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Primary adrenal insufficiency

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https://www.readbyqxmd.com/read/29156052/primary-adrenal-insufficiency-managing-mineralocorticoid-replacement-therapy
#1
Daniela Esposito, Daniela Pasquali, Gudmundur Johannsson
Context: Mineralocorticoid (MC) replacement therapy in patients with primary adrenal insufficiency (PAI) was introduced more than 60 years ago. Still, there are limited data on how MC substitution should be optimized, since MC dosing regimens have only been systematically investigated in a few studies. We review the management of current standard MC replacement therapy in PAI and its plausible impact on outcome. Evidence Acquisition: Using PubMed, we conducted a systematic review of the literature from 1939 to 2017, with the following keywords: 'adrenal insufficiency', 'mineralocorticoid deficiency', 'aldosterone', 'cardiovascular disease', 'hypertension', and 'heart failure'...
November 15, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29151085/a-novel-mutation-in-the-critical-p-box-residue-of-steroidogenic-factor-1-presenting-with-xy-sex-reversal-and-transient-adrenal-failure
#2
Anna S Orekhova, Natalia Kalinchenko, Ivan A Morozov, Evgeny V Vasilyev, Petr M Rubtsov, Ivan I Dedov, Anatoly Tiulpakov
BACKGROUND: Although the importance of steroidogenic factor-1 (SF1, NR5A1) for adrenal development is supported by numerous in vitro and in vivo studies, cases of SF1 deficiency associated with adrenal failure are exceptionally rare. The first human NR5A1 mutation was a heterozygous de novo p.G35E variant identified in a patient with disorder of sex development (DSD) 46,XY and primary adrenal insufficiency. Here we describe another association of the "classic" SF1 phenotype with a novel NR5A1 mutation affecting G35 residue...
November 17, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29146725/rare-cause-of-adrenal-insufficiency
#3
Justin Ws Hii, Michael M Page, Andrew Wesseldine
A 72-year-old man presented with weight loss, night sweats and haemoptysis and was hypotensive. CT imaging showed rapidly enlarging bilateral adrenal masses, and he was found to have primary adrenal insufficiency. An adrenal gland biopsy revealed the rare diagnosis of primary adrenal lymphoma. This unique case highlights possible rare causes of adrenal masses and adrenal insufficiency, their investigation and management principles.
November 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29116030/management-of-x-linked-adrenoleukodystrophy-in-morocco-actual-situation
#4
F Z Madani Benjelloun, Y Kriouile, D Cheillan, H Daoud-Tetouani, L Chabraoui
OBJECTIVES: X-linked adrenoleukodystrophy is a neurodegenerative disorder caused by mutations in the ABCD1 gene. Adrenomyeloneuropathy and childhood cerebral Adrenoleukodystrophy are the most common phenotypes. This paper focuses on a descriptive study of the first program of diagnosis, treatment, and follow-up of this disease in Morocco. RESULTS: We developed three protocols of X-linked Adrenoleukodystrophy management: general protocol, asymptomatic protocol, and heterozygous protocol...
November 7, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/29089364/primary-adrenal-insufficiency-in-adult-population-a-portuguese-multicentre-study-by-the-adrenal-tumours-study-group
#5
Lia Ferreira, Joao Silva, Susana Garrido, Carlos Bello, Diana Oliveira, Hélder Simões, Isabel Paiva, Joana Guimarães, Marta Ferreira, Maria Teresa Pereira, Rita Bettencourt-Silva, Ana Filipa Martins, Tiago Silva, Vera Fernandes, Maria Ferreira
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. METHODS: This multicentre retrospective study examined PAI patients in 12 Portuguese hospitals. RESULTS: We investigated 278 patients with PAI (55...
October 31, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/29067215/nivolumab-induced-hypothyroidism-and-selective-pituitary-insufficiency-in-a-patient-with-lung-adenocarcinoma-a-case-report-and-review-of-the-literature
#6
Myrto Kastrisiou, Fereniki-Lida Kostadima, Aristides Kefas, George Zarkavelis, Nikos Kapodistrias, Evangelos Ntouvelis, Dimitrios Petrakis, Alexandra Papadaki, Amalia Vassou, George Pentheroudakis
Immune checkpoint blockade including programmed cell death 1 pathway inhibition with agents such as nivolumab is gaining ground in a wide array of malignancies, so far demonstrating significantly improved survival rates even in metastatic, often multiply pretreated settings. Although targeted in nature and generally well-tolerated compared with conventional anticancer treatments, these agents are often linked to a newly emerged group of adverse reactions, referred to as immune-related adverse events, which can also affect endocrine organs...
