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    1 OF 29
    Recognizing and managing adrenal disorders in the emergency department
    Emerg Med Pract 2017 09;19(9):1-24
    Assistant Professor of Emergency Medicine, Department of Emergency Medicine, University of Nebraska Medical Center, Omaha, NE
    Primary and secondary adrenal insufficiency are underrecognized conditions among emergency department patients, affecting an estimated 10% to 20% of critically ill patients. The signs and symptoms of cortisol deficit can be nonspecific and wide-ranging, and identification and swift treatment with stress-dosing of hydrocortisone is vital to avoid life-threatening adrenal crisis. Laboratory evaluation focuses on identification of electrolyte abnormalities typical of adrenal insufficiency, and while additional testing may depend on the type and severity of symptoms, it should not delay corticosteroid replacement. Read More
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    [Adrenal crisis].
    Internist (Berl) 2017 Aug 16. Epub 2017 Aug 16.
    Medizinische Klinik und Poliklinik, Schwerpunkt Endokrinologie und Diabetologie, Universitätsklinikum Würzburg, Oberdürrbacherstr. 6, 97080, Würzburg, Deutschland.
    Patients with chronic adrenal insufficiency suffer from reduced quality of life and increased mortality. An association between mortality and adrenal crisis is assumed. The frequency of adrenal crisis is about 8/100 patient years. Read More
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    Fatal Systemic Vasoconstriction in a Case of Metastatic Small-Intestinal NET.
    Case Rep Gastrointest Med 2017 18;2017:9810194. Epub 2017 Jul 18.
    Department of Clinical Toxicology, Klinikum rechts der Isar, Technical University of Munich, Ismaningerstrasse 22, 81675 Munich, Germany.
    An increased release of serotonin secreted by ileal NETs is thought to be the major factor causing the carcinoid syndrome. However, in acutely arising carcinoid crisis also other vasoactive factors may lead to hazardous fluctuations in blood pressure and bronchial constriction. In rare cases, systemic vasoconstriction can be observed, probably caused by catecholamines or similar acting substances. Read More
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    Parental management of adrenal crisis in children with congenital adrenal hyperplasia.
    J Spec Pediatr Nurs 2017 Aug 3. Epub 2017 Aug 3.
    University of North Carolina at Chapel Hill School of Nursing, Chapel Hill, NC, USA.
    Purpose: Congenital adrenal hyperplasia (CAH) requires parents to inject their child with hydrocortisone intramuscularly during times of illness and adrenal crisis. The purpose of this study was to describe circumstances surrounding adrenal crisis events in children with CAH; to explore parents' perceptions of the consequences of having a child with a life-threatening condition; and to examine a relationship between parents' perceived management ability and the impact CAH has on the family.

    Methods: In Phase 1 of this mixed-methods, cross-sectional study, 77 parents were asked to complete questionnaires comprising measures of family life in the context of childhood illness. Read More
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    [Treatment of an uncommon case of a cardiogenic shock : Simultaneous use of a VA-ECMO and an Impella-CP®].
    Med Klin Intensivmed Notfmed 2017 Aug 2. Epub 2017 Aug 2.
    Abteilung für Kardiologie und Intensivmedizin, Kliniken Maria Hilf, Lehrkrankenhaus der Uniklinik RWTH Aachen, Viersener Straße 450, 41063, Mönchengladbach, Deutschland.
    We report the case of a 48-year old woman where probably the intramuscular administration of glucocorticoids by an orthopedist induced a pheochromocytoma crisis. The development of a cardiogenic shock with a cardiac arrest made the use of a venoarterial extra corporeal membrane oxygenation (VA-ECMO, Cardiohelp®, Maquet, Rastatt) necessary. To treat a pulmonary edema under VA-ECMO an Impella-CP® (Abiomed, Aachen) was implanted. Read More
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    Exploration of knowledge and understanding in patients with primary adrenal insufficiency: a mixed methods study.
    BMC Endocr Disord 2017 Aug 1;17(1):47. Epub 2017 Aug 1.
    School of Nursing, Institute of Clinical Sciences, College of Medical & Dental Sciences, University of Birmingham, Edgbaston, Birmingham, West Midlands, B15 2TT, UK.
