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    1 OF 30

    Steroid Prophylaxis: The knowledge and practices of New Zealand General Dental Practitioners.
    N Z Dent J 2016 Dec;112(4):102-107
    Objective: To describe the knowledge and practices of New Zealand (NZ) General Dental Practitioners (GDPs) concerning the use of steroid prophylaxis.

    Methods: An online survey was conducted in 2013 involving a sample of 500 NZ GDPs who met inclusion criteria and had email addresses on the Dental Council register.

    Results: A total of 214 dentists responded, giving a 44. Read More

    Mortality data from the European Adrenal Insufficiency Registry-Patient characterization and associations.
    Clin Endocrinol (Oxf) 2018 Apr 22. Epub 2018 Apr 22.
    Department of Endocrinology, Leeds Teaching Hospitals NHS Trust, St James's University Hospital, Leeds, UK.
    Objective: Mortality from primary and secondary adrenal insufficiency (AI; PAI and SAI, respectively) is 2-3-fold higher than in the general population. Mortality relates to cardiovascular disease, acute adrenal crisis (AC), cancer and infections; however, there has been little further characterization of patients who have died.

    Design/methods: We analysed real-world data from 2034 patients (801 PAI, 1233 SAI) in the European Adrenal Insufficiency Registry (EU-AIR; NCT01661387). Read More

    Adrenocorticotropic Hormone Secreting Pheochromocytoma Underlying Glucocorticoid Induced Pheochromocytoma Crisis.
    Case Rep Endocrinol 2018 20;2018:3963274. Epub 2018 Feb 20.
    Department of Internal Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
    Context: Pheochromocytomas are hormone secreting tumors of the medulla of the adrenal glands found in 0.1-0.5% of patients with hypertension. Read More

    Livedo reticularis: a cutaneous clue to an underlying endocrine crisis.
    Endocrinol Diabetes Metab Case Rep 2018 12;2018. Epub 2018 Apr 12.
    Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK.
    A 54-year-old woman was admitted to hospital with a presumed allergic reaction to a single dose of amoxicillin given for a suspected upper respiratory tract infection. She complained of chest tightness although there was no wheeze or stridor. On examination, she was pyrexial, tachycardic, hypertensive and had a diffuse mottled rash on her lower limbs. Read More

    Treatment of Addison's disease during pregnancy.
    Endocrinol Diabetes Metab Case Rep 2018 12;2018. Epub 2018 Apr 12.
    Endocrinology, Diabetes and Metabolism Department, Coimbra Hospital and University Center, Coimbra, Portugal.
    Addison's disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison's disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. Read More

    Partial Cystectomy of Paraganglioma of the Urinary Bladder Before Living Kidney Transplantation: Case Report.
    Transplant Proc 2018 Apr;50(3):898-901
    Department of Urology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan; Department of Advanced Transplant and Regenerative Medicine, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
    Background: Paraganglioma (extra-adrenal pheochromocytoma) of the bladder is a very rare disease, accounting for 0.06% of all bladder tumors. Optimal management of bladder paraganglioma before kidney transplantation is unknown. Read More

    Management of adrenal emergencies in educated patients with adrenal insufficiency-A prospective study.
    Clin Endocrinol (Oxf) 2018 Apr 4. Epub 2018 Apr 4.
    Department of Medicine I, Endocrinology and Diabetes Unit, University Hospital Wuerzburg, Wuerzburg, Germany.
    Objective: To evaluate the management of adrenal emergencies (AE) requiring parenteral glucocorticoid (GC) treatment in patients with chronic adrenal insufficiency (AI).

    Design: Prospective, multicentre, questionnaire-based study.

    Patients And Measurements: Participating patients (n = 150) with chronic AI were provided with a questionnaire on the management of emergency situations, which had to be completed and sent back in case of an AE. Read More

    Therapeutic patient education in adrenal insufficiency.
    Ann Endocrinol (Paris) 2018 Mar 29. Epub 2018 Mar 29.
    Service des maladies endocriniennes et métaboliques, hôpital Cochin, CHU Paris-centre, centre de référence des maladies rares de la surrénale, 75014 Paris, France. Electronic address:
    It is essential to encourage patient autonomy in the management of their illness, and notably their participation in treatment education programs; specific programs target avoidance or early preventive treatment of acute adrenal insufficiency, which is a life-threatening complication. Therapeutic patient education is recommended by the two international consensus statements on the management of primary adrenal insufficiency and the French consensus on adrenal insufficiency. Although there is no common international reference framework to date, the objective of the French consensus was to provide a frame of reference to facilitate the development of therapeutic education for patients with adrenal insufficiency. Read More

