the adrenals) 3. as a life-threatening emergency in which the primary manifestation is hypotension 4. catecholamine and IV fluid administration! If the missing cortisol is not replaced, death will ensue!

Question 41

1. Presence of unexplained hyponatremia and hyperkalemia in setting of hypotension unresponsive to catecholamine and fluid administration is strong evidence of what? 2. One of the best opportunities to diagnose a pheochromocytoma is in the ED. Why?

Answer 41

1. adrenal crisis 2. as the person has likely come in because he/she is in the middle of an attack

Question 42

1. If absolutely everything is slowed down in the patient with long-standing hypothyroidism, then consider what? 2. Think of thyroid storm as what? 3 Tx should not be delayed due to waiting for laboratory results

Answer 42

1. myxedema 2. - hyperthyroid symptoms - plus fever and - CNS disturbances