    Background: Primary adrenal insufficiency (PAI) is a rare and severe condition requiring lifelong steroid replacement. During acute illness or stressful events, it is important to appropriately adjust glucocorticoid dose; failure to do so may lead to an adrenal crisis. The aim of the study was to explore patients PAI knowledge and understanding of the condition, steroid replacement adjustment during acute illness or stress and provided education. Read More
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    The PJ Nicholoff Steroid Protocol for Duchenne and Becker Muscular Dystrophy and Adrenal Suppression.
    PLoS Curr 2017 Jun 27;9. Epub 2017 Jun 27.
    Complex Care, Nationwide Children's Hospital, Columbus, OH, USA.
    Duchenne muscular dystrophy (DMD or Duchenne) is a progressive, life-limiting muscle-wasting disease that requires comprehensive, multidisciplinary care. This care, at minimum, should include neuromuscular, respiratory, cardiac, orthopedic, endocrine and rehabilitative interventions that address both the primary and secondary manifestations of the disease. The care needs of patients evolve over the cdourse of the disease and as they transition from childhood into young adulthood. Read More
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    Adrenal crisis in metastatic breast cancer.
    BMJ Case Rep 2017 Jul 6;2017. Epub 2017 Jul 6.
    Interne I: Internistische Onkologie, Hämatologie u. Gastroenterologie, Ordensklinikum Linz Krankenhaus d. Barmherzigen Schwestern Linz Betriebs GmbH, Linz, Austria.
    A female patient with oestrogen receptor-positive and human epidermal growth factor receptor 2 (HER2)-positive invasive lobular breast cancer presented with progressive disease on CT scan. Some days after initiation of antineoplastic chemotherapy and anti-HER2 targeted antibody therapy, the patient presented with profuse diarrhoea, neutropaenia, nausea and weakness. Although Clostridium difficile was rapidly tackled as a causative agent of gastrointestinal complaints, clinical situation did not markedly improve despite proper antimicrobial treatment. Read More
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    Hypertensive crisis secondary to pheochromocytoma.
    Proc (Bayl Univ Med Cent) 2017 Jul;30(3):314-315
    Department of Surgery, University of Mississippi Medical Center, Jackson, Mississippi.
    Pheochromocytoma is an uncommon tumor of the adrenal glands that can present with headaches, sweating, palpitations, and paroxysmal hypertension. Pheochromocytoma crisis can lead to cardiomyopathy, pulmonary edema, and even total circulatory collapse. We describe a patient with hypoxic respiratory failure requiring extracorporeal membrane oxygenation to stabilize until the pheochromocytoma was discovered and treated. Read More
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    The Key to Adrenal Insufficiency Education: Repetition, Repetition, Repetition.
    Pediatr Endocrinol Rev 2017 Jun;14(Suppl 2):448-453
    Organic Acid Research Section (OARS) of the Medical Genomics and Metabolic Genetics Branch (MGMGB) National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA.
    Described more than 150 years ago by Thomas Addison, adrenal gland dysfunction, while treatable, remains a clinically significant and potentially fatal disease. Vague and non-specific symptomatology can delay diagnosis of adrenal insufficiency and lead to adrenal crisis. Affected individuals may delay self-management due to knowledge deficits or lack of required therapies. Read More
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    Thyrotoxic and pheochromocytoma multisystem crisis: a case report.
    J Med Case Rep 2017 Jun 23;11(1):173. Epub 2017 Jun 23.
    Department of Emergency and Disaster Medicine, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan.
    Background: Thyrotoxic crisis and pheochromocytoma multisystem crisis are rare, life-threatening, emergency endocrine diseases with various clinical manifestations. Here we report a case of a patient who simultaneously developed thyrotoxic crisis and pheochromocytoma multisystem crisis and required intensive cardiovascular management.

    Case Presentation: A 60-year-old Asian man experienced nausea and vomiting, and subsequently developed dyspnea and cold sweats while farming. Read More
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    Adrenal insufficiency - recognition and management.