    Adrenal crisis after first infusion of zoledronic acid: a case report.
    Osteoporos Int 2018 Mar 28. Epub 2018 Mar 28.
    Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre Ljubljana, Zaloska 7, 1525, Ljubljana, Slovenia.
    Patients with Addison's disease are at greater risk of having reduced bone mineral density and hip fractures and are thus more likely to receive a bisphosphonate than their peers. Potent intravenous bisphosphonates could provoke an acute phase reaction. An 80-year-old female with Addison's disease received her first infusion of zoledronic acid for osteoporosis at our outpatient clinic around noon. Read More

    Longitudinal assessment of illnesses, stress dosing and illness sequelae in patients with congenital adrenal hyperplasia.
    J Clin Endocrinol Metab 2018 Mar 22. Epub 2018 Mar 22.
    National Institutes of Health Clinical Center, Bethesda, MD.
    Context: Congenital adrenal hyperplasia (CAH) patients are at risk for life-threatening adrenal crises. Management of illness episodes aims to prevent adrenal crises.

    Objective: We evaluated rates of illnesses and associated factors in a cohort of CAH patients followed prospectively and receiving repeated glucocorticoid stress dosing education. Read More

    A rare cause of primary adrenal insufficiency due to a homozygous Arg188Cys mutation in the gene.
    Endocrinol Diabetes Metab Case Rep 2018 21;2018. Epub 2018 Mar 21.
    Pediatric Endocrinology, Diabetology and Metabolism, Bern University Children's Hospital and Department of BioMedical Research, University of Bern, Bern, Switzerland.
    Steroidogenic acute regulatory protein () is a key protein for the intracellular transport of cholesterol to the mitochondrium in endocrine organs (e.g. adrenal gland, ovaries, testes) and essential for the synthesis of all steroid hormones. Read More

    [Addisonian Crisis - Risk Assessment and Appropriate Treatment].
    Dtsch Med Wochenschr 2018 Mar 15;143(6):392-396. Epub 2018 Mar 15.
    An adrenal crisis (Addisonian crisis) is an acute life-threatening complication of adrenal insufficiency. It occurs when hydrocortisone demand is not met by supplementation in the context of an infection - often gastrointestinal, fever, trauma, acute psychological or physical stress. Symptoms of weakness, nausea, muscle/joint pain and drowsiness may develop out of robust health within few hours. Read More

    Metoclopramide induced pheochromocytoma crisis.
    Am J Emerg Med 2018 Mar 5. Epub 2018 Mar 5.
    Maryland Poison Center, Baltimore, MD, United States; Department of Emergency Medicine, University of Maryland School of Medicine, Baltimore, MD, United States.
    Metoclopramide (MCP) is a commonly used anti-emetic in the emergency department (ED). Its use is generally well tolerated; although infrequent adverse reactions such as extrapyramidal reactions or tardive dyskinesia are reported. However, many ED providers are not familiar with the potentially life-threatening hypertensive emergency that can be precipitated by MCP administration in patients with pheochromocytoma. Read More

    Iatrogenic water intoxication in a female adolescent with hypopituitarism.
    BMJ Case Rep 2018 Mar 9;2018. Epub 2018 Mar 9.
    Pediatric Endocrinology Unit, Hospital de Braga, Braga, Portugal.
    The authors report a case of a 15-year-old girl with hypopituitarism due to pituitary stalk interruption syndrome diagnosed in the neonatal period. The patient was admitted to the emergency room with impaired consciousness and hypoglycaemia. The day before, she increased her water intake to about 1. Read More

    Acute Primary Adrenal Insufficiency after Hip Replacement in a Patient with Acute Intermittent Porphyria.
    Case Rep Endocrinol 2018 4;2018:2353172. Epub 2018 Jan 4.
    Division of Endocrinology, Diabetology and Metabolism, Santa Croce e Carle Hospital, Via M. Coppino 26, 12100 Cuneo, Italy.
    Adrenal insufficiency is a potentially life-threatening condition when it occurs acutely, as in adrenal hemorrhage. Generally it is not reversible and requires chronic replacement therapy. Acute intermittent porphyria (AIP) is a rare genetic disease characterized by alterations in heme biosynthesis that result in accumulation of precursors in tissues. Read More

