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Hypercortisolism

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https://www.readbyqxmd.com/read/28614036/an-individualized-approach-to-the-evaluation-of-cushing-syndrome
#1
Susmeeta T Sharma
Cushing syndrome (CS) is caused by chronic exposure to excess glucocorticoids. Early recognition and treatment of hypercortisolemia can lead to decreased morbidity and mortality. The diagnosis of CS and thereafter, establishing the cause can often be difficult, especially in patients with mild and cyclic hypercortisolism. Surgical excision of the cause of excess glucocorticoids is the optimal treatment for CS. Medical therapy (steroidogenesis inhibitors, medications that decrease adrenocorticotropic hormone [ACTH] levels or glucocorticoid antagonists) and pituitary radiotherapy may be needed as adjunctive treatment modalities in patients with residual, recurrent or metastatic disease, in preparation for surgery, or when surgery is contraindicated...
June 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28597198/long-term-treatment-of-cushing-s-disease-with-pasireotide-5-year-results-from-an-open-label-extension-study-of-a-phase-iii-trial
#2
S Petersenn, L R Salgado, J Schopohl, L Portocarrero-Ortiz, G Arnaldi, A Lacroix, C Scaroni, S Ravichandran, A Kandra, B M K Biller
BACKGROUND: Treating hypercortisolism in patients with Cushing's disease after failed surgery often requires chronic medication, underlining the need for therapies with favourable long-term efficacy and safety profiles. METHODS: In a randomised, double-blind study, 162 adult patients with persistent/recurrent or de novo Cushing's disease received pasireotide. Patients with mean urinary free cortisol at/below the upper limit of normal or clinical benefit at month 12 could continue receiving pasireotide during an open-ended, open-label phase, the outcomes of which are described herein...
June 9, 2017: Endocrine
https://www.readbyqxmd.com/read/28594137/increased-adipocyte-size-macrophage-infiltration-and-adverse-local-adipokine-profile-in-perirenal-fat-in-cushing-s-syndrome
#3
Sean H P P Roerink, Margreet A E M Wagenmakers, Johan F Langenhuijsen, Dov B Ballak, Hanne M M Rooijackers, Frank C d'Ancona, François M van Dielen, Jan W A Smit, Theo S Plantinga, Romana T Netea-Maier, Ad R M M Hermus
OBJECTIVE: To analyze changes in fat cell size, macrophage infiltration, and local adipose tissue adipokine profiles in different fat depots in patients with active Cushing's syndrome. METHODS: Subcutaneous (SC) and perirenal (PR) adipose tissue of 10 patients with Cushing's syndrome was compared to adipose tissue of 10 gender-, age-, and BMI-matched controls with regard to adipocyte size determined by digital image analysis on hematoxylin and eosin stainings, macrophage infiltration determined by digital image analysis on CD68 stainings, and adipose tissue leptin and adiponectin levels using fluorescent bead immunoassays and ELISA techniques...
June 8, 2017: Obesity
https://www.readbyqxmd.com/read/28566446/cortisol-related-metabolic-alterations-assessed-by-mass-spectrometry-assay-in-patients-with-cushing-s-syndrome
#4
Guido Di Dalmazi, Marcus Quinkler, Timo Deutschbein, Cornelia Prehn, Nada Rayes, Matthias Kroiss, Christina Maria Berr, Günter K Stalla, Martin Fassnacht, Jerzy Adamski, Martin Reincke, Felix Beuschlein
OBJECTIVE: Endogenous hypercortisolism is a chronic condition associated with severe metabolic disturbances and cardiovascular sequela. The aim of this study was to characterize metabolic alterations in patients with different degrees of hypercortisolism by mass-spectrometry-based targeted plasma metabolomic profiling and correlate the metabolomic profile with clinical and hormonal data. DESIGN: Cross-sectional study. METHODS: Subjects (n=149) were classified according to clinical and hormonal characteristics: Cushing's syndrome (n=46), adrenocortical adenomas with autonomous cortisol secretion (n=31) or without hypercortisolism (n=27)...
May 31, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28543178/volumetric-mri-analysis-in-patients-with-short-term-remission-of-cushing-s-disease
#5
Hong Jiang, Jie Ren, Na-Ying He, Chang Liu, Yu-Hao Sun, Fang-Fang Jian, Liu-Guan Bian, Jian-Kang Shen, Fu-Hua Yan, Si-Jan Pan, Qing-Fang Sun
OBJECTIVE: The data on patients with short-term remission of Cushing's disease (CD) might provide information that is not available from previous long-term remission studies. We aimed to investigate structural changes in the brain in these patients and to examine whether these changes were associated with clinical characteristics. DESIGN: A cross-sectional study was performed. METHODS: Thirty-four CD patients (14 with CD in short-term remission and 20 with active CD) and 34 controls matched for age, sex, and education underwent clinical evaluation and magnetic resonance imaging brain scans...