    Clin Med (Lond) 2017 Jun;17(3):258-262
    Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK
    Adrenal insufficiency is characterised by inadequate -glucocorticoid production owing to destruction of the adrenal cortex or lack of adrenocorticotropic hormone stimulation. In primary adrenal insufficiency, lack of mineralocorticoids is also a feature. Patients can present with an insidious onset of symptoms, or acutely in adrenal crisis, which requires prompt recognition and treatment. Read More
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    Endocrine Emergencies With Neurologic Manifestations.
    Continuum (Minneap Minn) 2017 Jun;23(3, Neurology of Systemic Disease):778-801
    Purpose Of Review: This article provides an overview of endocrine emergencies with potentially devastating neurologic manifestations that may be fatal if left untreated. Pituitary apoplexy, adrenal crisis, myxedema coma, thyroid storm, acute hypercalcemia and hypocalcemia, hyperglycemic emergencies (diabetic ketoacidosis and hyperglycemic hyperosmolar state), and acute hypoglycemia are discussed, with an emphasis on identifying the signs and symptoms as well as diagnosing and managing these clinical entities.

    Recent Findings: To identify the optimal management of endocrine emergencies, using formal clinical diagnostic criteria and grading scales such as those recently proposed for pituitary apoplexy will be beneficial in future prospective studies. Read More
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    Thyrotoxicosis leading to adrenal crises reveals primary bilateral adrenal lymphoma.
    Endocrinol Diabetes Metab Case Rep 2017 16;2017. Epub 2017 May 16.
    Anatomopathology Department, Hospital de Egas Moniz C.H.L.O.-E.P.E, LisbonPortugal.
    Primary adrenal lymphoma is a rare malignancy. It frequently presents bilaterally and with symptoms of adrenal insufficiency. Amiodarone may induce secondary organ dysfunction, and thyrotoxicosis develops in 15% of cases. Read More
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    A case report of pedigree of a homozygous mutation of the steroidogenic acute regulatory protein causing lipoid congenital adrenal hyperplasia.
    Medicine (Baltimore) 2017 May;96(21):e6994
    aDepartment of Endocrinology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Key Laboratory of Health and Family Planning Commission, Beijing, China bThe Key Laboratory of Genome Sciences and Information, Beijing Institute of Genomics, Chinese Academy of Sciences, Beijing, China.
    Rationale: Lipoid congenital adrenal hyperplasia (LCAH) is extremely rare, but is the most fatal form of congenital adrenal hyperplasia resulting from mutations in the steroidogenic acute regulatory protein (STAR) gene. LCAH arises from severe defects in the conversion of cholesterol to pregnenolone, the precursor of all steroids.

    Patient Concerns: A case was reported that an 11-month-old Chinese girl who presented with a sex development disorder and hyponatremia. Read More
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    Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome.
    Horm Res Paediatr 2017 May 19. Epub 2017 May 19.
    Department of Pediatrics, Oita University Faculty of Medicine, Oita, Japan.
    Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Read More
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    Quality of compounded hydrocortisone capsules used in the treatment of children.
    Eur J Endocrinol 2017 Aug 16;177(2):239-242. Epub 2017 May 16.
    Institute for Experimental Paediatric EndocrinologyCharité Berlin, Berlin, Germany
    Objectives: Due to the lack of paediatric-licensed formulations, children are often treated with individualized pharmacy-compounded adult medication. An international web-based survey about the types of medication in children with adrenal insufficiency (AI) revealed that the majority of paediatric physicians are using pharmacy-compounded medication to treat children with AI. Observations of loss of therapy control in children with congenital adrenal hyperplasia with compounded hydrocortisone capsules and regained control after prescribing a new hydrocortisone batch led to this 'real world' evaluation of pharmacy-compounded paediatric hydrocortisone capsules. Read More
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    Patients treated with immunosuppressive steroids are less aware of sick day rules Than those on endocrine replacement therapy and may be at greater risk of adrenal crisis.
    Ir J Med Sci 2017 May 4. Epub 2017 May 4.
    Academic Department of Endocrinology, Beaumont Hospital/RCSI Medical School, Beaumont Road, Dublin 9, Co. Dublin, Ireland.
    Background: Patients taking corticosteroids for immune suppression are vulnerable to adrenal crisis during acute illness or if steroids are stopped abruptly. Although patients treated for adrenal failure in endocrine units are routinely provided with sick day guidelines, we wished to ascertain whether patients on immunosuppressive steroids are appropriately advised.

