Paediatric Adrenal Crisis
Lesson Objectives
By the end of this lesson, learners should be able to:
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Define adrenal crisis and understand its pathophysiology in children.
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Recognize the clinical features of adrenal crisis.
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List the investigations needed for diagnosis and monitoring.
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Describe stepwise management of adrenal crisis according to Zambian Paediatric Protocol.
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Understand the importance of hydrocortisone therapy and the limitations of other steroids.
Description
Adrenal crisis is a severe adrenocortical insufficiency resulting in peripheral shutdown, cyanosis, tachycardia, tachypnoea, hypotension, drowsiness, and coma.
It can be fatal if not quickly recognised and urgently treated.
Causes:
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Congenital: Congenital adrenal hyperplasia, congenital adrenal hypoplasia, adrenoleukodystrophy.
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Acquired: Acute bilateral adrenal haemorrhage (often due to sepsis, e.g., meningococcemia), rapid withdrawal of steroids, autoimmune Addison’s disease, tuberculosis, birth asphyxia, hypopituitarism.
Signs and Symptoms
| Category | Symptoms |
|---|---|
| Gastrointestinal | Vomiting, diarrhoea, nausea, weight loss |
| Cardiovascular | Hypotension, tachycardia, cyanosis |
| Metabolic | Acidosis, hypoglycaemia |
| Neurological | Drowsiness, coma, convulsions |
| Infectious / Dermatologic | High fever, rash (petechiae, ecchymoses, purpura, dermal gangrene), neck stiffness in meningococcaemia |
Investigations
| Test Type | Findings / Purpose |
|---|---|
| Random glucose | Hypoglycaemia |
| Urinalysis | Ketosis |
| ECG | Hyperkalaemia |
| Serum cortisol | <3 µg/dL at 9:00 AM defines low cortisol |
| ACTH | Often elevated |
| Urea / Electrolytes | Hyponatraemia, hyperkalaemia; monitor GFR via creatinine |
| Aldosterone | Often normal |
| Urinary electrolytes | Increased sodium and chloride, decreased potassium |
| Imaging | Abdominal ultrasound, CT scan, MRI for adrenal size; CXR for infection or TB |
| Microbiology | Gene Xpert for TB if indicated |
Treatment
| Step | Action |
|---|---|
| Airway and Breathing | Ensure airway is patent and ventilatory function is supported. |
| Circulation | Correct shock with normal saline at 20 mL/kg; subsequently correct dehydration. Treat hypoglycaemia. Administer normal saline to correct salt deficit. |
| Steroid Therapy | Hydrocortisone 8 mg/kg IV initially, then 4 mg/kg IV every 6 hours as daily maintenance. Note: Dexamethasone should not be used because it lacks mineralocorticoid activity. |
| Treat Underlying Cause | Manage sepsis, infection, or other precipitating factors. |
Key Points Summary
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Adrenal crisis is life-threatening and requires immediate recognition.
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Early fluid resuscitation, correction of hypoglycaemia, and IV hydrocortisone are lifesaving.
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Investigations guide diagnosis and monitor electrolyte and metabolic derangements.
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Treat any underlying cause promptly (infection, TB, autoimmune).