2017: ESMO Open
https://www.readbyqxmd.com/read/29062486/cardiac-tamponade-in-a-patient-with-autoimmune-polyglandular-syndrome-type-2
#7
Andromachi Vryonidou, Stavroula A Paschou, Fotini Dimitropoulou, Panagiotis Anagnostis, Vasiliki Tzavara, Apostolos Katsivas
We describe a case of a 40-year-old woman who was admitted to the intensive care unit with a rapid onset of dyspnea and orthopnea. She presented progressive weakness, weight loss and secondary amenorrhea during last year, while intermittent fever was present for the last two months. Initial biochemical evaluation showed anemia, hyponatremia and increased C-reactive protein levels. Clinical and echocardiographic evaluation revealed cardiac tamponade, which was treated with pericardiocentesis. Pleural fluid samples were negative for malignancy, tuberculosis or bacterial infection...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29056270/primary-adrenal-insufficiency-due-to-x-linked-adrenoleukodystrophy-diagnosed-in-adulthood
#8
Guillermo Serra Soler, María Soledad Gogorza Pérez, Ana Jiménez Portilla, Vicente Pereg Macazaga
No abstract text is available yet for this article.
October 2017: Endocrinología, diabetes y nutrición
https://www.readbyqxmd.com/read/29025857/endocrine-side-effects-of-cancer-immunotherapy
#9
REVIEW
Priscilla Cukier, Fernando C Santini, Mariana Scaranti, Ana O Hoff
Immune checkpoint inhibitors have recently become a cornerstone for the treatment of different advanced cancers. These drugs, represented mainly by monoclonal antibodies anti-cytotoxic T-lymphocyte antigen 4 (CTLA-4), anti-programmed cell death protein-1 (PD-1) and anti-PD-1 ligand molecules (PD-L1 and L2), have the ability to reactivate the immune system against tumor cells, but can also trigger a myriad of autoimmune side effects, termed immune-related adverse events (irAEs). In particular, there are a number of endocrine-related irAEs...
December 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29018153/prednisolone-has-the-same-cardiovascular-risk-profile-as-hydrocortisone-in-glucocorticoid-replacement
#10
David J F Smith, Hemanth Prabhudev, Sirazum Choudhury, Karim Meeran
INTRODUCTION: Patients who need glucocorticoid replacement in both primary and secondary adrenal insufficiency (AI) have the choice of either once-daily prednisolone or thrice-daily hydrocortisone. A recent European study found no difference between prednisolone and hydrocortisone users in several markers including glucose, weight, body mass index, systolic and diastolic blood pressure and waist circumference, although an increase in cholesterol and low-density lipoprotein (LDL) was suggested in a subgroup of these patients...
November 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28989892/management-of-primary-adrenal-insufficiency-review-of-current-clinical-practice-in-a-developed-and-a-developing-country
#11
Uzma Khan, Om J Lakhani
Treatment of primary adrenal insufficiency (PAI) requires lifelong hormone replacement with glucocorticoids (GCs) and mineralocorticoids. Impaired quality of life and increased standardized mortality ratio in these patients emphasize the importance of tailoring therapy to individual needs. Role of education is paramount in improving patient compliance and in anticipating and preventing adrenal crises. Although discovery of synthetic GCs was a major breakthrough in treatment of patients with this life-threatening condition, management of PAI continues to be challenging...
September 2017: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28988646/pembrolizumab-in-advanced-soft-tissue-sarcoma-and-bone-sarcoma-sarc028-a-multicentre-two-cohort-single-arm-open-label-phase-2-trial
#12
Hussein A Tawbi, Melissa Burgess, Vanessa Bolejack, Brian A Van Tine, Scott M Schuetze, James Hu, Sandra D'Angelo, Steven Attia, Richard F Riedel, Dennis A Priebat, Sujana Movva, Lara E Davis, Scott H Okuno, Damon R Reed, John Crowley, Lisa H Butterfield, Ruth Salazar, Jaime Rodriguez-Canales, Alexander J Lazar, Ignacio I Wistuba, Laurence H Baker, Robert G Maki, Denise Reinke, Shreyaskumar Patel
BACKGROUND: Patients with advanced sarcomas have a poor prognosis and few treatment options that improve overall survival. Chemotherapy and targeted therapies offer short-lived disease control. We assessed pembrolizumab, an anti-PD-1 antibody, for safety and activity in patients with advanced soft-tissue sarcoma or bone sarcoma. METHODS: In this two-cohort, single-arm, open-label, phase 2 study, we enrolled patients with soft-tissue sarcoma or bone sarcoma from 12 academic centres in the USA that were members of the Sarcoma Alliance for Research through Collaboration (SARC)...
October 4, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28973656/incidence-of-endocrine-dysfunction-following-the-use-of-different-immune-checkpoint-inhibitor-regimens-a-systematic-review-and-meta-analysis
#13
Romualdo Barroso-Sousa, William T Barry, Ana C Garrido-Castro, F Stephen Hodi, Le Min, Ian E Krop, Sara M Tolaney
Importance: If not promptly recognized, endocrine dysfunction can be life threatening. The incidence and risk of developing such adverse events (AEs) following the use of immune checkpoint inhibitor (ICI) regimens are unknown. Objective: To compare the incidence and risk of endocrine AEs following treatment with US Food and Drug Administration-approved ICI regimens. Data Sources: A PubMed search through July 18, 2016, using the following keywords was performed: "ipilimumab," "MDX-010," "nivolumab," "BMS-963558," "pembrolizumab," "MK-3475," "atezolizumab," "MPDL3280A," and "phase...
