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Mawson Wang Nepean Blue Mountains Local Health District, Katoomba, New South Wales, Australia

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Catherine Cho Nepean Blue Mountains Local Health District, Katoomba, New South Wales, Australia

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Callum Gray Nepean Blue Mountains Local Health District, Katoomba, New South Wales, Australia

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Thora Y Chai Department of Endocrinology, Nepean Blue Mountains Local Health District, Kingswood, New South Wales, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia

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Ruhaida Daud Nepean Blue Mountains Local Health District, Katoomba, New South Wales, Australia

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Matthew Luttrell Department of Endocrinology, Nepean Blue Mountains Local Health District, Kingswood, New South Wales, Australia

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Summary

We report the case of a 65-year-old female who presented with symptomatic hypercalcaemia (corrected calcium of 4.57 mmol/L) with confusion, myalgias and abdominal discomfort. She had a concomitant metabolic alkalosis (pH 7.46, HCO3 - 40 mmol/L, pCO2 54.6 mmHg). A history of significant Quick-Eze use (a calcium carbonate based antacid) for abdominal discomfort, for 2 weeks prior to presentation, suggested a diagnosis of milk-alkali syndrome (MAS). Further investigations did not demonstrate malignancy or primary hyperparathyroidism. Following management with i.v. fluid rehydration and a single dose of i.v. bisphosphonate, she developed symptomatic hypocalcaemia requiring oral and parenteral calcium replacement. She was discharged from the hospital with stable biochemistry on follow-up. This case demonstrates the importance of a detailed history in the diagnosis of severe hypercalcaemia, with MAS representing the third most common cause of hypercalcaemia. We discuss its pathophysiology and clinical importance, which can often present with severe hypercalcaemia that can respond precipitously to calcium-lowering therapy.

Learning points:

  • Milk-alkali syndrome is an often unrecognised cause for hypercalcaemia, but is the third most common cause of admission for hypercalcaemia.

  • Calcium ingestion leading to MAS can occur at intakes as low as 1.0–1.5 g per day in those with risk factors.

  • Early recognition of this syndrome can avoid the use of calcium-lowering therapy such as bisphosphonates which can precipitate hypocalcaemia.

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Kaja Grønning Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Archana Sharma Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Maria Adele Mastroianni Department of Haematology, Akershus University Hospital, Lorenskog, Norway

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Bo Daniel Karlsson Department of Radiology, Akershus University Hospital, Lorenskog, Norway

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Eystein S Husebye Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Kristian Løvås Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

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Summary

Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.

Learning points:

  • Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency.

  • Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically.

  • Steroid treatment before biopsy may affect diagnosis.

  • Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.

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Mohammed Faraz Rafey Galway University Hospitals, Galway, Ireland
HRB Clinical Research Facility, National University of Ireland Galway, Galway, Ireland

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Arslan Butt Galway University Hospitals, Galway, Ireland

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Barry Coffey Galway University Hospitals, Galway, Ireland

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Lisa Reddington Galway University Hospitals, Galway, Ireland

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Aiden Devitt Galway University Hospitals, Galway, Ireland

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David Lappin Galway University Hospitals, Galway, Ireland

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Francis M Finucane Galway University Hospitals, Galway, Ireland
HRB Clinical Research Facility, National University of Ireland Galway, Galway, Ireland

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Summary

We describe two cases of SGLT2i-induced euglycaemic diabetic ketoacidosis, which took longer than we anticipated to treat despite initiation of our DKA protocol. Both patients had an unequivocal diagnosis of type 2 diabetes, had poor glycaemic control with a history of metformin intolerance and presented with relatively vague symptoms post-operatively. Neither patient had stopped their SGLT2i pre-operatively, but ought to have by current treatment guidelines.

Learning points:

  • SGLT2i-induced EDKA is a more protracted and prolonged metabolic derangement and takes approximately twice as long to treat as hyperglycaemic ketoacidosis.

  • Surgical patients ought to stop SGLT2i medications routinely pre-operatively and only resume them after they have made a full recovery from the operation.

  • While the mechanistic basis for EDKA remains unclear, our observation of marked ketonuria in both patients suggests that impaired ketone excretion may not be the predominant metabolic lesion in every case.

  • Measurement of insulin, C-Peptide, blood and urine ketones as well as glucagon and renal function at the time of initial presentation with EDKA may help to establish why this problem occurs in specific patients.

