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cushing's syndrome

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https://www.readbyqxmd.com/read/28938458/corticotroph-pituitary-carcinoma-in-a-patient-with-lynch-syndrome-ls-and-pituitary-tumors-in-a-nationwide-ls-cohort
#1
Daniel Bengtsson, Patrick Joost, Christos Aravidis, Marie Askmalm Stenmark, Ann-Sofie Backman, Beatrice Melin, Jenny von Salomé, Theofanis Zagoras, Samuel Gebre-Medhin, Pia Burman
Context: Lynch syndrome is a cancer predisposing syndrome caused by germline mutations in genes involved in DNA mismatch repair (MMR). Patients are at high risk for several types of cancer, but pituitary tumors have not previously been reported. Case: A 51-year old man with LS (MSH2 mutation) and a history of colon carcinoma presented with severe Cushing's disease and a locally aggressive pituitary tumor. The tumor harbored a mutation consistent with the patient's germline mutation, and displayed defect MMR function...
August 18, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28937294/hypertensive-crisis-in-pregnancy-due-to-a-metamorphosing-pheochromocytoma-with-postdelivery-cushing-s-syndrome
#2
Katharina Langton, Matthias Gruber, Jimmy Masjkur, Charlotte Steenblock, Mirko Peitzsch, Jörn Meinel, Jacques Lenders, Stefan Bornstein, Graeme Eisenhofer
Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing's syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. A cesarean section was performed at 32 weeks following a hypertensive crisis after routine administration of betamethasone. Postnatal persistence of signs and symptoms of catecholamine excess led to the diagnosis of a left adrenal pheochromocytoma...
September 22, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28931750/adrenal-gipr-expression-and-chromosome-19q13-microduplications-in-gip-dependent-cushing-s-syndrome
#3
Anne-Lise Lecoq, Constantine A Stratakis, Say Viengchareun, Ronan Chaligné, Lucie Tosca, Vianney Deméocq, Mirella Hage, Annabel Berthon, Fabio R Faucz, Patrick Hanna, Hadrien-Gaël Boyer, Nicolas Servant, Sylvie Salenave, Gérard Tachdjian, Clovis Adam, Vanessa Benhamo, Eric Clauser, Anne Guiochon-Mantel, Jacques Young, Marc Lombès, Isabelle Bourdeau, Dominique Maiter, Antoine Tabarin, Jérôme Bertherat, Hervé Lefebvre, Wouter de Herder, Estelle Louiset, André Lacroix, Philippe Chanson, Jérôme Bouligand, Peter Kamenický
GIP-dependent Cushing's syndrome is caused by ectopic expression of glucose-dependent insulinotropic polypeptide receptor (GIPR) in cortisol-producing adrenal adenomas or in bilateral macronodular adrenal hyperplasias. Molecular mechanisms leading to ectopic GIPR expression in adrenal tissue are not known. Here we performed molecular analyses on adrenocortical adenomas and bilateral macronodular adrenal hyperplasias obtained from 14 patients with GIP-dependent adrenal Cushing's syndrome and one patient with GIP-dependent aldosteronism...
September 21, 2017: JCI Insight
https://www.readbyqxmd.com/read/28929506/factitious-acth-dependent-apparent-hypercortisolism-the-problem-with-late-night-salivary-cortisol-measurements-collected-at-home
#4
G A Kline, J D Buse, J G Van Der Gugten, D T Holmes, A C Chin, S M H Sadrzadeh
Cushing's Syndrome (CS) is critical to identify because of the numerous and sometimes irreversible sequelae. Surreptitious glucocorticoid use is usually a straightforward consideration in the Cushingoid patient having suppressed ACTH levels, in the absence of adrenal mass or hyperplasia, and in whom screening tests for CS have unexpectedly low or high results depending on the specific steroid used and vulnerability of the assay employed to analytical cross-reactivity. We present a case of a patient with factitious hypercortisolism without suppressed ACTH...
