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cushing s disease

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https://www.readbyqxmd.com/read/28642375/acquired-resistance-to-corticotropin-therapy-in-nephrotic-syndrome-role-of-de-novo-neutralizing-antibody
#1
Pei Wang, Yan Zhang, Yu Wang, Andrew S Brem, Zhangsuo Liu, Rujun Gong
There is increasing evidence supporting the use of corticotropin as an alternative treatment of refractory proteinuric glomerulopathies. The efficacy of short-acting corticotropin, however, remains unknown and was tested here in an adolescent with steroid-dependent nephrotic syndrome caused by minimal change disease. After developing Cushing syndrome and recently being afflicted with severe cellulitis, the patient was weaned off all immunosuppressants, including corticosteroids. This resulted in a relapse of generalized anasarca, associated with massive proteinuria and hypoalbuminemia...
June 22, 2017: Pediatrics
https://www.readbyqxmd.com/read/28614036/an-individualized-approach-to-the-evaluation-of-cushing-syndrome
#2
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/28614003/biochemical-control-during-long-term-follow-up-of-230-adult-patients-with-cushing-s-disease-a-multi-center-retrospective-study
#3
Eliza B Geer, Ismat Shafiq, Murray B Gordon, Vivien Bonert, Alejandro Ayala, Ronald S Swerdloff, Laurence Katznelson, Yelena Lalazar, Ekaterina Manuylova, Karen J Pulaski-Liebert, John D Carmichael, Zeina Hannoush, Vijaya Surampudi, Michael S Broder, Dasha Cherepanov, Marianne Eagan, Jackie Lee, Qayyim Said, Maureen P Neary, Beverly Mk Biller
OBJECTIVES: Cushing's disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately-treated CD is associated with significant morbidity and elevated mortality. Multi-center data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. The study purpose was to describe the burden of illness and treatment outcomes for CD patients. METHODS: Eight pituitary centers in 4 US regions participated in this multi-center retrospective chart review study...
June 14, 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
#4
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/28532906/first-report-of-coexistence-of-2-ectopic-pituitary-tumors-rathke-cleft-cyst-and-silent-acth-adenoma
#5
Mirza Pojskić, Blazej Zbytek, Neal S Beckford, Frederick A Boop, Kenan I Arnautović
Rathke cleft cysts (RCCs) and pituitary adenomas (PAs) are thought to have a common embryonic ancestry. Despite this, PAs with a concomitant RCC inside the sella turcica are rarely observed; ectopic pituitary tumors are also rare. We present the case of a 65-year-old woman with an ectopic RCC in the sphenoid sinus and outside of the sella turcica concomitant with an adrenocorticotropic hormone (ACTH)-staining, clinically silent PA. The patient presented with headache but no endocrine or visual disturbances...
May 19, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28487882/identification-of-a-novel-rasd1-somatic-mutation-in-a-usp8-mutated-corticotroph-adenoma
#6
Andrew V Uzilov, Khadeen C Cheesman, Marc Y Fink, Leah C Newman, Chetanya Pandya, Yelena Lalazar, Marco Hefti, Mary Fowkes, Gintaras Deikus, Chun Yee Lau, Aye S Moe, Yayoi Kinoshita, Yumi Kasai, Micol Zweig, Arpeta Gupta, Daniela Starcevic, Milind Mahajan, Eric E Schadt, Kalmon D Post, Michael J Donovan, Robert Sebra, Rong Chen, Eliza B Geer
Cushing's disease (CD) is caused by pituitary corticotroph adenomas that secrete excess adrenocorticotropic hormone (ACTH). In these tumors, somatic mutations in the gene USP8 have been identified as recurrent and pathogenic and are the sole known molecular driver for CD. Although other somatic mutations were reported in these studies, their contribution to the pathogenesis of CD remains unexplored. No molecular drivers have been established for a large proportion of CD cases and tumor heterogeneity has not yet been investigated using genomics methods...
May 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28450229/endoscopic-endonasal-surgery-for-tumors-of-the-cavernous-sinus-a-series-of-234-patients
#7
Maria Koutourousiou, Francisco Vaz Guimaraes Filho, Juan C Fernandez-Miranda, Eric W Wang, S Tonya Stefko, Carl H Snyderman, Paul A Gardner
BACKGROUND: Cavernous sinus (CS) tumors are often considered inoperable. We present our experience with endoscopic endonasal surgery (EES) and compare the outcomes for different tumor. METHODS: EES (medial or lateral approach) was used in 234 patients with CS tumors. The cohort included 175 (75%) pituitary adenomas and 59 (25%) non-adenomatous lesions. RESULTS: Presenting symptoms were significantly different between the two groups, with cranial neuropathies occurring mainly in non-adenomas (p<...
