Recognise and manage adrenal crisis in a steroid-dependent child — OSCE

Candidate brief

You have eight minutes to manage an emergency presentation in a steroid-dependent child. Use a structured approach: recognise that this is adrenal crisis, state the immediate hydrocortisone and resuscitation, run the precipitant and differential work-up in parallel, and then explain the diagnosis and the emergency plan to the family in plain language. The examiners are watching for the speed and confidence with which you reach for empirical hydrocortisone before any confirmatory test. [9]

Key teaching and decision objectives

Recognise adrenal crisis. The child has the three ingredients: long-term corticosteroid exposure with hypothalamic-pituitary-adrenal suppression, a febrile precipitant, and the classic collapse with hypotension and hypoglycaemia. Hypoglycaemia is prominent in younger children because their glycogen reserves are small. Do not attribute this to a simple viral illness — treat it as adrenal crisis and sepsis at once, because sepsis is both a precipitant and a differential and the steroid-dependent child is also immunosuppressed. [9] [12]

Give empirical hydrocortisone before the cortisol result. The first action is to replace the cortisol the child cannot make. Give intravenous or intramuscular hydrocortisone immediately at the age-banded stress dose — 50 mg for this five-year-old — followed by a continuous infusion around 2 to 3 mg/kg per 24 hours or repeated six-hourly doses. Hydrocortisone, not dexamethasone, is the crisis drug, because at stress doses it carries the mineralocorticoid activity the child also needs; dexamethasone alone would leave her salt-wasting. Draw a cortisol and ACTH sample first if it can be done without delay, but do not let blood tests delay the steroid. [7] [9]

Resuscitate in parallel. Give a 10 to 20 mL/kg isotonic saline bolus, repeated for shock, because the cortisol-deficient child is hypovolaemic from salt-wasting and vomiting. Correct the hypoglycaemia with intravenous dextrose and monitor glucose because it can rebound. Take cultures and start broad-spectrum antibiotics after cultures, because sepsis must be assumed. Reassess blood pressure, capillary refill, and glucose within the first hour; escalate to PICU if there is no improvement. [9]

Explain to the family in plain language. Reassure while being honest: the body that makes cortisol was switched off by the long-term medicine, the fever tipped the balance, and the first dose of steroid will start to bring her back. Give the parents the sick-day rules and the hydrocortisone injection teaching before discharge, because the risk of recurrence is high without a robust plan. [9] [12]

Communication to the family

To the family (plain language): "Your daughter's body normally makes a hormone called cortisol that helps it cope with stress like a fever. Because she has been taking a steroid medicine for her kidney condition for a long time, her body stopped making its own cortisol — which is exactly what we expect, and it is why we plan a careful reducing schedule. What has happened today is that the fever asked her body for more cortisol than it could make, and she became very low on it. That is why she is drowsy and her blood pressure and sugar are low. The good news is that we can replace it straight away — I am giving her a dose of hydrocortisone now, through a drip, along with fluid and sugar, and we expect her to pick up within the next hour. While we do this we are also treating her for any infection, because that can be the trigger. Before she goes home we will teach you how to give an emergency injection and what to do when she is unwell, so you are ready next time." [9] [12]

Marking domains

• Clinical reasoning (30 per cent): recognises adrenal crisis from the steroid history, stress and hypoglycaemia; identifies sepsis as a parallel differential; knows the HPA suppression mechanism.
• Decision-making and resuscitation (25 per cent): gives empirical age-banded hydrocortisone (50 mg) before the cortisol result; uses hydrocortisone not dexamethasone; runs fluids, glucose and antibiotics in parallel; escalates to PICU if no response.
• Communication to the family (20 per cent): explains cortisol deficiency and the crisis in plain language; reassures while being honest; introduces sick-day rules and emergency injection.
• Safety and follow-up (15 per cent): arranges endocrinology review; MedicAlert identifier; school notification; written emergency plan; checks the axis before weaning stops.
• Pharmacology knowledge (10 per cent): states the equivalent-dose ladder and the mineralocorticoid independence of glucocorticoid potency. [7] [9] [12]