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Primary adrenal insufficiency

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https://www.readbyqxmd.com/read/27903023/-adrenal-insufficiency
#1
Stephanie Burger-Stritt, Stefanie Hahner
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...
November 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27900224/perioperative-severe-hypotension-in-a-patient-with-multiple-endocrine-neoplasia-type-iib-and-bilateral-adrenalectomies-time-to-review-the-evidence-for-stress-dose-steroids
#2
Jens Tan, Acsa Zavala, Katherine B Hagan, Antoinette Van Meter, Uduak Ursula Williams, Wei Zhang, Pascal Owusu-Agyemang
Multiple endocrine neoplasia type IIb (MEN IIb) is an endocrine disorder which can manifest with tumors such as pheochromocytomas and neuromas. We present the case of a patient with MEN IIb, after bilateral adrenalectomies, on maintenance steroid replacement, who underwent a neuroma resection and developed severe hypotension. There is persistent controversy regarding the general administration of perioperative "stress dose" steroids for patients with adrenal insufficiency. While the most recent literature suggests that stress dose steroids are unnecessary for secondary adrenal insufficiency, the rarer form of primary adrenal insufficiency always requires supplemental steroids, specifically hydrocortisone, when undergoing surgical procedures...
2016: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/27899384/rifampicin-induced-adrenal-crisis-in-a-patient-with-tuberculosis-a-therapeutic-challenge
#3
Nicholas Denny, Sarika Raghunath, Praveen Bhatia, Muntasir Abdelaziz
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. He was transferred to critical care and only improved after starting hydrocortisone and stopping rifampicin. His short synACTHen test subsequently confirmed primary adrenal insufficiency, and a CT of the abdomen showed bilateral adrenal enlargement...
November 29, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27878773/ramadan-fasting-in-patients-with-adrenal-insufficiency
#4
Mélika Chihaoui, Fatma Chaker, Meriem Yazidi, Wafa Grira, Zohra Ben Amor, Ons Rejeb, Hedia Slimane
AIM: The risks of fasting during Ramadan in patients with adrenal insufficiency are unknown. The aims of this study were to evaluate these risks in such patients, to determine the risk factors and finally to set some recommendations. METHODS: It is a cross-sectional study about 180 patients with known and treated adrenal insufficiency. The patients responded to a 14-item questionnaire concerning their knowledge about the disease and fasting during the last month of Ramadan...
November 23, 2016: Endocrine
https://www.readbyqxmd.com/read/27857838/hyponatraemia-secondary-to-nivolumab-induced-primary-adrenal-failure
#5
Harris Trainer, Paul Hulse, Claire E Higham, Peter Trainer, Paul Lorigan
: Checkpoint inhibitors, such as ipilimumab and pembrolizumab, have transformed the prognosis for patients with advanced malignant melanoma and squamous non-small-cell lung cancer, and their use will only expand as experience is gained in a variety of other malignancies, for instance, renal and lymphoma. As the use of checkpoint inhibitors increases, so too will the incidence of their unique side effects, termed immune-related adverse events (irAEs), which can affect dermatological, gastrointestinal, hepatic, endocrine and other systems...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27855238/adrenal-crisis-secondary-to-bilateral-adrenal-haemorrhage-after-hemicolectomy
#6
Anthony Logaraj, Venessa H M Tsang, Shahrir Kabir, Julian C Y Ip
: 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...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27827529/a-design-thinking-approach-to-primary-ovarian-insufficiency
#7
Lisa A Martin, Alison G Porter, Vincent A Pelligrini, Peter A Schnatz, Xuezhi Jiang, Nicole Kleinstreuer, Janet E Hall, Sarah Verbiest, Jill Olmstead, Ryan Fair, Alberto Falorni, Luca Persani, Aleksandar Rajkovic, Khanjan Mehta, Lawrence M Nelson
Most clinicians are not prepared to provide integrated personal care to address all the clinical needs of women with primary ovarian insufficiency. Design thinking is an engineering methodology used to develop and evaluate novel concepts for systems operation. Here we articulate the need for a seamlessly integrated mobile health system to support genomic research as well as patient care. We also review the pathophysiology and management of primary ovarian insufficiency. Molecular understanding regarding the pathogenesis is essential to developing strategies for prevention, earlier diagnosis, and appropriate management of the disorder...