September 28, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28966452/adrenal-crisis-and-autoimmune-polyglandular-syndromes
#14
Zachary K Freeland, Richard Lueking, Ginger Tsai-Nguyen, Thoris Pan, Adan Mora
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.
October 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28954735/bcli-polymorphism-of-the-glucocorticoid-receptor-and-adrenal-crisis-in-primary-adrenal-insufficiency
#15
Kathrin Zopf, Kathrin R Frey, Tina Kienitz, Manfred Ventz, Britta Bauer, Marcus Quinkler
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 polymorphism BclI in patients with PAI and CAH. DESIGN AND PATIENTS: This prospective, longitudinal study over 37...
November 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28940017/critical-illness-related-corticosteroid-insufficiency-circi-a-narrative-review-from-a-multispecialty-task-force-of-the-society-of-critical-care-medicine-sccm-and-the-european-society-of-intensive-care-medicine-esicm
#16
Djillali Annane, Stephen M Pastores, Wiebke Arlt, Robert A Balk, Albertus Beishuizen, Josef Briegel, Joseph Carcillo, Mirjam Christ-Crain, Mark S Cooper, Paul E Marik, Gianfranco Umberto Meduri, Keith M Olsen, Bram Rochwerg, Sophia C Rodgers, James A Russell, Greet Van den Berghe
OBJECTIVE: To provide a narrative review of the latest concepts and understanding of the pathophysiology of critical illness-related corticosteroid insufficiency (CIRCI). PARTICIPANTS: A multispecialty task force of international experts in critical care medicine and endocrinology and members of the Society of Critical Care Medicine (SCCM) and the European Society of Intensive Care Medicine (ESICM). DATA SOURCES: Medline, Database of Abstracts of Reviews of Effects (DARE), Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Database of Systematic Reviews...
September 21, 2017: Intensive Care Medicine
https://www.readbyqxmd.com/read/28938251/critical-illness-related-corticosteroid-insufficiency-circi-a-narrative-review-from-a-multispecialty-task-force-of-the-society-of-critical-care-medicine-sccm-and-the-european-society-of-intensive-care-medicine-esicm
#17
Djillali Annane, Stephen M Pastores, Wiebke Arlt, Robert A Balk, Albertus Beishuizen, Josef Briegel, Joseph Carcillo, Mirjam Christ-Crain, Mark S Cooper, Paul E Marik, Gianfranco Umberto Meduri, Keith M Olsen, Bram Rochwerg, Sophia C Rodgers, James A Russell, Greet Van den Berghe
OBJECTIVE: To provide a narrative review of the latest concepts and understanding of the pathophysiology of critical illness-related corticosteroid insufficiency (CIRCI). PARTICIPANTS: A multi-specialty task force of international experts in critical care medicine and endocrinology and members of the Society of Critical Care Medicine and the European Society of Intensive Care Medicine. DATA SOURCES: Medline, Database of Abstracts of Reviews of Effects (DARE), Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Database of Systematic Reviews...
December 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28933807/recognizing-and-managing-adrenal-disorders-in-the-emergency-department-digest
#18
Amy Cutright, Stephen Ducey, Claudia L Barthold, Jeremy Kim
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...
September 22, 2017: Emergency Medicine Practice
https://www.readbyqxmd.com/read/28924487/a-novel-stop-mutation-p-gln22-of-dax1-nr0b1-results-in-late-onset-x-linked-adrenal-hypoplasia-congenita
#19
Judith Gerards, Michael M Ritter, Elke Kaminsky, Andreas Gal, Wolfgang Hoeppner, Marcus Quinkler
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. However, patients with a late-onset form of X-linked AHC have also been described in the past years...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28911151/expanding-the-phenotypic-and-genotypic-landscape-of-autoimmune-polyendocrine-syndrome-type-1
#20
COMPARATIVE STUDY
Elizaveta M Orlova, Leila S Sozaeva, Maria A Kareva, Bergithe E Oftedal, Anette S B Wolff, Lars Breivik, Ekaterina Y Zakharova, Olga N Ivanova, Olle Kämpe, Ivan I Dedov, Per M Knappskog, Valentina A Peterkova, Eystein S Husebye
Context: Autoimmune polyendocrine syndrome type 1 (APS-1) is a rare monogenic autoimmune disease caused by mutations in the autoimmune regulator (AIRE) gene and characterized by chronic mucocutaneous candidiasis, hypoparathyroidism, and primary adrenal insufficiency. Comprehensive characterizations of large patient cohorts are rare. Objective: To perform an extensive clinical, immunological, and genetic characterization of a large nationwide Russian APS-1 cohort...
September 1, 2017: Journal of Clinical Endocrinology and Metabolism
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