Makindo Medical Notes

⚡ Adrenocortical crisis is a life-threatening emergency in infants and young children, caused by acute adrenal insufficiency. It is frequently triggered by stress (infection, trauma, surgery) in a child with underlying adrenal dysfunction (e.g., Congenital Adrenal Hyperplasia). Because symptoms such as vomiting, lethargy, and seizures are non-specific, the condition is often misdiagnosed as sepsis or febrile convulsions. 👉 Early recognition and immediate treatment with IV hydrocortisone and fluids is lifesaving.

🌟 Common Causes of Adrenocortical Crisis in Babies

• Congenital Adrenal Hyperplasia (CAH): Most common cause in neonates; enzyme defects impair cortisol ± aldosterone synthesis, leading to salt-wasting crisis.
• Severe Infections: Sepsis, gastroenteritis, or meningitis can precipitate crisis in infants with borderline adrenal reserve.
• Surgery or Major Trauma: Physiological stress can overwhelm adrenal output.
• Inadequate Corticosteroid Replacement: Missed doses, abrupt steroid withdrawal, or underdosing in children with known adrenal insufficiency.
• Adrenal Hemorrhage/Waterhouse-Friderichsen Syndrome: Classically due to meningococcal sepsis.
• Hypoglycemia: From inadequate cortisol-mediated gluconeogenesis.

🔎 Symptoms and Signs

• 🤢 Nausea, Vomiting, Abdominal Pain – early non-specific features, often mistaken for gastroenteritis.
• 😴 Lethargy, Weakness – due to cortisol deficiency and electrolyte derangements.
• ⬇️ Hypotension / Shock – refractory to fluids alone; hallmark of crisis.
• 💧 Hyponatremia (Na loss) and 🔺 Hyperkalemia (K retention) from aldosterone deficiency.
• 🩸 Hypoglycemia – especially in neonates, presenting as jitteriness or seizures.
• 🧠 Seizures – commonly due to severe hyponatremia, often mislabelled as “febrile convulsions.”

🧪 Key Investigations (Do not delay treatment)

• Serum Cortisol: <5 µg/dL highly suggestive during crisis (but send before hydrocortisone if possible).
• ACTH Stimulation Test: Definitive, but performed later once stable.
• Electrolytes: Hyponatremia, hyperkalemia, metabolic acidosis.
• Blood Glucose: Hypoglycemia is common in infants.
• 17-Hydroxyprogesterone: Elevated in CAH (confirmatory).
• Genetic Testing: For congenital adrenal enzyme defects.

🚑 Emergency Management (Do not wait for results)

• 🔴 Airway, Breathing, Circulation → give high-flow O₂, monitor cardiac rhythm.
• 💉 Hydrocortisone IV/IM (STAT, weight-based):
  • Neonate: 10 mg IV bolus, then 100 mg/m²/day by infusion.
  • 1 month – 12 yrs: 2–4 mg/kg IV every 6 hrs.
  • ≥12 yrs: 100 mg IV every 6–8 hrs.
• 💧 IV 0.9% Saline bolus (10–20 mL/kg) → repeat if hypotension persists. Add 5% dextrose if hypoglycemia present.
• 🍬 Correct hypoglycemia – 10% dextrose IV bolus (2 mL/kg), then infusion.
• 💊 Fludrocortisone 0.1 mg/day PO (for chronic mineralocorticoid replacement, not acute).
• 📈 Monitor: BP, glucose, U&Es, fluid balance closely.

💡 Clinical Pearls

• 🍼 In neonates, salt-wasting CAH often presents at 1–3 weeks with vomiting, poor feeding, weight loss, and shock.
• 💊 Children on long-term steroids require “stress doses” during illness, surgery, or trauma to prevent crisis.
• 🧠 Always consider adrenal crisis in an infant with seizures + hyponatremia + hypoglycemia.
• 📢 Do not delay hydrocortisone for tests if the child is unstable—treat first, investigate later.

✅ Summary

Adrenocortical crisis in babies is a medical emergency. Key features: vomiting, lethargy, hypotension, hyponatremia, hyperkalemia, hypoglycemia. Immediate treatment with IV hydrocortisone, saline, and glucose is lifesaving. Prevention relies on early diagnosis of CAH and educating families on stress dosing of steroids.