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Clemens Gardemann FH Münster Oecotrophologie, Münster, Germany
Clinic for Pediatrics and Adolescent Medicine/Metabolism Laboratory, Universitätsklinikum Münster, Münster, Germany

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Sonja Knowles FH Münster Oecotrophologie, Münster, Germany

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Thorsten Marquardt Clinic for Pediatrics and Adolescent Medicine/Metabolism Laboratory, Universitätsklinikum Münster, Münster, Germany

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). Case presentation The patient presented is a German male in his 20s who was diagnosed with type 1 diabetes mellitus at the age of 2 years. He weighs 61 kg and has a height of 1.75 m. He had no known comorbidities, did not smoke and drank no alcohol at

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Fiona Melzer Institute of Diabetes and Clinical Metabolic Research, Kiel, Germany

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Corinna Geisler Institute of Diabetes and Clinical Metabolic Research, Kiel, Germany

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Dominik M Schulte Institute of Diabetes and Clinical Metabolic Research, Kiel, Germany
Department of Medicine 1, Division of Endocrinology, Diabetes and Clinical Nutrition, University Hospital of Schleswig-Holstein, Kiel, Germany

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Matthias Laudes Institute of Diabetes and Clinical Metabolic Research, Kiel, Germany
Department of Medicine 1, Division of Endocrinology, Diabetes and Clinical Nutrition, University Hospital of Schleswig-Holstein, Kiel, Germany

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bioelectrical impedance analysis (BIA, seca mBCA 515 device, seca Deutschland, Hamburg, Germany). The patient presented a body fat content of 33.2% with a fat mass index of 8.8 kg/m² (slightly above the normal range of 3.8–8.7 kg/m²). The BIA also showed that

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Tina Kienitz Endocrinology in Charlottenburg, Stuttgarter Platz 1, Berlin, Germany

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Jörg Schwander Endocrinology in Charlottenburg, Stuttgarter Platz 1, Berlin, Germany

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Ulrich Bogner Endocrinology in Charlottenburg, Stuttgarter Platz 1, Berlin, Germany

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Michael Schwabe Pathology, DRK-Kliniken Westend, Berlin, Germany

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Thomas Steinmüller Department of Abdominal and Endocrine Surgery, DRK-Kliniken Westend, Berlin, Germany

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Marcus Quinkler Endocrinology in Charlottenburg, Stuttgarter Platz 1, Berlin, Germany

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receptor expression in a giant adrenal myelolipoma associated with 21-hydroxylase deficiency . Endocrine Pathology 2008 19 122 – 127 . ( https://doi.org/10.1007/s12022-008-9034-2 ) 5 German-Mena E Zibari GB Levine SN Adrenal myelolipomas in

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Ellada Sotiridou Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK

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Henrike Hoermann Department of General Paediatrics, Neonatology and Paediatric Cardiology, University Children’s Hospital, Medical Faculty, Heinrich-Heine University, Duesseldorf, Germany

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Sommayya Aftab Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK

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Antonia Dastamani Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK

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Eva Thimm Department of General Paediatrics, Neonatology and Paediatric Cardiology, University Children’s Hospital, Medical Faculty, Heinrich-Heine University, Duesseldorf, Germany

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Louise Doodson Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK

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Spyros Batzios Metabolic Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK

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Sebastian Kummer Department of General Paediatrics, Neonatology and Paediatric Cardiology, University Children’s Hospital, Medical Faculty, Heinrich-Heine University, Duesseldorf, Germany

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Pratik Shah Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK

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-appropriate fasting tolerance without any supplemental carbohydrates. His treatment was discontinued after 6 months when he tolerated a 12 h fast, off diazoxide. Case 3 The third patient was male, born in Germany at 36 + 4 weeks of gestation to consanguineous

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Viktoria F Koehler Department of Internal Medicine IV, University Hospital of Munich, LMU Munich, Munich, Germany

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Patrick Keller Department of Urology, University Hospital of Munich, LMU Munich, Munich, Germany

