Clinical Overview > Gland/Organ

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Viviana Ostrovsky Kaplan Medical Center, Diabetes, Endocrinology and Metabolic Disease Institute, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Mira Ulman Endocrinology Laboratory, Kaplan Medical Center, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Rina Hemi Endocrine Laboratory, Division of Endocrinology, Diabetes and Metabolism, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel

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Samuel Lurie Women’s Health Center, Ramat Aviv Gimel, Clalit Health Services, Tel Aviv, Israel

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Inon Hazan Kaplan Medical Center, Gynecology Department, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Alon Ben Ari Kaplan Medical Center, Gynecology Department, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Oleg Sukmanov Kaplan Medical Center, Pathology Department, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Tal Schiller Kaplan Medical Center, Diabetes, Endocrinology and Metabolic Disease Institute, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Alena Kirzhner Kaplan Medical Center, Diabetes, Endocrinology and Metabolic Disease Institute, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Taiba Zornitzki Kaplan Medical Center, Diabetes, Endocrinology and Metabolic Disease Institute, Hebrew University of Jerusalem, Medical School, Rehovot, Israel

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Summary

Total testosterone, which is peripherally converted to its biologically active form dihydrotestosterone (DHT), is the first-line hormone investigation in hyperandrogenic states and infertility in premenopausal women. Polycystic ovary syndrome (PCOS), the most common cause of hyperandrogenism and infertility in young women, is often associated with mild elevations of total testosterone. Whereas very high levels of total testosterone (>2–3 SD of normal reference), are most often associated with hyperandrogenic signs, menstrual irregularity, rapid onset of virilization, and demand a prompt investigation. Herein, we report a case of a 32-year-old woman who was referred to the endocrinology outpatient clinic due to secondary amenorrhea and extremely high testosterone levels without any virilization signs. We initially suspected pitfalls in the testosterone laboratory test. Total serum testosterone decreased after a diethyl-ether extraction procedure was done prior to the immunoassay, but testosterone levels were still elevated. An ovarian steroid-cell tumor (SCT) was then revealed, which was thereby resected. Twenty-four hours post surgery, the total testosterone level returned to normal, and a month later menstruation resumed. This case emphasizes that any discrepancy between laboratory tests and the clinical scenario deserves a rigorous evaluation to minimize misinterpretation and errors in diagnosis and therapeutic approach. Additionally, we describe a possible mechanism of disease: a selective peripheral target-tissue response to high testosterone levels that did not cause virilization but did suppress ovulation and menstruation.

Learning points

  • Total testosterone is the most clinically relevant hormone in investigating hyperandrogenic states and infertility in premenopausal women.

  • Very high total testosterone levels in women (>2–3 SD of normal reference) are most often associated with hyperandrogenic signs, menstrual irregularities, and a rapid onset of virilization.

  • In women with very elevated testosterone levels and the absence of clinical manifestations, laboratory interference should be suspected, and diethyl ether extraction is a useful technique when other methods fail to detect it.

  • Ovarian steroid cell tumors (SCT) encompass a rare subgroup of sex cord-stromal tumors and usually secrete androgen hormones. SCTs are clinically malignant in 25–43% of cases.

  • A selective response of peripheral target tissues to testosterone levels, with clinical manifestations in some tissues and no expression in others, may reflect differences in the conformation of tumor-produced testosterone molecules.

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Melanie Nana Department of Obstetric Medicine, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Catherine Nelson-Piercy Department of Obstetric Medicine, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Summary

COVID-19 is associated with severe disease in pregnancy. Complications of the disease, or simultaneous diagnoses, may be missed if clinicians do not retain a large differential diagnosis when assessing such women. Starvation ketoacidosis is one such diagnosis which may complicate the disease and should not be missed. A 37-year-old woman, 33 weeks’ gestation presented with breathlessness. Clinical history, examination and investigations supported a diagnosis of starvation ketosis of pregnancy complicating COVID-19 pneumonitis. Prompt correction of the metabolic disturbance resulted in resolution, and preterm delivery was avoided at this time. Early recognition and prompt management of starvation ketosis of pregnancy in women with COVID-19 are important in reducing maternal and neonatal morbidity and mortality. Preterm delivery may be avoided with prompt resolution of the metabolic disturbance. Clinicians should keep a wide differential diagnosis when assessing women with breathlessness. A multidisciplinary team (MDT) approach is required to facilitate optimal care.

Learning points

  • Clinicians should maintain a wide differential when assessing women who are unwell with COVID-19 in pregnancy.

  • Complications such as starvation ketoacidosis are rare but life-threatening.

  • An awareness of such complications facilitates early identification of the condition, and involvement of appropriate specialists who can initiate optimal and timely management.

  • In the context of pregnancy, where ketoacidosis poses a threat to the mother or baby, prompt management and resolution may avoid preterm delivery.

  • Conditions that may increase the risk of developing starvation ketoacidosis include pregnancy, medication use such as corticosteroids or tocolytic therapies, previous gastric surgery, intercurrent illness and pregnancy-related conditions that might contribute towards a degree of chronic starvation.

  • Multidisciplinary input supports the delivery of best practice and care for the patients.

