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    1 OF 28

    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

    Clinical Characteristics of Patients with Adrenal Insufficiency in a General Hospital.
    Endocrinol Metab (Seoul) 2017 Feb 28. Epub 2017 Feb 28.
    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

    [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

    Nivolumab induced myxedema crisis.
    J Immunother Cancer 2017 21;5:13. Epub 2017 Feb 21.
    grid.416413.5Department 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

    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

    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

    Two Moroccan Sisters Presenting with a Severe Salt-Wasting Form of Congenital Adrenal Hyperplasia but Normal Female Genitalia.
    Sex Dev 2017 Feb 11. 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

    [Recommendations for the diagnosis and treatment of classic forms of 21-hydroxylase-deficient congenital adrenal hyperplasia].
    An Pediatr (Barc) 2017 Feb 1. 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

    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

    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

    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

    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

    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

    Effect of Exercise on Ovulation: A Systematic Review.
    Sports Med 2016 Dec 29. Epub 2016 Dec 29.
    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

    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

    Adrenal crises: perspectives and research directions.
    Endocrine 2017 Feb 19;55(2):336-345. Epub 2016 Dec 19.
    Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden.
    Adrenal crises are life-threatening complications of adrenal insufficiency. These events have an estimated incidence of between 5 and 10  adrenal crises/100 patient years and are responsible for some of the increased morbidity and excess mortality experienced by patients with adrenal insufficiency. Treatment involves urgent administration of IV/IM hydrocortisone and IV fluids. Read More

    Anesthetic Management of Clinically Silent Familial Pheochromocytoma with MEN 2A: A Report of Four Cases.
    Indian J Surg 2016 Oct 15;78(5):414-417. Epub 2016 Aug 15.
    Department of Anesthesia, V.M.M.C. and Safdarjung Hospital, A7/14 Mianwali Nagar Paschim Vihar, New Delhi, 110087 India.
    Familial pheochromocytomas are commonly associated with multiple endocrine neoplasia type 2 (MEN 2) syndrome. Majority of the patients present with normal clinical and biochemical parameters in the preoperative period, the incidence of hypertension being only 50 %. Even though patients may be clinically asymptomatic, surveillance and proper preoperative evaluation is important, as surgery for associated tumors may precipitate a hypertensive crisis and result in severe complications. Read More

    Conduct protocol in emergency: Acute adrenal insufficiency.
    Rev Assoc Med Bras (1992) 2016 Nov;62(8):728-734
    Degree in Endocrinology and Metabology from Sociedade Brasileira de Endocrinologia e Metabologia (SBEM). Assistant Physician at the Internal Medicine Service of Hospital de Base. Researcher at Centro Integrado de Pesquisa (CIP), Hospital de Base, São José do Rio Preto. Endocrinology Coordinator of the Specialties Outpatient Clinic (AME), São José do Rio Preto, SP, Brazil.
    Introduction:: Acute adrenal insufficiency or addisonian crisis is a rare comorbidity in emergency; however, if not properly diagnosed and treated, it may progress unfavorably.

    Objective:: To alert all health professionals about the diagnosis and correct treatment of this complication.

    Method:: We performed an extensive search of the medical literature using specific search tools, retrieving 20 articles on the topic. Read More

    Hypertensive Crisis From the Aquamantys Bipolar Sealing System.
    A A Case Rep 2016 Dec;7(12):270-271
    From the Department of Anesthesiology, University of Florida College of Medicine, Gainesville, Florida.
    Hypertension is a common occurrence during general anesthesia. Apart from pathological causes of hypertension, it is rarely extreme enough to be classified as a hypertensive crisis (systolic blood pressure >180 mm Hg or diastolic blood pressure >120 mm Hg). There is literature concerning the unintentional electrocauterization of the adrenal gland leading to hypertensive crisis, but to date, no reports have been made of adrenal stimulation from the use of an Aquamantys for hemostasis. Read More

    [Adrenal Insufficiency].
    Dtsch Med Wochenschr 2016 Nov 30;141(24):1740-1742. Epub 2016 Nov 30.
    Even under established replacement therapy, chronic adrenal insufficiency is associated with an impairment in daily life activities and a higher number of sick-days. In patients with individually adjusted glucocorticoid doses (avoiding over-replacement) the frequency of glucocorticoid side effects is low. Besides the standard treatment with conventional hydrocortisone, new formulas aiming at more adjusted physiological circadian cortisol profiles or improved individual dosing have been evaluated in recent clinical trials. Read More

