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https://www.readbyqxmd.com/read/29664314/iran-pituitary-tumor-registry-description-of-the-program-and-initial-results
#1
Mohammad Ebrahim Khamseh, Mohammad Reza Mohajeri Tehrani, Zohreh Mousavi, Mojtaba Malek, Mehrnaz Imani, Nasim Hoshangian Tehrani, Mohammad Ghorbani, Hamideh Akbari, Farzaneh Sarvghadi, Atieh Amouzegar, Fatemeh Esfahanian, Nahid Hashemi Madani, Zahra Emami
BACKGROUND: This study was designed to present initial results on clinical presentation, therapeutic modalities, and outcome information of patients with pituitary tumors registered in Iran Pituitary Tumor Registry (IPTR). METHODS: We collected data from a web-based electronic medical records of patients with various pituitary tumors referred to four tertiary care centers in the country. Retrospective analysis was performed on demographic, clinical, and therapeutic information of 298 patients including 51 clinically nonfunctioning adenoma (CNFA), 85 acromegaly, 135 prolactinoma, and 27 Cushing's disease (CD)...
December 31, 2017: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/29661784/mechanisms-in-endocrinology-a-sense-of-time-of-the-glucocorticoid-circadian-clock-from-the-ontogeny-to-the-diagnosis-of-cushing-s-syndrome
#2
Ayrton Custodio Moreira, Sonir Roberto R Antonini, Margaret De Castro
The circadian rhythm of glucocorticoids has long been recognised within the last 75 years. Since the beginning, researchers have sought to identify basic mechanisms underlying the origin and emergence of the corticosteroid circadian rhythmicity among mammals. Accordingly, Young, Hall and Rosbash, laureates of the 2017 Nobel Prize in Physiology or Medicine, as well as Takahashi's group among others, have characterised the molecular cogwheels of the circadian system, describing interlocking transcription/translation feedback loops essential for normal circadian rhythms...
April 16, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29650225/an-update-on-cushing-syndrome-in-pediatrics
#3
Constantine A Stratakis
Cushing syndrome (CS) in childhood results mostly from the exogenous administration of glucocorticoids; endogenous CS is a rare disease. The latter is the main reason pediatric patients with CS escape diagnosis for too long. Other barriers to optimal care of a pediatric patient with CS include improper following of the proper sequence of testing for diagnosing CS, which stems from lack of understanding of pathophysiology of the hypothalamic-pituitary-adrenal axis; lack of access to proper (i.e., experienced, state-of-the-art) surgical treatment; and unavailability of well-tolerated and effective medications to control hypercortisolemia...
April 9, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29626059/evaluation-of-lipid-profile-and-its-relationship-with-blood-pressure-in-patients-with-cushing-s-disease
#4
Lang Qin, Xiaoming Zhu, Xiaoxia Liu, Meifang Zeng, Ran Tao, Yan Zhuang, Yiting Zhou, Zhaoyun Zhang, Yehong Yang, Yi-Ming Li, Yongfei Wang, Hongying Ye
INTRODUCTION: The purpose of the study was to describe lipid profile and explore pathogenetic role of LDL-c on hypertension in patients with Cushing's disease (CD).Hypertension is a common feature in patients with CD. Previous study found low-density lipoprotein-cholesterol (LDL-c) uptake in vascular cells might be involved in vascular remodeling in patients with CD. Therefore, we evaluated the relationship between lipid profile and the blood pressure in patients with CD. METHODS: This retrospective study included 84 patients referred to Huashan Hospital for evaluation and diagnosis of CD from January 2012 to December 2013...
April 6, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29625700/quality-of-life-in-cushing-s-disease-a-long-term-issue
#5
Susan M Webb, Alicia Santos, Eugenia Resmini, Maria-Antonia Martínez-Momblán, Luciana Martel, Elena Valassi
The purpose of this review is to describe how quality of life (QoL) is impaired in patients with hypercortisolism due to Cushing's syndrome of any aetiology, including pituitary-dependent Cushing's disease. It is worse in active disease, but improvement after successful therapy is often incomplete, due to persistent physical and psychological co-morbidities, even years after endocrine "cure". Physical symptoms like extreme fatigability, central obesity with limb atrophy, hypertension, fractures, and different skin abnormalities severely impair the affected patients' everyday life...