    Adrenal insufficiency, steroid sick day rules and the paediatric endocrine nurse.
    Nurs Child Young People 2018 Mar;30(2):26-31
    Oxford University Hospitals NHS Foundation Trust, Oxford, England.
    Clinical governance processes are important for improving patient care. Patients with adrenal insufficiency are at significant risk if they have an adrenal crisis and require steroid therapy. Families should receive education on managing illness or stress, that is, steroid sick day rules. Read More

    More, less or both?
    BMJ Case Rep 2018 Mar 5;2018. Epub 2018 Mar 5.
    Department of Internal medicine, VU Medical Center, Amsterdam, The Netherlands.
    A 67-year-old Caucasian woman with no prior medical history was admitted to our hospital with complaints of generalised weakness, nausea, diarrhoea and weight loss. The patient suffered from tachycardia and hypotension. Blood tests revealed Graves' thyrotoxicosis and the patient was treated accordingly. Read More

    Endocr Pract 2018 Mar 2. Epub 2018 Mar 2.
    Endocrine and Metabolic Unit, Royal Adelaide Hospital and University of Adelaide, North Terrace, Adelaide, SA, 5000, Australia.
    Background: Glucocorticoid pharmacotherapy is an effective treatment for a range of diseases but exposure can cause suppression of the HPA axis, leading to glucocorticoid-induced adrenal insufficiency (GC-AI) in some patients. However, the incidence of diagnosed GC-AI and the health burden it imposes on patients, including the incidence of adrenal crises (ACs), are unknown. Although, treatment of GC-AI is based on well-established principles, there are no agreed protocols on the perioperative management of exposed patients. Read More

    Nivolumab, a new immunomodulatory drug, a new adverse effect; adrenal crisis.
    Turk J Emerg Med 2017 Dec 20;17(4):157-159. Epub 2017 Jun 20.
    Ege University, Faculty of Medicine, Department of Emergency Medicine, Izmir, Turkey.
    Owing to the advancements in medicine, new information is obtained regarding cancer, new antineoplastic agents are developed. Frequent use of these new pharmacological agents emergency physicians to be vigilant about their side effects. We present a case of adrenal crisis in a patient with non-small cell lung cancer (NSCLC), caused by an immunomodulatory drug; nivolumab. Read More

    Acute pituitary disease in pregnancy: how to handle hypophysitis and Sheehan's syndrome?
    Minerva Endocrinol 2018 Feb 19. Epub 2018 Feb 19.
    Department of Neurosurgery, University of Tübingen, Tübingen, Germany.
    Pregnancy-related hypophysitis (PR-Hy) that typically occurs during late pregnancy and the early postpartum period is considered an autoimmune disorder. Knowledge and awareness of characteristic clinical symptoms and specific endocrine and neuroradiological features mostly enables a correct diagnosis and guarantees best possible management. In the presence of mild clinical symptoms, a "wait and watch" policy is recommended. Read More

    Incidence and Characteristics of Adrenal Crisis in Children Younger than 7 Years with 21-Hydroxylase Deficiency: A Nationwide Survey in Japan.
    Horm Res Paediatr 2018 16;89(3):166-171. Epub 2018 Feb 16.
    The Committee on Mass Screening, Japanese Society for Pediatric Endocrinology, Kyoto, Japan.
    Background/aims: We aimed to evaluate the incidence and characteristics of adrenal crisis in Japanese children with 21-hydroxylase deficiency (21-OHD).

    Methods: We conducted a retrospective nationwide survey for the councilors of the Japanese Society for Pediatric Endocrinology (JSPE) regarding adrenal crisis in children under 7 years with 21-OHD, admitted to hospitals from 2011 through 2016. We defined adrenal crisis as the acute impairment of general health due to glucocorticoid deficiency with at least two of symptoms, signs, or biochemical abnormalities. Read More

    Impact of opioid therapy on gonadal hormones: focus on buprenorphine.
    Horm Mol Biol Clin Investig 2018 Feb 17. Epub 2018 Feb 17.
    Veterans Affairs Medical Centre, Salem, VA, USA.
    Objective The USA is in the midst of an opioid crisis. Understanding the impact of opioids and commonly used treatments for opioid dependence is essential for clinicians and researchers in order to educate and treat the nation's growing population with opioid use disorders. As a relatively new treatment for opioid dependence, buprenorphine is gaining popularity to the extent of becoming not only a preferred approach to the maintenance of opiate addiction, but also an option for chronic pain management. Read More

    Retroperitoneal laparoscopic adrenalectomy with transient renal artery occlusion for large adrenal tumors (≥8 cm).
    J Surg Oncol 2018 Feb 15. Epub 2018 Feb 15.
    The Department of Urology, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi Province, China.
    Objectives: To analyze our experience in retroperitoneal laparoscopic adrenalectomy (RLA) with transient renal artery occlusion for large adrenal tumors (≥8 cm) and to explore the safety and feasibility of this surgical procedure.