    Rifampicin-induced adrenal crisis in a patient with tuberculosis: a therapeutic challenge.
    BMJ Case Rep 2016 Nov 29;2016. Epub 2016 Nov 29.
    Department of Respiratory Medicine, Tameside General Hospital, Ashton-under-Lyne, UK.
    A 55-year-old Indian man presented with productive cough and a large left pleural effusion. Pleural fluid culture grew Mycobacterium tuberculosis, and he was started on antituberculosis therapy. One week later, the patient presented to hospital with drowsiness, dehydration and hypotension. Read More

    Adrenal crisis secondary to bilateral adrenal haemorrhage after hemicolectomy.
    Endocrinol Diabetes Metab Case Rep 2016 25;2016. Epub 2016 Oct 25.
    Department of Surgery, Royal North Shore Hospital, St Leonards, New South Wales, Australia; The University of Sydney, Sydney, Australia.
    Adrenal haemorrhage is a rare cause of adrenal crisis, which requires rapid diagnosis, prompt initiation of parenteral hydrocortisone and haemodynamic monitoring to avoid hypotensive crises. We herein describe a case of bilateral adrenal haemorrhage after hemicolectomy in a 93-year-old female with high-grade colonic adenocarcinoma. This patient's post-operative recovery was complicated by an acute hypotensive episode, hypoglycaemia and syncope, and subsequent computed tomography (CT) scan of the abdomen revealed bilateral adrenal haemorrhage. Read More

    Novel SCC mutation in a patient of Mexican descent with sex reversal, salt-losing crisis and adrenal failure.
    Endocrinol Diabetes Metab Case Rep 2016 4;2016. Epub 2016 Oct 4.
    Laboratory of Biochemistry, Biomedical Sciences, Mercer University School of Medicine, Savannah, Georgia, USA; Anderson Cancer Institute, Memorial University Medical Center, Savannah, Georgia, USA.
    Congenital adrenal hyperplasia (CAH) is caused by mutations in cytochrome P450 side chain cleavage enzyme (CYP11A1 and old name, SCC). Errors in cholesterol side chain cleavage by the mitochondrial resident CYP11A1 results in an inadequate amount of pregnenolone production. This study was performed to evaluate the cause of salt-losing crisis and possible adrenal failure in a pediatric patient whose mother had a history of two previous stillbirths and loss of another baby within a week of birth. Read More

    Short-Term Outcomes and Safety of Computed Tomography-Guided Percutaneous Microwave Ablation of Solitary Adrenal Metastasis from Lung Cancer: A Multi-Center Retrospective Study.
    Korean J Radiol 2016 Nov-Dec;17(6):864-873. Epub 2016 Oct 31.
    Department of Oncology, Shandong Provincial Hospital Affiliated with Shandong University, Jinan, Shandong Province 250021, China.
    Objective: To retrospectively evaluate the short-term outcomes and safety of computed tomography (CT)-guided percutaneous microwave ablation (MWA) of solitary adrenal metastasis from lung cancer.

    Materials And Methods: From May 2010 to April 2014, 31 patients with unilateral adrenal metastasis from lung cancer who were treated with CT-guided percutaneous MWA were enrolled. This study was conducted with approval from local Institutional Review Board. Read More

    Pheochromocytoma Multisystem Crisis Triggered by Glucocorticoid Administration and Aggravated by Citrate Dialysis.
    A A Case Rep 2017 Feb;8(3):58-60
    From the Departments of *Anaesthesiology and †Hepato-Pancreato-Biliary and Transplant Surgery, Singapore General Hospital, Singapore.
    Pheochromocytoma multisystem crisis is the most severe presentation of pheochromocytoma. We report on a 68-year-old survivor of pheochromocytoma multisystem crisis, whose clinical course was triggered inadvertently by a short innocuous course of oral dexamethasone to suppress inflammation and swelling after a left orbital floor fracture repair. He presented first with severe epigastric pain and headache, and subsequently experienced insults to neurological, cardiac, respiratory, hepatobiliary, renal, and immune system in his prolonged intensive care unit stay. Read More

