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OBJECTIVE: Primary aldosteronism (PA) is one of the most frequent causes of secondary hypertension. Although clinical practice guidelines recommend a diagnostic process, details of the steps remain incompletely standardized. DESIGN: In the present SCOT-PA survey, we have investigated the diversity of approaches utilized for each diagnostic step in different expert centers through a survey using Google questionnaires. A total of 33 centers from 3 continents participated. RESULTS: We demonstrated a prominent diversity in the conditions of blood sampling, assay methods for aldosterone and renin, and the methods and diagnostic cutoff for screening and confirmatory tests. The most standard measures were modification of antihypertensive medication and sitting posture for blood sampling, measurement of plasma aldosterone concentration (PAC) and active renin concentration by chemiluminescence enzyme immunoassay, a combination of aldosterone-to-renin ratio with PAC as an index for screening, and saline infusion test in a seated position for confirmatory testing. The cutoff values for screening and confirmatory testing showed significant variation among centers. CONCLUSIONS: Diversity of the diagnostic steps may lead to an inconsistent diagnosis of PA among centers and limit comparison of evidence for PA between different centers. We expect the impact of this diversity to be most prominent in patients with mild PA. The survey raises 2 issues: the need for standardization of the diagnostic process and revisiting the concept of mild PA. Further standardization of the diagnostic process/criteria will improve the quality of evidence and management of patients with PA.

A 52-year-old female presented with recurrent episodes of fasting or post-absorptive hypoglycemia. A 72-h fasting test confirmed endogenous hyperinsulinemia. Conventional imaging was unremarkable. Selective pancreatic arterial calcium stimulation and hepatic venous sampling showed a maximum calcium-stimulated insulin concentration from several pancreatic areas, mainly the proximal splenic artery and the proximal gastroduodenal artery, suggesting the presence of one or more occult insulinoma(s) in the region of the pancreatic body. 68Ga-DOTA-exendin-4 PET/CT showed however generalized increased uptake in the pancreas and a diagnosis of nesidioblastosis was therefore suspected. The patient has been since successfully treated with dietetic measures and diazoxide. Treatment efficacy was confirmed by a flash glucose monitoring system with a follow-up of 7 months. Learning points: Adult nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia. The distinction between insulinoma and nesidioblastosis is essential since the therapeutic strategies are different. 68Ga-DOTA-exendin-4 PET/CT emerges as a new noninvasive diagnostic tool for the localization of an endogenous source of hyperinsulinemic hypoglycemia. Medical management with dietetic measures and diazoxide need to be considered as a valuable option to treat patients with adult nesidioblastosis. Flash glucose monitoring system is helpful for the evaluation of treatment efficacy.

Complicated Rathke's cleft cyst (RCC) is a rare occurrence of symptomatic bleeding or growth of a previously asymptomatic (and often undiagnosed) intrasellar cyst derived from remnants of Rathke's pouch, situated on the midline between the adeno- and neurohypophysis. Symptoms may be identical to those of pituitary apoplexy: acute onset of headache, hypopituitarism, and neurological disturbances. Both syndromes may also exhibit a similar appearance of a large haemorrhagic sellar mass at initial radiological evaluation. We report on two patients who presented with headache and complete hypopituitarism. Based on the initial MRI, they were first diagnosed with pituitary apoplexy but managed conservatively with hormone therapy alone because of the absence of severe visual or neurological threat. Upon follow-up at 4 months, clinical evolution was good in both patients but their pituitary mass had not reduced in size and, after careful radiologic reviewing, was more indicative of a large midline complicated RCC. In conclusion, the diagnosis of complicated RCC is challenging because it can mimic pituitary apoplexy clinically, biologically, and radiologically. Clinicians should distinguish between the two entities using specific radiological signs or evolution of the mass at MRI if the patient does not undergo surgery. To our knowledge, we report conservative management of this rare condition for the first time, though it seems appropriate in the absence of neurological compromise or visual compression. Long-term follow-up is however mandatory. Learning points: Complicated Rathke's cleft cyst can mimic pituitary apoplexy, presenting with sudden onset of headache, hypopituitarism, and visual and neurological compromise in the most severe cases. At diagnosis, pituitary MRI may not be able to differentiate between the two entities, showing a large haemorrhagic mass inside the sella, with little or no normal pituitary tissue visible. Patients are often diagnosed with apoplexy at