    Methods: A retrospective cohort study was conducted with a surgical data review of 18 patients with large adrenal tumors who underwent RLA with transient renal artery occlusion in our hospital.

    Results: Eighteen patients were treated by RLA with transient occlusion of the renal artery, and none were converted to open adrenalectomy. Read More

    Primary adrenal insufficiency due to hereditary apolipoprotein AI amyloidosis: endocrine involvement beyond hypogonadism.
    Amyloid 2018 Feb 15:1-4. Epub 2018 Feb 15.
    a Department of Endocrinology and Nutrition , Hospital Clínic , Barcelona , Spain.
    Several mutations in the gene encoding apolipoprotein AI (apoAI) have been described as a cause of familial amyloidosis. Individuals with apoAI-derived (AApoAI) amyloidosis frequently manifest with liver, kidney, laryngeal, skin and myocardial involvement. Although primary hypogonadism (PH) is considered almost pathognomonic of this disease, until now, primary adrenal insufficiency (PAI) has not been described as a common clinical feature. Read More

    [Steroid 21-hydroxylase deficiency, the most frequent cause of congenital adrenal hyperplasia].
    Orv Hetil 2018 Feb;159(7):269-277
    II. Belgyógyászati Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
    Congenital adrenal hyperplasia is a group of genetic diseases due to the disablement of 7 genes; one of them is steroid 21-hydroxylase deficiency. The genes of congenital adrenal hyperplasia encode enzymes taking part in the steroidogenesis of adrenal gland. Steroid 21-hydroxylase deficiency is an autosomal recessive disorder caused by mutations of the steroid 21-hydroxylase gene. Read More

    Evaluation of the frequency of adrenal crises and preventive measures in patients with primary and secondary adrenal insufficiency in Switzerland.
    Swiss Med Wkly 2018 Jan 29;148:w14586. Epub 2018 Jan 29.
    Division of Diabetology, Endocrinology and Metabolism, University Hospital of Bern, Inselspital, Switzerland / Division of Endocrinology, Diabetology and Metabolism, University Hospital of Basel, Switzerland.
    Aims Of The Study: Adrenal insufficiency is a dangerous clinical condition, leading to significant morbidity or mortality in situations with inadequate glucocorticoid replacement treatment. We aimed to assess preventive measures in adrenal insufficiency and the incidence and risk factors of adrenal crisis, as well as to test the patients' knowledge about their disease.

    Methods: All patients in May and June 2016 and December 2016 and January 2017 with primary (17. Read More

    Effectiveness and safety of CT-guided percutaneous radiofrequency ablation of adrenal metastases.
    Br J Radiol 2018 May 7;91(1085):20170607. Epub 2018 Mar 7.
    2 Department of Anesthesiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences , Beijing , China.
    Objective: The imaging-guided percutaneous radiofrequency (RF) ablation of adrenal metastases is a relatively new treatment procedure, compared to the more widespread application of the technique for the treatment of liver and renal cancers. The present study aims to evaluate the safety and efficacy of the CT-guided percutaneous RF ablation of adrenal metastases in a cohort of patients.

    Methods: 33 patients with 38 adrenal metastases who received percutaneous CT-guided RF ablation between 2012 to 2015 were retrospectively reviewed. Read More

    Evaluation of quantitative parameters for distinguishing pheochromocytoma from other adrenal tumors.
    Hypertens Res 2018 Mar 18;41(3):165-175. Epub 2018 Jan 18.
    Department of Diabetes, Endocrinology and Nutrition, Kyoto University Graduate School of Medicine, Kyoto, Japan.
    Adrenal tumors are increasingly found incidentally during imaging examinations. It is important to distinguish pheochromocytomas from other adrenal tumors because of the risk of hypertensive crisis. Although catecholamines and their metabolites are generally used to diagnose pheochromocytoma, false-positive test results are common. Read More

