{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:48Z","timestamp":1784557068141,"version":"3.55.0"},"reference-count":24,"publisher":"Bioscientifica","license":[{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,3,1]]},"abstract":"<jats:sec>\n                    <jats:title>Summary<\/jats:title>\n                    <jats:p>\n                      A 49-year-old teacher presented to his general physician with lethargy and lower limb weakness. He had noticed polydipsia, polyuria, and had experienced weight loss, albeit with an increase in central adiposity. He had no concomitant illnesses and took no regular medications. He had hypercalcaemia (adjusted calcium: 3.34 mmol\/L) with hyperparathyroidism (parathyroid hormone: 356 ng\/L) and hypokalaemia (K: 2.7 mmol\/L) and was admitted for i.v. potassium replacement. A contrast-enhanced CT chest\/abdomen\/pelvis scan revealed a well-encapsulated anterior mediastinal mass measuring 17 \u00d7 11 cm with central necrosis, compressing rather than invading adjacent structures. A neck ultrasound revealed a 2 cm right inferior parathyroid lesion. On review of CT imaging, the adrenals appeared normal, but a pancreatic lesion was noted adjacent to the uncinate process. His serum cortisol was 2612 nmol\/L, and adrenocorticotrophic hormone was elevated at 67 ng\/L, followed by inadequate cortisol suppression to 575 nmol\/L from an overnight dexamethasone suppression test. His pituitary MRI was normal, with unremarkable remaining anterior pituitary biochemistry. His admission was further complicated by increased urine output to 10 L\/24 h and despite three precipitating factors for the development of diabetes insipidus including hypercalcaemia, hypokalaemia, and hypercortisolaemia, due to academic interest, a water deprivation test was conducted. An\n                      <jats:sup>18<\/jats:sup>\n                      flurodeoxyglucose-PET (FDG-PET) scan demonstrated high avidity of the mediastinal mass with additionally active bilateral superior mediastinal nodes. The pancreatic lesion was not FDG avid. On 68Ga DOTATE-PET scan, the mediastinal mass was moderately avid, and the 32 mm pancreatic uncinate process mass showed significant uptake. Genetic testing confirmed multiple endocrine neoplasia type 1.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Learning points<\/jats:title>\n                    <jats:list list-type=\"bullet\">\n                      <jats:list-item>\n                        <jats:p>In young patients presenting with primary hyperparathyroidism, clinicians should be alerted to the possibility of other underlying endocrinopathies.<\/jats:p>\n                        <jats:p>In patients with multiple endocrine neoplasia type 1 (MEN-1) and ectopic adrenocorticotrophic hormone syndrome (EAS), clinicians should be alerted to the possibility of this originating from a neoplasm above or below the diaphragm.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Although relatively rare compared with sporadic cases, thymic carcinoids secondary to MEN-1 may also be associated with EAS.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Electrolyte derangement, in particular hypokalaemia and hypercalcaemia, can precipitate mild nephrogenic diabetes insipidus.<\/jats:p>\n                      <\/jats:list-item>\n                    <\/jats:list>\n                  <\/jats:sec>","DOI":"10.1530\/edm-21-0207","type":"journal-article","created":{"date-parts":[[2022,3,10]],"date-time":"2022-03-10T04:15:08Z","timestamp":1646885708000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":1,"title":["Multiple electrolyte disturbances as the presenting feature of multiple endocrine neoplasia type 1 (MEN-1)"],"prefix":"10.1530","volume":"2022","author":[{"given":"Adrian Po Zhu","family":"Li","sequence":"first","affiliation":[{"name":"Department of Endocrinology ASO\/EASO COM, King \u2019s College Hospital NHS Foundation Trust, Denmark Hill, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheela","family":"Sathyanarayan","sequence":"additional","affiliation":[{"name":"Department of Endocrinology ASO\/EASO COM, King \u2019s College Hospital NHS Foundation Trust, Denmark Hill, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salvador","family":"Diaz-Cano","sequence":"additional","affiliation":[{"name":"Departments of Cellular Pathology and Molecular Pathology, Queen Elizabeth Hospital, Birmingham, UK"},{"name":"Division of Cancer Studies, King\u2019s College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sobia","family":"Arshad","sequence":"additional","affiliation":[{"name":"Department of Endocrinology ASO\/EASO COM, King \u2019s College Hospital NHS Foundation Trust, Denmark Hill, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eftychia E","family":"Drakou","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology, Guy\u2019s Cancer Centre \u2013 Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, Great