OBJECTIVES: Surgery of pheochromocytomas (PCs) still carries a high risk of haemodynamic complications during the perioperative period. We aimed to evaluate the influence of their secretory phenotype and preoperative alpha-blocker treatment on surgical outcome. DESIGN: A retrospective monocentric study at a tertiary medical centre. PATIENTS: In this study, 80 consecutive patients operated by the same team for a PC between 1988 and 2018. RESULTS: Diagnosis was based on typical symptoms and signs in 58 patients, genetic testing in 12 and work-up of an adrenal incidentaloma in 9. It was made during surgery in one patient. A genetic predisposition was found in one-third of index cases (21/62). The majority of the patients (73/79) had a secreting PC; more than 2/3 had an adrenergic phenotype and less than 1/3 a noradrenergic phenotype. The rate of perioperative haemodynamic complications was not influenced by the secretory phenotype, but persistent hypertension after surgery, recurrence and malignancy were more frequently observed in patients with a noradrenergic tumour. Preoperative alpha-blocker treatment was given for ≥ 14 days in 29 patients and, although being more symptomatic at diagnosis, these patients had less haemodynamic complications (3/29 vs 12/51 non-treated patients, P = 0.05). CONCLUSIONS: The occurrence of haemodynamic complications during surgery was not significantly affected by the secretory phenotype in our study, but noradrenergic tumours show a worse post-surgical outcome. Our data also provide additional support in favour of a sufficient preoperative alpha-blockade in patients with pheochromocytoma.

BACKGROUND: Insulinomas are usually benign, small-sized, well-encapsulated and often solitary pancreatic tumors. Currently, enucleation is the treatment of choice for sporadic solitary insulinoma if diameter is less than 2cm and the structural integrity of the pancreatic duct can be maintained. However, the procedure has a risk of postoperative complications, and especially of pancreatic fistula. There is growing interest in endoscopic ultrasound-guided radiofrequency ablation (EUS-RFA) as an effective and less invasive alternative treatment for benign sporadic insulinoma. METHOD: We retrospectively analyzed the efficacy and safety of EUS-RFA in four patients with benign localized insulinoma treated in our tertiary care center between June 2018 and November 2019. EUS-RFA was performed with a EUS-guided RFA 19-gauge needle electrode (Starmed; Taewoong Medical, Seoul, South Korea) that released energy at 50W up to 100 Ohms impedance. RESULTS: The series comprised three women and one man, with a median age of 58 years (range 52-82 years). Mean tumor size was 12mm. Although three of the four patients would have been eligible for surgery, EUS-RFA was proposed to them. Symptomatic and biological improvement occurred immediately, generally straight after the procedure, in all patients, and no serious complications were observed. During the mean follow-up period of 22 months, no symptom recurrence was observed. CONCLUSIONS: This preliminary report in 4 patients showed that EUS-RFA was an effective and relatively safe alternative treatment, devoid of major complications, for benign sporadic insulinoma. Larger-scale prospective multicenter studies are, however, needed to confirm the long-term effectiveness and safety of this novel technique.

The authors of the aforementioned article published in this journal (vol 183 Iss 2 pages: 161–167) apologise for errors in reporting the dosage of etomidate in several places in their article. [...]

Intravenous etomidate infusion is effective to rapidly lower cortisol levels in severe Cushing's syndrome (CS) in the intensive care unit (ICU). Recently, etomidate treatment has also been proposed at lower doses in non-ICU wards, but it is not yet clear how this approach compares to ICU treatment. We compared data from patients with severe CS treated with high starting doses of etomidate (median: 0.30 mg/kg BW/day) in ICU or with lower starting doses (median: 0.025 mg/kg BW/day) in non-ICU medical wards. Fourteen patients were included, among which ten were treated with low starting doses (LD) and four with high starting doses etomidate (HD). All patients had severe and complicated CS related to adrenal carcinoma (n = 8) or ectopic ACTH secretion (n = 6). Etomidate was effective in reducing cortisol levels below 500 nmol/L in a median of 1 day in the HD group compared to 3 days in the LD group (P = 0.013). However, all patients of the HD group had etomidate-induced cortisol insufficiency and needed frequent monitoring, while no patient from the LD group required hydrocortisone supplementation. No patient in either group died from complications of CS or etomidate treatment, but final outcome was poor as six patients in the LD group and all four patients in the HD group died from their cancer during follow-up. Our study suggests that, for patients with severe CS who do not require intensive organ-supporting therapy, the use of very low doses of etomidate in medical wards should be considered.