    MANAGEMENT OF ENDOCRINE DISEASE: Regenerative therapies in autoimmune Addison's disease.
    Eur J Endocrinol 2017 Mar 3;176(3):R123-R135. Epub 2016 Nov 3.
    Institute of Genetic MedicineInternational Centre for Life, Centre Parkway, Newcastle upon Tyne, UK.
    The treatment for autoimmune Addison's disease (AAD) has remained virtually unchanged in the last 60 years. Most patients have symptoms that are relatively well controlled with exogenous steroid replacement, but there may be persistent symptoms, recurrent adrenal crisis and poor quality of life, despite good compliance with optimal current treatments. Treatment with conventional exogenous steroid therapy is also associated with premature mortality, increased cardiovascular risk and complications related to excessive steroid replacement. Read More

    Adrenal crisis while on high-dose steroid treatment: what rheumatologist should consider?
    Rheumatol Int 2017 Apr 1;37(4):657-662. Epub 2016 Nov 1.
    Division of Rheumatology, Department of Internal Medicine, School of Medicine, Eskişehir Osmangazi University, 26480, Eskişehir, Turkey.
    Steroid treatment is commonly recommended for autoimmune disorders in rheumatology practice. While adrenal crisis may occur upon existence of an inducing factor in patients with known or unknown adrenal insufficiency as well as in those with a suppressed hypothalamic-pituitary-adrenal (HPA) axis due to chronic steroid use, addisonian crisis rarely develops in patients on supraphysiological doses of steroid and, when emerged, it might be very difficult to recognize. Here, we present a patient who developed adrenal crisis while receiving high-dose methylprednisolone treatment due to retroperitoneal fibrosis and we also discuss possible mechanisms with a brief literature review. Read More

    Uniparental Isodisomy of Chromosome 1 Unmasking an Autosomal Recessive 3-Beta Hydroxysteroid Dehydrogenase Type II-Related Congenital Adrenal Hyperplasia.
    J Clin Res Pediatr Endocrinol 2017 Mar 31;9(1):70-73. Epub 2016 Oct 31.
    The Children's Hospital of Philadelphia, Division of Neonatal and Perinatal Medicine, Philadelphia, USA Phone: +1 319 855-9093 E-mail:
    Steroid 3-beta hydroxysteroid dehydrogenase type II (3β-HSD2) deficiency is a rare autosomal recessive form of congenital adrenal hyperplasia (CAH). We report the genetic basis of 3β-HSD2 deficiency arising from uniparental isodisomy (UPD) of chromosome 1. We describe a term undervirilized male whose newborn screen indicated borderline CAH. Read More

    Adrenal suppression in children treated with swallowed fluticasone and oral viscous budesonide for eosinophilic esophagitis.
    Allergy Asthma Clin Immunol 2016 10;12:49. Epub 2016 Oct 10.
    Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON Canada ; Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON Canada ; Department of Pediatrics, University of Ottawa, Ottawa, ON Canada.
    Background: Adrenal suppression (AS), a glucocorticoid (GC) side effect associated with significant morbidity, is well described related to inhaled corticosteroid therapy for asthma. Swallowed topical glucocorticoid therapy is the main pharmacotherapy treatment for eosinophilic esophagitis (EoE) and therefore children with EoE are potentially at increased risk of AS.

    Methods: In this prospective cohort study, we included children and youth <18 years diagnosed with EoE and treated with swallowed fluticasone or oral viscous budesonide for more than 1 month. Read More

    Pheochromocytoma presenting as a mimic of acute coronary syndrome.
    Acute Med 2016 ;15(3):145-148
    King's College Hospital, Denmark Hill, London.
    Chest pain with elevated serum troponin is a common clinical presentation and is normally managed as suspected myocardial infarction or acute coronary syndrome (ACS). We report a 49 year old man who presented with central chest pain sweating and breathlessness. He had a significantly elevated serum troponin I level and a subsequent angiogram showed near normal coronary arteries. Read More

