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Nicola Tufton, Kirun Gunganah, Shazia Hussain, Maralyn Druce, Robert Carpenter, Michael Ashby, William M Drake, Scott A Akker
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...
October 19, 2016: Clinical Endocrinology
William Grey, Rosaline Hulse, Anna Yakovleva, Dilyana Genkova, Benjamin Whitelaw, Ellen Solomon, Salvador J Diaz-Cano, Louise Izatt
The REarranged during Transfection (RET) proto-oncogene is a receptor tyrosine kinase involved in growth and differentiation during embryogenesis and maintenance of the urogenital and nervous systems in mammals. Distinct mutations across hotspot RET exons can cause Multiple Endocrine Neoplasia Type 2A (MEN2A) characterised by development of medullary thyroid cancer (MTC), phaeochromocytoma (PCC) and primary hyperparathyroidism (PHPT), with a strong correlation between genotype and phenotype. Here, we report a 42-year-old man presented in the clinic with a unilateral PCC, with subsequent investigations revealing a nodular and cystic thyroid gland...
October 5, 2016: Endocrine Pathology
James Roy, Zakir Akhunji, Virag Kushwaha, James Mackie, Nigel Jepson
Pheochromocytomas have been reported prior to and during coronary artery bypass surgery. We present a patient with an undiagnosed pheochromocytoma who presented with labile hypertension following coronary artery bypass surgery. This case calls attention to the inclusion of an undiagnosed pheochromocytoma in the differential diagnosis for all patients who develop labile hypertension in the postoperative period following cardiac surgery.
October 3, 2016: Journal of Cardiac Surgery
B G Challis, R T Casey, H L Simpson, M Gurnell
Phaeochromocytomas and paragangliomas (PPGLs) are catecholamine secreting neuroendocrine tumours that predispose to haemodynamic instability. Currently, surgery is the only available curative treatment, but carries potential risks including hypertensive and hypotensive crises, cardiac arrhythmias, myocardial infarction and stroke, due to tumoral release of catecholamines during anaesthetic induction and tumour manipulation. The mortality associated with surgical resection of PPGL has significantly improved from 20-45% in the early 20(th) century(1) to 0-2...
October 3, 2016: Clinical Endocrinology
Nicola Tufton, Lucy Shapiro, Umasuthan Srirangalingam, Polly Richards, Anju Sahdev, V K Ajith Kumar, Lorraine McAndrew, Lee Martin, Daniel Berney, John Monson, Shern L Chew, Mona Waterhouse, Maralyn Druce, Márta Korbonits, Karl Metcalfe, William M Drake, Helen L Storr, Scott A Akker
OBJECTIVE: For 'asymptomatic carriers' of the succinate dehydrogenase subunit B (SDHB) gene mutations, there is currently no consensus as to the appropriate modality or frequency of surveillance imaging. We present the results of a surveillance programme of SDHB mutation carriers. DESIGN: Review of clinical outcomes of a surveillance regimen in patients identified to have a SDHB gene mutation, based on annual MRI, in a single UK tertiary referral centre. PATIENTS: A total of 92 patients were identified with an SDHB gene mutation...
September 28, 2016: Clinical Endocrinology
Eleni Daniel, Robert Jones, Matthew Bull, John Newell-Price
INTRODUCTION: Patients with SDHx mutations need long-term radiological surveillance for the development of paragangliomas and phaeochromocytomas, but no longitudinal data exist. We assessed the performance of rapid-sequence non-contrast magnetic resonance imaging (MRI) in the long-term monitoring of patients with SDHx mutations. METHODS: Retrospective study between 2005-2015 at a University Hospital and regional endocrine genetics referral center. Clinical and imaging data of forty-seven patients with SDHx mutations [SDHB (36), SDHC (6), SDHD (5)] who had surveillance for detection of paragangliomas by rapid-sequence non-contrast MRI (base of skull to pubic symphysis) were collected...
September 15, 2016: European Journal of Endocrinology
Henrique V Luiz, Run Yu, Katherine Wolf, Ning Miao, Andrew Mannes, Karel Pacak
No abstract text is available yet for this article.
