Efficient Short-Term Control of Hypercortisolaemia by Low-Dose Etomidate in Severe Paediatric Cushing’s Disease

Greening, James; Brain, Caroline; Perry, Leslie; Mushtaq, Imran; Marques, Jorge Sales; Grossman, Ashley; Savage, Martin O.

doi:10.1159/000088587

Cited by 63 publications

(48 citation statements)

References 35 publications

(23 reference statements)

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“…Several publications that followed report sub-hypnotic i.v. 2.5-3.5 mg/h doses of etomidate completely inhibiting endogenous cortisol production (21,23,24). In the case reports where patients were rendered hypoadrenal, this was demonstrated by serum cortisol levels !100 nmol/l and glucocorticoid replacement therapy was provided by a continuous i.v.…”

Section: Clinical Experiencementioning

confidence: 99%

THERAPY IN ENDOCRINE DISEASE: Etomidate in the management of hypercortisolaemia in Cushing's syndrome: a review

Preda

Sen

Karavitaki

et al. 2012

European Journal of Endocrinology

132

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This review addresses the practical usage of intravenous etomidate as a medical therapy in Cushing's syndrome. We reviewed the relevant literature, using search terms 'etomidate', 'Cushing's syndrome', 'adrenocortical hyperfunction', 'drug therapy' and 'hypercortisolaemia' in a series of public databases. There is a paucity of large randomised controlled trials, and data on its use rely only on small series, case study reports and international consensus guideline recommendations. Based on these, etomidate is an effective parenteral medication for the management of endogenous hypercortisolaemia, particularly in cases with significant biochemical disturbance, sepsis and other serious complications such as severe psychosis, as well as in preoperative instability. We suggest treatment protocols for the safe and effective use of etomidate in Cushing's syndrome.

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Section: Clinical Experiencementioning

confidence: 99%

THERAPY IN ENDOCRINE DISEASE: Etomidate in the management of hypercortisolaemia in Cushing's syndrome: a review

Preda

Sen

Karavitaki

et al. 2012

European Journal of Endocrinology

132

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“…Despite advances in the understanding and treatment of CD, the condition still carries with it significant morbidity and mortality (5)(6)(7). The management of this condition requires a multidisciplinary approach involving neurosurgeons, endocrinologists, biochemists, radiologists and radiotherapists.…”

Section: Introductionmentioning

confidence: 99%

Long-term anterior pituitary function in patients with paediatric Cushing’s disease treated with pituitary radiotherapy

Chan

Storr

Plowman³

et al. 2007

eur j endocrinol

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Background/objective: Pituitary radiotherapy (RT) is an effective second-line treatment for paediatric Cushing's disease (CD). Although the short-term effects of pituitary RT are well documented, there are less data on possible long-term sequelae. We report the long-term anterior pituitary function in a cohort of paediatric CD patients treated with pituitary RT. Patients and methods: Between 1983 and 2006, 12 paediatric CD patients (10 males and 2 females) of mean age 11.4 years at diagnosis (range 6.4-17.4) underwent second-line pituitary RT (45 Gy in 25 fractions), following unsuccessful transsphenoidal surgery. Out of 12, 11 patients were cured by RT (cure interval 0.13-2.86 years) defined by mean serum cortisol of !150 nmol/l on 5-point day curve and midnight sleeping cortisol of !50 nmol/l. Long-term data are available for six male patients, who received RT at the age of 7.0-17.6 years. The mean follow-up from the completion of RT was 10.5 years (6.6-16.5). Results: At a mean of 1.0 year (0.11-2.54) following RT, GH deficiency (peak GH !1-17.9 mU/l) was present in five out of six patients. On retesting at a mean of 9.3 years (7.6-11.3) after RT, three out of four patients were GH sufficient (peak GH 19.2-50.4 mU/l). Other anterior pituitary functions including serum prolactin in five out of six patients were normal on follow-up. All the six patients had testicular volumes of 20-25 ml at the age of 14.5-28.5 years. Conclusion: This series of patients illustrates the absence of serious long-term pituitary deficiency after RT and emphasises the importance of continued surveillance.

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“…This compares to 49-71% in adult CS (11,12). The median age of presentation is 14.1 yrs (141 cases taken from the literature), with the youngest reported case being 6.2 years (14). Despite advances in the diagnosis and management of CD, the condition still causes significant morbidity and mortality (9,14,15).…”

Section: Acth-dependent Csmentioning

confidence: 99%

“…The median age of presentation is 14.1 yrs (141 cases taken from the literature), with the youngest reported case being 6.2 years (14). Despite advances in the diagnosis and management of CD, the condition still causes significant morbidity and mortality (9,14,15). Unlike adult CD where the number of female patients predominates, pediatric CD is characterized by significant male preponderance in prepubertal years (4).…”

Section: Acth-dependent Csmentioning

confidence: 99%

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Pediatric Cushing's syndrome: clinical features, diagnosis, and treatment

Chan

Storr

Grossman

et al. 2007

Arq Bras Endocrinol Metab

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Cushing's syndrome (CS) results from prolonged exposure to supraphysiological levels of circulating glucocorticoids, endogenously or exogenously derived. Although rare in childhood, CS remains a difficult condition to diagnose and treat. A multidisciplinary approach and close collaboration with adult colleagues is adopted at most large centres that manage pediatric CS patients. Although pediatric protocols are derived from adult data, significant differences exist between adult and childhood CS. Furthermore, long term outcome parameters including final height, bone mineral density, reproductive function, body composition and psychological health pose challenges for pediatric care. This article will aim to provide an overall view of pediatric CS highlighting some of the differences between adult and pediatric CS. A síndrome de Cushing (SC) resulta da exposição prolongada a níveis suprafisiológicos de glicocorticóides circulantes, tanto endógenos como de seus derivados exógenos. Embora rara na infância, a SC permanece uma condição difícil de ser diagnosticada e tratada. Uma avaliação multidisciplinar e a colaboração próxima com colegas da área não-pediátrica são adotadas na maioria dos grandes centros que cuidam de pacientes pediátricos com SC. Embora os protocolos pediátricos sejam derivados de dados em adultos, existem diferenças significativas entre a SC no adulto e na infância. Além disso, parâmetros evolutivos finais, incluindo altura final, densidade mineral óssea, função reprodutiva, composição corporal e saúde psicológica trazem desafios no cuidado pediátrico. Este artigo procura oferecer uma visão geral da SC pediátrica, focalizando algumas das diferenças entre a SC adulta e a pediátrica.

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Efficient Short-Term Control of Hypercortisolaemia by Low-Dose Etomidate in Severe Paediatric Cushing’s Disease

Cited by 63 publications

References 35 publications

THERAPY IN ENDOCRINE DISEASE: Etomidate in the management of hypercortisolaemia in Cushing's syndrome: a review

THERAPY IN ENDOCRINE DISEASE: Etomidate in the management of hypercortisolaemia in Cushing's syndrome: a review

Long-term anterior pituitary function in patients with paediatric Cushing’s disease treated with pituitary radiotherapy

Pediatric Cushing's syndrome: clinical features, diagnosis, and treatment

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