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Primary adrenal insufficiency

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https://www.readbyqxmd.com/read/28223394/primary-adrenal-insufficiency-is-associated-with-impaired-natural-killer-cell-function-a-potential-link-to-increased-mortality
#1
Irina Bancos, Jon Hazeldine, Vasileios Chortis, Peter Hampson, Angela E Taylor, Janet M Lord, Wiebke Arlt
OBJECTIVE: Mortality in patients with primary adrenal insufficiency (PAI) is significantly increased, with respiratory infections as a major cause of death. Moreover, patients with PAI report an increased rate of non-fatal infections. Neutrophils and natural killer (NK) cells are innate immune cells that provide frontline protection against invading pathogens. Thus, we compared the function and phenotype of NK cells and neutrophils isolated from PAI patients and healthy controls to ascertain whether altered innate immune responses could be a contributory factor for the increased susceptibility of PAI patients to infection...
April 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28190868/self-management-in-adrenal-insufficiency-towards-a-better-understanding
#2
Daniela Kampmeyer, Christian Stefan Haas, Heiner Moenig, Birgit Harbeck
Patients with adrenal insufficiency (AI) require life-long glucocorticoid (GC) replacement treatment and dose adjustment in stress situations to prevent life-threatening adrenal crises. Herein this study we evaluated the patients' healthcare situation and their knowledge on AI, comparing various aspects to a prior survey in 209 physicians. Using a questionnaire, we conducted a comprehensive survey among 33 AI patients who were treated at the endocrine outpatient clinics of two University Hospitals in Germany...
February 10, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28165343/sphingosine-1-phosphate-lyase-mutations-cause-primary-adrenal-insufficiency-and-steroid-resistant-nephrotic-syndrome
#3
Rathi Prasad, Irene Hadjidemetriou, Avinaash Maharaj, Eirini Meimaridou, Federica Buonocore, Moin Saleem, Jenny Hurcombe, Agnieszka Bierzynska, Eliana Barbagelata, Ignacio Bergadá, Hamilton Cassinelli, Urmi Das, Ruth Krone, Bulent Hacihamdioglu, Erkan Sari, Ediz Yesilkaya, Helen L Storr, Maria Clemente, Monica Fernandez-Cancio, Nuria Camats, Nanik Ram, John C Achermann, Paul P Van Veldhoven, Leonardo Guasti, Debora Braslavsky, Tulay Guran, Louise A Metherell
Primary adrenal insufficiency is life threatening and can present alone or in combination with other comorbidities. Here, we have described a primary adrenal insufficiency syndrome and steroid-resistant nephrotic syndrome caused by loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1). SGPL1 executes the final decisive step of the sphingolipid breakdown pathway, mediating the irreversible cleavage of the lipid-signaling molecule sphingosine-1-phosphate (S1P). Mutations in other upstream components of the pathway lead to harmful accumulation of lysosomal sphingolipid species, which are associated with a series of conditions known as the sphingolipidoses...
February 6, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28143809/non-hodgkin-s-lymphoma-involving-a-femur-bone-and-bilateral-adrenal-glands-alone-with-adrenal-insufficiency
#4
Yoshihito Iwahara, Tsutomu Shinohara, Keishi Naruse, Yukihisa Komatsu
Primary bone lymphoma and primary adrenal lymphoma are rare clinicopathological entities of non-Hodgkin's lymphoma (NHL). We present the first case of diffuse large B-cell lymphoma with the involvement of a single bone and both adrenal glands alone with adrenal insufficiency. As primary extranodal NHL may have other unusual extranodal lesions, which may present unexplained clinical findings, patients with primary extranodal NHL require careful systemic examination, even when lymphadenopathy is absent.
January 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28130352/clinical-features-and-practice-patterns-of-treatment-for-adrenal-crisis-a-nationwide-cross-sectional-study-in-japan
#5
Yosuke Ono, Sachiko Ono, Hideo Yasunaga, Hiroki Matsui, Kiyohide Fushimi, Yuji Tanaka
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. METHODS: We conducted a cross-sectional study of patients who received intravenous glucocorticoids for adrenal crisis at admission from 1 July 2007 to 31 March 2014, using a national inpatient database in Japan...