    Genetic defect of a combined 17 α-hydroxylase/17,20-lyase deficiency patient with adrenal crisis.
    Gynecol Endocrinol 2018 Jan 18:1-5. Epub 2018 Jan 18.
    a Department of Gynecology , The Obstetrics and Gynecology Hospital , Shanghai , PR China.
    Combined 17 α-hydroxylase/17,20-lyase deficiency (17OHD) is a rare autosomal recessive disease that is a type of congenital adrenal hyperplasia, which results in hypertension, hypokalemia, sexual infantilism, primary amenorrhea in females (46,XX), or pseudohermaphroditism in males (46,XY). It is mainly caused by mutation in the CYP17A1 gene, which encodes a key enzyme in the steroidogenic pathway. However, these patients rarely experience adrenal crisis, due to abnormally high corticosterone levels. Read More

    Extent of surgery for phaeochromocytomas in the genomic era.
    Br J Surg 2018 Jan;105(2):e84-e98
    Department of Clinical and Experimental Medicine, Faculty of Medicine and Health Sciences, Linköping University, Linköping, Sweden.
    Background: Germline mutations are present in 20-30 per cent of patients with phaeochromocytoma. For patients who develop bilateral disease, complete removal of both adrenal glands (total adrenalectomy) will result in lifelong adrenal insufficiency with an increased risk of death from adrenal crisis. Unilateral/bilateral adrenal-sparing surgery (subtotal adrenalectomy) offers preservation of cortical function and independence from steroids, but leaves the adrenal medulla in situ and thus at risk of developing new and possibly malignant disease. Read More

    SFE/SFEDP adrenal insufficiency French consensus: Introduction and handbook.
    Ann Endocrinol (Paris) 2018 Feb 12;79(1):1-22. Epub 2018 Jan 12.
    Service d'endocrinologie diabétologie pédiatrique, hôpital Robert-Debré, centre de référence des maladies endocriniennes rares de la croissance et du développement, université Paris Diderot, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75019 Paris, France.
    The French endocrinology society (SFE) and the French pediatric endocrinology society (DFSDP) have drawn up recommendations for the management of primary and secondary adrenal insufficiency in the adult and child, based on an analysis of the literature by 19 experts in 6 work-groups. A diagnosis of adrenal insufficiency should be suspected in the presence of a number of non-specific symptoms except hyperpigmentation which is observed in primary adrenal insufficiency. Diagnosis rely on plasma cortisol and ACTH measurement at 8am and/or the cortisol increase after synacthen administration. Read More

    Improved medical-alert ID ownership and utilization in youth with congenital adrenal hyperplasia following a parent educational intervention.
    J Pediatr Endocrinol Metab 2018 Jan;31(2):213-219
    Center for Endocrinology, Diabetes and Metabolism, Children's Hospital Los Angeles, Los Angeles, CA, USA.
    Background: Classical congenital adrenal hyperplasia (CAH) is a potentially life-threatening condition, and adrenal crisis is a major cause of morbidity and mortality in affected children. Medical-alert identification (ID) could prevent complications of adrenal crisis by identifying the need for time-sensitive, critical treatment. Our objectives were to evaluate usage of medical-alert IDs by CAH youth, ownership and awareness of IDs amongst their parents, and the effect of an in-clinic educational intervention on ID utilization. Read More

    Isolated unilateral adrenal gland hemorrhage following motor vehicle collision: a case report and review of the literature.
    J Med Case Rep 2017 Dec 26;11(1):358. Epub 2017 Dec 26.
    Department of General Surgery, Henry Ford Wyandotte Hospital, 2333 Biddle Ave, Wyandotte, MI, 48192, USA.
    Background: Adrenal gland trauma is a rare condition that typically stems from blunt force trauma, and is associated with multiple organ injuries. Alternatively, isolated adrenal gland trauma is extremely rare, accounting for only 1.5 to 4% of all adrenal trauma cases. Read More

    Stress-Dosed Glucocorticoids and Mineralocorticoids Before Intensive Endurance Exercise in Primary Adrenal Insufficiency.
    Clin J Sport Med 2017 Dec 19. Epub 2017 Dec 19.
    Endocrinology and Metabolism, Wake Forest University, Winston-Salem, NC.
    Patients with primary adrenal insufficiency (PAI) require increased doses of glucocorticoids and mineralocorticoids during stressors, such as surgery, trauma, and sepsis. Although current guidelines exist for dose adjustments in these situations, there is no accepted dosing regimen for patients with PAI participating in intensive endurance exercise. Given the extensive physiologic stress of events, such as marathons, triathlons, and similar events, it is likely that a "stress-dose" of adrenal replacement therapy will not only prevent adrenal crisis, but also improve performance. Read More