April 24, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28422946/pediatric-cushing-disease-disparities-in-disease-severity-and-outcomes-in-the-hispanic-and-african-american-populations
#8
Alexandra Gkourogianni, Ninet Sinaii, Sharon H Jackson, Alexander S Karageorgiadis, Charalampos Lyssikatos, Elena Belyavskaya, Margaret F Keil, Mihail Zilbermint, Prashant Chittiboina, Constantine A Stratakis, Maya B Lodish
BACKGROUND: Little is known about the contribution of racial and socioeconomic disparities to severity and outcomes for children with Cushing disease (CD). METHODS: 129 children with CD, 45 Hispanic/Latino or African American (HI/AA) and 84 non-Hispanic White (non-HW), are included. A 10-point index for rating severity (CD-severity) incorporated degree of hypercortisolemia, glucose tolerance, hypertension, anthropomorphic measurements, disease duration, and tumor characteristics...
April 19, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28420444/case-report-acth-secreting-pituitary-carcinoma-metastatic-to-the-liver-in-a-patient-with-a-history-of-atypical-pituitary-adenoma-and-cushing-s-disease
#9
Amy S Joehlin-Price, Douglas A Hardesty, Christina A Arnold, Lawrence S Kirschner, Daniel M Prevedello, Norman L Lehman
BACKGROUND: Pituitary carcinoma is a rare entity requiring the presence of metastasis to confirm its malignant potential. We report a case of pituitary carcinoma and discuss the diagnosis and management of this lesion in relation to the existing literature. CASE PRESENTATION: The patient is a 51-year-old woman with Cushing's disease and intact adrenal glands who was diagnosed with metastatic pituitary carcinoma to the liver, 29 months after initial resection of an ACTH-secreting primary atypical pituitary adenoma (APA)...
April 18, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28380523/genomewide-association-study-reveals-a-risk-locus-for-equine-metabolic-syndrome-in-the-arabian-horse
#10
S L Lewis, H M Holl, C Streeter, C Posbergh, B J Schanbacher, N J Place, M F Mallicote, M T Long, S A Brooks
Equine obesity can cause life-threatening secondary chronic conditions, similar to those in humans and other animal species. Equine metabolic syndrome (EMS), primarily characterized by hyperinsulinemia, is often present in obese horses and ponies. Due to clinical similarities to conditions such as pituitary pars intermedia dysfunction (formerly equine Cushing's disease), conclusive diagnosis of EMS often proves challenging. Aside from changes in diet and exercise, few targeted treatments are available for EMS, emphasizing the need for early identification of at-risk individuals to enable implementation of preventative measures...
March 2017: Journal of Animal Science
https://www.readbyqxmd.com/read/28364356/diabetes-in-cushing-disease
#11
REVIEW
G Mazziotti, A M Formenti, S Frara, F Maffezzoni, M Doga, A Giustina
PURPOSE OF REVIEW: This review focuses on the pathophysiological and clinical aspects of diabetes mellitus occurring in patients with Cushing disease (CD). RECENT FINDINGS: Insulin resistance and impairment in insulin secretion are both involved in the pathogenesis of glucocorticoid-induced diabetes. Correction of glucocorticoid excess does not always resolve abnormalities of glucose homeostasis, and correction of hyperglycaemia is specifically required. In fact, insulin resistance may persist even after correction of glucocorticoid excess and diabetes needs to be treated for long term...
May 2017: Current Diabetes Reports
https://www.readbyqxmd.com/read/28355719/-a-clinical-analysis-of-123-cases-of-primary-empty-sella
#12
J Li, H W Jia, C L Wang, R Zhang, M Y Qu, W Li, M H Yuan, J Cui, Q He, H Y Wei, T H Zhu, Z S Ma, W Liu, Z L Dong, Z G Gao
Objective: This study was conducted to analyze the clinical characteristics and pituitary function of patients with primary empty sella (PES). Methods: The clinical data from 123 hospitalized adult patients with PES from January 2010 to May 2016 were retrospectively studied. Results: (1) The average age of the 123 (male 43, female 80) PES patients was (59.2±13.6) years (ranging 24-92 years), among whom 61% patients were in the age group between 50-69 years. (2) The symptoms of the patients included fatigue (56...
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28275992/follow-up-intervals-in-patients-with-cushing-s-disease-recommendations-from-a-panel-of-experienced-pituitary-clinicians
#13
Eliza B Geer, Alejandro Ayala, Vivien Bonert, John D Carmichael, Murray B Gordon, Laurence Katznelson, Ekaterina Manuylova, Ismat Shafiq, Vijaya Surampudi, Ronald S Swerdloff, Michael S Broder, Dasha Cherepanov, Marianne Eagan, Jackie Lee, Qayyim Said, Maureen P Neary, Beverly M K Biller
PURPOSE: Follow-up guidelines are needed to assess quality of care and to ensure best long-term outcomes for patients with Cushing's disease (CD). The purpose of this study was to assess agreement by experts on recommended follow-up intervals for CD patients at different phases in their treatment course. METHODS: The RAND/UCLA modified Delphi process was used to assess expert consensus. Eleven clinicians who regularly manage CD patients rated 79 hypothetical patient scenarios before and after ("second round") an in-person panel discussion to clarify definitions...