November 9, 2016: Panminerva Medica
https://www.readbyqxmd.com/read/27801983/management-of-glucocorticoid-replacement-in-adrenal-insufficiency-shows-notable-heterogeneity-data-from-the-eu-air
#8
Robert D Murray, Bertil Ekman, Sharif Uddin, Claudio Marelli, Marcus Quinkler, Pierre M J Zelissen
CONTEXT AND OBJECTIVE: Treatment for adrenal insufficiency (AI) remains suboptimal. Despite glucocorticoid replacement, patients with AI have reduced life expectancy and quality of life. This study aimed to describe the spectrum of management of glucocorticoid replacement in patients with AI enrolled in the European Adrenal Insufficiency Registry (EU-AIR). DESIGN, SETTING AND PATIENTS: EU-AIR is a prospective, multinational, multicenter, observational study initiated in August 2012 to monitor the long-term safety of glucocorticoid replacement in routine clinical practice in Germany, the Netherlands, Sweden and the UK (ClinicalTrials...
November 1, 2016: Clinical Endocrinology
https://www.readbyqxmd.com/read/27797844/bilateral-primary-adrenal-lymphoma-with-adrenal-insufficiency
#9
Aishah Ekhzaimy, Ahmad Mujamammi
Primary adrenal lymphoma is an extremely rare condition. We describe a case of bilateral adrenal lymphoma in a man aged 55 years who was admitted to our hospital. He had a 3-month history of left flank pain, nausea and vomiting with weight loss. A CT scan at a private hospital revealed bilateral large adrenal masses; the patient was referred to our centre based on these findings. He was evaluated for pheochromocytoma by an endocrinology team; however, all findings were negative. In addition, a cosyntropin stimulation test indicated adrenal insufficiency...
October 26, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27795295/adrenal-lymphoma-presentation-management-and-prognosis
#10
C Laurent, O Casasnovas, L Martin, A Chauchet, H Ghesquieres, G Aussedat, L M Fornecker, S Bologna, S Borot, K Laurent, B Bouillet, B Verges, J-M Petit
AIM: This study aimed to identify the clinical, radiological and prognostic features of primary adrenal lymphoma (PAL) in order to diagnose the disease more accurately. MATERIALS AND METHODS: A retrospective multi-centre study was conducted on the clinical, biological and radiological features as well as the treatment and overall survival outcomes in PAL. RESULTS: Between 1994 and 2014, 28 patients from five regions of eastern France were diagnosed with primary adrenal lymphoma...
October 18, 2016: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/27683652/atypical-manifestation-of-lps-responsive-beige-like-anchor-deficiency-syndrome-as-an-autoimmune-endocrine-disorder-without-enteropathy-and-immunodeficiency
#11
Shahrzad Bakhtiar, Frank Ruemmele, Fabienne Charbit-Henrion, Eva Lévy, Frédéric Rieux-Laucat, Nadine Cerf-Bensussan, Peter Bader, Ulrich Paetow
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27633717/idiopathic-intracranial-hypertension-after-surgical-treatment-of-cushing-disease-case-report-and-review-of-management-strategies
#12
Jeffrey Wagner, Cara M Fleseriu, Aly Ibrahim, Justin S Cetas
BACKGROUND: Idiopathic intracranial hypertension (IIH) in patients with Cushing disease (CD), after treatment, is rarely described, in adults. The cause is believed to be multifactorial, potentially related to a relative decrease in cortisol after surgical resection or medical treatment of a corticotroph pituitary adenoma. We investigate our center's CD database (140 surgically and 60 medically [primary or adjunct] treated patients) for cases of IIH, describe our center's experience with symptomatic IIH, and review treatment strategies in adults with CD after transsphenoidal resection...