April 3, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29606280/cardiovascular-mortality-in-patients-with-subclinical-cushing
#6
Valentina Morelli, Maura Arosio, Iacopo Chiodini
Patients with adrenal incidentaloma (AI) and subclinical hypercortisolism (SH) show a high prevalence of cardiovascular risk factors and an increased prevalence and incidence of cardiovascular events. Furthermore, some recent data suggest that in these patients, the cardiovascular mortality is also increased. Unfortunately, to date, the diagnosis of SH is still a matter of debate, and, therefore, it is still not possible to address the treatment of choice (i.e. surgical or conservative approach) in many AI patients...
March 29, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29600293/-usp8-mutations-in-pituitary-cushing-adenomas-targeted-analysis-by-next-generation-sequencing
#7
Cora Ballmann, Anne Thiel, Hannah E Korah, Anna-Carinna Reis, Wolfgang Saeger, Stefanie Stepanow, Karl Köhrer, Guido Reifenberger, Christiane B Knobbe-Thomsen, Ulrich J Knappe, Ute I Scholl
Gain-of-function somatic mutations in the ubiquitin specific protease 8 ( USP8 ) gene have recently been reported as a cause of pituitary adenomas in Cushing disease. Molecular diagnostic testing of tumor tissue may aid in the diagnosis of specimens obtained through therapeutic transsphenoidal surgery; however, for small tumors, availability of fresh tissue is limited, and contamination with normal tissue is frequent. We performed molecular testing of DNA isolated from single formalin-fixed and paraffin-embedded (FFPE) tissue sections of 42 pituitary adenomas from patients with Cushing disease (27 female patients and 15 male patients; mean age at surgery, 42...
March 1, 2018: Journal of the Endocrine Society
https://www.readbyqxmd.com/read/29587644/a-novel-germline-armc5-mutation-in-a-patient-with-bilateral-macronodular-adrenal-hyperplasia-a-case-report
#8
Qiuli Liu, Dali Tong, Jing Xu, Xingxia Yang, Yuting Yi, Dianzheng Zhang, Luofu Wang, Jun Zhang, Yao Zhang, Yaoming Li, Lianpeng Chang, Rongrong Chen, Yanfang Guan, Xin Yi, Jun Jiang
BACKGROUND: Bilateral macronodular adrenal hyperplasia (BMAH) is a rare cause of Cushing's syndrome (CS). BMAH is predominantly believed to be caused by two mutations, a germline and somatic one, respectively, as described in the two-hit hypothesis. In many familial cases of BMAH, mutations in armadillo repeat containing 5 (ARMC5), a putative tumor suppressor gene, are thought to induce the disorder. The objective of this study was to report a case in which the patient presented with BMAH induced by a novel heterozygous germline ARMC5 mutation (c...
March 27, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29576599/new-diagnostic-criteria-of-adrenal-subclinical-cushing-s-syndrome-opinion-from-the-japan-endocrine-society
#9
Toshihiko Yanase, Yutaka Oki, Takuyuki Katabami, Michio Otsuki, Kazunori Kageyama, Tomoaki Tanaka, Hisaya Kawate, Makito Tanabe, Masaru Doi, Yuko Akehi, Takamasa Ichijo
New diagnostic criteria and the treatment policy for adrenal subclinical Cushing's syndrome (SCS) are proposed on behalf of the Japan Endocrine Society. The Japanese version has been published, and the essential contents are presented in this English-language version. The current diagnostic criteria for SCS have elicited two main problems: (i) the relatively low reliability of a low range of serum cortisol essential for the diagnosis by an overnight 1-mg dexamethasone suppression test (DST); (ii) different cutoff values for serum cortisol after a 1-mg DST compared with those of other countries...
March 23, 2018: Endocrine Journal
https://www.readbyqxmd.com/read/29575160/association-of-serious-infections-with-pemphigus-and-pemphigoid-analysis-of-the-nationwide-inpatient-sample
#10
Ziyou Ren, Shanthi Narla, Derek Y Hsu, Jonathan I Silverberg
BACKGROUND: Pemphigus and pemphigoid are blistering disorders associated with barrier disruption, immune dysregulation and use of immunosuppressing systemic therapy, which may predispose toward serious infections. OBJECTIVES: To determine whether pemphigus and pemphigoid are associated with increased likelihood of serious infections, and the impact of such infections on mortality and cost of care. METHODS: We analyzed data from the 2002-2012 National Inpatient Sample, including a representative 20% sample of all hospitalizations in the US (total n=72,108,077 adults)...