    Aim: This study sets out to compare patient awareness of steroid sick day rules in endocrine and non-endocrine patients. Read More
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    De novo disruption of promoter and exon 1 of STAR gene reveals essential role for gonadal development.
    Endocrinol Diabetes Metab Case Rep 2017 13;2017. Epub 2017 Mar 13.
    Laboratory of Biochemistry, Mercer University School of Medicine, Savannah, GeorgiaUSA.
    Summary: Cholesterol transport into the mitochondria is required for synthesis of the first steroid, pregnenolone. Cholesterol is transported by the steroidogenic acute regulatory protein (STAR), which acts at the outer mitochondrial membrane prior to its import. Mutations in the STAR protein result in lipoid congenital adrenal hyperplasia (CAH). Read More
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    Autoimmune polyendocrine syndrome type 1 in an Indian cohort: a longitudinal study.
    Endocr Connect 2017 Jul 26;6(5):289-296. Epub 2017 Apr 26.
    Departments of EndocrinologySanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
    Objective: Autoimmune polyendocrine syndrome type 1 (APS1) is a rare autosomal recessive disorder characterized by progressive organ-specific autoimmunity. There is scant information on APS1 in ethnic groups other than European Caucasians. We studied clinical aspects and autoimmune regulator (AIRE) gene mutations in a cohort of Indian APS1 patients. Read More
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    Management of Childhood Congenital Adrenal Hyperplasia-An Integrative Review of the Literature.
    J Pediatr Health Care 2017 Apr 13. Epub 2017 Apr 13.
    Congenital adrenal hyperplasia (CAH) is an endocrine condition that requires parents to administer steroids up to three times daily, supplementing maintenance doses with oral or injectable doses during times of illness. The purpose of this integrative review was to explore the management, care, and associated health issues for children with CAH and the family response to the condition. Four literature indexes were searched, with 39 articles included. Read More
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    Paraganglioma of the bladder in a kidney transplant recipient: A case report.
    Mol Clin Oncol 2017 Apr 6;6(4):553-555. Epub 2017 Mar 6.
    Department of Nephrology and Kidney Transplantation, Necker Enfants-Malades Hospital, Paris Descartes University, Assistance Publique-Hôpitaux de Paris, 75015 Paris, France.
    Renal transplantation has been associated with a significantly increased risk of developing cancer, including bladder neoplasia, with urothelial carcinoma being the most frequent type of bladder cancer. Bladder paraganglioma, also referred to as extra-adrenal pheochromocytoma, is a rare but severe condition that may cause a severe hypertensive crisis during handling and mobilization of the tumor. We herein present the case of a 67-year-old kidney transplant recipient with a bladder polyp consistent with paraganglioma of the bladder. Read More
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    Pheochromocytoma Crisis in the ICU: A French Multicenter Cohort Study With Emphasis on Rescue Extracorporeal Membrane Oxygenation.
    Crit Care Med 2017 Jul;45(7):e657-e665
    1Service de Réanimation Médicale Polyvalente, Centre Hospitalier Public du Cotentin, Cherbourg-en-Cotentin, France.2Service de Réanimation Médicale, Centre Hospitalier Universitaire, Avenue de la Côte de Nacre, Caen, France.3Service de Réanimation Médicale et Médecine hyperbare, Centre Hospitalier Universitaire, Angers, France.4Service de Réanimation Médicale, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.5Service d'Anesthésie Réanimation, Groupement des Hôpitaux de l'Institut Catholique de Lille, Lille, France.6Service de Réanimation Chirurgicale, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.7Service de Réanimation Médico-chirurgicale, Centre Hospitalier Universitaire Dupuytren, Limoges Cedex, France.8Département d'Anesthésie-Réanimation, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.9Service de Réanimation Médicale et infectieuse, Hôpital Bichat Claude Bernard, Assistance Publique-Hôpitaux de Paris, Paris, France.10Service de Réanimation Médicale, Centre Hospitalier Universitaire, Rouen, France.11Service de Réanimation Médico-chirurgicale, Centre Hospitalier de Longjumeau, Longjumeau, France.12Service de Réanimation Médicale, Hôpital Brabois, Centre Hospitalier Universitaire, Vandoeuvre les Nancy, France.13Service de Réanimation Polyvalente, Hôpital Félix-Guyon, Centre Hospitalier Universitaire, Saint-Denis, La Réunion, France.14Service de Réanimation Polyvalente, Centre Hospitalier de Cholet, Cholet Cedex, France.15Service de Réanimation Polyvalente, Centre Hospitalier Robert Ballanger, boulevard Robert Ballanger, Aulnay sous Bois, France.16Service de Réanimation Polyvalente, Centre Hospitalier Versailles, Le Chesnay, France.17Service de Médecine intensive, Centre Hospitalier Marc Jacquet, Melun cedex, France.18Service d'Anesthésie Réanimation, Centre Hospitalier Universitaire, Caen, France.19Université de Caen Normandie, Esplanade de la Paix, Caen, France.20Service de Réanimation Médicale, CHU Grenoble Alpes, Grenoble, France.