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Joanna Prokop Departments of Endocrinology, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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João Estorninho Departments of Endocrinology, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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Sara Marote Departments of Internal Medicine, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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Teresa Sabino Departments of Endocrinology, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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Aida Botelho de Sousa Departments of Hemato-Oncology, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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Eduardo Silva Departments of Internal Medicine, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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Ana Agapito Departments of Endocrinology, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal

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Summary

POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a rare multisystemic disease. Clinical presentation is variable, the only mandatory criteria being polyneuropathy and monoclonal gammapathy in association with one major and one minor criterion. Primary adrenal insufficiency is rarely reported. We describe a case of a 33-year-old patient, in whom the presenting symptoms were mandibular mass, chronic sensory-motor peripheral polyneuropathy and adrenal insufficiency. The laboratory evaluation revealed thrombocytosis, severe hyperkalemia with normal renal function, normal protein electrophoresis and negative serum immunofixation for monoclonal protein. Endocrinologic laboratory work-up confirmed Addison’s disease and revealed subclinical primary hypothyroidism. Thoracic abdominal CT showed hepatosplenomegaly, multiple sclerotic lesions in thoracic vertebra and ribs. The histopathologic examination of the mandibular mass was nondiagnostic. Bone marrow biopsy revealed plasma cell dyscrasia and confirmed POEMS syndrome. Axillary lymphadenopathy biopsy: Castleman’s disease. Gluco-mineralocorticoid substitution and levothyroxine therapy were started with clinical improvement. Autologous hematopoietic cell transplantation (HCT) was planned, cyclophosphamide induction was started. Meanwhile the patient suffered two ischemic strokes which resulted in aphasia and hemiparesis. Cerebral angiography revealed vascular lesions compatible with vasculitis and stenosis of two cerebral arteries. The patient deceased 14 months after the diagnosis. The young age at presentation, multiplicity of manifestations and difficulties in investigation along with the absence of serum monoclonal protein made the diagnosis challenging. We report this case to highlight the need to consider POEMS syndrome in differential diagnosis of peripheral neuropathy in association with endocrine abnormalities even in young patients.

Learning points:

  • POEMS syndrome is considered a ‘low tumor burden disease’ and the monoclonal protein in 15% of cases is not found by immunofixation.

  • Neuropathy is the dominant characteristic of POEMS syndrome and it is peripheral, ascending, symmetric and affecting both sensation and motor function.

  • Endocrinopathies are a frequent feature of POEMS syndrome, but the cause is unknown.

  • The most common endocrinopathies are hypogonadism, primary hypothyroidism and abnormalities in glucose metabolism.

  • There is no standard therapy; however, patients with disseminated bone marrow involvement are treated with chemotherapy with or without HCT.

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Taisuke Uchida Department of Neurology, Respirology, Endocrinology and Metabolism, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Hideki Yamaguchi Department of Neurology, Respirology, Endocrinology and Metabolism, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Kazuhiro Nagamine Department of Neurology, Respirology, Endocrinology and Metabolism, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Tadato Yonekawa Department of Neurology, Respirology, Endocrinology and Metabolism, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Eriko Nakamura Department of Diagnostic Pathology, University of Miyazaki Hospital, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Nobuhiro Shibata Department of Clinical Oncology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Fumiaki Kawano Division of the Gastrointestinal, Endocrine and Pediatric Surgery, Department of Surgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Yujiro Asada Department of Diagnostic Pathology, University of Miyazaki Hospital, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Masamitsu Nakazato Department of Neurology, Respirology, Endocrinology and Metabolism, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

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Summary

We report a case of rapid pleural effusion after discontinuation of lenvatinib. A 73-year-old woman was diagnosed with poorly differentiated thyroid cancer with right pleural metastasis. Weekly paclitaxel treatment was performed for 18 weeks, but it was not effective. Oral administration of lenvatinib, a multi-target tyrosine kinase inhibitor, reduced the size of cervical and thoracic tumors and lowered serum thyroglobulin levels. Lenvatinib was discontinued on day 28 because of Grade 2 thrombocytopenia and Grade 3 petechiae. Seven days after discontinuation of lenvatinib, the patient was hospitalized because of dyspnea and right pleural effusion. Pleural effusion rapidly improved with drainage and re-initiation of lenvatinib and did not recur. Anorexia caused by lenvatinib led to undernutrition, which resulted in death 13 months after initiation of lenvatinib. Autopsy revealed extensive necrosis with primary and metastatic lesions, suggesting that the patient responded to lenvatinib. Physicians should be aware of the possibility of flare-up in patients with thyroid cancer treated with lenvatinib.

Learning points:

  • Autopsy findings revealed that lenvatinib was efficacious in treating poorly differentiated thyroid cancer without primary lesion resection.

  • Flare-up phenomenon may occur in thyroid cancer treated with lenvatinib.

  • Attention should be paid to flare-up phenomenon within a few days of discontinuing lenvatinib.