    Delayed diagnosis with autoimmune polyglandular syndrome type 2 causing acute adrenal crisis: A case report.
    Medicine (Baltimore) 2016 Oct;95(42):e5062
    Key laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
    Background: Autoimmune polyglandular syndrome type 2 (APS-2), also known as Schmidt's syndrome, is an uncommon disorder characterized by the coexistence of Addison's disease with thyroid autoimmune disease and/or type 1 diabetes mellitus. Addison's disease as the obligatory component is potentially life-threatening. Unfortunately, the delayed diagnosis of Addison's disease is common owing to its rarity and the nonspecific clinical manifestation. Read More

    NFKB2 mutation in common variable immunodeficiency and isolated adrenocorticotropic hormone deficiency: A case report and review of literature.
    Medicine (Baltimore) 2016 Oct;95(40):e5081
    aDepartment of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health bDepartment of Internal Medicine cDepartment of Pediatrics dDepartment of Infectious Diseases eDepartment of Dermatology, Peking Union Medical College Hospital, Beijing, P.R. China.
    Background: Common variable immunodeficiency (CVID) with central adrenal insufficiency is a recently defined clinical syndrome caused by mutations in the nuclear factor kappa-B subunit 2 (NFKB2) gene. We present the first case of NFKB2 mutation in Asian population.

    Methods And Results: An 18-year-old Chinese female with adrenocorticotropic hormone (ACTH) deficiency was admitted due to adrenal crisis and pneumonia. Read More

    Histopathological analysis of spontaneous large necrosis of adrenal pheochromocytoma manifested as acute attacks of alternating hypertension and hypotension: a case report.
    J Med Case Rep 2016 Oct 12;10(1):279. Epub 2016 Oct 12.
    Department of Internal Medicine, Ojiya General Hospital, Niigata, Japan.
    Background: Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors. Hypertension secondary to pheochromocytoma is often paroxysmal, and patients occasionally present with sudden attacks of alternating hypertension and hypotension. Spontaneous, extensive necrosis within the tumor that is associated with catecholamine crisis is an infrequent complication of adrenal pheochromocytoma, but its pathogenesis remains unclear. Read More

    Frequency and Duration of Adrenal Suppression Following Glucocorticoid Therapy in Children with Rheumatic Diseases.
    Arthritis Care Res (Hoboken) 2016 Oct 9. Epub 2016 Oct 9.
    Department of Pediatrics, University of Ottawa, Ottawa, Ontario, Canada.
    Objective: Adrenal suppression (AS), a glucocorticoid (GC) side effect with potentially significant morbidity, is poorly understood. The purpose of our study was to determine frequency, duration and predictors of AS following a gradual taper of GC in children with rheumatologic conditions.

    Methods: Prospective observational cohort study was conducted. Read More

    Alacrima as a Harbinger of Adrenal Insufficiency in a Child with Allgrove (AAA) Syndrome.
    Am J Case Rep 2016 Oct 4;17:703-706. Epub 2016 Oct 4.
    Department of Genetics, New York Methodist Hospital, Brooklyn, NY, USA.
    BACKGROUND Allgrove syndrome, or triple "A" syndrome (3A syndrome), is a rare autosomal recessive syndrome with variable phenotype, and an estimated prevalence of 1 per 1,000,000 individuals. Patients usually display the triad of achalasia, alacrima, and adrenocorticotropin (ACTH) insensitive adrenal insufficiency, though the presentation is inconsistent. CASE REPORT Here, the authors report a case of Allgrove syndrome in a pediatric patient with delayed diagnosis in order to raise awareness of this potentially fatal disease as a differential diagnosis of alacrima. Read More

    Pheochromocytoma presenting as an acute coronary syndrome complicated by acute heart failure: The challenge of a great mimic.
    J Saudi Heart Assoc 2016 Oct 10;28(4):278-82. Epub 2016 Feb 10.
    Unità Operativa di Cardiologia, Ospedale SS Annunzia, Sassari, aItaly.
    Pheochromocytoma is a rare neuroendocrine tumor with a highly variable clinical presentation. The serious and potentially lethal cardiovascular complications of these tumors are related to the effects of secreted catecholamines. We describe a case of a 50-year-old woman urgently admitted to our hospital because of symptoms and clinical and instrumental findings consistent with an acute coronary syndrome complicated by acute heart failure. Read More

