ADRENOCORTICAL INSUFFICIENCY   65A

A 28-year-old man presents to the emergency department with a headache, stiff neck, and fever. After a lumbar puncture, he is admitted to the intensive care unit with meningococcal meningitis. On hospital day 3, despite antibiotic treatment, he continues to be hypotensive and in shock and complains of new abdominal pain when he is awake. On physical examination, he has multiple areas of purpura on his skin. Serum testing reveals hyponatremia, hyperkalemia, and hypoglycemia.

What are the salient features of this patient’s problem? How do you think through his problem?

Salient features: Meningococcal meningitis with purpura; hypotension and shock; abdominal pain; hyponatremia, hyperkalemia, hypoglycemia

How to think through: This case shows the importance of assessing the full differential diagnosis when treating a patient in shock. This patient has an infection, but what other causes of hypotension, beyond septic shock, could be at play? (Cardiogenic shock, anaphylactic shock, neurogenic shock, adrenal insufficiency.) How should adrenal function be evaluated? (Morning plasma cortisol level <3 mg/dL is diagnostic. Cosyntropin stimulation test indicates primary adrenal insufficiency if the rise in cortisol is <20 mcg/dL 30 to 60 minutes after administration of cosyntropin. The test is less reliable in the setting of acute illness, and thus empiric corticosteroid treatment is often warranted.) If adrenal insufficiency is suspected, what are the possible causes? (Bilateral hemorrhagic adrenal infarction; autoimmune adrenal insufficiency [Addison disease]; indolent adrenal infection such as tuberculosis; adrenal atrophy caused by chronic glucocorticoid use; secondary [pituitary level] or tertiary [hypothalamic] lesions are possible but rare.) What is the eponym for adrenal infarction in the setting of meningococcemia? (Waterhouse-Friderichsen syndrome.). How do hyponatremia and hyperkalemia help localize the problem? (Only primary adrenal disease impacts aldosterone production.) Is imaging indicated? (Yes. Computed tomography [CT] of the abdomen may show adrenal hemorrhage.) How should he be treated? (Draw a plasma cortisol level; then start intravenous hydrocortisone for hypocortisolism and 50% dextrose in water [D50W] for hypoglycemia.)

ADRENOCORTICAL INSUFFICIENCY   65B

What are the essentials of diagnosis and general considerations regarding adrenocortical insufficiency?

Essentials of Diagnosis

Weakness, anorexia, weight loss; abdominal pain, muscle and joint pains; amenorrhea

Increased skin pigmentation in chronic disease, especially of creases, pressure areas, and nipples

Hypotension, dehydration, hyponatremia, hyperkalemia, hypercalcemia

Plasma cortisol levels low or fail to rise after administration of cosyntropin

General Considerations

Adrenocortical insufficiency can be chronic or result from an acute deficiency of cortisol.

Causes of chronic adrenocortical insufficiency include autoimmune destruction of adrenal glands; congenital; and infections such as HIV, cytomegalovirus, fungal, and tuberculosis.

Acute adrenocortical insufficiency is an emergency condition caused by primary adrenal gland or pituitary dysfunction; it may be caused by withdrawal of cortisol replacement in patients with chronic insufficiency or by increased cortisol need in stress, trauma, surgery, or infection.

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