Maze Pond, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Royce P","family":"Vincent","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry, King\u2019s College Hospital NHS Foundation Trust, Denmark Hill, London, UK"},{"name":"Faculty of Life Sciences and Medicine, School of Life Course Sciences, King\u2019s College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley B","family":"Grossman","sequence":"additional","affiliation":[{"name":"Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK"},{"name":"Barts and the London School of Medicine, Centre for Endocrinology, William Harvey Institute, London, UK"},{"name":"Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon J B","family":"Aylwin","sequence":"additional","affiliation":[{"name":"Department of Endocrinology ASO\/EASO COM, King \u2019s College Hospital NHS Foundation Trust, Denmark Hill, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6662-804X","authenticated-orcid":true,"given":"Georgios K","family":"Dimitriadis","sequence":"additional","affiliation":[{"name":"Department of Endocrinology ASO\/EASO COM, King \u2019s College Hospital NHS Foundation Trust, Denmark Hill, London, UK"},{"name":"Obesity, Type 2 Diabetes and Immunometabolism Research Group, Department of Diabetes, Faculty of Life Sciences, School of Life Course Sciences, King\u2019s College London, London, UK"},{"name":"Division of Reproductive Health, Warwick Medical School, University of Warwick, Coventry, 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Treasure Island [FL), StatPearls Publishing 2021."},{"key":"bib4","series-title":"Endocrine-Related Cancer","doi-asserted-by":"publisher","first-page":"R173","DOI":"10.1530\/ERC-17-0036","article-title":"Paraneoplastic endocrine syndromes","volume":"24","author":"Dimitriadis","year":"2017","unstructured":"Dimitriadis GKAngelousi AWeickert MORandeva HSKaltsas GGrossman A. Paraneoplastic endocrine syndromes. Endocrine-Related Cancer201724R173\u2013R190. 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(https:\/\/doi.org\/10.1002\/cncr.28341)10.1002\/cncr.28341"},{"key":"bib14","series-title":"Journal of Clinical Endocrinology and Metabolism","doi-asserted-by":"publisher","first-page":"3449","DOI":"10.1210\/clinem\/dgaa507","article-title":"The clinical features and molecular mechanisms of ACTH-secreting pancreatic neuroendocrine tumors","volume":"105","author":"Zhang","year":"2020","unstructured":"Zhang CJin JXie JYe LSu TJiang LZhou WJiang YWu LWang TThe clinical features and molecular mechanisms of ACTH-secreting pancreatic neuroendocrine tumors. Journal of Clinical Endocrinology and Metabolism20201053449\u20133458. 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(https:\/\/doi.org\/10.1200\/JCO.2003.12.142)"},{"key":"bib18","series-title":"Neuroendocrinology","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1159\/000443171","article-title":"Enets consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors","volume":"103","author":"Falconi","year":"2016","unstructured":"Falconi MEriksson BKaltsas GBartsch DKCapdevila JCaplin MKos-Kudla BKwekkeboom DRindi GKl\u00f6ppel GEnets consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology2016103153\u2013171. (https:\/\/doi.org\/10.1159\/000443171)10.1159\/000443171"},{"key":"bib19","series-title":"American Journal of Physiology: Renal Physiology","first-page":"F1313","article-title":"AQP3, p-AQP2 and AQP2 expression is reduced in polyuric rats with hypercalcemia: prevention by cAMP-PDE inhibitors","volume":"283","author":"Wang","year":"2002","unstructured":"Wang WLi CKwon THKnepper MAFrokiaer JNielsen S. AQP3, p-AQP2 and AQP2 expression is reduced in polyuric rats with hypercalcemia: prevention by cAMP-PDE inhibitors. American Journal of Physiology: Renal Physiology2002283F1313\u2013F1325. (https:\/\/doi.org\/10.1152\/ajprenal.00040.2002)"},{"key":"bib20","series-title":"Journal of Clinical Investigation","doi-asserted-by":"publisher","first-page":"1960","DOI":"10.1172\/JCI118628","article-title":"Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex","volume":"97","author":"Marples","year":"1996","unstructured":"Marples DFrokiaer JDorup JKnepper MANielsen S. Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex. Journal of Clinical Investigation1996971960\u20131968. 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(https:\/\/doi.org\/10.4158\/EP11038.CR)10.4158\/EP11038.CR"},{"key":"bib24","series-title":"Endocrine","doi-asserted-by":"publisher","first-page":"183","DOI":"10.1007\/s12020-013-0141-6","article-title":"Familial Cushing syndrome due to thymic carcinoids in a multiple endocrine neoplasia type 1 kindred","volume":"47","author":"Li","year":"2014","unstructured":"Li XSu JZhao LWu JDing XFang FWu YSun HPeng Y. Familial Cushing syndrome due to thymic carcinoids in a multiple endocrine neoplasia type 1 kindred. Endocrine201447183\u2013190. 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