    Objectives: To describe the characteristics, management, and outcome of patients admitted to ICUs for pheochromocytoma crisis.

    Design: A 16-year multicenter retrospective study.

    Setting: Fifteen university and nonuniversity ICUs in France. Read More
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    Clinical characteristics of Taiwanese children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency detected by neonatal screening.
    J Formos Med Assoc 2017 Apr 6. Epub 2017 Apr 6.
    Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address:
    Background/purpose: Neonatal screening for congenital adrenal hyperplasia (CAH) has been conducted in Taiwan since 2000. This study aimed to determine the clinical characteristics of Taiwanese children with CAH due to 21-hydroxylase deficiency (21-OHD) detected by neonatal screening.

    Methods: From 2000 to 2015, 26 neonates (14 boys and 12 girls) with classic 21-OHD detected by neonatal screening and confirmed at National Taiwan University Hospital were enrolled. Read More
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    Primary pigmented nodular adrenocortical disease: literature review and case report of a 6-year-old boy.
    J Pediatr Endocrinol Metab 2017 May;30(5):603-609
    University of Novi Sad, Faculty of Medicine, Novi Sad.
    Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. Read More
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    A Complex Interplay: Cognitive Behavioural Therapy for Severe Health Anxiety in Addison's Disease to Reduce Emergency Department Admissions.
    Behav Cogn Psychother 2017 Jul 9;45(4):419-426. Epub 2017 Apr 9.
    Department of Psychology,University of Bath,Bath,UK.
    Background: Addison's disease (AD) is a rare chronic illness caused by adrenocortical insufficiency. Due to the pivotal role of the regulating hormone cortisol in AD, there is a common symptom overlap between the presentation of anxiety and adrenal crisis. Previous literature has identified the prevalence of anxiety in endocrinological disorders, however there is a paucity of research examining the complex interplay between AD and anxiety. Read More
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    [A clinical analysis of 123 cases of primary empty sella].
    Zhonghua Nei Ke Za Zhi 2017 Apr;56(4):268-272
    Department of Endocrinology and Metabolism, General Hospital of Tianjin Medical University, Tianjin 300052, China.
    Objective: This study was conducted to analyze the clinical characteristics and pituitary function of patients with primary empty sella (PES). Methods: The clinical data from 123 hospitalized adult patients with PES from January 2010 to May 2016 were retrospectively studied. Results: (1) The average age of the 123 (male 43, female 80) PES patients was (59. Read More
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    Symptomatic adrenal suppression among children in Canada.
    Arch Dis Child 2017 Apr 9;102(4):338-339. Epub 2016 Nov 9.
    Department of Pediatrics (Endocrinology), Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.
    Background: Adrenal suppression (AS) is an under-recognised side effect of glucocorticoid (GC) use. AS may go undetected until a physiological stress precipitates an adrenal crisis. The incidence of AS has not been established. Read More
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    Clinical Characteristics of Patients with Adrenal Insufficiency in a General Hospital.
    Endocrinol Metab (Seoul) 2017 Mar 19;32(1):83-89. Epub 2017 Jan 19.
    Division of Endocrinology and Metabolism, Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea.
    Background: Adrenal insufficiency (AI) is a life-threatening disorder caused by the deficiency of adrenal steroid hormones. This retrospective cross-sectional study investigated the characteristics of patients with AI in Korea.