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Benedetta Zampetti Endocrinology Niguarda Hospital, Galeazzi Institute IRCCS, Milan, Italy

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Roberto Attanasio Endocrinology, Galeazzi Institute IRCCS, Milan, Italy

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Renato Cozzi Endocrinology Niguarda Hospital, Galeazzi Institute IRCCS, Milan, Italy

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Summary

A 69-year-old male was admitted for severe hyponatremia disclosed after an accidental fall. He was anticoagulated from 2 months after the implantation of a biologic aortic valve prosthesis. The work-up disclosed adrenal failure and MRI showed bilateral adrenal hemorrhage. Clinical picture and lab parameters normalized quickly after the appropriate replacement treatment. Anticoagulation excess should be added to the list of drugs potentially causing hyponatremia.

Learning points:

  • Hyponatremia requires a complete and timely workup in order to start an appropriate treatment for the improvement of clinical conditions.

  • History is crucial: a detailed list of drugs potentially causing hyponatremia should be collected. Anticoagulants should be added to the list, mostly in the event of excessive anticoagulation.

  • Intra-adrenal hemorrhage is a rare cause of hyponatremia and adrenal failure.

  • The ACTH test is still the gold standard for the diagnosis of hypoadrenalism.

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Bernardo Marques Endocrinology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Raquel G Martins Endocrinology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Guilherme Tralhão Surgery Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal

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Joana Couto Endocrinology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Sandra Saraiva Gastroenterology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Henrique Ferrão Surgery Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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João Ribeiro Oncology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Jacinta Santos Endocrinology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Teresa Martins Endocrinology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Ana Teresa Cadime Gastroenterology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Fernando Rodrigues Endocrinology Department, Instituto Português de Oncologia de Coimbra Franscisco Gentil, EPE, Coimbra, Portugal

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Summary

Gastric neuroendocrine neoplasms (GNENs) are classified into three types according to their aetiology. We present a clinical case of a female patient of 66 years and a well-differentiated (grade 2), type 3 GNEN with late liver metastasis (LM). The patient underwent surgical excision of a gastric lesion at 50 years of age, without any type of follow-up. Sixteen years later, she was found to have a neuroendocrine tumour (NET) metastatic to the liver. The histological review of the gastric lesion previously removed confirmed that it was a NET measuring 8 mm, pT1NxMx (Ki67 = 4%). 68Ga-DOTANOC PET/CT reported two LM and a possible pancreatic tumour/gastric adenopathy. Biopsies of the lesion were repeatedly inconclusive. She had a high chromogranin A, normal gastrin levels and negative anti-parietal cell and intrinsic factor antibodies, which is suggestive of type 3 GNEN. She underwent total gastrectomy and liver segmentectomies (segment IV and VII) with proven metastasis in two perigastric lymph nodes and both with hepatic lesions (Ki67 = 5%), yet no evidence of local recurrence. A 68Ga-DOTANOC PET/CT was performed 3 months after surgery, showing no tumour lesions and normalisation of CgA. Two years after surgery, the patient had no evidence of disease. This case illustrates a rare situation, being a type 3, well-differentiated (grade 2) GNEN, with late LM. Despite this, it was possible to perform surgery with curative intent, which is crucial in these cases, as systemic therapies have limited efficacy. We emphasise the need for extended follow-up in these patients.

Learning points:

  • GNENs have a very heterogeneous biological behaviour.

  • Clinical distinction between the three types of GNEN is essential to plan the correct management strategy.

  • LMs are rare and more common in type 3 and grade 3 GNEN.

  • Adequate follow-up is crucial for detection of disease recurrence.

  • Curative intent surgery is the optimal therapy for patients with limited and resectable LM, especially in well-differentiated tumours (grade 1 and 2).

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Su Ann Tee Department of Endocrinology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK

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Earn Hui Gan Department of Endocrinology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
Institute of Genetic Medicine, Newcastle University, Newcastle Upon Tyne, UK

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Mohamad Zaher Kanaan Departments of Ophthalmology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK

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David Ashley Price Departments of Infectious Disease and Tropical Medicine, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK

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Tim Hoare Radiology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK

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Simon H S Pearce Department of Endocrinology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
Institute of Genetic Medicine, Newcastle University, Newcastle Upon Tyne, UK