March 25, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29574994/worse-health-related-quality-of-life-at-long-term-follow-up-in-patients-with-cushing-s-disease-than-patients-with-cortisol-producing-adenoma-data-from-the-ercusyn
#11
Elena Valassi, Richard Feelders, Dominique Maiter, Philippe Chanson, Maria Yaneva, Martin Reincke, Michal Krsek, Miklós Tóth, Susan M Webb, Alicia Santos, Isabel Paiva, Irina Komerdus, Michael Droste, Antoine Tabarin, Christian J Strasburger, Holger Franz, Peter J Trainer, John Newell-Price, John A H Wass, Eleni Papakokkinou, Oskar Ragnarsson
OBJECTIVE: Hypercortisolism in Cushing's syndrome (CS) is associated with impaired Health-Related Quality of Life (HRQoL), which may persist despite remission. We used the data entered into the European Registry on Cushing's syndrome (ERCUSYN) to evaluate if patients with CS of pituitary origin (PIT-CS) have worse HRQoL, both before and after treatment, than patients with adrenal causes (ADR-CS). METHODS: Data from 595 patients (492 women; 83%) who completed the CushingQoL and/or EQ-5D questionnaires at baseline and/or following treatment were analyzed...
March 24, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29554355/bilateral-adrenalectomy-in-congenital-adrenal-hyperplasia-a-systematic-review-and-meta-analysis
#12
Diana MacKay, Anna Nordenström, Henrik Falhammar
Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal (HPA) axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing's syndrome being a frequent complication of adequate suppression. Bilateral adrenalectomy, with subsequent replacement of glucocorticoids and mineralocorticoids at physiological doses, has been proposed as an alternative therapeutic strategy. Objective: To review the outcomes following bilateral adrenalectomy for CAH...
March 15, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29550934/specificity-of-late-night-salivary-cortisol-measured-by-automated-electrochemiluminescence-immunoassay-for-cushing-s-disease-in-an-obese-population
#13
J Aberle, C Schulze Zur Wiesch, J Flitsch, J Veigel, G Schön, R Jung, F Reining, A Lautenbach, R Rotermund, N Riedel
PURPOSE: Data about the specificity of late-night salivary cortisol (LNSC) in obese subjects are still conflicting. Therefore, with this study, we aimed to evaluate the specificity of LNSC measurement in an obese cohort with or without type 2 diabetes mellitus (T2DM) using an automated electrochemiluminescence immunoassay (ECLIA). METHODS: A total number of 157 patients involving 40 healthy subjects (HS) with BMI < 25 kg/m2 , 83 obese subjects (OS) with BMI ≥ 35 kg/m2 , and 34 histopathologically proven Cushing's disease (CD) were included...
March 17, 2018: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/29535866/case-series-of-paraneoplastic-cushing-syndrome-in-small-cell-lung-cancer
#14
Carine Ghassan Richa, Khadija Jamal Saad, Georges Habib Halabi, Elie Mekhael Gharios, Fadi Louis Nasr, Marie Tanios Merheb
The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29532430/complete-evaluation-of-pituitary-tumours-in-a-single-tertiary-care-institution
#15
Dimitrios Askitis, Damianos Tsitlakidis, Nicolle Müller, Albrecht Waschke, Gunter Wolf, Ulrich Alfons Müller, Christof Kloos
INTRODUCTION: We retrospectively evaluated all patients with pituitary tumours treated in our department from 1/1/1997 to 01/11/2014. PATIENTS AND METHODS: Two hundred and fifteen patients (124 females, 91 males, mean age 50.9 years) were treated because of pituitary tumours. All patients underwent basal hormonal analysis and when required dynamic testing in order to check for hormonal activity. Pituitary masses were divided into groups concerning their hormonal status and were further classified according to gender, age at diagnosis, tumour size, and the development of postoperative pituitary insufficiency when neurosurgical intervention was conducted...