September 20, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28928236/histone-deacetylase-inhibition-ameliorates-hypertension-and-hyperglycemia-in-a-model-of-cushing-s-syndrome
#5
Hae-Ahm Lee, Seol-Hee Kang, Mina Kim, Eunjo Lee, Hyun-Min Cho, Eun-Kyung Moon, Inkyeom Kim
Cushing's syndrome (CS) caused by hypercortisolism is occasionally accompanied by metabolic disorders such as hypertension, diabetes mellitus (DM), dyslipidemia and central obesity. Thus, morbidity and mortality, observed in cardiovascular disease, are elevated in patients with CS. We hypothesized that HDAC inhibition (HDACi) decreased transcriptional activity of glucocorticoid receptor (GR), which ameliorates hypertension and hyperglycemia in patients with CS. To establish an animal model of hypercortisolism, Sprague-Dawley rats were infused with adrenocorticotropic hormone (ACTH, 40 ng/day) or dexamethasone (Dex, 10 μg/day) via osmotic minipumps for 4 weeks...
September 19, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28915330/impact-of-adrenal-steroids-on-regulation-of-adipose-tissue
#6
Marco Infante, Andrea Armani, Caterina Mammi, Andrea Fabbri, Massimiliano Caprio
Corticosteroids are secreted by the adrenal glands and control the functions of adipose tissue via the activation of mineralocorticoid receptor (MR) and glucocorticoid receptor (GR). In turn, adipocytes release a large variety of adipokines into the bloodstream, regulating the function of several organs and tissues, including the adrenal glands, hereby controlling corticosteroid production. In adipose tissue, the activation of the MR by glucocorticoids (GC) and aldosterone affects important processes such as adipocyte differentiation, oxidative stress, autophagic flux, adipokine expression as well as local production of GC through upregulation of the enzyme 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1)...
September 12, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28911199/age-dependent-effects-of-armc5-haploinsufficiency-on-adrenocortical-function
#7
A Berthon, F R Faucz, S Espiard, L Drougat, J Bertherat, C A Stratakis
Inactivating mutations in the Armadillo repeat-containing 5 (ARMC5) gene have recently been discovered in primary macronodular adrenal hyperplasia (PMAH), a cause of Cushing syndrome. Biallelic ARMC5 inactivation in PMAH suggested that ARMC5 may have tumor suppressor functions in the adrenal cortex. We generated and characterized a new mouse model of Armc5 deficiency. Almost all Armc5 knockout mice died during early embryonic development, around 6.5 and 8.5 days. Knockout embryos did not undergo gastrulation, as demonstrated by the absence of mesoderm development at E7...
September 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28910955/-the-localization-diagnosis-of-patients-with-adrenocorticotropic-hormone-dependent-cushing-s-syndrome-in-adolescence
#8
S Chen, Z X Pan, L Lu, A L Tong, H Pan, H J Zhu, Y Yao, M Feng, R Z Wang, Z L Lu
Objective: To elucidate the clinical characteristics and localization diagnosis of patients with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) in adolescence. Methods: The clinical data, laboratory examination and localization diagnosis from 35 patients aged less than 18 years old with adolescent CS who were treated at Peking Union Medical College Hospital between January 1990 and March 2012 were analyzed. Results: There were 29 cases of Cushing's disease (CD) and 6 cases of ectopic ACTH syndrome (EAS)...
September 12, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28906379/accessory-ovarian-steroid-cell-tumor-producing-testosterone-and-cortisol-a-case-report
#9
Li Zang, Mingxia Ye, Guoqing Yang, Jinlong Li, Mei Liu, Jin Du, Weijun Gu, Nan Jin, Lijuan Yang, Jianming Ba, Jingtao Dou, Wensheng Fan, Yiming Mu, Yuanguang Meng, Zhaohui Lyu
RATIONALE: An accessory ovary is a rare structure containing normal ovarian tissue, which has a direct or ligamentous connection with a normal and eutopic ovary. PATIENT CONCERNS: In the study, we reported a 46-year-old woman presented with secondary amenorrhea and virilization symptoms for 1 year. DIAGNOSES: Endocrine evaluation revealed slightly elevated serum cortisol, extremely elevated 24-hour urinary-free cortisol and serum testosterone...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28905825/intraocular-pressure-and-its-correlation-with-midnight-plasma-cortisol-level-in-cushing-s-disease-and-other-endogenous-cushing-s-syndrome
#10
Priyadarshini Mishra, Alok Pratap Singh, Vikas Kanaujia, Rachna Agarwal, Prabhaker Mishra, Ashwani Guleria, Alka Tripathi
PURPOSE: The purpose of this study is to measure intraocular pressure (IOP) and evaluate the correlation between IOP and midnight plasma cortisol (MPC) level in patients with Cushing's disease (CD) and other endogenous Cushing's syndrome (ECS). METHODS: This is a cross-sectional study from a single center including newly diagnosed patients with CD or ECS. All patients underwent detailed ophthalmological evaluation. IOP was measured by Goldmann applanation tonometry in the morning and evening on two consecutive days...
