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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Aim: To study the prevalence of germline mutations of the aryl-hydrocarbon receptor interacting protein (AIP) gene in a large cohort of patients seen in the Oxford Centre for Diabetes Endocrinology and Metabolism (OCDEM), UK, with apparently sporadic pituitary adenomas, who were either diagnosed or had relevant clinical manifestations by the age of 40 years. Patients: We prospectively investigated all patients who were seen at Oxford University Hospital, OCDEM, and a tertiary referral centre, between 2012 and 2013, and presented with pituitary tumours under the age of 40 years and with no family history: a total of 127 patients were enrolled in the study. Methods: Leukocyte-origin genomic DNA underwent sequence analysis of exons 1-6 and the flanking intronic regions of the AIP gene (NM_003977.2), with dosage analysis by multiplex ligation-dependent probe amplification. Results: AIP variants were detected in 3% of the 127 patients, comprising four of 48 patients with acromegaly (8%), 0 of 43 with prolactinomas, 0 of the 20 patients with non-functioning adenomas, 0 of 15 with corticotroph adenomas and 0 of one with a thyrotroph adenomas. Definite pathogenetic mutations were seen in 2/4 variants, comprising 4.2% of patients with acromegaly. Conclusions: This prospective cohort study suggests a relatively low prevalence of AIP gene mutations in young patients with apparently sporadic pituitary adenomas presenting to a tertiary pituitary UK centre. Those with somatotroph macroadenomas have a higher rate of AIP mutation. These findings should inform discussion of genetic testing guidelines.

Primary ovarian NET is rare and likely underrepresented in the literature. It can no longer be simply dismissed as a generally benign entity and may present with both the carcinoid syndrome and carcinoid heart disease. It requires careful evaluation and long-term review.

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The authors apologise for the publication of an error in Table 2 of this article published in the European Journal of Endocrinology 167 137–143. They wish to make clear in Table 2 that they are stipulating the dose of etomidate and that the corresponding dose of hydrocortisone for complete blockade should be 0.5–1.0 mg/h. The correct table is published in full below.Table 2Treatment of hypercorticolism with etomidate: Recommendations.Infusion rate optionsBlockadeCortisol levelBiochemical monitoringOtherEtomidate (IV) 0.04–0.05 m/kg per h=2.5–3.0 mg/hPartial to complete depending on clinical circumstance of the patientTitrate to serum cortisol 500–800 nmol/l in physiologically stressed patient, 150–300 nmol/l in non-physiologically stressed patientPotassium level Cortisol levelSedation scoring initially every two hours then every 12 hours after first 24 hoursHydrocortisone IV 0.5–1.0 mg/hComplete (will need steroid replacement)<150 nmol/lPotassium level Cortisol levelThis table could now be used as a practical guide for clinicians commencing infusions on the ward of etomidate and required hydrocortisone replacement.

Optimal management depends on recognition of immune reconstitution inflammatory syndrome as a unique syndrome by healthcare providers, rather than a failure of highly active antiretroviral therapy or an adverse drug reaction. Our understanding of dermatologic immune reconstitution inflammatory syndrome continues to evolve as the diversity of reported cutaneous immune reconstitution inflammatory syndrome-associated disorders expands.