    Methods: All consecutive patients with suspected AI who received care at a tertiary referral center in Korea in 2014 and underwent adrenocorticotropic hormone stimulation or insulin-tolerance testing were identified through a review of medical charts. Read More
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    [Improvement in Hyperglysemia Following Unilateral Adrenalectomy for ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH) : A Case Report].
    Hinyokika Kiyo 2017 Jan;63(1):7-10
    The Department of Radiology, Kansai Medecal University.
    Adrenal corticotropin (ACTH) -independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is the treatment of choice, but lifetime steroid replacement is essential. Here we report a case of AIMAH whose hyperglycemia was improved following unilateral adrenalectomy. Read More
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    Nivolumab induced myxedema crisis.
    J Immunother Cancer 2017 21;5:13. Epub 2017 Feb 21.
    Department of Hematology/Oncology, St. John Hospital and Medical Center, 19229 Mack Ave Suite 23 Grosse Pointe Woods, Detroit, 48236 Michigan USA.
    Background: Nivolumab is an anti-programmed cell death (anti-PD-1) monoclonal antibody that is approved by Food and Drug Administration for treatment of metastatic non-small cell lung cancer, metastatic melanoma, relapsed Hodgkin lymphoma and advanced renal cell cancer. We report a rare case of myxedema crisis induced by nivolumab in a patient with metastatic squamous cell carcinoma of lung.

    Case Presentation: Fifty three-year old woman with metastatic squamous cell carcinoma currently on treatment with nivolumab presented with diffuse facial and tongue swelling, slurred speech, depressed mentation, fatigue and weakness. Read More
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    Septo-optic dysplasia/de Morsier's syndrome.
    Saudi J Anaesth 2017 Jan-Mar;11(1):106-107
    Department of Anesthesia, São João Hospital Centre, Porto, Portugal; Department of Anesthesia, Medical Faculty, Oporto University, Porto, Portugal.
    Septo-optic dysplasia (SOD)/de Morsier's syndrome is characterized by optic nerve hypoplasia, pituitary endocrine dysfunction, and midline brain abnormalities. Hypopituitarism, hypothyroidism, hypogonadism, and adrenal insufficiency can lead to severe hypoglycemia, adrenal crisis, seizures, and sudden death. Anesthetic management of SOD was associated with high perioperative mortality. Read More
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    From Bad to Worse: Paraganglioma Diagnosis during Induction of Labor for Coexisting Preeclampsia.
    Case Rep Anesthesiol 2017 18;2017:5495808. Epub 2017 Jan 18.
    Department of Anesthesiology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
    Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. Read More
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    Two Moroccan Sisters Presenting with a Severe Salt-Wasting Form of Congenital Adrenal Hyperplasia but Normal Female Genitalia.
    Sex Dev 2017 11;11(2):82-85. Epub 2017 Feb 11.
    Neonatology Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
    We report the case of 2 sisters (46,XX) born from consanguineous Moroccan parents. Both sisters had normal female genitalia, but within 2 weeks after birth, they presented with a severe salt-wasting crisis. Hormonal investigations suggested the diagnosis of congenital adrenal hyperplasia, which was confirmed by subsequent molecular analysis to be caused by 3β-hydroxysteroid dehydrogenase type 2 deficiency. Read More
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    [Recommendations for the diagnosis and treatment of classic forms of 21-hydroxylase-deficient congenital adrenal hyperplasia].
    An Pediatr (Barc) 2017 Aug 1;87(2):116.e1-116.e10. Epub 2017 Feb 1.
    Hospital Clínico Universitario Virgen Arrixaca, Murcia, España.
    Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is an autosomal recessive disorder caused by mutations in the CYP21A2 gene. Cortisol and aldosterone synthesis are impaired in the classic forms (adrenal insufficiency and salt-wasting crisis). Females affected are virilised at birth, and are at risk for genital ambiguity. Read More
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    Clinical features and practice patterns of treatment for adrenal crisis: a nationwide cross-sectional study in Japan.
    Eur J Endocrinol 2017 Mar;176(3):329-337
    Department of General MedicineNational Defense Medical College, Tokorozawa, Saitama, Japan.