September 13, 2016: Clinical Endocrinology
Paul Trayhurn
Brown adipose tissue (BAT) was identified as a thermogenic organ in 1961, and in 1978 shown to be the major site of thermoregulatory non-shivering thermogenesis in rats acclimated to the cold. Investigations in the mid-late 1970s established the uncoupling of oxidative phosphorylation through a proton conductance pathway across the mitochondrial inner membrane as the mechanism for heat production in BAT, this being regulated by UCP1 which was first discovered as a 32,000 Mr cold-inducible protein. These developments came when those concerned with nutritional energetics were proposing that thermogenesis is a significant factor in energy balance and the aetiology of obesity...
September 10, 2016: Biochimie
Victor Srougi, Jose L Chambo, Fabio Y Tanno, Iracy S Soares, Madson Q Almeida, Maria A A Pereira, Miguel Srougi, Maria C Fragoso
PURPOSE: To evaluate the presentation and early surgical outcomes of elderly patients undergoing adrenalectomy for phaeochromocytoma. PATIENTS AND METHODS: A retrospective search was performed of our adrenal disorders database for patients who underwent surgery for phaeochromocytoma or paraganglioma between 2009 and 2014. Patients >60 years old were classified as elderly. The clinical manifestations, intraoperative course, and early postoperative outcomes of elderly patients were compared to those of younger individuals (<60 years old)...
July 2016: International Braz J Urol: Official Journal of the Brazilian Society of Urology
D Maccora, G V Walls, G P Sadler, R Mihai
INTRODUCTION The 2012 British Association of Endocrine and Thyroid Surgeons audit report showed that only 86 of 1359 patients who underwent adrenalectomy had a bilateral operation; thus the experience with this procedure remains limited. METHODS Retrospective review of patients undergoing bilateral adrenalectomy in a tertiary referral centre. RESULTS Between November 2005 and January 2016, bilateral adrenalectomy was performed in 23 patients (6 male, 17 female, age 43 ± 4 years) diagnosed with Cushing's disease (n = 13), hereditary phaeochromocytomas (n = 6), adrenocortical cancer (n = 2), colorectal metastatic disease (n = 1) and adrenocortical adenomas (n = 1)...
August 23, 2016: Annals of the Royal College of Surgeons of England
Géza Tóth, Attila Patócs, Miklós Tóth
Phaeochromocytoma is a tumor of the catecholamine-producing cells of the adrenal gland. Extraadrenal phaeochromocytomas are frequently called paragangliomas. The majority of phaeochromocytomas are sporadic, however, about 25-30% are caused by genetic mutation. These tumor are frequently referred as hereditary phaeochromocytomas/paragangliomas. Their incidence increases continuously which can be attributed to availability of genetic examination and to the discovery of novel genes. The 47-year-old female patient underwent abdominal computed tomography which revealed bilateral adrenal gland enlargement...
August 2016: Orvosi Hetilap
M H Petersen, T B Christophersen, P S Hansen, J Hangaard
No abstract text is available yet for this article.
November 1, 2016: International Journal of Cardiology
Mehmet Ali Erdoğan, Muharrem Uçar, Ahmet Selim Özkan, Ülkü Özgül, Mahmut Durmuş
Phaeochromocytoma is a catecholamine-secreting vascular tumour that is derived from chromaffin cell. Lethal cardiovascular complications, such as serious hypertension, myocardial infarction and aortic dissection, may occur because of uncontrolled catecholamine release. Each stage of anaesthesia management has vital importance because of this destructive catecholamine secretion that may occur during induction, perioperative stage and surgical manipulation. In this study, we report regarding the preoperative preparation and severe, persistent hypertension attack management with a combination of α-adrenergic blockade, β-adrenergic blockade, sodium nitroprusside and remifentanil in a patient who underwent laparoscopic surgery for phaeochromocytoma...
February 2016: Turk J Anaesthesiol Reanim
Claude Lentschener, Sebastien Gaujoux, Christophe Baillard, Bertrand Dousset
No abstract text is available yet for this article.