May 20, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28542805/downregulation-of-cholesteryl-ester-transfer-protein-by-glucocorticoids-a-randomized-study-on-hdl
#6
Jorien Werumeus Buning, Lidya G Dimova, Frank G Perton, Uwe J F Tietge, André P van Beek, Robin P F Dullaart
BACKGROUND: High density lipoprotein (HDL) cholesterol is not decreased in hypercortisolism despite high triglycerides, which may be ascribed to effects on the cholesteryl ester transfer protein (CETP) pathway. We explored if CETP mRNA expression is modulated by glucocorticoid treatment in vitro. Effects of doubling the hydrocortisone (HCT) replacement dose on plasma CETP activity and HDL characteristics were tested in patients with secondary adrenal insufficiency. MATERIALS AND METHODS: Human THP-1 macrophages were incubated with corticosterone in vitro in the presence or absence of a liver X receptor (LXR) agonist, followed by determination of CETP mRNA levels by quantitative real-time PCR...
May 24, 2017: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28529722/recent-advances-in-understanding-cushing-disease-resistance-to-glucocorticoid-negative-feedback-and-somatic-usp8-mutations
#7
REVIEW
Eleni Daniel, John Newell-Price
Cushing's disease is a rare disease with a characteristic phenotype due to significant hypercortisolism driven by over-secretion of adrenocorticotropic hormone and to high morbidity and mortality if untreated. It is caused by a corticotroph adenoma of the pituitary, but the exact mechanisms leading to tumorigenesis are not clear. Recent advances in molecular biology such as the discovery of somatic mutations of the ubiquitin-specific peptidase 8 ( USP8) gene allow new insights into the pathogenesis, which could be translated into exciting and much-needed therapeutic applications...
2017: F1000Research
https://www.readbyqxmd.com/read/28528397/perioperative-hypothalamic-pituitary-adrenal-function-in-patients-with-silent-corticotroph-adenomas
#8
Abdelle F Cheres, Nadine ElAsmar, Aman Rajpal, Warren R Selman, Baha M Arafah
BACKGROUND: Silent corticotroph adenomas (SCAs) are characterized by strong ACTH immunostaining without clinical manifestations of hypercortisolism. Patients with SCAs often present with mechanical symptoms related to tumor growth. This study investigates the hypothalamic pituitary adrenal axis (HPA) characteristics after adenomectomy in patients with SCAs. METHODS: Biochemical parameters of HPA function were monitored frequently after surgical resection of non-functioning macroadenomas...
May 20, 2017: Pituitary
https://www.readbyqxmd.com/read/28528327/three-quarters-adrenalectomy-for-infantile-onset-cushing-syndrome-due-to-bilateral-adrenal-hyperplasia-in-mccune-albright-syndrome
#9
Tomoyo Itonaga, Hironori Goto, Manabu Toujigamori, Yasuharu Ohno, Seigo Korematsu, Tatsuro Izumi, Satoshi Narumi, Tomonobu Hasegawa, Kenji Ihara
BACKGROUND: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS...
May 19, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28515793/does-the-diurnal-cycle-of-cortisol-explain-the-relationship-between-physical-performance-and-cognitive-function-in-older-adults
#10
B Dijckmans, J Tortosa-Martínez, N Caus, G González-Caballero, B Martínez-Pelegrin, C Manchado-Lopez, J M Cortell-Tormo, I Chulvi-Medrano, A Clow
BACKGROUND: Regular physical activity is a promising strategy to treat and prevent cognitive decline. The mechanisms that mediate these benefits are not fully clear but physical activity is thought to attenuate the harmful effects of chronic psychological stress and hypercortisolism on cognition. However, the circadian pattern of cortisol secretion is complex and it is not known which aspects are most closely associated with increased cognitive function and better physical performance...