    Context: Adrenal crisis is an endocrine emergency that requires prompt diagnosis and treatment. However, the clinical features and practice patterns of treatment for adrenal crisis are not completely understood.

    Objective: To investigate patient characteristics, comorbidities and treatments of adrenal crisis. Read More
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    Consensus guideline for the diagnosis and treatment of aromatic l-amino acid decarboxylase (AADC) deficiency.
    Orphanet J Rare Dis 2017 Jan 18;12(1):12. Epub 2017 Jan 18.
    Department of Child Neurology and Metabolic Disorders, University Children's Hospital, Heidelberg, Germany.
    Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare, autosomal recessive neurometabolic disorder that leads to a severe combined deficiency of serotonin, dopamine, norepinephrine and epinephrine. Onset is early in life, and key clinical symptoms are hypotonia, movement disorders (oculogyric crisis, dystonia, and hypokinesia), developmental delay, and autonomic symptoms.In this consensus guideline, representatives of the International Working Group on Neurotransmitter Related Disorders (iNTD) and patient representatives evaluated all available evidence for diagnosis and treatment of AADCD and made recommendations using SIGN and GRADE methodology. Read More
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    Acute management of autoimmune toxicity in cancer patients on immunotherapy: Common toxicities and the approach for the emergency physician.
    Emerg Med Australas 2017 Apr 16;29(2):245-251. Epub 2017 Jan 16.
    Medical Oncology, Chris O'Brien Lifehouse, Camperdown, New South Wales, Australia.
    When a patient receiving anti-cancer treatment presents acutely unwell, an understanding of associated side effects of their therapy is critical. This review will discuss the approach to patients receiving anti-cancer treatment with immunotherapy presenting with autoimmune toxicities in the emergency setting. These toxicities are commonly referred to as immune-related adverse events (irAE). Read More
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    Pheochromocytoma Multisystem Crisis Behaving Like Interstitial Pneumonia: An Autopsy Case.
    Intern Med 2017 15;56(2):149-152. Epub 2017 Jan 15.
    Department of Cardiology, Fuchu Hospital, Japan.
    Pheochromocytoma multisystem crisis is a rare and life-threatening disease that is associated with numerous symptoms and which is also difficult to diagnose. We herein report an autopsy case of a 61-year-old man who died due to pheochromocytoma multisystem crisis. The patient complained of vomiting and breathlessness. Read More
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    Cystic Pheochromocytoma Presenting as Adrenal Cyst.
    J Clin Diagn Res 2016 Nov 1;10(11):OD09-OD10. Epub 2016 Nov 1.
    Senior Medical Specialist, GB Pant Hospital , Port Blair, Andaman and Nicobar Islands, India .
    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. Read More
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    Effect of Exercise on Ovulation: A Systematic Review.
    Sports Med 2017 Aug;47(8):1555-1567
    Laboratory of Protein Biochemistry, Federal University of State of Rio de Janeiro, Rio de Janeiro, Brazil.
    Background: Infertility has been described as a devastating life crisis for couples, and has a particularly severe effect on women, in terms of anxiety and depression. Anovulation accounts for around 30% of female infertility, and while lifestyle factors such as physical activity are known to be important, the relationship between exercise and ovulation is multi-factorial and complex, and to date there are no clear recommendations concerning exercise regimes.

    Objectives: The objective of this review was to systematically assess the effect of physical activity on ovulation and to discuss the possible mechanisms by which exercise acts to modulate ovulation in reproductive-age women. Read More
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    Sheehan syndrome.
    Nat Rev Dis Primers 2016 12 22;2:16092. Epub 2016 Dec 22.
    Department of Endocrinology, Erciyes University Medical School, 38039, Kayseri, Turkey.
    Sheehan syndrome or postpartum hypopituitarism is a condition characterized by hypopituitarism due to necrosis of the pituitary gland. The initial insult is caused by massive postpartum haemorrhage (PPH), leading to impaired blood supply to the pituitary gland, which has become enlarged during pregnancy. Small sella turcica size, vasospasms (caused by PPH) and/or thrombosis (associated with pregnancy or coagulation disorders) are predisposing factors; autoimmunity might be involved in the progressive worsening of pituitary functions. Read More
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