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Elisa Waldmann Department of Internal Medicine IV, University Hospital of Munich, LMU Munich, Munich, Germany

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Nathalie Schwenk Department of Internal Medicine IV, University Hospital of Munich, LMU Munich, Munich, Germany

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Carolin Kitzberger Department of Internal Medicine IV, University Hospital of Munich, LMU Munich, Munich, Germany

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Kathrin A Schmohl Department of Internal Medicine IV, University Hospital of Munich, LMU Munich, Munich, Germany

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Thomas Knösel Department of Pathology, University Hospital of Munich, LMU Munich, Munich, Germany

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Christian Georg Stief Department of Urology, University Hospital of Munich, LMU Munich, Munich, Germany

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Christine Spitzweg Department of Internal Medicine IV, University Hospital of Munich, LMU Munich, Munich, Germany
Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic Rochester, Minnesota, USA

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Summary

Struma ovarii is a teratoma of the ovaries predominantly composed of thyroid tissue. Hyperthyroidism associated with struma ovarii is rare, occurring in approximately 8% of cases. Due to the rarity of struma ovarii, available data are limited to case reports and small case series.We report on a 61-year-old female patient with known Hashimoto’s thyroiditis on levothyroxine replacement therapy for years with transition to clinical and biochemical hyperthyroidism despite antithyroid medication with carbimazole (10 mg/day), new diagnosis of urothelial carcinoma and an adnexal mass suspicious of ovarian cancer. The patient underwent resection of the adnexal mass and histopathology revealed a mature teratoma predominantly composed of thyroid tissue showing high levels of sodium iodide symporter protein expression. Following struma ovarii resection and disappearance of autonomous production of thyroid hormones, the patient developed hypothyroidism with severely decreased thyroid hormone levels fT4 and fT3 (fT4 0.4 ng/dL, reference interval 0.9–1.7 and fT3 < 1.0 pg/mL, reference interval 2.0–4.4). This has previously been masked by continued thyroid-stimulating hormone suppression due to long-term hyperthyroidism pre-surgery indicating secondary hypothyroidism, in addition to primary hypothyroidism based on the known co-existing chronic lymphocytic thyroiditis of the orthotopic thyroid gland. Levothyroxine administration was started immediately restoring euthyroidism.This case illustrates possible diagnostic pitfalls in a patient with two concurrent causes of abnormal thyroid function.

Learning points:

  • Struma ovarii is an ovarian tumor containing either entirely or predominantly thyroid tissue and accounts for approximately 5% of all ovarian teratomas.

  • In rare cases, both benign and malignant struma ovarii can secrete thyroid hormones, causing clinical and biochemical features of hyperthyroidism.

  • Biochemical features of patients with struma ovarii and hyperthyroidism are similar to those of patients with primary hyperthyroidism. In such cases, thyroid scintigraphy should reveal low or absent radioiodine uptake in the thyroid gland, but the presence of radioiodine uptake in the pelvis in a whole body radioiodine scintigraphy.

  • We give advice on possible diagnostic pitfalls in a case with two simultaneous causes of abnormal thyroid function due to the co-existence of struma ovarii.

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Nina Dauth MVZ Diamedicum Würzburg GmbH, Würzburg, Germany

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Victoria T Mücke Medical Department 1, University Hospital of the Goethe-University Frankfurt, Frankfurt am Main, Germany

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Marcus M Mücke Medical Department 1, University Hospital of the Goethe-University Frankfurt, Frankfurt am Main, Germany

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Christian M Lange Clinic for Gastroenterology and Hepatology, University Hospital Essen and University Duisburg-Essen, Essen, Germany

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Martin Welker Medical Department 1, University Hospital of the Goethe-University Frankfurt, Frankfurt am Main, Germany

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Stefan Zeuzem Medical Department 1, University Hospital of the Goethe-University Frankfurt, Frankfurt am Main, Germany