Introduction: Intravenous etomidate infusion is effective to rapidly lower cortisol levels in severe Cushing's syndrome (CS) in the intensive care unit (ICU). Recently, etomidate treatment has also been proposed at lower doses in non-ICU wards, but it is not yet clear how this approach compares to ICU treatment. Methods: We compared data from patients with severe CS treated with high starting doses of etomidate (median: 0.30 mg/kg BW/day) in ICU or with lower starting doses (median: 0.025 mg/kg BW/day) in non-ICU medical wards. Results: Fourteen patients were included, among which ten were treated with low starting doses (LD) and four with high starting doses etomidate (HD). All patients had severe and complicated CS related to adrenal carcinoma (n = 8) or ectopic ACTH secretion (n = 6). Etomidate was effective in reducing cortisol levels below 500 nmol/L in a median of 1 day in the HD group compared to 3 days in the LD group (P = 0.013). However, all patients of the HD group had etomidate-induced cortisol insufficiency and needed frequent monitoring, while no patient from the LD group required hydrocortisone supplementation. No patient in either group died from complications of CS or etomidate treatment, but final outcome was poor as six patients in the LD group and all four patients in the HD group died from their cancer during follow-up. Conclusion: Our study suggests that, for patients with severe CS who do not require intensive organ-supporting therapy, the use of very low doses of etomidate in medical wards should be considered., SCOPUS: ar.j, info:eu-repo/semantics/published

Background: Hypoglycemic manifestations are highly variable in patients with an insulinoma and largely independent of tumour size and severity of insulin hypersecretion. Objectives: We investigated the clinical, biological and tumoral characteristics of insulinomas in a large monocentric series of patients and we evaluated their insulin sensitivity before and after successful pancreatic surgery. Patients and methods: This was a retrospective analysis of 40 patients treated for an insulinoma between 1982 and 2012 in our academic hospital. Insulin sensitivity and beta cell function were evaluated by a HOMA test outside hypoglycaemic episodes in a large subset of these patients. Results: The mean age at onset of symptoms was 48.8. ±. 20.1 years and the mean age at diagnosis was 50.7. ±. 19.9 years. Neuroglycopenic symptoms were observed in 90% of patients. The most effective preoperative imaging technique to localize the tumour was endoscopic ultrasound. Insulin sensitivity was greatly reduced in patients with insulinoma (38.9%. ±. 22.3%), while beta cells function was increased (359.0. ±. 171.5%), but to a variable extent (range: 110.6-678.6%). After complete resection of the tumour and remission of hypoglycemic episodes, insulin sensitivity increased in all evaluated subjects (72.8. ±. 36.7%) and normalized in the majority. Conclusion: Although neuroglycopenic symptoms are present in most patients, diagnosis of insulinoma is often delayed. Endoscopic ultrasound remains the most sensitive preoperative technique to localize the tumour. We also show that in response to chronic hyperinsulinemia, patients with insulinoma develop protective mechanisms responsible for a marked insulin resistance, which is reversible after complete resection of the tumour.

A 26-year-old woman presented with severe postpartum headaches. Magnetic resonance imaging (MRI) revealed a symmetric, heterogeneous enlargement of the pituitary gland. Three months later, she developed central diabetes insipidus. A diagnosis of postpartum hypophysitis was suspected and corticosteroids were prescribed. Six months later, the pituitary mass showed further enlargement and characteristics of a necrotic abscess with a peripheral shell and infiltration of the hypothalamus. Transsphenoidal surgery was performed, disclosing a pus-filled cavity which was drained. No bacterial growth was observed, except a single positive blood culture for , considered at that time as a potential contaminant. A short antibiotic course was, however, administered together with hormonal substitution for panhypopituitarism. Four months after her discharge, severe headaches recurred. Pituitary MRI was suggestive of a persistent inflammatory mass of the sellar region. She underwent a new transsphenoidal resection of a residual abscess. At that time, the sellar aspiration fluid was positive for and she was treated with antibiotics for 6 weeks, after which she had complete resolution of her infection. The possibility of a pituitary abscess, although rare, should be kept in mind during evaluation for a necrotic inflammatory pituitary mass with severe headaches and hormonal deficiencies. The possibility of a pituitary abscess, although rare, should be kept in mind during evaluation for a necrotic inflammatory pituitary mass with severe headaches and hormonal deficiencies.In a significant proportion of cases no pathogenic organism can be isolated.A close follow-up is necessary given the risk of recurrence and the high rate of postoperative pituitary deficiencies.