September 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28895535/an-approach-to-screening-for-cushing-s-syndrome-in-non-specialized-health-care-settings
#11
David A Cano, Alfonso Leal-Cerro
No abstract text is available yet for this article.
October 2017: Endocrinología, diabetes y nutrición
https://www.readbyqxmd.com/read/28895486/iatrogenic-adrenal-insufficiency-secondary-to-combination-therapy-with-elvitegravir-cobicistat-tenofovir-disoproxil-fumarate-emtricitabine-and-interlaminar-triamcinolone-injection-in-an-aids-patient
#12
Chanie Wassner, Sutapa Maiti, Kurt Kodroff, Henry Cohen
We report the first identified case of suspected iatrogenic adrenal insufficiency after an interlaminar injection of triamcinolone acetonide while on concomitant Stribild (elvitegravir 150 mg/cobicistat 150 mg/tenofovir disoproxil fumarate 300 mg/emtricitabine 200 mg [EVG/c/TDF/FTC]). A 49-year-old female with HIV on EVG/c/TDF/FTC therapy presented to our endocrinology clinic to be evaluated for suspected Cushing syndrome. Prior to presentation, the patient had been given 2 interlaminar spinal injections of triamcinolone...
January 1, 2017: Journal of the International Association of Providers of AIDS Care
https://www.readbyqxmd.com/read/28895465/paraneoplastic-cushing-syndrome-case-series-and-review-of-the-literature
#13
Annelies Deldycke, Christel Haenebalcke, Youri Taes
OBJECTIVES: Paraneoplastic Cushing syndrome is a rare condition, representing a small fraction of the adrenocorticotropic hormone (ACTH)-dependent cases of Cushing syndrome Methods: Four case descriptions and literature review, highlighting the diagnostic challenges and treatment options are presented. RESULTS: Different tumor types can be associated with ectopic ACTH secretion. The most common types are bronchial carcinoids and small cell lung carcinoma (SCLC)...
September 12, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28885500/endocrine-surgery-a-hopkins-legacy
#14
Robert Udelsman
: The field of Endocrine Surgery is linked to extraordinary contributions made by Hopkins leaders in surgery including William Stewart Halsted, Harvey Cushing, and John L Cameron. Halsted's contributions to the anatomic basis of thyroid and parathyroid surgery were based on his experimental and clinical work performed at Johns Hopkins Hospital. Halsted's disciple, Harvey Cushing, created the field of modern neurosurgery and recognized the disease and syndrome that are immortalized with his name. The Halstedian principles promulgated and transmitted by John L Cameron to subsequent generations of endocrine surgeons at Hopkins have transformed the field of Endocrine Surgery with the stamp of Johns Hopkins Hospital...
September 6, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28878664/a-novel-prkar1a-mutation-identified-in-a-patient-with-isolated-primary-pigmented-nodular-adrenocortical-disease
#15
Sira Korpaisarn, Objoon Trachoo, Bhakbhoom Panthan, Rangsima Aroonroch, Ronnarat Suvikapakornkul, Chutintorn Sriphrapradang
Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome, especially the isolated form without Carney complex, associated with germline mutations in PRKAR1A, the protein kinase A regulatory subunit type 1 alpha gene. We report a 31-year-old female who presented with secondary amenorrhea, cushingoid appearance, and hypertension without Carney complex. Biochemical laboratory examinations confirmed the ACTH-independent adrenal Cushing syndrome with negative Liddle test. A small right adrenal adenoma of 0...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28877721/a-case-of-adrenocoricotrophic-hormone-independent-bilateral-adrenocortical-macronodular-hyperplasia-concomitant-with-primary-aldosteronism
#16
Mao Tokumoto, Naoyoshi Onoda, Yukie Tauchi, Shinichiro Kashiwagi, Satoru Noda, Norikazu Toi, Masahumi Kurajoh, Masahiko Ohsawa, Yuto Yamazaki, Hironobu Sasano, Kosei Hirakawa, Masaichi Ohira
BACKGROUND: Adrenocoricotrophic hormone (ACTH) - independent bilateral adrenocortical macronodular hyperplasia (AIMAH) is a rare cause of Cushing's syndrome, and is characterized by bilateral adrenal hyperplasia. However, Primary aldosteronism (PA) is a relatively common adrenal disease. CASE PRESENTATION: A 56-year-old man who has been treated hypertension and diabetes mellitus was detected low plasma potassium level with an elevated level of plasma aldosterone concentration and bilateral adrenal swelling...