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Klaus Badenhoop Medical Department 1, University Hospital of the Goethe-University Frankfurt, Frankfurt am Main, Germany

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Summary

Wilson’s disease (WD) is a rare disorder of copper metabolism usually presenting with variable liver damage and neuropsychiatric symptoms. Here we report a 39-year-old Taiwanese female with late manifestation of WD presenting with gonadotroph, thyreotroph and corticotroph hypopituitarism. Molecular genetic testing revealed compound heterozygosity for two mutations in exons 12 and 14 (c.2828G>A and c.3140A>T). Copper-chelating therapy with D-penicillamine and zinc was initiated along with supplementation of hydrocortisone and L-thyroxine. Hypopituitarism resolved when urinary copper excretion returned to normal levels under copper chelation. This case should raise awareness of pituitary function in WD patients.

Learning points

  • Hypopituitarism can complicate Wilson’s disease (WD) and endocrinologists should be aware of it when caring for hypopituitary patients.

  • Hepatologists should consider endocrinologic testing for hypopituitarism when WD patients present with symptoms of adrenal insufficiency, thyroid or gonadal dysfunction.

  • Copper-chelating treatment is mandatory and may lead to the recovery of pituitary function in such patients.

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Carmina Teresa Fuss Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

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Stephanie Burger-Stritt Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

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Silke Horn Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

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Ann-Cathrin Koschker Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

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Kathrin Frey Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

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Almuth Meyer Division of Endocrinology and Diabetology, Department of Internal Medicine, Helios Klinikum Erfurt, Erfurt, Germany

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Stefanie Hahner Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

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several years ago. Since 2017, rhPTH 1–84 is also available for patients with treatment-refractory hypoparathyroidism in Germany. Recent trials studied substitution therapy in patients with primary hypoparathyroidism. Under treatment with PTH, serum

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Ana Dugic Department for Gastroenterology, Endocrinology and Metabolic Diseases, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Michael Kryk Department for Gastroenterology, Endocrinology and Metabolic Diseases, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Claudia Mellenthin Department of Surgery, HFR Fribourg, Fribourg, Switzerland

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Christoph Braig Department for Gastroenterology, Endocrinology and Metabolic Diseases, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Lorenzo Catanese Department for Nephrology, Angiology and Rheumatology, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Sandy Petermann Department for Gastroenterology, Endocrinology and Metabolic Diseases, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Jürgen Kothmann Department for Nephrology, Angiology and Rheumatology, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Steffen Mühldorfer Department for Gastroenterology, Endocrinology and Metabolic Diseases, Bayreuth University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Bayreuth, Germany

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Summary

Drinking fruit juice is an increasingly popular health trend, as it is widely perceived as a source of vitamins and nutrients. However, high fructose load in fruit beverages can have harmful metabolic effects. When consumed in high amounts, fructose is linked with hypertriglyceridemia, fatty liver and insulin resistance. We present an unusual case of a patient with severe asymptomatic hypertriglyceridemia (triglycerides of 9182 mg/dL) and newly diagnosed type 2 diabetes mellitus, who reported a daily intake of 15 L of fruit juice over several weeks before presentation. The patient was referred to our emergency department with blood glucose of 527 mg/dL and glycated hemoglobin (HbA1c) of 17.3%. Interestingly, features of diabetic ketoacidosis or hyperosmolar hyperglycemic state were absent. The patient was overweight with an otherwise unremarkable physical exam. Lipase levels, liver function tests and inflammatory markers were closely monitored and remained unremarkable. The initial therapeutic approach included i.v. volume resuscitation, insulin and heparin. Additionally, plasmapheresis was performed to prevent potentially fatal complications of hypertriglyceridemia. The patient was counseled on balanced nutrition and detrimental effects of fruit beverages. He was discharged home 6 days after admission. At a 2-week follow-up visit, his triglyceride level was 419 mg/dL, total cholesterol was 221 mg/dL and HbA1c was 12.7%. The present case highlights the role of fructose overconsumption as a contributory factor for severe hypertriglyceridemia in a patient with newly diagnosed diabetes. We discuss metabolic effects of uncontrolled fructose ingestion, as well as the interplay of primary and secondary factors, in the pathogenesis of hypertriglyceridemia accompanied by diabetes.