September 12, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27623069/exploring-inpatient-hospitalizations-and-morbidity-in-patients-with-adrenal-insufficiency
#13
Paul M Stewart, Beverly Mk Biller, Claudio Marelli, Candace Gunnarsson, Michael P Ryan, Gudmundur Johannsson
CONTEXT: Patients with adrenal insufficiency (AI) (primary [PAI], secondary to pituitary disease [PIT] and congenital adrenal hyperplasia [CAH]) have reduced life expectancy; however, the underlying explanation remains unknown. OBJECTIVE: To evaluate characteristics, comorbidities and hospitalizations in AI patients. DESIGN: Retrospective Observational Setting and Population: Using a US-based national payer database comprising >108 million members, strict inclusion criteria including diagnostic codes and steroid prescription records were used to identify 10,383 adults with AI; 1,014 with PAI, 8,818 with PIT and 551 with CAH...
September 13, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27609733/bilateral-adrenal-hemorrhage-in-polycythemia-vera
#14
Shruti Bhandari, Katrina Agito, Esther I Krug
Bilateral adrenal hemorrhage (BAH) is a rare complication typically seen in critically ill patients, which can lead to acute adrenal insufficiency and death unless it is recognized promptly and treated appropriately. We describe the case of a 64-year-old man with polycythemia vera found to be unresponsive with fever, hypotension, tachycardia, and hypoglycemia. Electrocardiogram showed ST-elevation with elevated troponin, hemoglobin, prothrombin time, and partial thromboplastin time. He required aggressive ventilator and vasopressor support...
2016: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/27588307/redefined-clinical-features-and-diagnostic-criteria-in-autoimmune-polyendocrinopathy-candidiasis-ectodermal-dystrophy
#15
Elise M N Ferre, Stacey R Rose, Sergio D Rosenzweig, Peter D Burbelo, Kimberly R Romito, Julie E Niemela, Lindsey B Rosen, Timothy J Break, Wenjuan Gu, Sally Hunsberger, Sarah K Browne, Amy P Hsu, Shakuntala Rampertaap, Muthulekha Swamydas, Amanda L Collar, Heidi H Kong, Chyi-Chia Richard Lee, David Chascsa, Thomas Simcox, Angela Pham, Anamaria Bondici, Mukil Natarajan, Joseph Monsale, David E Kleiner, Martha Quezado, Ilias Alevizos, Niki M Moutsopoulos, Lynne Yockey, Cathleen Frein, Ariane Soldatos, Katherine R Calvo, Jennifer Adjemian, Morgan N Similuk, David M Lang, Kelly D Stone, Gulbu Uzel, Jeffrey B Kopp, Rachel J Bishop, Steven M Holland, Kenneth N Olivier, Thomas A Fleisher, Theo Heller, Karen K Winer, Michail S Lionakis
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare primary immunodeficiency disorder typically caused by homozygous AIRE mutations. It classically presents with chronic mucocutaneous candidiasis and autoimmunity that primarily targets endocrine tissues; hypoparathyroidism and adrenal insufficiency are most common. Developing any two of these classic triad manifestations establishes the diagnosis. Although widely recognized in Europe, where nonendocrine autoimmune manifestations are uncommon, APECED is less defined in patients from the Western Hemisphere...
August 18, 2016: JCI Insight
https://www.readbyqxmd.com/read/27540463/methimazole-induced-insulin-autoimmune-syndrome
#16
REVIEW
Nidhi Jain, Malvi Savani, Manyoo Agarwal, Dipen Kadaria
BACKGROUND: Hypoglycemia in a critical care setting is often multifactorial with iatrogenic insulin use, sulfonylurea (SU) use, sepsis, adrenal insufficiency and insulinoma among the common causes. Insulin autoimmune syndrome (IAS) is a rare cause of hypoglycemia characterized by the presence of insulin-binding autoantibodies to the sulfhydryl group-containing agents. We report a case of methimazole-induced IAS managed in the intensive care unit. CASE PRESENTATION: A 76-year-old woman with a history of primary hyperthyroidism was sent from a nursing home for unresponsiveness...