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Ulla Kampmann Steno Diabetes Center Aarhus, Aarhus University Hospital, Hedeager, Aarhus N, Denmark

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Per Glud Ovesen Department of Obstetrics and Gynecology, Department of Diabetes and Endocrinology, Aarhus University Hospital, Palle Juul Jensens Boulevard, Aarhus N, Denmark

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Niels Møller Medical Research Laboratories, Department of Diabetes and Endocrinology, Aarhus University Hospital, Palle Juul Jensens Boulevard, Aarhus N, Denmark

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Jens Fuglsang Department of Obstetrics and Gynecology, Department of Diabetes and Endocrinology, Aarhus University Hospital, Palle Juul Jensens Boulevard, Aarhus N, Denmark

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Summary

During pregnancy, maternal tissues become increasingly insensitive to insulin in order to liberate nutritional supply to the growing fetus, but occasionally insulin resistance in pregnancy becomes severe and the treatment challenging. We report a rare and clinically difficult case of extreme insulin resistance with daily insulin requirements of 1420 IU/day during pregnancy in an obese 36-year-old woman with type 2 diabetes (T2D) and polycystic ovary syndrome (PCOS). The woman was referred to the outpatient clinic at gestational week 12 + 2 with a hemoglobin A1c (HbA1c) at 59 mmol/mol. Insulin treatment was initiated immediately using Novomix 30, and the doses were progressively increased, peaking at 1420 units/day at week 34 + 4. At week 35 + 0, there was an abrupt fall in insulin requirements, but with no signs of placental insufficiency. At week 36 + 1 a, healthy baby with no hypoglycemia was delivered by cesarean section. Blood samples were taken late in pregnancy to search for causes of extreme insulin resistance and showed high levels of C-peptide, proinsulin, insulin-like growth factor (IGF-1), mannan-binding-lectin (MBL) and leptin. CRP was mildly elevated, but otherwise, levels of inflammatory markers were normal. Insulin antibodies were undetectable, and no mutations in the insulin receptor (INSR) gene were found. The explanation for the severe insulin resistance, in this case, can be ascribed to PCOS, obesity, profound weight gain, hyperleptinemia and inactivity. This is the first case of extreme insulin resistance during pregnancy, with insulin requirements close to 1500 IU/day with a successful outcome, illustrating the importance of a close interdisciplinary collaboration between patient, obstetricians and endocrinologists.

Learning points

  • This is the first case of extreme insulin resistance during pregnancy, with insulin requirements of up to 1420 IU/day with a successful outcome without significant fetal macrosomia and hypoglycemia.

  • Obesity, PCOS, T2D and high levels of leptin and IGF-1 are predictors of severe insulin resistance in pregnancy.

  • A close collaboration between patient, obstetricians and endocrinologists is crucial for tailoring the best possible treatment for pregnant women with diabetes, beneficial for both the mother and her child.

Open access
Pedro Marques Endocrinology Department, Instituto Português de Oncologia de Lisboa, Francisco Gentil, Rua Professor Lima Basto1099-023, Lisboa, Portugal
Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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Kavinga Gunawardana Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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Ashley Grossman Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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Summary

Gestational diabetes insipidus (DI) is a rare complication of pregnancy, usually developing in the third trimester and remitting spontaneously 4–6 weeks post-partum. It is mainly caused by excessive vasopressinase activity, an enzyme expressed by placental trophoblasts which metabolises arginine vasopressin (AVP). Its diagnosis is challenging, and the treatment requires desmopressin. A 38-year-old Chinese woman was referred in the 37th week of her first single-gestation due to polyuria, nocturia and polydipsia. She was known to have gestational diabetes mellitus diagnosed in the second trimester, well-controlled with diet. Her medical history was unremarkable. Physical examination demonstrated decreased skin turgor; her blood pressure was 102/63 mmHg, heart rate 78 beats/min and weight 53 kg (BMI 22.6 kg/m2). Laboratory data revealed low urine osmolality 89 mOsmol/kg (350–1000), serum osmolality 293 mOsmol/kg (278–295), serum sodium 144 mmol/l (135–145), potassium 4.1 mmol/l (3.5–5.0), urea 2.2 mmol/l (2.5–6.7), glucose 3.5 mmol/l and HbA1c 5.3%. Bilirubin, alanine transaminase, alkaline phosphatase and full blood count were normal. The patient was started on desmopressin with improvement in her symptoms, and normalisation of serum and urine osmolality (280 and 310 mOsmol/kg respectively). A fetus was delivered at the 39th week without major problems. After delivery, desmopressin was stopped and she had no further evidence of polyuria, polydipsia or nocturia. Her sodium, serum/urine osmolality at 12-weeks post-partum were normal. A pituitary magnetic resonance imaging (MRI) revealed the neurohypophyseal T1-bright spot situated ectopically, with a normal adenohypophysis and infundibulum. She remains clinically well, currently breastfeeding, and off all medication. This case illustrates some challenges in the diagnosis and management of transient gestational DI.

Learning points

  • Gestational DI is a rare complication of pregnancy occurring in two to four out of 100 000 pregnancies. It usually develops at the end of the second or third trimester of pregnancy and remits spontaneously 4–6 weeks after delivery.

  • Gestational DI occurrence is related to excessive vasopressinase activity, an enzyme expressed by placental trophoblasts during pregnancy, which metabolises AVP. Its activity is proportional to the placental weight, explaining the higher vasopressinase activity in third trimester or in multiple pregnancies.

  • Vasopressinase is metabolised by the liver, which most likely explains its higher concentrations in pregnant women with hepatic dysfunction, such acute fatty liver of pregnancy, HELLP syndrome, hepatitis and cirrhosis. Therefore, it is important to assess liver function in patients with gestational DI, and to be aware of the risk of DI in pregnant women with liver disease.

  • Serum and urine osmolality are essential for the diagnosis, but other tests such as serum sodium, glucose, urea, creatinine, liver function may be informative. The water deprivation test is normally not recommended during pregnancy because it may lead to significant dehydration, but a pituitary MRI should be performed at some point to exclude lesions in the hypothalamo-pituitary region.

  • These patients should be monitored for vital signs, fluid balance, body weight, fetal status, renal and liver function, and treated with desmopressin. The recommended doses are similar or slightly higher than those recommended for central DI in non-pregnant women, and should be titrated individually.

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