September 6, 2017: BMC Surgery
https://www.readbyqxmd.com/read/28874240/-review-of-online-prescriptions-of-topical-steroids-for-an-obese-boy-with-iatrogenic-cushing-s-syndrome
#17
Sina Dalby, Inge Østergaard
A 14-year-old boy with obesity developed iatrogenic Cushing's syndrome after having received topical steroid therapy for psoriasis. The diagnosis was suspected when he developed striae, moon face and stunted growth. A review of the Danish online registration of prescriptions: Shared Medicine Card, revealed that an amount of 5.1 kg topical steroids had been prescribed during a period of twelve months. Blood tests showed stunted cortisol release. Primary obesity in children is associated with increased growth...
August 28, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28872770/hormone-secreting-adrenal-tumours-cause-severe-hypertension-and-high-rates-of-poor-pregnancy-outcome-a-ukoss-study-with-case-control-comparisons
#18
G Quartermaine, K Lambert, K Rees, P T Seed, M K Dhanjal, M Knight, D R McCance, C Williamson
OBJECTIVE: To examine the management and outcomes of adrenal tumours in pregnancy. DESIGN: A national observational, cohort study over four years using the UK Obstetric Surveillance System (UKOSS). SETTING: Consultant led obstetric units. PATIENTS: Women with phaeochromocytoma, primary aldosteronism or Cushing's syndrome diagnosed before or during pregnancy. METHODS: Clinical features of UKOSS cases were compared to those of women with adrenal tumours reported from 1985-2015...
September 5, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28870984/optimal-follow-up-strategies-for-adrenal-incidentalomas-reappraisal-of-the-2016-ese-ensat-guidelines-in-real-clinical-practice
#19
A Ram Hong, Jung Hee Kim, Kyeong Seon Park, Kyong Young Kim, Ji Hyun Lee, Sung Hye Kong, Seo Young Lee, Chan Soo Shin, Sang Wan Kim, Seong-Yeon Kim
OBJECTIVE: Recently, the European Society of Endocrinology (ESE), in collaboration with the European Network for the Study of Adrenal Tumors (ENSAT), asserted that adrenal incidentalomas (AIs) <4 cm and ≤10 Hounsfield units (HU) do not require further follow-up imaging. In order to validate the clinical application of the follow-up strategies suggested by the 2016 ESE-ENSAT guidelines, we explored the clinical characteristics and natural course of AIs in a single center over 13 years...
September 4, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28862247/ectopic-adrenocorticotropic-hormone-syndrome-in-a-case-of-duodenal-neuroendocrine-tumor-presenting-with-liver-metastasis
#20
J Khare, S Daga, S Nalla, P Deb
Ectopic adrenocorticotropic hormone (ACTH) syndrome is an uncommon disorder and comprises about 15% of all patients with Cushing's syndrome (CS). Duodenal carcinoids are rare, indolent tumors usually associated with a benign progression. We hereby report a rare case of CS resulting from ectopic ACTH secretion from a duodenal neuroendocrine tumor (NET) presenting with liver metastasis. A 37-year-old female presented with abdominal discomfort and dyspepsia of 1-month duration. Ultrasound abdomen suggested a well-defined hypoechoic lesion in the left lobe of the liver, suggestive of neoplasia...
August 31, 2017: Journal of Postgraduate Medicine
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