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Hypercortisolism

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https://www.readbyqxmd.com/read/28718178/body-composition-is-different-after-surgical-or-pharmacological-remission-of-cushing-s-syndrome-a-prospective-dxa-study
#1
Filippo Ceccato, Michela Boccato, Marialuisa Zilio, Mattia Barbot, Anna Chiara Frigo, Giovanni Luisetto, Marco Boscaro, Carla Scaroni, Valentina Camozzi
Patients with active Cushing's syndrome (CS) exhibit an increase of the visceral adipose tissue, increasing the risk of cardiovascular events. Until now, it is not yet clear whether remission of CS leads to a normalization of body composition, or if different strategies to control hypercortisolism could result in a different clinical outcome concerning adipose tissue distribution. Therefore, we analyzed body composition changes using dual-energy X-ray absorptiometry (DXA) in patients with CS in a prospective and controlled study...
July 17, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28710189/use-of-cabergoline-for-the-management-of-persistent-cushing-s-disease-in-pregnancy
#2
Kathleen Su-Yen Sek, Doddabele Srinivasa Deepak, Kok Onn Lee
Cushing's disease (CD) is rare during pregnancy and is associated with significant maternal and fetal complications. It is important to control hypercortisolism during pregnancy, either surgically or medically, for a successful maternal and fetal outcome. We report a patient with recurrent CD who was treated with low-dose cabergoline (CAB) for persistent hypercortisolism throughout pregnancy. A 36-year-old woman was diagnosed with CD at the age of 23. She underwent trans-sphenoidal surgery with initial complete remission...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28701629/atypical-pituitary-adenoma-with-men1-somatic-mutation-associated-with-abnormalities-of-dna-mismatch-repair-genes-mlh1-germline-mutation-and-msh6-somatic-mutation
#3
Shinsuke Uraki, Hiroyuki Ariyasu, Asako Doi, Hiroto Furuta, Masahiro Nishi, Kokichi Sugano, Naoko Inoshita, Naoyuki Nakao, Shozo Yamada, Takashi Akamizu
The mechanism of pituitary tumorigenesis remains largely unknown. Lynch syndrome is an autosomal, dominantly inherited syndrome caused by a defective mismatch repair (MMR) mechanism involved in the development of various tumors at an early age. In this case study, we showed the occurrence of pituitary tumors associated with Lynch syndrome for the first time and performed genetic and immunohistochemical analysis to evaluate the genetic aberrations that might be related to the tumorigenesis and proliferation...
July 13, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28698013/links-between-early-child-maltreatment-mental-disorders-and-cortisol-secretion-anomalies
#4
Bronsard Guillaume, Auquier Pascal, Boyer Laurent
Early child maltreatment has been widely associated with the development of mental disorders in both childhood and adulthood. However, such association cannot be systematically established, as only few factors are observed regularly, such as high prevalence of comorbidities and externalized disorders. Similarly, the association between early abuse and cortisol secretion anomalies has been well-documented. Whereas early hypercortisolism followed by hypocortisolism was often described, the results proved inconsistent and at times contradictory...
July 8, 2017: Journal of Physiology, Paris
https://www.readbyqxmd.com/read/28688648/nationwide-review-of-hormonally-active-adrenal-tumors-highlights-high-morbidity-in-pheochromocytoma
#5
Punam P Parikh, Gustavo A Rubio, Josefina C Farra, John I Lew
BACKGROUND: Adrenal adenomas are benign tumors often discovered incidentally, and >70% are hormonally inactive. The remaining subset may produce excess aldosterone, cortisol, or catecholamine. Perioperative outcomes after adrenalectomy for such "hormonally active" tumors remain unclear. This study examines in-hospital outcomes after unilateral adrenalectomy for hormonally active tumors. METHODS: A retrospective review was performed using the Nationwide Inpatient Sample (2006-2011) to identify patients undergoing unilateral adrenalectomy for hormonally active or inactive tumors...
