Makindo Medical Notes

Adrenocortical crisis, a life-threatening condition, can occur in babies due to acute adrenal insufficiency. This condition is often triggered by stressors such as infections, trauma, or surgery, leading to circulatory collapse and severe metabolic disturbances. In infants, adrenal insufficiency may present with nonspecific symptoms, such as hyponatremic seizures, which are frequently misdiagnosed as febrile convulsions. Early recognition and prompt treatment are critical to prevent fatal outcomes.

Common Causes of Adrenocortical Crisis in Babies

• Congenital Adrenal Hyperplasia (CAH): A genetic disorder affecting cortisol production.
• Severe Infections: Sepsis, gastroenteritis, or other systemic infections.
• Surgery or Major Trauma: Physical stress that overwhelms the adrenal glands.
• Inadequate Corticosteroid Replacement or Withdrawal: Sudden discontinuation or insufficient dosing of corticosteroids in patients with known adrenal insufficiency.
• Hemorrhagic Shock: Severe blood loss leading to adrenal hypoperfusion.
• Hypoglycemia: Low blood sugar due to insufficient cortisol production.

Symptoms and Signs

• Nausea and Vomiting: Common early signs of adrenal crisis.
• Abdominal Pain: Often severe and nonspecific.
• Lethargy or Weakness: Due to low cortisol and electrolyte imbalances.
• Hypotension: Low blood pressure, a hallmark of circulatory collapse.
• Hyponatremia and Hyperkalemia: Electrolyte imbalances caused by aldosterone deficiency.
• Hypoglycemia: Low blood sugar, particularly in neonates and infants.

Investigations

• Serum Cortisol: Levels <5 mcg/dL indicate adrenal insufficiency.
• ACTH Stimulation Test: A lack of adequate cortisol response confirms adrenal insufficiency.
• Electrolytes (Na+, K+, Cl-): Hyponatremia and hyperkalemia are typical findings.
• Blood Glucose: Hypoglycemia is common during an adrenal crisis.
• Urinary Free Cortisol: Low levels suggest chronic adrenal insufficiency.
• Genetic Testing: Recommended for suspected Congenital Adrenal Hyperplasia (CAH) or other genetic causes.

Urgent Treatment

• ABC, Resuscitation. IV fluids.
• Hydrocortisone:
  • Neonate: 10 mg slow IV stat, followed by 100 mg/m² daily by IV infusion.
  • Child (1 month–12 years): 2–4 mg/kg every 6 hours.
  • Over 12 years: 100 mg every 6–8 hours slow IV.
• 0.9% Saline IV Infusion: To correct hypotension and hyponatremia.
• Glucose: To address hypoglycemia.
• Fludrocortisone: 0.1 mg/day orally for mineralocorticoid replacement in chronic adrenal insufficiency.