Learning points

  • Excessive dietary fructose intake can exacerbate hypertriglyceridemia in patients with underlying type 2 diabetes mellitus (T2DM) and absence of diabetic ketoacidosis or hyperosmolar hyperglycemic state.

  • When consumed in large amounts, fructose is considered a highly lipogenic nutrient linked with postprandial hypertriglyceridemia and de novo hepatic lipogenesis (DNL).

  • Severe lipemia (triglyceride plasma level > 9000 mg/dL) could be asymptomatic and not necessarily complicated by acute pancreatitis, although lipase levels should be closely monitored.

  • Plasmapheresis is an effective adjunct treatment option for rapid lowering of high serum lipids, which is paramount to prevent acute complications of severe hypertriglyceridemia.

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Sebastian Hörber Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany
Institute for Diabetes Research and Metabolic Diseases of the Helmholtz Center Munich at the University of Tübingen, Tübingen, Germany
German Center for Diabetes Research (DZD), München-Neuherberg, Germany

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Sarah Hudak Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany

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Martin Kächele Department of Internal Medicine, Medical Intensive Care Unit, University of Tübingen, Tübingen, Germany

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Dietrich Overkamp Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany

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Andreas Fritsche Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany
Institute for Diabetes Research and Metabolic Diseases of the Helmholtz Center Munich at the University of Tübingen, Tübingen, Germany
German Center for Diabetes Research (DZD), München-Neuherberg, Germany

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Hans-Ulrich Häring Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany
Institute for Diabetes Research and Metabolic Diseases of the Helmholtz Center Munich at the University of Tübingen, Tübingen, Germany
German Center for Diabetes Research (DZD), München-Neuherberg, Germany

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Andreas Peter Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany
Institute for Diabetes Research and Metabolic Diseases of the Helmholtz Center Munich at the University of Tübingen, Tübingen, Germany
German Center for Diabetes Research (DZD), München-Neuherberg, Germany

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Martin Heni Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry, Department of Internal Medicine, University of Tübingen, Tübingen, Germany
Institute for Diabetes Research and Metabolic Diseases of the Helmholtz Center Munich at the University of Tübingen, Tübingen, Germany
German Center for Diabetes Research (DZD), München-Neuherberg, Germany

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Forschungsgemeinschaft and the Open Access Publishing Fund of the University of Tübingen. Part of this work was supported by a grant (01Gbib925) from the Federal Ministry of Education and Research (BMBF) to the German Centre for Diabetes Research (DZD e.V.). Author

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Ahmad Haider Private Urology Practice, Bremerhaven, Germany

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Karim S Haider Private Urology Practice, Bremerhaven, Germany

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Farid Saad Global Medical Affairs Andrology, Bayer AG, Berlin, Germany
Research Department, Gulf Medical University, Ajman, UAE

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Summary

In daily practice, clinicians are often confronted with obese type 2 diabetes mellitus (T2DM) patients for whom the treatment plan fails and who show an inadequate glycemic control and/or no sustainable weight loss. Untreated hypogonadism can be the reason for such treatment failure. This case describes the profound impact testosterone therapy can have on a male hypogonadal patient with metabolic syndrome, resulting in a substantial and sustained loss of body weight, pronounced improvement of all critical laboratory values and finally complete remission of diabetes.

Learning points:

  • Hypogonadism occurs frequently in men with T2DM.

  • In case of pronounced abdominal fat deposition and T2DM, the male patient should be evaluated for testosterone deficiency.

  • Untreated hypogonadism can complicate the successful treatment of patients with T2DM.

  • Under testosterone therapy, critical laboratory values are facilitated to return back to normal ranges and even complete remission of diabetes can be achieved.

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