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Primary adrenal insufficiency

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https://www.readbyqxmd.com/read/28423475/primary-lymphocytic-hypophysitis-clinical-characteristics-and-treatment-of-50-cases-in-a-single-centre-in-china-over-18-years
#1
Shuchang Wang, Linjie Wang, Yong Yao, Feng Feng, Hongbo Yang, Zhiyong Liang, Kan Deng, Hui You, Jian Sun, Bing Xing, Zimeng Jin, Renzhi Wang, Hui Pan, Huijuan Zhu
OBJECTIVE: Primary lymphocytic hypophysitis (LYH) is rare and it is often evaluated in a small case series. This study aimed to describe the diagnosis and treatment of primary LYH in a larger cohort. DESIGN: A retrospective study of the diagnosis and treatment of primary LYH was conducted at Peking Union Medical College Hospital from 1999 to 2016. PATIENTS: Fifty patients (28 histologically-diagnosed and 22 clinically-diagnosed) were eligible for inclusion...
April 19, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28422753/steroid-metabolome-analysis-reveals-prevalent-glucocorticoid-excess-in-primary-aldosteronism
#2
Wiebke Arlt, Katharina Lang, Alice J Sitch, Anna S Dietz, Yara Rhayem, Irina Bancos, Annette Feuchtinger, Vasileios Chortis, Lorna C Gilligan, Philippe Ludwig, Anna Riester, Evelyn Asbach, Beverly A Hughes, Donna M O'Neil, Martin Bidlingmaier, Jeremy W Tomlinson, Zaki K Hassan-Smith, D Aled Rees, Christian Adolf, Stefanie Hahner, Marcus Quinkler, Tanja Dekkers, Jaap Deinum, Michael Biehl, Brian G Keevil, Cedric H L Shackleton, Jonathan J Deeks, Axel K Walch, Felix Beuschlein, Martin Reincke
BACKGROUND: Adrenal aldosterone excess is the most common cause of secondary hypertension and is associated with increased cardiovascular morbidity. However, adverse metabolic risk in primary aldosteronism extends beyond hypertension, with increased rates of insulin resistance, type 2 diabetes, and osteoporosis, which cannot be easily explained by aldosterone excess. METHODS: We performed mass spectrometry-based analysis of a 24-hour urine steroid metabolome in 174 newly diagnosed patients with primary aldosteronism (103 unilateral adenomas, 71 bilateral adrenal hyperplasias) in comparison to 162 healthy controls, 56 patients with endocrine inactive adrenal adenoma, 104 patients with mild subclinical, and 47 with clinically overt adrenal cortisol excess...
April 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28398936/endogenous-glucocorticoid-response-to-single-dose-dexamethasone-for-croup-in-children-a-pharmacodynamic-study
#3
Natasha Gill, Natalie Sirizzotti, David Johnson, Gary Joubert, Andrew S Kucey, Alvin Tieu, Brad L Urquhart, Melanie Columbus, Rodrick Lim, Michael Rieder, Shruti Mehrotra, Emily D Hartjes, Naveen Poonai
OBJECTIVES: Dexamethasone is associated with adrenal insufficiency in adults and children with chronic disease. This association has not been studied after single-dose oral dexamethasone, the standard of care for children with croup. We hypothesized that single-dose oral dexamethasone in children with croup is associated with a transient decrease in endogenous glucocorticoids. METHODS: We conducted a prospective, 2-arm, pharmacodynamic study of single-dose oral dexamethasone 0...
April 11, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28377801/current-best-practice-in-the-management-of-patients-after-pituitary-surgery
#4
REVIEW
Alessandro Prete, Salvatore Maria Corsello, Roberto Salvatori
Sellar and parasellar masses are a common finding, and most of them are treated surgically via transsphenoidal approach. This type of surgery has revolutionized the approach to several hypothalamic-pituitary diseases and is usually effective, and well-tolerated by the patient. However, given the complex anatomy and high density of glandular, neurological and vascular structures in a confined space, transsphenoidal surgery harbors a substantial risk of complications. Hypopituitarism is one of the most frequent sequelae, with central adrenal insufficiency being the deficit that requires a timely diagnosis and treatment...
March 2017: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28339821/russell-s-viper-envenomation-acute-hypopituitarism-or-acute-primary-adrenal-insufficiency
#5
A Krishnamurthy, V Y Vishnu
No abstract text is available yet for this article.
