Kirun Gunganah

Etomidate in the emergency management of hypercortisolemia

We describe a protocol for etomidate infusion for the emergency management of hypercortisolemia. Etomidate is commenced at 2.5 mg/h and titrated subsequently according to cortisol levels. It is well tolerated without any sedative effects and can be administered safely via peripheral access for use in the general ward setting.

Recurrent pancreatitis in a patient with familial hypocalciuric hypercalcaemia treated successfully with cinacalcet

Summary A 22-year-old female student presented with a history of recurrent pancreatitis. The commonest causes of pancreatitis, including drugs, gallstones, corticosteroids, excess alcohol and hypertriglyceridaemia, were excluded. She was found to have an elevated serum calcium level that was considered to be the cause of her pancreatitis, with a detectable serum parathyroid hormone (PTH). An initial diagnosis of primary hyperparathyroidism was made. However, two neck explorations failed to reveal a parathyroid adenoma. She was referred to our unit three years later as her episodes of pancreatitis were becoming more frequent and her calcium level remained persistently elevated. Her investigations were as follows: elevated adjusted calcium level of 2.79 mmol/l (2.2–2.58), PTH level of 4.2 pmol/l (0.6–6.0), low 24 h urine calcium of 0.3 mmol/l and a urine calcium:creatinine ratio of <0.003. A clinical diagnosis of familial hypocalciuric hypercalcaemia (FHH) was made and confirmed on genetic testing that showed a c.1703 G>A mutation in the calcium-sensing receptor gene. Although the hypercalcaemia of FHH is usually without sequelae due to the generalised changes in calcium sensing, in the presence of this complication she was started on cinacalcet 30 mg daily. She had one further episode of pancreatitis with calcium levels ranging between 2.53 and 2.66 mmol/l. Her cinacalcet was gradually increased to 30 mg three times daily, maintaining her calcium levels in the range of 2.15–2.20 mmol/l. She has not had a further episode of pancreatitis for more than 2 years. FHH is usually a benign condition with minimal complications from hypercalcaemia. Pancreatitis has been reported rarely, and no clear management strategy has been defined in these cases. Cinacalcet was successfully used in treating recurrent pancreatitis in a patient with FHH by maintaining calcium levels in the lower part of the reference range. Whether or not this is an effective long-term treatment remains yet to be seen. Learning points FHH is an important differential diagnosis for hypercalcaemia. FHH can rarely cause pancreatitis. No clear strategy is available to help in the management of patients with pancreatitis due to FHH. Cinacalcet was effective in lowering serum calcium levels and reducing the frequency of pancreatitis in our patient with FHH.

Circadian variation in serum cortisol during hydrocortisone replacement is not attributable to changes in cortisol binding globulin concentrations

Patients taking hydrocortisone (HC) replacement for primary or secondary adrenal failure require individual adjustment of their dose. In addition to modifying the administered doses of HC for each patient, physicians are increasingly interested in variations in the bioavailability of glucocorticoid replacement. One potential determinant of the bioavailability of replaced HC is a variation in serum cortisol‐binding globulin (CBG) concentration, which may, in turn, affect interpretation of cortisol profiles and individual dose selection for patients on hydrocortisone replacement therapy.

Eplerenone use in primary aldosteronism during pregnancy.

Primary aldosteronism (PA) in pregnancy is rare. Due to pharmacological limitations and risks associated with surgical intervention during pregnancy, clinical decision making in this area is difficult. We report the short‐term use of eplerenone in the management of hypertension and hypokalemia due to PA in pregnancy.

Phaeochromocytoma and Paraganglioma Excision Involving the Great Vessels

Objective/background Phaeochromocytomas and paragangliomas are vascular neuroendocrine tumours distributed between the neck and the pelvis and may be associated with catecholamine secretion. The aim of the study was to describe the complex surgical management required to excise these tumours when in close proximity to the great vessels (aorta and vena cava). Methods This was a retrospective case series. Patients included those undergoing surgical excision of a phaeochromocytoma or paraganglioma involving the great vessels. Data on clinical presentation; genetic mutations; tumour location; catecholamine/metanephrine secretion; surgical strategy; pre-, intra-, and post-operative course were collated. Results Five patients (age range 16–60 years) were identified; three had thoracic paragangliomas located under the arch of the aorta, one had an abdominal paraganglioma invading the aorta, and one had a massive phaeochromocytoma invading the inferior vena cava via the adrenal vein. Three patients had predisposing germline mutations. All patients had adrenergic blockade prior to surgery. A diverse range of complex surgical techniques were employed to excise tumours, including cardiopulmonary bypass, aortic resection, grafting and venotomy of the vena cava. Early post-operative complications were limited. Conclusions Excision of phaeochromocytomas and paragangliomas involving the great vessels is high risk surgery optimally undertaken within a multidisciplinary setting in a tertiary referral centre. Comprehensive radiological and biochemical assessment, meticulous pre-operative preparation and close intra- and post-operative monitoring are essential. Radiological imaging may be unable to resolve the tumour extent and anatomy pre-operatively and direct visualisation of the tumour may be the only way to clarify the surgical strategy. Pre-operative knowledge of the genetic predisposition may influence surgical management.

Alpha blockade-not to be underdone.

We read with interest the article on whether patients with hormonally functioning phaeochromocytomas and paragangliomas receive proper adrenoreceptor blockade (1). Since the removal of intravenous phenoxybenzamine (Goldshield) from the UK formulae due to worldwide lack of availability, providing patients with adequate alpha adrenoreceptor blockade has become more challenging. We conducted a small retrospective audit in our department to investigate the efficacy of oral Phenoxybenzamine compared to intravenous Phenoxybenzamine as we had formed the clinical impression that that pre-operative preparation is currently less safe than it used to be. This article is protected by copyright. All rights reserved.