July 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28680643/a-rare-case-of-an-acth-crh-co-secreting-midgut-neuroendocrine-tumor-mimicking-cushing-s-disease
#6
Regina Streuli, Ina Krull, Michael Brändle, Walter Kolb, Günter Stalla, Marily Theodoropoulou, Annette Enzler-Tschudy, Stefan Bilz
Ectopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing's syndrome and only a few cases have been reported in the literature. Differentiating between Cushing's disease and ectopic Cushing's syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary corticotroph hyperplasia that may mimic Cushing's disease during dynamic testing with both dexamethasone and CRH as well as bilateral inferior petrosal sinus sampling (BIPSS). We present the case of a 24-year-old man diagnosed with ACTH-dependent Cushing's syndrome caused by an ACTH/CRH co-secreting midgut NET...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28676429/the-role-of-armc5-in-human-cell-cultures-from-nodules-of-primary-macronodular-adrenocortical-hyperplasia-pmah
#7
Isadora P Cavalcante, Mirian Nishi, Maria Claudia N Zerbini, Madson Q Almeida, Vania B Brondani, Maria Luiza Anhaia de Arruda Botelho, Fabio Y Tanno, Victor Srougi, José Luis Chambo, Berenice B Mendonca, Jérôme Bertherat, Claudimara F P Lotfi, Maria Candida B V Fragoso
The participation of aberrant receptors and intra-adrenal ACTH in hyperplastic tissue are considered mechanisms that regulate hypercortisolism in PMAH. Additionally, germline ARMC5 mutations have been described as the most frequent genetic abnormality found in patients diagnosed with PMAH. Previous functional studies analyzed ARMC5 role using H295R cells. Therefore, we investigated the role of ARMC5 in cell cultures obtained from PMAH nodules containing steroidogenic cells, aberrant receptors and intra-adrenal ACTH...
July 1, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28670192/the-occurrence-of-subclinical-hypercortisolism-and-osteoporosis-in-patients-with-incidentally-discovered-unilateral-and-bilateral-adrenal-tumors
#8
Sanja Ognjanović, Djuro Macut, Milan Petakov, Valentina Elezović Kovačević, Tatjana Isailović, Ivana Bozić Antić, Dušan Ilić, Bojana Popović, Tamara Bogavac, Tatjana Pekmezović, Svetozar Damjanović
BACKGROUND: Adrenal incidentalomas (AI) are clinically silent adrenal masses that are detected incidentally during imaging procedures performed for unrelated diseases. The aim of this study was to investigate the prevalence of sub-clinical hypercortisolism (SH) and associated co-morbidities in patients with unilateral AI (UAI) and bilateral AI (BAI). METHODS: We evaluated 152 patients, 105 (69.1%) with UAI and 47 (30.9%) with BAI. SH was diagnosed in the presence of serum cortisol levels after 1 mg dexamethasone suppression test (DST) or after 2-day low-dose DST (LDDST) > 50 nmol/L with at least one of the following parameters: midnight serum cortisol > 208 nmol/L, 24-h urinary free cortisol > 245 nmol/24 h, or ACTH < 10 ng/L...
October 2016: Journal of Medical Biochemistry
https://www.readbyqxmd.com/read/28665508/a-polymorphism-in-the-cyp17a1-gene-influences-the-therapeutic-response-to-steroidogenesis-inhibitors-in-cushing-s-syndrome
#9
Elena Valassi, Anna Aulinas, Camilla Am Glad, Gudmundur Johannsson, Oskar Ragnarsson, Susan M Webb
CONTEXT: Steroidogenesis inhibitors, such as ketoconazole (KTZ) and metyrapone (MTP) are used to lower hypercortisolism in patients with Cushing's syndrome (CS). Cortisol normalization is not reached in all patients taking these medications. OBJECTIVE: To test the hypothesis that variants in genes affecting steroidogenesis contribute to different responses to KTZ and/or MTP in patients with CS. PATIENTS AND METHODS: Fifty-four CS patients [46 women; mean (±SD) age, 39...