March 12, 2018: Endocrine
https://www.readbyqxmd.com/read/29525561/secreting-ectopic-adrenal-adenoma-a-rare-condition-to-be-aware-of
#16
Yang Zhao, Hui Guo, Ying Zhao, Bingyin Shi
Ectopic adrenal adenoma causing chronic Cushing's syndrome (CS) is a rare phenomenon. Diagnosis is usually made years after disease onset because of the insidious nature of the ectopic adrenal gland and because it overlaps with common symptoms, such as overweight and hypertension, in the general population (Kreitschmann-Andermahr et al., 2015). Here, we report the case of a 46-year-old male with a 15-year history of severe hypertension, facial plethora, and centripetal obesity. During treatment for herpes zoster, the patient presented with severe hypokalemia and flaccid paralysis, characteristic changes associated with CS...
March 7, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29510671/an-unexpected-mild-phenotype-of-glucocorticoid-resistance-associated-with-glucocorticoid-receptor-gene-mutation-case-report-and-review-of-the-literature
#17
Ágnes Molnár, Attila Patócs, István Likó, Gábor Nyírő, Károly Rácz, Miklós Tóth, Beatrix Sármán
BACKGROUND: Glucocorticoid resistance is a rare, sporadic or familial condition caused by mutation of the gene encoding the glucocorticoid receptor (GR). Clinically it is characterized by symptoms developed due to local, tissue-specific, or generalized partial insensitivity to glucocorticoids. CASE PRESENTATION: A 31-year-old woman was evaluated because of infertility at the Endocrine Unit of the 2nd Department of Medicine, Semmelweis University. During her laboratory investigations, elevated serum and salivary cortisol were observed which failed to be suppressed after administration of 1 mg dexamethasone...
March 6, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29472379/diagnosis-of-endocrine-disease-the-role-of-the-desmopressin-test-in-the-diagnosis-and-follow-up-of-cushing-s-syndrome
#18
Dimitra Argyro Vassiliadi, Stylianos Tsagarakis
Desmopressin is a vasopressin analogue selective for type 2 vasopressin receptors that mediate renal water retention. In contrast to the native hormone arginine-vasopressin, a well-known ACTH secretagogue, desmopressin exerts minimal or no activity on ACTH excretion. However, in a substantial proportion of patients with ACTH-dependent Cushing's syndrome (CS), desmopressin elicits an ACTH and cortisol response, which contrasts with the minimal responses obtained in healthy subjects. The mechanism underlying this paradoxical response involves upregulation of vasopressin type 3 and/or the aberrant expression of type 2 receptors by neoplastic ACTH-producing cells...
February 22, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29460202/clinicopathological-significance-of-baseline-t2-weighted-signal-intensity-in-functional-pituitary-adenomas
#19
Sema Ciftci Dogansen, Gulsah Yenidunya Yalin, Seher Tanrikulu, Sakin Tekin, Nihan Nizam, Bilge Bilgic, Serra Sencer, Sema Yarman
PURPOSE: To assess baseline T2-weighted signal intensity (T2-WSI) of functional pituitary adenomas (FPA), and to investigate the relationship of baseline T2-WSI with clinical features, histopathological granulation patterns, and response to treatment in patients with acromegaly, prolactinoma and Cushing's disease (CD). METHODS: Somatotroph adenomas (n = 87), prolactinomas (n = 78) and corticotroph adenomas (n = 29) were included in the study. Baseline T2-WSI findings (grouped as hypo-, iso- and hyperintense) were compared with hormone levels, tumor diameter, granulation patterns and response to treatment...
February 19, 2018: Pituitary
https://www.readbyqxmd.com/read/29458220/incidence-of-autoimmune-and-related-disorders-after-resolution-of-endogenous-cushing-syndrome-in-children
#20
Christina Tatsi, Meg Keil, Charalampos Lyssikatos, Elena Belyavskaya, Constantine A Stratakis, Maya B Lodish
Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here we investigated the presence of autoimmune and related diseases that developed after cure of endogenous Cushing syndrome (CS) in children. We identified 129 children who were diagnosed and successfully treated for endogenous CS at the National Institutes of Health from 1997 until 2017, and who were followed for at least 6 months after treatment...
February 19, 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
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