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    1 OF 29

    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 Jan 19:20170607. Epub 2018 Jan 19.
    2 Department of Anesthesiology, Peking Union Medical College Hospital, 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 computed tomography (CT)-guided percutaneous radiofrequency ablation of adrenal metastases in a cohort of patients.

    Methods: Thirty-three patients with 38 adrenal metastases who received percutaneous CT-guided radiofrequency ablation between 2012 to 2015 were retrospectively reviewed. The average diameter of the treated adrenal metastases was 3. Read More

    Evaluation of quantitative parameters for distinguishing pheochromocytoma from other adrenal tumors.
    Hypertens Res 2018 Jan 18. 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 Jan 12. 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

    Acute development of Cushing syndrome in an HIV-infected child on atazanavir/ritonavir based antiretroviral therapy.
    Endocrinol Diabetes Metab Case Rep 2017 28;2017. Epub 2017 Oct 28.
    Divisions of Infectious Diseases.
    An 11-year-old male with perinatally acquired human immune deficiency virus (HIV) infection on antiretroviral regimen, which included abacavir plus lamivudine (Epzicom), didanosine, ritonavir and atazanavir presented with bilateral axillary striae, increased appetite, fatigue, facial swelling and acute weight gain. Two months prior to presentation, the patient had received a diagnostic and therapeutic intra-articular triamcinolone injection in the knee for pain relief and subsequently became progressively swollen in the face, developed striae bilaterally at the axillae, experienced increased appetite, fatigue and an 8 pound weight gain. During the endocrine workup, suspicion for adrenal insufficiency prompted 24-h urine collection for free cortisol, which was found to be undetectable (below LLQ of 1. Read More

    Acute-Onset Panhypopituitarism Nearly Missed by Initial Cosyntropin Testing.
    Case Rep Crit Care 2017 3;2017:7931438. Epub 2017 Oct 3.
    Medical University Clinic, Kantonsspital Aarau, Aarau, Switzerland.
    Introduction: Diagnosis of adrenal crisis and panhypopituitarism in patients with septic shock is difficult but crucial for outcome.

    Case: A 66-year-old woman with metastasized breast cancer presented to the ED with respiratory insufficiency and septic shock after a 2-day history of the flu. After transfer to the ICU, corticosteroids were started in addition to antibiotics, as the patient was vasopressor-nonresponsive. Read More

    Clues for early detection of autoimmune Addison's disease - myths and realities.
    J Intern Med 2018 Feb 3;283(2):190-199. Epub 2017 Nov 3.
    Department of Clinical Medicine, University of Bergen, Bergen, Norway.
    Background: Early detection of autoimmune Addison's disease (AAD) is important as delay in diagnosis may result in a life-threatening adrenal crisis and death. The classical clinical picture of untreated AAD is well-described, but methodical investigations are scarce.

    Objective: Perform a retrospective audit of patient records with the aim of identifying biochemical markers for early diagnosis of AAD. Read More

    Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group.
    Endocr Connect 2017 Nov 31;6(8):935-942. Epub 2017 Oct 31.
    Department of EndocrinologyHospital de Braga, Braga, Portugal.
    Introduction: Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI.

    Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow-up of Portuguese patients with confirmed PAI. Read More

    Sodium Chloride Supplementation Is Not Routinely Performed in the Majority of German and Austrian Infants with Classic Salt-Wasting Congenital Adrenal Hyperplasia and Has No Effect on Linear Growth and Hydrocortisone or Fludrocortisone Dose.
    Horm Res Paediatr 2018 26;89(1):7-12. Epub 2017 Oct 26.
    Pediatric Endocrinology, Department of Pediatrics, Otto von Guericke Universität Magdeburg, Magdeburg, Germany.
    Introduction: Sodium chloride supplementation in salt-wasting congenital adrenal hyperplasia (CAH) is generally recommended in infants, but its implementation in routine care is very heterogeneous.

    Objective: To evaluate oral sodium chloride supplementation, growth, and hydrocortisone and fludrocortisone dose in infants with salt-wasting CAH due to 21-hydroxylase in 311 infants from the AQUAPE CAH database.

