Efficient short-term control of hypercortisolaemia by low-dose etomidate in severe paediatric Cushing's disease.
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.


CONCLUSIONS
In our experience, etomidate was effective and safe for short-term control of severe hypercortisolaemia in a severely ill child.
Greening
J E
JE
Department of Paediatric, St. Bartholomew's and the Royal London Hospitals, London, UK.
Brain
C E
CE
Perry
L A
LA
Mushtaq
I
I
Sales Marques
J
J
Grossman
A B
AB
Savage
M O
MO
D000315:Adrenalectomy; D002648:Child; D000075202:Contraindications; D003480:Cushing Syndrome / Q000188:drug therapy* / Q000601:surgery; D005045:Etomidate / Q000008:administration & dosage*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D007654:Ketoconazole; D008297:Male; D008797:Metyrapone / Q000009:adverse effects
D007654:Ketoconazole; D006854:Hydrocortisone; D005045:Etomidate; D008797:Metyrapone