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Maria Flynn Department of Medicine, University of Calgary, Alberta, Canada

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Christopher Noss Department of Anesthesiology, Perioperative, and Pain Medicine, University of Calgary, Alberta, Canada

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Robert Miller Department of Cardiac Sciences, University of Calgary, Alberta, Canada

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Corey Adams Department of Cardiac Sciences, University of Calgary, Alberta, Canada

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Dean Ruether Department of Medicine, University of Calgary, Alberta, Canada

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Denise Chan Department of Radiology, University of Calgary, Alberta, Canada

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Janice Pasieka Department of Surgery, University of Calgary, Alberta, Canada

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Kirstie Lithgow Department of Medicine, University of Calgary, Alberta, Canada

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Summary

Carcinoid heart disease is a rare complication of carcinoid syndrome, resulting in right-sided valvular heart disease and subsequent heart failure due to long-term exposure to vasoactive substances. The management of this condition is complex, often requiring surgical intervention. Current perioperative regimens entail the use of prophylactic somatostatin analogs to prevent carcinoid crisis; however, regimens vary widely among practitioners and evidence supporting their efficacy in this clinical setting is mixed. This case report describes the perioperative management of a 65-year-old man with carcinoid heart disease requiring tricuspid and pulmonary valve replacement surgery. As an adjunct to somatostatin analog therapy, the novel tyrosine hydroxylase inhibitor, telotristat, was initiated preoperatively. This combination resulted in normalization of preoperative urinary 5-HIAA levels. The patient successfully underwent tricuspid and pulmonic valve replacement without evidence of carcinoid crisis. This clinical case is the first published documenting the use of telotristat in the perioperative period in a patient with carcinoid syndrome and carcinoid heart disease and was associated with a good long-term outcome despite the high-risk nature of the case.

Learning points

  • Carcinoid crisis is a life-threatening complication of carcinoid syndrome, resulting in hemodynamic instability, bronchospasm, and arrhythmia.

  • Cardiac surgical patients with carcinoid syndrome present a unique challenge as they are subject to physiologic conditions and medications which can potentiate intraoperative carcinoid crisis.

  • Perioperative management of patients with carcinoid syndrome currently entails the use of prophylactic somatostatin analogs; however, these agents do not prevent carcinoid crisis in all cases.

  • Telotristat, a tryptophan hydroxylase inhibitor, shows promise as an adjunctive therapy to somatostatin analogs to reduce the risk of intraoperative carcinoid crisis.

Open access
Sabine Kleissl-Muir Deakin University School of Nursing and Midwifery, Geelong, Victoria, Australia

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Bodil Rasmussen Deakin University School of Nursing and Midwifery, Geelong, Victoria, Australia
School of Nursing and Midwifery, Centre for Quality and Patient Safety, Institute for Health Transformation, Deakin University, Geelong, Victoria, Australia
The Centre for Quality and Patient Safety, Institute of Health Transformation -Western Health Partnership, Western Health, St Albans, Victoria, Australia
Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark
Faculty of Health Sciences, University of Southern Denmark and Steno Diabetes Centre, Odense M, Denmark

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Alice Owen School of Public Health and Preventive Medicine, Monash University, Melbourne, Victoria, Australia

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Caryn Zinn Human Potential Centre, Faculty of Health and Environmental Sciences, Auckland University of Technology, Auckland, New Zealand

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Andrea Driscoll Deakin University School of Nursing and Midwifery, Geelong, Victoria, Australia
School of Nursing and Midwifery, Centre for Quality and Patient Safety, Institute for Health Transformation, Deakin University, Geelong, Victoria, Australia
School of Public Health and Preventive Medicine, Monash University, Melbourne, Victoria, Australia
Department of Cardiology, Austin Health, Heidelberg, Victoria, Australia

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Summary

In patients with diabetes mellitus, the toxic milieu caused by abnormal glucose and free fatty acid handling can lead to heart failure (HF). Referred to as diabetic cardiomyopathy (DMCM), this syndrome often exists in the absence of conventional risk factors for HF such as history of myocardial infarction or hypertension. Low-carbohydrate diets (LCDs) have recently been endorsed as an efficacious therapeutic dietary approach to prevent and reverse cardiometabolic disease including type 2 diabetes mellitus (T2DM). LCDs improve systemic insulin resistance (IR), reverses cardiac remodelling in a rodent model and downregulates the expression of sodium–glucose co-transporter 2 (SGLT2) receptors in the kidney. It is therefore conceivable that a lifestyle approach such as adopting an LCD can be offered to patients with DMCM. The reported case is that of a 45-year-old man with a 15-year history of non-ischaemic cardiomyopathy, T2DM and obesity. The patient volunteered to engage in a 16-week low-carbohydrate dietary intervention trial and then self-selected to remain on this diet for 1 year. The whole-food LCD was based on simple ‘traffic light’ style food lists and not designed to restrict calories, protein, fat or salt. After 1 year, the patient had lost 39 kg and his cardiometabolic markers had significantly improved. LCDs present a potentially beneficial approach for patients with DMCM and could be considered as a lifestyle intervention before SGLT2i therapy is commenced.