June 22, 2016: Clinical Endocrinology
Christopher Boot, Barry Toole, Sarah J Johnson, Steve G Ball, Dermot Neely
BACKGROUND: Measurement of plasma metanephrines (PMETS) is regarded as one of the best screening tests for phaeochromocytoma/paraganglioma (P/PGL). Current guidelines recommend that samples are ideally collected in the supine position after 30 minutes rest and interpreted using supine reference ranges, in order to optimise the diagnostic performance of the test. Current practice in our centre is to collect samples for PMETS from seated patients. The aim of the study was to determine if seated sampling for PMETS provides acceptable diagnostic performance in our centre...
May 11, 2016: Annals of Clinical Biochemistry
Ruth Casey, Tomas Patrick Griffin, Deirdre Wall, Michael C Dennedy, Marcia Bell, Paula O'Shea
BACKGROUND: The Endocrine Society Clinical Practice Guideline on Phaeochomocytoma and Paraganglioma (PPGL) recommends phlebotomy for plasma-free metanephrines (PMets) with patients fasted and supine using appropriately defined reference intervals. Studies have shown higher diagnostic sensitivities using these criteria. Further, with seated-sampling protocols, for result interpretation, reference intervals that do not compromise diagnostic sensitivity should be employed. OBJECTIVE: To determine the impact on diagnostic performance and financial cost of using supine reference intervals for result interpretation with our current PMets fasted/seated-sampling protocol...
May 10, 2016: Annals of Clinical Biochemistry
D C D Hope, J M Palan
A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia, hyperglycaemia, nausea and vomiting. She was initially managed for diabetic ketoacidosis with severe dehydration. However, persistent episodic hypertension and tachycardia led the investigating team to identify significantly raised urinary metanephrines and a left-sided adrenal mass; Iodine-123-meta-iodobenzylguanidine single photon emission computer tomography scan (MIBG SPECT/CT) showed avid uptake of tracer, confirming a left-sided phaeochromocytoma...
2016: BMJ Case Reports
Laurence Amar, Charlotte Lussey-Lepoutre, Jacques W M Lenders, Juliette Djadi-Prat, Pierre-Francois Plouin, Olivier Steichen
OBJECTIVES: To systematically review the incidence and factors associated with recurrences or new tumors after apparent complete resection of pheochromocytoma or thoraco-abdomino-pelvic paraganglioma. DESIGN: A systematic review and meta-analysis of published literature was performed. METHODS: Pubmed and Embase from 1980 to 2012 were searched for studies published in English on patients with non-metastatic pheochromocytoma or thoraco-abdomino-pelvic paraganglioma, complete tumor resection, postoperative follow-up exceeding 1 month, and recurrence or new tumor documented by pathology, hormonal dosages, or imaging tests...
October 2016: European Journal of Endocrinology
Fiona Lalloo
About 30% of phaeochromocytomas or paragangliomas are genetic. Whilst some individuals will have clinical features or a family history of inherited cancer syndrome such as neurofibromatosis type 1 (NF1) or multiple endocrine neoplasia 2 (MEN2), the majority will present as an isolated case. To date, 14 genes have been described in which pathogenic mutations have been demonstrated to cause paraganglioma or phaeochromocytoma . Many cases with a pathogenic mutation may be at risk of developing further tumours...
2016: Recent Results in Cancer Research
Elizabeth M Winter, Alberto M Pereira, Eleonora P Corssmit
This is the first report of unilateral hypercortisolism and phaeochromocytoma that cannot be explained by medullary tumourigenic adrenocorticotropic hormone (ACTH) excretion. The patient was referred for an adrenal incidentaloma with hypertension but no Cushingoid features, disturbed glucose tolerance and osteopaenia. Additional testing revealed hypercortisolism with suppressed ACTH, and a right-sided phaeochromocytoma with typical radiographic appearance. Resection of the right adrenal completely normalised the clinical symptoms and biochemistry, and increased ACTH concentrations, implicating initial suppression...
2016: BMJ Case Reports
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