Acute adrenal insufficiency

Mechanisms and causes of development

Globally, pathogenesis is divided into two types: primary insufficiency (destruction of the adrenals themselves) and secondary/tertiary (disruption of the hypothalamic-pituitary-adrenal axis).

The most common etiological factors in children:

• Decompensation of chronic adrenal insufficiency. Can be the first manifestation of a previously latent pathology or develop in diagnosed patients during stress and with inadequate therapy doses.
• Withdrawal syndrome. Occurs with a sharp dose reduction or discontinuation of drugs in patients who have been taking high doses of glucocorticoids for a long time.
• Iatrogenic causes. Complications of hypercortisolism treatment (adrenalectomy or adenoma resection, use of steroidogenesis inhibitors).
• Sudden destruction of the cortex. Vascular catastrophes (thrombosis, infarction) or infectious-toxic processes. A classic example is Waterhouse-Friderichsen syndrome: massive bilateral adrenal hemorrhage, often associated with severe meningococcal infection.

Development of the crisis is provoked by factors that deplete the body's reserves or impair microcirculation: acute stress (surgery, burns, sepsis), coagulopathies, systemic lupus erythematosus, antiphospholipid syndrome.

Clinical presentation and diagnostics

Clinical manifestations of addisonian crisis are non-specific and do not depend on the primary cause.

The leading syndrome is hemodynamic disorders:

• Critical decrease in blood pressure (shock).
• Pronounced tachycardia.
• Impaired peripheral perfusion (acrocyanosis).
• Decreased urine output down to anuria.

Due to the non-specific clinical picture, medical history plays a crucial role in identifying risk factors.

Blood chemistry reveals the classic triad: hyponatremia, hyperkalemia, and hypoglycemia. The hormonal profile shows a sharp drop in cortisol and aldosterone. ACTH levels depend on whether the insufficiency is primary or secondary. ECG may show signs of hyperkalemia.

In pediatrics, the condition must be differentiated from acute intestinal infections, "acute abdomen", exogenous poisonings, and hypoglycemic coma of other causes.

Emergency Medicine

Main rule: therapy is initiated immediately, before receiving laboratory results. The management protocol includes three areas:

1. Hormone replacement therapy. The drug of choice is hydrocortisone as it has a glucocorticoid to mineralocorticoid activity ratio of 1:1. The starting dose is administered as an intravenous bolus (3–5 mg/kg), followed by administration every 2–6 hours. After stabilization, the dose is gradually tapered, the patient is switched to oral administration, and mineralocorticoids are added.
2. Infusion therapy (massive rehydration). Performed with crystalloids (0.9% sodium chloride solution) along with a 5–10% dextrose solution. The goal is to restore blood volume, correct blood pressure, sodium, and glucose levels.
3. Etiotropic therapy. Treatment of the underlying disease that triggered the crisis (e.g., treating an infection).

Prevention

The basis of prevention is control of the underlying disease and dosage adjustment.

• "Stress dose" rule: patients with chronic insufficiency must temporarily increase their glucocorticoid dose during periods of stress (infection, trauma, surgery).
• Patient education: patients are advised to wear medical alert bracelets with their diagnosis and first-aid instructions in case of loss of consciousness.

Class and exam questions

What else to study on this topic

• Pathogenesis of primary and secondary adrenal insufficiency
• Comparative pharmacological activity of other glucocorticoids
• Differential diagnosis of addisonian crisis and acute abdomen
• Algorithm for calculating the hydrocortisone dose by glucocorticoid equivalent
• Management of patients with glucocorticoid withdrawal syndrome

Other topics in "Chapter 13. Main syndromes requiring emergency care"

Prepared by WhiteCoat Pro faculty in accordance with the medical school curriculum. For educational purposes only.