Learning points

  • Diabetic cardiomyopathy (DMCM) is a syndrome precipitated mainly by the detrimental effects of glucose metabolism disorders such as insulin resistance and diabetes.

  • Low-carbohydrate diets (LCD) mimic many effects of sodium–glucose co-transporter 2 inhibitors (SGLT2i).

  • LCDs are a dietary pattern which can have significant and beneficial effects on metabolic and anthropometric markers in patients with DMCM.

  • LCDs and SGLT2i therapy could be combined and may achieve better clinical outcomes for patients with DMCM.

  • Combination therapy may be carried out under close supervision as the real risk for diabetic ketoacidosis remains.

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Václav Hána Jr 3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic

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Tomáš Brutvan 3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic

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Adéla Krausová 3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic

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Michal Kršek 3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic

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Václav Hána 3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic

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Summary

Severe Cushing’s syndrome from an ectopic adrenocorticotropic hormone-producing tumour is rare but often demands rapid diagnostics and treatment of hypercortisolism with its comorbidities. Pharmacotherapy of hypercortisolism by ketoconazole, metyrapone and osilodrostat is currently available. If unsuccessful or insufficient a bilateral adrenalectomy is an option. We present a 28-year-old female with severe Cushing’s syndrome caused by a bronchial metastatic neuroendocrine tumour (NET). Hypercortisolism was efficiently treated by osilodrostat with block–replace and then titration regimen. A once-daily dose was finally used with normalised cortisol levels. Androgen levels measured by liquid chromatography–mass spectrometry were slightly elevated during the treatment but without any symptoms. A simple once-daily use of osilodrostat with titration regimen led to normalised cortisol levels in a severe Cushing’s syndrome patient with an uncurable bronchial NET. Transient hypocortisolism during treatment appeared but was easily treated by hydrocortisone.

Learning points

  • Cushing’s syndrome from an ectopic adrenocorticotropic hormone-producing tumour is rare.

  • Cortisol upregulation is often severe and rapid, though clinical signs are not always fully pronounced.

  • Rapid treatment is a key for preventing and reducing complications such as fractures, thromboembolism, bleeding, hyperglycaemia, and arterial hypertension.

  • The novel potent steroidogenesis inhibitor osilodrostat can be used as first-line treatment for reducing hypercortisolism.

Open access
Bronwyn G A Stuckey Keogh Institute for Medical Research, Nedlands, Western Australia, Australia
Department of Endocrinology and Diabetes, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia
School of Medicine, University of Western Australia, Nedlands, Western Australia, Australia

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James D Nolan Department of Endocrinology and Diabetes, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia

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David M Hurley Department of Endocrinology and Diabetes, Royal Perth Hospital, Perth, Western Australia, Australia

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Graeme B Martin School of Agriculture and Environment, University of Western Australia, Nedlands, Western Australia, Australia

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Summary

A 33-year-old man with Kallmann syndrome had received pulsatile GnRH as an infant for the treatment of cryptorchidism. As an adult, his treatment for fertility with gonadotrophins was unusually rapid compared with expectations, with a total sperm count of 25 million after only 12 months of gonadotrophin therapy. We propose that pulsatile GnRH treatment as an infant induced minipuberty and facilitated his successful, rapid response to therapy. We also propose that identification of the absence of minipuberty in infants with clinical signs suggesting congenital hypogonadotrophic hypogonadism (CHH) is an opportunity for intervention with pulsatile GnRH yielding benefits for fertility decades later.

Learning points

  • Absence of minipuberty in males with CHH results in low Sertoli cell numbers and delayed response to induction of spermatogenesis in adulthood.

  • Presentation with 'red flags' for androgen deficiency including cryptorchidism at birth, with or without micropenis, should prompt screening for CHH and minipuberty by measurement of gonadotrophins and testosterone in the first 2 months after birth.

  • Pulsatile GnRH therapy in patients with CHH, given prior to age of attainment of Sertoli cell maturation, can replicate the normal physiology of minipuberty, thereby priming the testis for future fertility.

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Seigo Konishi Department of Health Promotion and Medicine of the Future, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Ishikawa, Japan

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Mitsuhiro Kometani Department of Health Promotion and Medicine of the Future, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Ishikawa, Japan

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