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Summary

Primary adrenal insufficiency secondary to syphilis is extremely rare, with only five cases being reported in the literature. We report a case of adrenal insufficiency as a manifestation of Treponema pallidum infection (tertiary syphilis). A 69-year-old, previously fit and well Caucasian male was found to have adrenal insufficiency after being admitted with weight loss, anorexia and postural dizziness resulting in a fall. Biochemical testing showed hyponatraemia, hyperkalaemia, and an inadequate response to Synacthen testing, with a peak cortisol level of 302 nmol/L after administration of 250 µg Synacthen. Abdominal imaging revealed bilateral adrenal hyperplasia with inguinal and retroperitoneal lymphadenopathy. He was started on hydrocortisone replacement; however, it was not until he re-attended ophthalmology with a red eye and visual loss 1 month later, that further work-up revealed the diagnosis of tertiary syphilis. Following a course of penicillin, repeat imaging 5 months later showed resolution of the abnormal radiological appearances. However, adrenal function has not recovered and 3 years following initial presentation, the patient remains on both glucocorticoid and mineralocorticoid replacement. In conclusion, this case highlights the importance of considering syphilis as a potential differential diagnosis in patients presenting with adrenal insufficiency and bilateral adrenal masses, given the recent re-emergence of this condition. The relative ease of treating infectious causes of adrenal lesions makes accurate and timely diagnosis crucial.

Learning points:

  • Infectious causes, including syphilis, should be excluded before considering adrenalectomy or biopsy for any patient presenting with an adrenal mass.

  • It is important to perform a full infection screen including tests for human immunodeficiency virus, other blood-borne viruses and concurrent sexually transmitted diseases in patients presenting with bilateral adrenal hyperplasia with primary adrenal insufficiency.

  • Awareness of syphilis as a potential differential diagnosis is important, as it not only has a wide range of clinical presentations, but its prevalence has been increasing in recent times.

Open access
Gordon Sloan Diabetes and Endocrinology Department, Barnsley District General Hospital, Barnsley, UK

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Tania Kakoudaki Diabetes and Endocrinology Department, Barnsley District General Hospital, Barnsley, UK

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Nishant Ranjan Diabetes and Endocrinology Department, Barnsley District General Hospital, Barnsley, UK

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Summary

We report a case of a 63-year-old man who developed diabetic ketoacidosis (DKA) associated with canagliflozin, a sodium glucose co-transporter 2 (SGLT-2) inhibitor. He presented acutely unwell with a silent myocardial infarction, diverticulitis and DKA with a minimally raised blood glucose level. Standard therapy for DKA was initiated. Despite this, ketonaemia persisted for a total of 12 days after discontinuation of canagliflozin. Glucosuria lasting for several days despite discontinuation of the medications is a recognised phenomenon. However, this is the longest duration of ketonaemia to be reported. The cause of prolonged SGLT-2 inhibition remains uncertain. Deviation from the normal DKA treatment protocol and use of personalised regimens may be required in order to prevent relapse into ketoacidosis while avoiding hypoglycaemia in those that develop this condition.

Learning points:

  • Diabetic ketoacidosis (DKA) may develop in the presence of lower-than-expected blood glucose levels in patients treated with a sodium glucose co-transporter 2 (SGLT-2) inhibitor.

  • Certain individuals prescribed with SGLT-2 inhibitors may be more at risk of DKA, for example, those with a low beta cell function reserve, excessive alcohol consumption and a low carbohydrate diet.

  • In order to reduce the risk of SGLT-2 inhibitor-associated DKA, all patients must be carefully selected before prescription of the medication and appropriately educated.

  • Increased serum ketone levels and glucosuria have been reported to persist for several days despite discontinuation of their SGLT-2 inhibitor.

  • Physicians should consider individualised treatment regimens for subjects with prolonged DKA in the presence of SGLT-2 inhibition.

Open access
Carine Ghassan Richa Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon
Lebanese University, Hadath, Lebanon

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Khadija Jamal Saad Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon
Lebanese University, Hadath, Lebanon

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Georges Habib Halabi Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon
Mount Lebanon Hospital, Beirut, Lebanon

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Elie Mekhael Gharios Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon
Mount Lebanon Hospital, Beirut, Lebanon

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Fadi Louis Nasr Mount Lebanon Hospital, Beirut, Lebanon

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Marie Tanios Merheb Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon
Mount Lebanon Hospital, Beirut, Lebanon

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Summary

The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome. The latter is present in two out of three cases and in the remaining one, primary tumor could not be localized, representing a small fraction of patients with paraneoplastic Cushing. Diagnosis is established in the setting of high clinical suspicion by documenting an elevated cortisol level, ACTH and doing dexamethasone suppression test. Treatment options include management of the primary tumor by surgery and chemotherapy and treating Cushing syndrome. Prognosis is poor in SCLC. We concluded that in front of a high clinical suspicion, ectopic Cushing syndrome diagnosis should be considered, and identification of the primary tumor is essential.

Learning points:

  • Learning how to suspect ectopic Cushing syndrome and confirm it among all the causes of excess cortisol.

  • Distinguish between occult and severe ectopic Cushing syndrome and etiology.

  • Providing the adequate treatment of the primary tumor as well as for the cortisol excess.

  • Prognosis depends on the differentiation and type of the primary malignancy.

Open access