June 30, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28660991/insulin-resistance-in-endocrine-disorders-treatment-options
#10
Anita Rogowicz-Frontczak, Anna Majchrzak, Dorota Zozulińska-Ziółkiewicz
Changes in sensitivity to insulin occur in the course of a number of endocrine disorders. Most of the hormones through their antagonistic action to insulin lead to increased hepatic glucose output and its decreased utilisation in peripheral tissues. Carbohydrate disorders observed in endocrine diseases result from the phenomenon of insulin resistance, and in some cases also a reduction in insulin secretion is present. Abnormalities of glucose metabolism are observed in acromegaly, but also in growth hormone deficiency, hypercortisolism in the course of Cushing's syndrome, hyper- or hypothyroidism, primary hyperparathyroidism, aldosteronism, pheochromocytoma, congenital hypertrophy of the adrenal glands, polycystic ovaries syndrome, hypogonadism, or other hormonally active neuroendocrine tumours...
2017: Endokrynologia Polska
https://www.readbyqxmd.com/read/28656606/preventive-effects-of-ginseng-total-saponins-on-chronic-corticosterone-induced-impairment-in-astrocyte-structural-plasticity-and-hippocampal-atrophy
#11
Lin Chen, Xin Wang, Zi-Xuan Lin, Jian-Guo Dai, Yu-Fang Huang, Yu-Nan Zhao
To further explore the underlying antidepressant mechanism of ginseng total saponins (GTS), this study observed the effects on hippocampal astrocyte structural plasticity and hippocampal volume in the corticosterone-induced mouse depression model. Corticosterone (20 mg/kg/day) was administered subcutaneously for 5 weeks, and GTS (12.5, 25, and 50 mg/kg/day; namely GTSL, GTSM, and GTSH) or fluoxetine (10 mg/kg/day) were given intragastrically during the last 3 weeks. On day 33 and day 34, depression-like behavior was observed via a forced swimming test and a tail suspension test, respectively...
June 28, 2017: Phytotherapy Research: PTR
https://www.readbyqxmd.com/read/28642733/a-case-of-pulmonary-carcinoid-tumor-with-a-superimposed-aspergilloma-presenting-as-a-covert-ectopic-adrenocorticotropic-hormone-syndrome
#12
Kyoung Jin Kim, Ji Hee Yu, Nan Hee Kim, Young Hye Kim, Young Sik Kim, Ji A Seo
Ectopic adrenocorticotropic hormone (ACTH) syndrome is a challenging diagnosis only responsible for approximately 10% of Cushing syndrome cases. It has been associated with a variety of benign and malignant tumors including a carcinoid tumor accompanied by aspergilloma in our case that was significantly difficult to be detected. We report a patient over 70 years old with uncontrolled hypertension and hypokalemia presenting with generalized edema. Laboratory results revealed ACTH-dependent Cushing syndrome, but imaging studies did not show any discrete lesions secreting ACTH...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28638307/rectal-metyrapone-for-treatment-of-hypercortisolism-in-an-infant-with-mccune-albright-syndrome
#13
Kate Verbeeten, Stasia Hadjiyannakis, Melody Cameron, Jaime McDonald
Infantile Cushing syndrome is an infrequent yet potentially fatal manifestation of McCune-Albright syndrome, for which there are few safe treatments available. Ketoconazole is limited by potential hepatotoxicity in this population. Metyrapone may be an effective treatment, but it may not be tolerated when given orally. An infant with McCune-Albright syndrome presented with severe Cushing syndrome. Oral metyrapone resulted in feeding refusal, and ketoconazole caused an increase in liver enzymes; however, she was successfully treated with metyrapone given rectally...
May 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28614036/an-individualized-approach-to-the-evaluation-of-cushing-syndrome
#14
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
#15
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...
July 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
#16
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
#17
Guido Di Dalmazi, Marcus Quinkler, Timo Deutschbein, Cornelia Prehn, Nada Rayes, Matthias Kroiss, Christina M Berr, Günter 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)...
August 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28543178/volumetric-mri-analysis-in-patients-with-short-term-remission-of-cushing-s-disease
#18
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-randomised-study-on-hdl
#19
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
#20
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
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