    Polyendocrinopathy Resulting From Pembrolizumab in a Patient With a Malignant Melanoma.
    J Endocr Soc 2017 Jun 28;1(6):646-649. Epub 2017 Apr 28.
    Department of Endocrinology, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris Descartes University, 75014 Paris, France.
    Introduction: Checkpoint inhibitors have significantly improved the prognosis of patients with advanced melanoma. These cancer immunotherapy drugs have specific endocrine autoimmune toxicity. We describe a case of an adrenal insufficiency secondary to pembrolizumab, an anti-programmed cell death-1 monoclonal antibody. Read More

    Evaluation of Evidence of Adrenal Insufficiency in Trials of Normocortisolemic Patients Treated With Mifepristone.
    J Endocr Soc 2017 Apr 21;1(4):237-246. Epub 2017 Feb 21.
    Corcept Therapeutics, Menlo Park, California 94025.
    Context: Adrenal insufficiency (AI) is an important medical concern for clinicians when normocortisolemia is achieved during treatment of endogenous Cushing syndrome (CS).

    Objective: To examine symptoms of potential AI in a large population of normocortisolemic patients without CS treated with mifepristone, a glucocorticoid receptor antagonist indicated for the treatment of patients with CS.

    Methods: We conducted a pooled safety analysis of five phase 3, placebo-controlled clinical trials of normocortisolemic adults without CS but diagnosed with psychotic depression (n = 1460). Read More

    Different evolution in the treatment of a severe persistent asthma in 2 twins: Case report and review of the literature.
    Medicine (Baltimore) 2017 Dec;96(49):e8820
    aPediatrics Department, University of Medicine and Pharmacy "Grigore T. Popa"bClinic of Pulmonary Disease, Iasi, Romania.
    Rationale: Asthma is a multifactorial disease with complex genetic inheritance. In children under the age of 5 years, the diagnosis of asthma is a challenge.

    Patient Concern: We present the case of twin sisters under the same treatment for persistent asthma, but with different evolution over the time. Read More

    Initial clinical presentation and spectrum of pheochromocytoma: a study of 94 cases from a single center.
    Endocr Connect 2018 Jan 7;7(1):186-192. Epub 2017 Dec 7.
    Department of EndocrinologyMetabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden
    Background: With the increasing access to imaging more pheochromocytomas are diagnosed in the workup of adrenal incidentalomas. This may have changed the occurrence of the classic presentation with hypertension and the classic triad (headaches, sweating and palpitation).

    Methods: We reviewed 94 consecutive cases of pheochromocytomas. Read More

    An Autopsy Case of Sudden Death in Neurofibromatosis Type 1 With Pheochromocytoma and Myocarditis.
    Am J Forensic Med Pathol 2018 Mar;39(1):78-81
    An autopsy case of sudden death in a 33-year-old man with neurofibromatosis type 1 (von Recklinghausen disease), pheochromocytoma, and myocarditis is reported. The decedent was found in his bedroom in cardiopulmonary arrest. Polypoid, elastic dermal papules on the neck, chest, abdomen, and back, and flat dark-brown macules on the chest and abdomen were observed. Read More

    Optimal Management of a Synchronous Diagnosis of Phaeochromocytoma and Colorectal Neoplasia.
    Indian J Surg Oncol 2017 Dec 22;8(4):622-626. Epub 2017 Feb 22.
    University of Sheffield, Beech Hill Road, Sheffield, S10 2RX UK.
    The incidence rates of phaeochromocytoma and colorectal cancer (CRC) are approximately 1 and 65 per 100,000 per year, respectively. Simultaneous presentation of these conditions is rare and poses unique management challenges. We report on treatment strategies and clinical outcomes in a series of patients with colorectal neoplasia and phaeochromocytoma. Read More