2017: European Review of Aging and Physical Activity
https://www.readbyqxmd.com/read/28458898/efficacy-of-pasireotide-in-controlling-severe-hypercortisolism-until-cardiac-transplantation
#11
Roberto Attanasio, Liana Cortesi, Daniela Gianola, Claudia Vettori, Fulvio Sileo, Roberto Trevisan
SUMMARY: Cushing's syndrome is associated with increased morbidity and mortality. Although surgery is the first-line treatment, drugs can still play a role as an ancillary treatment to be employed while waiting for surgery, after unsuccessful operation or in patients unsuitable for surgery. We were asked to evaluate a 32-year-old male waiting for cardiac transplantation. Idiopathic hypokinetic cardiomyopathy had been diagnosed since 6 years. He was on treatment with multiple drugs, had a pacemaker, an implantable cardioverter and an external device for the support of systolic function...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28458897/armc5-mutation-in-a-portuguese-family-with-primary-bilateral-macronodular-adrenal-hyperplasia-pbmah
#12
Teresa Rego, Fernando Fonseca, Stéphanie Espiard, Karine Perlemoine, Jérôme Bertherat, Ana Agapito
SUMMARY: PBMAH is a rare etiology of Cushing syndrome (CS). Familial clustering suggested a genetic cause that was recently confirmed, after identification of inactivating germline mutations in armadillo repeat-containing 5 (ARMC5) gene. A 70-year-old female patient was admitted due to left femoral neck fracture in May 2014, in Orthopedics Department. During hospitalization, hypertension (HTA) and hypokalemia were diagnosed. She presented with clinical signs of hypercortisolism and was transferred to the Endocrinology ward for suspected CS...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28444400/effects-of-insufficient-sleep-on-pituitary-adrenocortical-response-to-crh-stimulation-in-healthy-men
#13
Aurore Guyon, Lisa L Morselli, Marcella L Balbo, Esra Tasali, Rachel Leproult, Mireille L'Hermite-Balériaux, Eve Van Cauter, Karine Spiegel
Study Objectives: Severe sleep restriction results in elevated evening cortisol levels. We examined whether this relative hypercortisolism is associated with alterations in the pituitary-adrenocortical response to evening corticotropin-releasing hormone (CRH) stimulation. Methods: Eleven subjects participated in 2 sessions (2 nights of 10 hours vs. 4 hours in bed) in randomized order. Sleep was polygraphically recorded. After the second night of each session, blood was sampled at 20-minute intervals from 09:00 to 24:00 for adrenocorticotropic hormone (ACTH) and cortisol measurements, and perceived stress was assessed hourly...
June 1, 2017: Sleep
https://www.readbyqxmd.com/read/28440767/zinc-alpha-2-glycoprotein-is-overproduced-in-cushing-s-syndrome
#14
Xavier Escoté, Gloria B Aranda, Mireia Mora, Gregori Casals, Joaquim Enseñat, Oscar Vidal, Yaiza Esteban, Irene Halperin, Felicia A Hanzu
INTRODUCTION: Cushing syndrome (CS), an endogenous hypercortisolemic condition with increased cardiometabolic morbidity, leads to development of abdominal obesity, insulin resistance, diabetes and proatherogenic dyslipidemia. Zinc alpha-2 glycoprotein (ZAG) is a recently characterized lipolytic adipokine implicated in regulation of adipose tissue metabolism and fat distribution. In vitro and animal studies suggest that glucocorticoids interact with ZAG secretion and action. To assess the relationship between ZAG and glucocorticoids in a human model of hypercortisolism, circulating ZAG levels were tested in patients with CS and its counterpart controls...
January 2017: Endocrinología, diabetes y nutrición
https://www.readbyqxmd.com/read/28438531/altered-gray-and-white-matter-microstructure-in-cushing-s-disease-a-diffusional-kurtosis-imaging-study
#15
Hong Jiang, Na-Ying He, Yu-Hao Sun, Fang-Fang Jian, Liu-Guan Bian, Jian-Kang Shen, Fu-Hua Yan, Si-Jian Pan, Qing-Fang Sun
Exposure to chronic hypercortisolism has multiple adverse effects on brain biology in humans. Cushing's disease (CD) represents a unique and natural human model for examining the effects of hypercortisolism on the brain. This cross-sectional study used Diffusional Kurtosis Imaging (DKI) to investigate the microstructure alterations in both white matter (WM) and gray matter (GM) of CD patients and to determine the relationship of these changes with clinical characteristics. DKI images were obtained from 15 active CD patients...