    Results: Of 358 patients with classic CAH born between 1999 and 2015, 311 patients had salt-wasting CAH (133 females, 178 males). Read More

    Adrenal Insufficiency in Pediatric Eosinophilic Esophagitis Patients Treated with Swallowed Topical Steroids.
    Pediatr Allergy Immunol Pulmonol 2017 Sep;30(3):135-140
    Gastrointestinal Eosinophilic Diseases Program, University of Colorado School of Medicine, Aurora, Colorado.
    Swallowed topical steroids (STS) are the only effective pharmacological therapy for eosinophilic esophagitis (EoE). Thus far, studies of small populations of EoE patients have reported conflicting results in relation to adrenal insufficiency (AI). We sought to measure AI in a clinical setting in children taking STS for EoE. Read More

    A Case of Hypertensive Crisis without a Surge in Adrenal Hormones after Radiofrequency Ablation as a Treatment for Primary Hepatocellular Carcinoma.
    Korean J Gastroenterol 2017 Oct;70(4):198-201
    Department of Internal Medicine, Seoul Paik Hospital, Inje University College of Medicine, Seoul, Korea.
    Radiofrequency ablation (RFA) is a minimally invasive procedure that has been considered as a relatively safe treatment for patients with small hepatocellular carcinoma (HCC). However, RFA has been shown to be associated with complications including mechanical and thermal damage. A 74-year-old man with hepatitis C virus-associated HCC was admitted to our hospital. Read More

    Adrenal crisis and autoimmune polyglandular syndromes.
    Proc (Bayl Univ Med Cent) 2017 Oct;30(4):427-428
    Department of Internal Medicine (Freeland) and Division of Pulmonary and Critical Care Medicine (Tsai-Nguyen, Pan, Mora), Baylor University Medical Center at Dallas; and the Texas A&M Health Science Center College of Medicine (Lueking).
    We report a 67-year-old woman who presented with adrenal crisis as a manifestation of autoimmune polyglandular syndrome 2, a polygenic disorder characterized by concurrent primary adrenal insufficiency and either autoimmune thyroid disease or type 1 diabetes mellitus. Read More

    I polymorphism of the glucocorticoid receptor and adrenal crisis in primary adrenal insufficiency.
    Endocr Connect 2017 Nov 27;6(8):685-691. Epub 2017 Sep 27.
    Endocrinology in CharlottenburgBerlin, Germany
    Context: Patients with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) are at a high risk of adrenal crisis (AC). Glucocorticoid sensitivity is at least partially genetically determined by polymorphisms of the glucocorticoid receptor (GR).

    Objectives: To determine if a number of intercurrent illnesses and AC are associated with the GR gene polymorphismI in patients with PAI and CAH. Read More

    Hypertensive crisis in pregnancy due to a metamorphosing pheochromocytoma with postdelivery Cushing's syndrome.
    Gynecol Endocrinol 2018 Jan 22;34(1):20-24. Epub 2017 Sep 22.
    a Institute of Clinical Chemistry and Laboratory Medicine , University Hospital and Medical Faculty Carl Gustav Carus, Technische Universität Dresden , Dresden , Germany.
    Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing's syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. Read More

    Recognizing and managing adrenal disorders in the emergency department [digest].
    Emerg Med Pract 2017 Sep 22;19(9 Suppl Points & Pearls):S1-S2. Epub 2017 Sep 22.
    Emergency Physician, St. David's South Austin Medical Center, Austin, TX.
    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

    A novel stop mutation (p.(Gln22*)) of DAX1 (NR0B1) results in late-onset X-linked adrenal hypoplasia congenita.
    Endocrinol Diabetes Metab Case Rep 2017 4;2017. Epub 2017 Sep 4.
    Endocrinology in Charlottenburg.
    DAX1 (NR0B1) is an orphan nuclear receptor, which plays an important role in development and function of the adrenal glands and gonads. Mutations in DAX1 cause X-linked adrenal hypoplasia congenita (X-linked AHC), which is characterized by adrenal insufficiency (AI) and hypogonadotropic hypogonadism (HHG). Affected boys present with adrenal failure usually in childhood and, later in life, with delayed puberty. Read More

    Addison's Disease: A Diagnostic Dilemma.
    Mymensingh Med J 2017 Jul;26(3):671-675
    Dr Shireen Afroz, Professor, Department of Pediatric Nephrology, Bangladesh Institute of Child Health & Dhaka Shishu (Children) Hospital, Sher-e-bangla Nagar Dhaka, Bangladesh; E-mail:
    Adrenal insufficiency is a rare disease, but is life threatening when overlooked. Addison's disease may be an acquired form of adrenal insufficiency due to the destruction or dysfunction of the adrenal cortex. It affects both glucocorticoid and mineralocorticoid function. Read More

    Hospitalisation in Children with Adrenal Insufficiency and Hypopituitarism: Is There a Differential Burden between Boys and Girls and between Age Groups?
    Horm Res Paediatr 2017 12;88(5):339-346. Epub 2017 Sep 12.
    Endocrine and Metabolic Unit, Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia, Australia.
    Background/aims: To determine the burden of hospitalisation in children with adrenal insufficiency (AI)/hypopituitarism in Australia.

