Endocrine Emergencies

Question 1

Cushing Syndrome Definition & Pathophysiology

Answer 1

Excess cortisol

Question 2

Possible Causes of Cushing Syndrome

Answer 2

1. Hormone secreting tumor
2. Exogenous corticosteroid supplementation

Question 3

Cushing Syndrome Presentation

Answer 3

Gradual Onset
Hypokalemia
Hypertensive Emergency (180/120 or higher) (organ damage causing)
Psychosis, Mania
Round face, striae
Glucose intolerance
Hyperlipidemia
Hepatic Steatosis (Fatty Liver)
Easily bruised

Question 4

Cushing Syndrome Treatment

Answer 4

1. Adrenal steroidogenesis inhibition
   (with oral agents: Metyrapone, Mitotane, or Ketoconazole or IV agents: Etomidate)
2. Surgical intervention

Question 5

How can Etomidate infusions be used to treat Cushing Syndrome in patients who cannot tolerate oral agents?

Answer 5

Etomidate can be used off-label because it inhibits 11beta-hydroxylase, thereby reducing the conversion of 11-deoxycortisol to cortisol.
Close monitoring is required to balance cortisol suppression with adverse effects.

Question 6

Adverse Effects of utilizing an Etomidate infusion as an acute Adrenal steroidogenesis inhibitor.

Answer 6

Sedation
Myoclonus
Nystagmus

Question 7

What are 3 types of adrenal insufficiency that can lead to adrenal crisis and what drives each of them?

Answer 7

1. Primary Adrenal Insufficiency- adrenal gland failure
2. Secondary Adrenal Insufficiency- caused by disorders of the hypothalamus or pituitary gland
3. Tertiary Adrenal Insufficiency- caused by the hypothalamic region being affected by endogenous factors such as inflammation or exogenous factors such as external glucocorticoid supplementation.

Question 8

What symptoms define Adrenal Crisis?

Answer 8

A SBP < 100 or 200 mmHg lower than patient’s baseline SBP that resolves upon administration of parenteral glucocorticoid supplementation.
Hyperkalemia
Hyponatremia
Hypercalcemia
Abdominal Pain
N/V
Diarrhea
Fatigue/Malaise
Weight Loss
Anxiety/Depression

Question 9

What is intermittent dosing of steroids more commonly named?

Answer 9

“Stress-dose” steroids

Question 10

Name 3 balanced glucocorticoid and mineralocorticoid steroids that could be prescribed to treat someone in the ACUTE phase of Adrenal Crisis?

Answer 10

1. Hydrocortisone (most-balanced and first-line usually)
2. Methylprednisolone
3. Prednisone

Question 11

What is the initial treatment (within the 1st hour) for a patient that arrives in Adrenal Crisis?

Answer 11

1. 1 L NS IV fluid resuscitation
2. IV steroid administration
3. Possible Fludrocortisone addition to prevent further crisis by replacing aldosterone

Question 12

What effects does cortisol have on the human body?

Answer 12

1. Maintenance of hemodynamics (ex: BP)
2. Effects metabolic function

Question 13

What is another name for adrenal insufficiency?

Answer 13

“Addison’s Disease”- named after Thomas Addison, a British physician who first described the condition in 1855

Question 14

What is a Pheochromocytoma?

Answer 14

A rare, neuroendocrine tumor that inappropriately secretes 1 or more catecholamines, including norepinephrine prompting vasoconstriction, vasospasm, and can result in deadly end-organ dysfunction and damage.

Commonly occurs with an infection.

Question 15

How might a patient with a pheochromocytoma present?

Answer 15

Paroxysmal Triad:
1. headaches
2. tachycardia
3. diaphoresis

Hypertensive Emergency- seen in most severe cases
AKI
Hyperglycemia
Lactic Acidosis
GI Symptoms
Cardiomyopathy
Cardiac Arrhythmias
Pulmonary Edema

Question 16

How is Myxedema Coma defined and what is the pathophysiology of the disease?

Answer 16

A chronic deficiency of thyroid hormone (T3 and T4), exacerbated by a trigger such as infection, electrolyte abnormality, thyroid hormone medication non-adherence, or an adverse medicinal effect (ex: Amiodarone, Lithium, CNS Depressants).

Question 17

What is the typical clinical presentation of a patient with Myxedema Coma?

Answer 17

AMS
Hypothermia
Hyponatremia
Hypoglycemia
Respiratory Failure
Cardiogenic Shock
N/V
Abdominal Pain
Constipation

Question 18

What are the general treatment guidelines for patients with Myxedema Coma?

Answer 18

IV thyroid hormone replacement (T4 preferred because it is more stable an converts to T3, replenishing both thyroid hormones anyways- ex: Levothyroxine).
IV Corticosteroid (usually given prior to thyroid hormone to prevent adrenal insufficiency)
IV Fluids
Vasopressors prn (Dopamine is preferred in this case)
Broad-spectrum abx (determine infectious source and treat underlying cause)

Question 19

What is “Thyroid Storm” and how does it occur?

Answer 19

A severe form of thyrotoxicosis d/t increased free fraction of thyroid hormone and hormone responsiveness. (Hyperthyroidism)
Seen most in people with Grave’s Disease
Hypermetabolic state
Commonly occurs w/an acute trigger like an infection, surgery, trauma, nonadherence to anti-thyroid medications or d/t an adverse effect from a medication (ex: Amiodarone, anesthetics)

Question 20

How would you expect a patient in Thryoid Storm to present?

Answer 20

Hyperreflexia
Tachyarrhythmias
Diaphoresis
AMS (including seizure or coma)
Hepatomegaly

Question 21

What is the general treatment outline for a patient in Thyroid Storm?

Answer 21

Anti-thyroid medication (ex: methimazole or propylthiouracil)
Inorganic Iodide solution (started no sooner than 30 mins after the anti-thyroid medication)
IV corticosteroids
B-blockers (ex: Esmolol or Propranolol)
Cholestyramine
Broad-spectrum antibiotics
And eventually, surgical thyroidectomy or radioactive iodine ablation