    MANAGEMENT OF ENDOCRINE DISEASE: Fertility, pregnancy and lactation in women with adrenal insufficiency.
    Eur J Endocrinol 2018 Feb 30;178(2):R45-R53. Epub 2017 Nov 30.
    Klinik für EndokrinologieDiabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zürich, Switzerland
    With the introduction of hormonal substitution therapy in the 1950s, adrenal insufficiency (AI) has been turned into a manageable disease in pregnant women. In fact, in the light of glucocorticoid replacement therapy and improved obstetric care, it is realistic to expect good maternal and fetal outcomes in patients with AI. However, there are still a number of challenges such as establishing the diagnosis of AI in pregnant women and optimizing the treatment of AI and related comorbidities prior to as well as during pregnancy. Read More

    Group 6. Modalities and frequency of monitoring of patients with adrenal insufficiency. Patient education.
    Ann Endocrinol (Paris) 2017 Dec 1;78(6):544-558. Epub 2017 Dec 1.
    Service d'endocrinologie diabétologie pédiatrique, hôpital Robert-Debré, université Paris-Diderot, centre de référence des maladies endocriniennes rares de la croissance et du développement, Assistance publique-hôpitaux de Paris, 48, boulevard Sérurier, 75019 Paris, France.
    Patients with adrenal insufficiency require regular, specialised monitoring in order to optimise their replacement therapy, to detect signs of under- and over-dosage, and to examine for possible associated disorders (auto-immune disorders in the case of auto-immune primary adrenal insufficiency either isolated or as part of auto-immune polyendocrinopathy syndrome type 1; illnesses with underlying monogenic causes). The transition period between adolescence and adulthood represents an added risk of a breakdown in monitoring which requires particular attention from medical teams and coordination between adult and pediatric medical teams. It is essential to encourage patient autonomy in the management of their illness, notably their participation in treatment education programs, in particular programs that target avoidance of, or early treatment of acute adrenal insufficiency. Read More

    Group 1. Epidemiology of primary and secondary adrenal insufficiency: Prevalence and incidence, acute adrenal insufficiency, long-term morbidity and mortality.
    Ann Endocrinol (Paris) 2017 Dec 27;78(6):490-494. Epub 2017 Nov 27.
    Service des maladies endocriniennes et métaboliques, hôpital Cochin, CHU Paris Centre, 75014 Paris, France.
    The prevalence of primary adrenal insufficiency is estimated at between 82-144/million, with auto-immunity being the most common cause in adults and genetic causes, especially enzyme defects, being the most common cause in children. The prevalence of secondary adrenal deficiency is estimated to be between 150-280/million. The most frequent occurrence is believed to be corticosteroid-induced insufficiency, despite the incidence of clinically relevant deficiency after cessation of glucocorticoid treatment being widely debated. Read More

    Group 5: Acute adrenal insufficiency in adults and pediatric patients.
    Ann Endocrinol (Paris) 2017 Dec 23;78(6):535-543. Epub 2017 Nov 23.
    Service d'endocrinologie et des maladies de la reproduction, hôpital de Bicêtre, hôpitaux universitaires Paris-Sud, AP-HP, 94275 Le Kremlin-Bicêtre, France; Inserm 1185, faculté médecine Paris-Sud, université Paris-Sud, université Paris-Saclay, 94276 Le Kremlin-Bicêtre, France.

    Management of an acute catecholamine-induced cardiomyopathy and circulatory collapse: a multidisciplinary approach.
    Endocrinol Diabetes Metab Case Rep 2017 9;2017. Epub 2017 Nov 9.
    Departments of Endocrinology and Diabetes.
    A phaeochromocytoma (PC) is a rare, catecholamine-secreting neuroendocrine tumour arising from the adrenal medulla. Presenting symptoms of this rare tumour are highly variable but life-threatening multiorgan dysfunction can occur secondary to catecholamine-induced hypertension or hypotension and subsequent cardiovascular collapse. High levels of circulating catecholamines can induce an acute stress cardiomyopathy, also known as Takotsubo cardiomyopathy. Read More

    Massive adrenal incidentalomas and late diagnosis of congenital adrenal hyperplasia in prostate cancer.
    Endocrinol Diabetes Metab Case Rep 2017 28;2017. Epub 2017 Oct 28.
    Division of Endocrinology, Department of Medicine, University of Calgary, Calgary, Alberta,Canada.
    In a 61-year-old Caucasian male with prostate cancer, leuprolide and bicalutamide failed to suppress the androgens. He presented to endocrinology with persistently normal testosterone and incidental massive (up to 18 cm) bilateral adrenal myelolipomas on CT scan. Blood test did not reveal metanephrine excess. Read More

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