March 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28033660/wide-spectrum-of-nr5a1-related-phenotypes-in-46-xy-and-46-xx-individuals
#6
REVIEW
Sorahia Domenice, Aline Zamboni Machado, Frederico Moraes Ferreira, Bruno Ferraz-de-Souza, Antonio Marcondes Lerario, Lin Lin, Mirian Yumie Nishi, Nathalia Lisboa Gomes, Thatiana Evelin da Silva, Rosana Barbosa Silva, Rafaela Vieira Correa, Luciana Ribeiro Montenegro, Amanda Narciso, Elaine Maria Frade Costa, John C Achermann, Berenice Bilharinho Mendonca
Steroidogenic factor 1 (NR5A1, SF-1, Ad4BP) is a transcriptional regulator of genes involved in adrenal and gonadal development and function. Mutations in NR5A1 have been among the most frequently identified genetic causes of gonadal development disorders and are associated with a wide phenotypic spectrum. In 46,XY individuals, NR5A1-related phenotypes may range from disorders of sex development (DSD) to oligo/azoospermia, and in 46,XX individuals, from 46,XX ovotesticular and testicular DSD to primary ovarian insufficiency (POI)...
December 2016: Birth Defects Research. Part C, Embryo Today: Reviews
https://www.readbyqxmd.com/read/27983741/psoriatic-arthritis-but-not-psoriasis-is-associated-with-primary-adrenal-insufficiency
#7
Peter Jensen, Alexander Egeberg, Jacob P Thyssen, Gunnar Gislason, Lone Skov
is missing (Short communication).
December 16, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27932067/nivolumab-plus-ipilimumab-as-first-line-treatment-for-advanced-non-small-cell-lung-cancer-checkmate-012-results-of-an-open-label-phase-1-multicohort-study
#8
Matthew D Hellmann, Naiyer A Rizvi, Jonathan W Goldman, Scott N Gettinger, Hossein Borghaei, Julie R Brahmer, Neal E Ready, David E Gerber, Laura Q Chow, Rosalyn A Juergens, Frances A Shepherd, Scott A Laurie, William J Geese, Shruti Agrawal, Tina C Young, Xuemei Li, Scott J Antonia
BACKGROUND: Nivolumab has shown improved survival in the treatment of advanced non-small-cell lung cancer (NSCLC) previously treated with chemotherapy. We assessed the safety and activity of combination nivolumab plus ipilimumab as first-line therapy for NSCLC. METHODS: The open-label, phase 1, multicohort study (CheckMate 012) cohorts reported here were enrolled at eight US academic centres. Eligible patients were aged 18 years or older with histologically or cytologically confirmed recurrent stage IIIb or stage IV, chemotherapy-naive NSCLC...
January 2017: Lancet Oncology
https://www.readbyqxmd.com/read/27919009/successful-percutaneous-ct-guided-microwave-ablation-of-adrenal-gland-for-ectopic-cushing-syndrome
#9
Rebecca Zener, Andrew Zaleski, Stan H Van Uum, Daryl K Gray, Amol Mujoomdar
Adrenocorticotropic hormone production by pancreatic neuroendocrine tumor (PNET) is rare and results in hyperstimulation of the adrenal gland to produce ectopic Cushing syndrome. Our case showcases the safety and effectiveness of percutaneous CT-guided microwave ablation of the adrenal gland in a 49-year-old female with PNET and hepatic metastases who presented with ectopic Cushing syndrome despite surgical resection of the primary pancreatic tumor and left adrenal gland. Prior to ablation, the right adrenal gland measured 4...
November 29, 2016: Clinical Imaging
https://www.readbyqxmd.com/read/27903023/-adrenal-insufficiency
#10
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
#11
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
#12
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
#13
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...
January 2017: Endocrine
https://www.readbyqxmd.com/read/27857838/hyponatraemia-secondary-to-nivolumab-induced-primary-adrenal-failure
#14
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
#15
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
#16
Lisa A Martin, Alison G Porter, Vincent A Pelligrini, Peter F 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...
March 2017: Panminerva Medica
https://www.readbyqxmd.com/read/27801983/management-of-glucocorticoid-replacement-in-adrenal-insufficiency-shows-notable-heterogeneity-data-from-the-eu-air
#17
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, multicentre, 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...
March 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/27797844/bilateral-primary-adrenal-lymphoma-with-adrenal-insufficiency
#18
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
#19
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
#20
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
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