    GLUCOCORTICOID REPLACEMENT REGIMENS IN CHRONIC ADRENAL INSUFFICIENCY: A SYSTEMATIC REVIEW AND META-ANALYSIS.
    Endocr Pract 2017 Jan 15;23(1):17-31. Epub 2016 Sep 15.
    Objective: Various glucocorticoid (GC) regimens have been used in the treatment of patients with adrenal insufficiency, yet the differences between such regimens on health outcomes are unclear. We performed a systematic review and meta-analysis to compare the effects of GC regimens on quality of life (QoL), bone density, incidence of adrenal crisis, and death. In pediatric studies, we also searched for final adult height. Read More

    [Analysis of phenotypes and genotypes in 66 patients with 21-hydroxylase deficiency identified by neonatal screening].
    Zhonghua Er Ke Za Zhi 2016 Sep;54(9):679-85
    Department of Pediatric Endocrinology/Genetics, Xin Hua Hospital, Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai Institute for Pediatric Research, Shanghai 200092, China.
    Objective: To analyze the phenotype-genotype correlation of 21-hydroxylase deficiency (21-OHD) patients found by neonatal screening, and to investigate the characteristics of gene frequency of these patients.

    Method: Clinical and biochemical data of 66 21-OHD patients diagnosed by neonatal screening in department of pediatric endocrinology and genetics and neonatal screening center of Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from 2009 to 2014 were retrospectively analyzed. Point mutations of CYP21A2 gene were analyzed by Sanger sequencing, and large gene deletions were detected by multiplex ligation-dependent probe amplification (MLPA). Read More

    Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult.
    IDCases 2016 16;6:1-4. Epub 2016 Aug 16.
    Division of Infectious Disease, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA; Department of Medicine, Alameda Health System, 1411 E 31st St, Oakland, CA 94602, USA.
    Asplenic patients are at increased risk for sepsis and fulminant infection. Sepsis in these patients is typically secondary to encapsulated bacteria, with Streptococcus pneumoniae being the most frequent pathogen. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). Read More

    Triple A to triple S: From diagnosis, to anesthetic management of Allgrove syndrome.
    J Clin Anesth 2016 Sep 29;33:141-3. Epub 2016 Apr 29.
    Department of Anaesthesiology and Critical Care, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
    Introduction: Allgrove syndrome (AS) is a rare autosomal recessive disorder characterized by achalasia cardia, alacrimia, and adrenocorticotropic hormone-resistant adrenal insufficiency which is sometimes associated with autonomic dysfunction. It has also been referred to as the triple A syndrome in view of the cardinal symptoms described above. First described by Allgrove et al in 1978, the disorder usually presents mostly during the first decade of life. Read More

    [The Use of Arginine Vasopressin and PhosphodiesteraseIII Inhibitor for Circulatory Shock after the Resection of a Massive Adrenal Pheochromocytoma].
    Masui 2016 Jun;65(6):624-7
    A 72-year-old man developed hypertensive crisis five month previously, and was diagnosed with massive adrenal pheochromocytoma, with a diameter of 14.5 cm. Preoperative echocardiography revealed normal cardiac function. Read More

    Darkened skin, vomiting, and salt cravings in a teenager · Dx?
    J Fam Pract 2016 Jun;65(6):E1-3
    Phoenix Children's Hospital, AZ, USA. Email:
    Acute adrenal insufficiency crisis usually occurs after a prolonged period of nonspecific complaints due to a loss of both glucocorticoids and mineralocorticoids; by the time overt symptoms occur, 90% of the adrenal gland may be destroyed. Patients (such as ours) may present with symptoms such as abdominal pain, weakness, vomiting, fever, and decreased responsiveness. Read More

    Pheochromocytoma of the pancreas: A report of three cases and a literature review.
    Oncol Lett 2016 Aug 15;12(2):959-962. Epub 2016 Jun 15.
    Department of Pancreatic Surgery, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China.
    Pheochromocytoma is primarily derived from the adrenal medulla. The majority of extra-adrenal pheochromocytoma cases occur in the superior para-aortic region and para-adrenal area. However, pheochromocytoma originating from the pancreas is rare. Read More

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