August 2016: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27536208/a-rare-case-of-addison-s-disease-hepatitis-thyreoiditis-positive-igg-anti-tissue-transglutaminase-antibodies-and-partial-iga-deficiency
#17
Marta P Baleva, Snejina Mihaylova, Petja Yankova, Iliana Atanasova, Milena Nikolova-Vlahova, Elissaveta Naumova
INTRODUCTION: Selective IgA deficiency (IgAD) is the most prevalent type of primary immune deficiencies, but partial IgA deficiency is even more common. Addison's disease is a rare condition associated with primary adrenal insufficiency due to infection or autoimmune destruction of the adrenals. The association between IgA deficiency and Addison's disease is very rare. CASE AND LABORATORY DATA: We observed a 22-year-old male patient with marked darkening of the skin, especially on the palms and areolae, jaundice on the skin and sclera, astheno-adynamia, hypotension (80/50 mm Hg), and pain in the right hypochondrium...
2016: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/27508646/-op-3a-04-adrenal-hemorrhage-following-adrenal-vein-sampling-in-primary-aldosteronism-a-six-center-experience
#18
S Monticone, F Satoh, A S Dietz, R Goupil, K Lang, F Pizzolo, R D Gordon, R Morimoto, M Reincke, M Stowasser, P Mulatero
OBJECTIVE: Adrenal vein sampling (AVS) is the only reliable means to distinguish between aldosterone-producing adenoma and bilateral adrenal hyperplasia, the two most common subtypes of primary aldosteronism (PA). To distinguish between unilateral and bilateral disease is of fundamental importance because it allows to allocate patients to the correct management: unilateral adrenalectomy for aldosterone-producing adenoma or pharmacotherapy with mineralocorticoid receptor antagonists for individuals with bilateral adrenal hyperplasia...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27507908/efficacy-of-single-serum-cortisol-reading-obtained-between-9-am-and-10-am-as-an-index-of-adrenal-function-in-children-treated-with-glucocorticoids-or-synthetic-adrenocorticotropic-hormone
#19
Masahiro Goto, Nao Shibata, Yukihiro Hasegawa
To find a simple method to screen for iatrogenic childhood adrenal insufficiency, we retrospectively examined the results of CRH stimulation tests performed 212 times on 111 subjects (68 males; age at commencement of initial treatment ranged 0.0-19.8 yr; median age, 5.8 yr). Before the commencement of this study, 97 subjects had been treated with glucocorticoids and 14 subjects with West syndrome had been treated with synthetic adrenocorticotropic hormone. Duration of the primary treatment ranged from 15 to 2150 days...
July 2016: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/27485500/congenital-primary-adrenal-insufficiency-and-selective-aldosterone-defects-presenting-as-salt-wasting-in-infancy-a-single-center-10-year-experience
#20
Carla Bizzarri, Nicole Olivini, Stefania Pedicelli, Romana Marini, Germana Giannone, Paola Cambiaso, Marco Cappa
BACKGROUND: Salt-wasting represents a relatively common cause of emergency admission in infants and may result in life-threatening complications. Neonatal kidneys show low glomerular filtration rate and immaturity of the distal nephron leading to reduced ability to concentrate urine. METHODS: A retrospective chart review was conducted for infants hospitalized in a single Institution from 1(st) January 2006 to 31(st) December 2015. The selection criterion was represented by the referral to the Endocrinology Unit for hyponatremia (serum sodium <130 mEq/L) of suspected endocrine origin at admission...
2016: Italian Journal of Pediatrics
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