February 28, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28339808/russell-s-viper-envenomation-acute-hypopituitarism-or-acute-primary-adrenal-insufficiency
#6
Srinivas Rajagopala, Molly Mary Thabah
No abstract text is available yet for this article.
February 28, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28329633/comorbidities-of-hospitalized-pemphigus-patients-in-the-united-states
#7
Derek Y Hsu Y Hsu
We sought to determine the comorbid health conditions and inpatient mortality associated with pemphigus in a U.S. inpatient cohort. The 2002-2012 Nationwide Inpatient Sample, which contains a representative 20% stratified sample of all inpatient hospitalizations in the US, was analyzed. Comorbidities were determined through ICD-9-CM codes. Survey weighted multivariate logistic regression models controlling for demographic factors were constructed to determine the association of pemphigus with various comorbidities...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28292927/long-term-safety-of-once-daily-dual-release-hydrocortisone-in-patients-with-adrenal-insufficiency-a-phase-3b-open-label-extension-study
#8
Anna G Nilsson, Ragnhildur Bergthorsdottir, Pia Burman, Per Dahlqvist, Bertil Ekman, Britt Edén Engstrom, Oskar Ragnarsson, Stanko Skrtic, Jeanette Wahlberg, Heinrich Achenbach, Sharif Uddin, Claudio Marelli, Gudmundur Johannsson
OBJECTIVE: To investigate the long-term safety and tolerability of a once-daily, dual-release hydrocortisone (DR-HC) tablet as oral glucocorticoid replacement therapy in patients with primary adrenal insufficiency (AI). DESIGN: Prospective, open-label, multicenter, 5-year extension study of DR-HC conducted at five university clinics in Sweden. METHODS: Seventy-one adult patients diagnosed with primary AI who were receiving stable glucocorticoid replacement therapy were recruited...
March 14, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28286277/extent-of-resection-visual-and-endocrinological-outcomes-for-endoscopic-endonasal-surgery-for-recurrent-pituitary-adenomas
#9
Hyunwoo Do, Varun R Kshettry, Alan Siu, Irina Belinsky, Christopher J Farrell, Gurston Nyquist, Marc Rosen, James J Evans
OBJECTIVE: To assess outcomes after endoscopic endonasal surgery for recurrent or residual pituitary adenomas. METHODS: We retrospectively analyzed 61 patients from 2009 - 2016 that underwent endoscopic endonasal surgery for recurrent or residual pituitary adenomas after prior microscopic or endoscopic transphenoidal operation. RESULTS: Prior surgical approach was endoscopic endonasal in 55.7% and microscopic in 44.2%. The mean preoperative maximal tumor diameter was 2...
March 9, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28248860/rapid-exacerbation-of-lymphocytic-infundibuloneurohypophysitis
#10
Kimitaka Shibue, Toshihito Fujii, Hisanori Goto, Yui Yamashita, Yoshihisa Sugimura, Masahiro Tanji, Akihiro Yasoda, Nobuya Inagaki
RATIONALE: Lymphocytic hypophysitis is a relatively rare autoimmune disease defined by lymphocytic infiltration to the pituitary. Its rarity and wide spectrum of clinical manifestations make clarification of the pathology difficult. Here, we describe a case we examined from the primary diagnosis to final discharge, showing the serial progression of lymphocytic infundibuloneurohypophysitis (LINH) to panhypopituitarism with extrapituitary inflammatory invasion in a short period, and responding favorably to high-dose glucocorticoid treatment...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28242047/simvastatin-treatment-aggravates-the-glucocorticoid-insufficiency-associated-with-hypocholesterolemia-in-mice
#11
Amber B Ouweneel, Ronald J van der Sluis, Joya E Nahon, Miranda Van Eck, Menno Hoekstra
BACKGROUND AND AIMS: Statin treatment disrupts HMG-CoA reductase-mediated endogenous cholesterol synthesis and lowers plasma LDL-cholesterol levels. Although statin treatment can theoretically impair adrenal steroid hormone synthesis, thus far, no effect on glucocorticoid output has been described, as LDL-cholesterol levels usually remain within the physiological range. However, novel statin-based treatment regimens that dramatically decrease LDL-cholesterol levels are currently employed...
February 20, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28223394/primary-adrenal-insufficiency-is-associated-with-impaired-natural-killer-cell-function-a-potential-link-to-increased-mortality
#12
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
#13
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
#14
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...
March 1, 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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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