March 9, 2017: Pituitary
https://www.readbyqxmd.com/read/28226363/coagulation-profile-in-patients-with-different-etiologies-for-cushing-syndrome-a-prospective-observational-study
#14
Amit Tirosh, Maya Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Richard A Feelders, Constantine A Stratakis
Previous studies reported a higher prevalence of venous-thromboembolic events among patients with Cushing disease (CD) compared to those with ACTH-independent Cushing syndrome (CS) from adrenal sources. The objective of the current study was to evaluate the coagulation profile of patients with CS from different etiologies. A prospective observational study was conducted at a clinical research center. The study included adult patients admitted for evaluation of suspected CS (n=85), that were divided into 3 groups: CD (n=22), ACTH-independent CS from an adrenal tumor/hyperplasia (adrenal CS, n=21), and a control group consisting of subjects with negative screening for CS (rule-out CS, n=42)...
May 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28149109/developing-cerebral-venous-infarct-presenting-with-seizure-occurring-after-lumbar-drain-placement-following-trans-sphenoidal-surgery-of-cushing-s-disease-review-of-literature
#15
Dattaraj Sawarkar, Guru Dutta Satyarthee, Pankaj Singh, Hitesh Gurjar, M M Singh, B S Sharma
No abstract text is available yet for this article.
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28079576/mixed-gangliocytoma-pituitary-adenoma-insights-on-the-pathogenesis-of-a-rare-sellar-tumor
#16
M Beatriz S Lopes, Emily Sloan, Julie Polder
Gangliocytomas originating in the sellar region are rare; most are tumors composed of gangliocytic and pituitary adenomatous elements, forming the so-called mixed gangliocytoma-pituitary adenoma. The majority of mixed gangliocytoma adenomas are associated with endocrinopathies, mainly acromegaly and less often Cushing disease and hyperprolactinemia. In the present study, 10 cases of mixed gangliocytoma and somatotroph adenomas were evaluated for patterns of cellular differentiation and expression of lineage-specific transcription factors...
May 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27941732/glycosylated-hemoglobin-in-different-levels-of-bmi
#17
R K Das, A Nessa, M Asrin, M Z Huq, M F Husain, S Sufrin, R Zannat, T Tajkia
Body mass index is an inexpensive and easy method of screening for weight categories that may lead to health problems. Increased BMI in overweight and obese persons is directly associated with an increase in metabolic disease, such as type 2 diabetes mellitus. This Analytical cross sectional study was undertaken to assess the relation between increasing BMI and glycemic control of body through measuring glycosylated hemoglobin. This study was carried out in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh from July 2011 to June 2012 on 180 equally divided male and female persons between the age of 25 to 55 years...
October 2016: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/27894193/currently-used-and-investigational-drugs-for-cushing%C3%A2-s-disease
#18
REVIEW
Denis Ciato, Aizhar G Mumbach, Marcelo Paez-Pereda, Günter K Stalla
Cushing's disease (CD) is caused by a corticotroph adenoma of the pituitary gland that secretes excess adrenocorticotropic hormone (ACTH) causing increased morbidity and mortality. Surgery is the treatment of choice, but is not always successful. Alternatives include radiotherapy, adrenal surgery, and pharmaceutical therapy. The latter is increasingly gaining momentum due to the recent development of compounds that reduce hypercortisolaemia or its symptoms, acting through different mechanisms. Areas covered: In this article, the authors provide a complete overview of the treatment options for Cushing´s disease, including adrenal-directed, tumor-targeted, and peripheral therapies that are currently used or in development, and discuss their potential advantages and limitations...
January 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/27882768/-adrenocortical-oncocytoma-presenting-as-cushing%C3%A2-s-syndrome-in-pregnancy-with-spontaneous-postpartum-uterine-rupture
#19
M Kotoulová, I Mikysková, J Dušková, J Vláčil, M Dvořák, M Halaška
OBJECTIVE: The description of rare case of the Cushings syndrome in pregnancy resulting in the spontaneous rupture of uterus. DESIGNS: A case report. SETTING: Department of Gynecology and Obstetrics, 1st Medical Faculty of Charles University and Hospital Na Bulovce. CASE REPORT: The authors report the case of a 33 year old woman, who was admitted to the clinic in 30th week of pregnancy for elevated blood pressure and hypokalemia...
2016: Ceská Gynekologie
https://www.readbyqxmd.com/read/27869719/targeted-molecular-imaging-in-adrenal-disease-an-emerging-role-for-metomidate-pet-ct
#20
REVIEW
Iosif A Mendichovszky, Andrew S Powlson, Roido Manavaki, Franklin I Aigbirhio, Heok Cheow, John R Buscombe, Mark Gurnell, Fiona J Gilbert
Adrenal lesions present a significant diagnostic burden for both radiologists and endocrinologists, especially with the increasing number of adrenal 'incidentalomas' detected on modern computed tomography (CT) or magnetic resonance imaging (MRI). A key objective is the reliable distinction of benign disease from either primary adrenal malignancy (e.g., adrenocortical carcinoma or malignant forms of pheochromocytoma/paraganglioma (PPGL)) or metastases (e.g., bronchial, renal). Benign lesions may still be associated with adverse sequelae through autonomous hormone hypersecretion (e...
November 18, 2016: Diagnostics
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