    Methods: A retrospective study of Australian hospitalisation data. All admissions between 2001 and 2014 for patients aged 0-19 years with a principal diagnosis of AI/hypopituitarism were included. Read More

    Clinical characteristics of adrenal crisis in adult population with and without predisposing chronic adrenal insufficiency: a retrospective cohort study.
    BMC Endocr Disord 2017 Sep 11;17(1):58. Epub 2017 Sep 11.
    Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Yoshida-Konoe-cho, Sakyo-ku, Kyoto, 606-8501, Japan.
    Background: Adrenal crisis (AC) occurs in various clinical conditions but previous epidemiological studies in AC are limited to chronic adrenal insufficiency (AI) and sepsis. The aim of this study was to investigate characteristics of AC patients, including predisposing diseases and to describe candidate risk factors for AC such as comorbidities and glucocorticoid (GC) therapy.

    Methods: We conducted a retrospective cohort study using a claims database on 7. Read More

    Mutational analysis of rare subtypes of congenital adrenal hyperplasia in a highly inbred population.
    Mol Cell Endocrinol 2018 Feb 6;461:105-111. Epub 2017 Sep 6.
    Department of Molecular Oncology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia; Department of Medicine, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia. Electronic address:
    Context: Apart from 21 Hydroxylase deficiency, other subtypes of congenital adrenal hyperplasia (CAH) are rare. We studied the clinical features and molecular genetics of a relatively large series of patients with CYP17A1, HSD3β2 and StAR deficiencies.

    Patients And Methods: We studied 21 patients including 7 patients with CYP17A1, 10 patients with HSD3β2 and 4 patients with StAR deficiencies. Read More

    An analysis of the relationship between serum cortisol and serum sodium in routine clinical patients.
    Pract Lab Med 2017 Aug 13;8:30-33. Epub 2017 Apr 13.
    Blood Sciences, Leeds General Infirmary, Leeds, UK.
    Objectives: Adrenal insufficiency is an uncommon cause of hyponatraemia that should not be overlooked due to the severe consequences of an Addisonian crisis. Using the laboratory database of a large teaching hospital, we have explored the relationship between serum sodium and serum cortisol, and have estimated the frequency of hypoadrenalism in severely hyponatraemic patients.

    Design And Methods: Data were gathered over a 23 month period from the Laboratory Information Management System at the Leeds Teaching Hospitals NHS Trust for instances where serum sodium and cortisol had been measured on a single sample. Read More

    Primary adrenal lymphoma with initial presentation concerning for bilateral adrenal pheochromocytomas.
    BMJ Case Rep 2017 Aug 22;2017. Epub 2017 Aug 22.
    Department of Endocrinology, Toowoomba Health Service, Toowoomba, Queensland, Australia.
    Primary adrenal non-Hodgkin's lymphoma is rarely encountered. Clinical presentation is non-specific with fatigue, abdominal pain and B-symptoms being more commonly reported. We report a case of primary bilateral adrenal lymphoma who initially presented with clinical features suspicious for pheochromocytoma. Read More

    Extracorporeal membrane oxygenation in pheochromocytoma-induced cardiogenic shock.
    Asian Cardiovasc Thorac Ann 2017 Jan 1:218492317727995. Epub 2017 Jan 1.
    2 Department of Surgery, Montreal Heart Institute, 25465 Université de Montréal, Montreal, Canada.
    Extracorporeal membrane oxygenation has been extensively used for cardiopulmonary support in cardiogenic shock. However, its clinical value in the management of pheochromocytoma crisis remains unclear. We report a rare case of life-threatening cardiogenic shock managed with peripheral venoarterial extracorporeal membrane oxygenation combined with endovascular left ventricular venting, in a 40-year-old female patient, in the setting of unknown adrenal pheochromocytoma. Read More

    Recognizing and managing adrenal disorders in the emergency department
    Emerg Med Pract 2017 09 1;19(9):1-24. Epub 2017 Sep 1.
    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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