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Efficient short-term control of hypercortisolaemia by low-dose etomidate in severe paediatric Cushing's disease
J E Greening1, C E Brain, L A Perry
1Department of Paediatric, St. Bartholomew's and the Royal London Hospitals, London, UK.
Insights
This study shows etomidate effectively controlled severe hypercortisolaemia in a pediatric Cushing's disease patient when other treatments failed. The drug provided safe, short-term cortisol management before surgery.
Area of Science:
- Pediatric Endocrinology
- Critical Care Medicine
Background:
- Paediatric Cushing's disease (CD) is a rare but serious condition requiring effective hypercortisolaemia management.
- Pre-surgical control of hypercortisolaemia is crucial for optimal outcomes in pediatric CD.
- This case highlights the challenges in managing severe hypercortisolaemia in a critically ill child.
Observation:
- A 6.2-year-old male presented with severe hypercortisolaemia and life-threatening complications of Cushing's disease.
- Metyrapone and ketoconazole were ineffective in controlling cortisol levels.
- The patient's deteriorating condition necessitated an alternative treatment approach.
Findings:
- Low-dose intravenous etomidate infusion effectively reduced serum cortisol from 1,250 to 250 nmol/l within 24 hours.
- Combined etomidate and hydrocortisone therapy stabilized serum cortisol levels for 12 days.
- Bilateral adrenalectomy was successfully performed following etomidate-induced cortisol control.
Implications:
- Etomidate offers a safe and effective option for short-term management of severe hypercortisolaemia in pediatric patients.
- This approach can stabilize critically ill children with Cushing's disease, facilitating definitive surgical treatment.
- Further research into etomidate's role in pediatric endocrine emergencies is warranted.
Background:
Paediatric Cushing's disease (CD) is rare, but is associated with considerable morbidity and requires effective treatment. Control of hypercortisolaemia is recommended prior to definitive therapy by transsphenoidal pituitary surgery with selective adenomectomy. We describe a 6.2-year-old male with severe hypercortisolaemia and life-threatening complications of Cushing's disease. Control of cortisol with metyrapone and ketoconazole was ineffective, and due to his deteriorating condition, the decision was taken to proceed to bilateral adrenalectomy.
Methods:
Low-dose IV infusion of etomidate, with dose titration according to serum cortisol levels, was administered.
Results:
Etomidate infusion (3.0 mg/h i.v.) decreased serum cortisol from 1,250 to 250 nmol/l within 24 h. Combined etomidate and hydrocortisone therapy was maintained to provide stable serum cortisol levels within the desired range for 12 days prior to successful bilateral adrenalectomy.
Conclusion:
In our experience, etomidate was effective and safe for short-term control of severe hypercortisolaemia in a severely ill child.
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