Our team of diabetologist is challenged by the case of a 41 year-old woman with recurrent hypoglycaemic episodes. Her clinical background was complex with, among others, a neuroendocrine tumor, a nonalcoholic steatohepatitis and an adrenal insufficiency; these conditions require the exploration of several potential causes. After excluding an endogenous etiology, a factitious hypoglycemia was quicky suspected by clinicians. However, several venous samples showed normal insulinemia and a moderately decreased C-peptide. After multidisciplinary team discussion and facing a strong clinical suspicion, samples were sent to another laboratory to confirm the insulin results. Substantially supratherapeutics insulin concentrations were highlighted. This confirms the previous suspicion of surreptitious insulin administration with a recombinant form unrecognized by our routinely used analyzer. This observation leads us to briefly discuss the lack of cross-reactivities observed with many different insulin assays.

À l’instar des années précédentes, l’année 2016 a été riche de nouveautés et d’innovations diagnostiques et thérapeutiques dans les domaines des pathologies endocriniennes, du diabète et des maladies métaboliques. Nous avons volontairement choisi de n’illustrer ici que celles qui, aujourd’hui déjà, ont modifié la prise en charge de ces pathologies, que ce soit par le médecin spécialiste ou le médecin généraliste. Tous les lecteurs devraient donc y trouver quelque intérêt. Ainsi dans le domaine du diabète, nous vous parlons de la nouvelle convention INAMI, d’un nouveau système remboursé de mesure en continu du taux de glucose interstitiel par capteur, des progrès technologiques impressionnants des nouvelles pompes à insuline ainsi que des bénéfices importants de l’utilisation des inhibiteurs des transporteurs SGLT-2 (les ‘glucorétiques’) en termes de complications cardiovasculaires et rénales chez le patient diabétique de type 2. Au plan des pathologies métaboliques, les inhibiteurs de la PCSK9 sont maintenant remboursés dans l’hypercholestérolémie familiale hétérozygote et certains analogues du GLP-1 sont disponibles pour un traitement efficace de l’obésité. Dans le domaine des pathologies thyroïdiennes, l’hypothyroxinémie maternelle isolée de la grossesse est aujourd’hui mieux caractérisée et de nouvelles recommandations ont été émises en 2016 concernant le diagnostic et le traitement de l’ophtalmopathie thyroïdienne. Enfin, concernant l’endocrinologie générale, de nouvelles recommandations clarifient et simplifient la prise en charge de l’incidentalome surrénalien dont la fréquence de découverte ne fait que croître, à l’ère d’une imagerie abdominale de plus en plus performante, [Innovations in endocrinology, diabetology, and nutrition: what 2016 brought us ?] The year 2016 was again full of novelties, with numerous diagnostic and therapeutic innovations in the fields of endocrine diseases, diabetes, and metabolic disorders. In this article, we deliberately chose to only discuss those which have already improved the current management of such diseases, be it for specialists or general practitioners. The article should therefore be interesting for every reader. In the field of diabetes, we have here reported on the main features of the new agreement between social security system, physicians, and diabetic patients (“Convention INAMI”). We have also reported on a newly reimbursed system for continuous measurement of interstitial glucose through a sensor, on the impressive technological advances in modern insulin pumps, as well as on the significant cardiovascular and renal benefits of SGLT2 transporter inhibitors in Type 2 diabetes patients. As for metabolic pathologies, the PCSK9 inhibitors are now reimbursed in heterozygous familial hypercholesterolemia, and specific GLP-1 analogues are available for an effective treatment of obesity. In the field of thyroid diseases, isolated maternal hypothyroxinemia during pregnancy is now better characterized, and new recommendations have been issued regarding the diagnosis and treatment of Grave’s ophthalmopathy. Finally, as regards general endocrinology, new recommendations clarify and simplify the management of adrenal incidentaloma, whose detection frequency continually increases in the era of common and more efficient abdominal imaging.

Isolated hypothyroxinemia (IH) is defined as a thyroxine level in the lower 5th (severe IH) or 10th percentile (mild IH) of the pregnancy-related reference range and a normal TSH. The etiology of IH remains unknown. This review aims to evaluate the biochemical criteria used to define IH in different published studies and to discuss potential maternal as well as fetal outcomes and whether treatment during early pregnancy can prevent the eventual adverse effects. For the current literature a better standardization of free thyroxine assays is needed, as well as the use of appropriated trimester-specific reference intervals for thyroid function tests. Today no study demonstrates a benefit from treating early pregnant IH women on perinatal and fetal outcomes.

Early diagnosis of Sheehan's syndrome.