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. Copyright (c) 2005 S. Karger AG, Basel.
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. Copyright (c) 2005 S. Karger AG, Basel.
Authors: Lynnette K Nieman; Beverly M K Biller; James W Findling; M Hassan Murad; John Newell-Price; Martin O Savage; Antoine Tabarin Journal: J Clin Endocrinol Metab Date: 2015-07-29 Impact factor: 5.958
Authors: B M K Biller; A B Grossman; P M Stewart; S Melmed; X Bertagna; J Bertherat; M Buchfelder; A Colao; A R Hermus; L J Hofland; A Klibanski; A Lacroix; J R Lindsay; J Newell-Price; L K Nieman; S Petersenn; N Sonino; G K Stalla; B Swearingen; M L Vance; J A H Wass; M Boscaro Journal: J Clin Endocrinol Metab Date: 2008-04-15 Impact factor: 5.958
Authors: John R Porterfield; Geoffrey B Thompson; William F Young; John T Chow; Raymond S Fryrear; Jon A van Heerden; David R Farley; John L D Atkinson; Fredric B Meyer; Charles F Abboud; Todd B Nippoldt; Neena Natt; Dana Erickson; Adrian Vella; Paul C Carpenter; Melanie Richards; J Aidan Carney; Dirk Larson; Cathy Schleck; Marilyn Churchward; Clive S Grant Journal: World J Surg Date: 2008-05 Impact factor: 3.352