April 21, 2017: Brain Research
https://www.readbyqxmd.com/read/28435794/cushing-disease-in-a-patient-with-multiple-endocrine-neoplasia-type-2b
#16
Kannan Kasturi, Lucas Fernandes, Martha Quezado, Mary Eid, Leigh Marcus, Prashant Chittiboina, Mark Rappaport, Constantine A Stratakis, Brigitte Widemann, Maya Lodish
CONTEXT: Multiple endocrine neoplasia type 2B (MEN2B) is a rare autosomal-dominant cancer syndrome characterized in part by metastatic medullary thyroid cancer (MTC) and pheochromocytoma. Cushing disease is a rare cause of endogenous hypercortisolism in children. CASE DESCRIPTION: We describe a 21-year-old African-American male who was diagnosed at age 10 with an ACTH-secreting pituitary microadenoma. At age 16 he developed medullary thyroid cancer and was found to have multiple endocrine neoplasia type 2B with the characteristic M918T mutation of the RET proto-oncogene...
June 2017: Journal of clinical and translational endocrinology case reports
https://www.readbyqxmd.com/read/28431167/performance-of-18f-fdg-pet-ct-in-the-characterization-of-adrenal-masses-in-non-cancer-patients-a-prospective-study
#17
Carole Guerin, François Pattou, Laurent Brunaud, Jean-Christophe Lifante, Eric Mirallié, Magalie Haissaguerre, Damien Huglo, Pierre Olivier, Claire Houzard, Catherine Ansquer, Elif Hindié, Anderson Loundou, Cendrine Archange, Antoine Tabarin, Fréderic Sebag, Karine Baumstarck, David Taïeb
Context: Few prospective studies have evaluated the role of 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the characterization of adrenal masses. Objective: To assess the performance of 18F-FDG PET/CT in the malignancy diagnosis of adrenal masses in non-cancer patients. Design: Prospective multicenter study. Material and methods: The study population consisted of 87 patients (87 adrenal masses) referred to endocrine surgeons: 56 with mass diameter ≥40 mm and 31 with a diameter <40 mm and of indeterminate nature based on unenhanced and washout CT attenuation densities...
April 20, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28407311/biological-variability-in-serum-cortisol-concentration-post-adrenocorticotropic-hormone-stimulation-in-healthy-dogs
#18
A Gal, K Weidgraaf, J P Bowden, N Lopez-Villalobos, N J Cave, J P Chambers, F Castillo-Alcala
BACKGROUND: The ACTH stimulation has low sensitivity for the diagnosis of hypercortisolism possibly as a result of biological and analytical variability. HYPOTHESIS/OBJECTIVES: To report the components of biological and analytical variability in serum cortisol concentration post-ACTH stimulation ([cortisol]) in healthy dogs. ANIMALS: Fourteen healthy harrier hound dogs. METHODS: The data were extracted from a separate, prospective, randomized, double-blinded, controlled discovery study in which dogs treated with vehicle control and 4 different doses of cortisone acetate (CA) for 7 days had an ACTH stimulation test performed to confirm the dose-dependent effect of CA...
May 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28405879/histopathological-and-genetic-characterization-of-aldosterone-producing-adenomas-with-concurrent-subclinical-cortisol-hypersecretion-a-case-series
#19
Francesco Fallo, Isabella Castellano, Celso E Gomez-Sanchez, Yara Rhayem, Catia Pilon, Valentina Vicennati, Donatella Santini, Valeria Maffeis, Ambrogio Fassina, Paolo Mulatero, Felix Beuschlein, Martin Reincke
PURPOSE: Aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion are reported in an increasing number of patients. Five aldosterone-producing adenomas from patients with primary aldosteronism and subclinical hypercortisolism were examined. THE AIMS OF OUR STUDY WERE: (1) to analyze pathological features and immunohistochemical expression of CYP11B1 (11β-hydroxylase) and CYP11B2 (aldosterone synthase) in these tumors; (2) to investigate somatic mutations involved in adrenal steroid hypersecretion and/or tumor growth...
April 12, 2017: Endocrine
https://www.readbyqxmd.com/read/28404594/health-related-quality-of-life-of-patients-with-hypothalamic-pituitary-adrenal-axis-dysregulations-a-cohort-study
#20
Charlotte De Bucy, Laurence Guignat, Tanya Niati, Jerome Bertherat, Joel Coste
OBJECTIVE: Health-related quality of life (HrQoL) is increasingly considered to be an important outcome of care for hypothalamic-pituitary-adrenal (HPA) axis dysregulation. The objective of this study was to assess the influence of type of HPA axis dysregulation and cortisol status on HrQOL and its evolution with time and treatment. DESIGN: Prospective cohort study. METHODS: Between September 2007 and April 2014, HrQoL questionnaires were administered during routine management to all patients with HPA axis dysregulation hospitalized in a single department and this was repeated after 6- 12-, 24- and 36-month during standard follow-up...
April 12, 2017: European Journal of Endocrinology
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