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Cushing's disease

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https://www.readbyqxmd.com/read/29675075/pediatric-cushing-s-disease-dichotomy-in-lateralization-between-imaging-and-inferior-petrosal-sinus-sampling-with-review-of-literature
#1
Salman Tehran Shaikh, Vikram S Karmarkar, Chandrashekhar Eknath Deopujari
Inferior petrosal sinus sampling (IPSS) is a minimally invasive method done in adrenocorticotrophic hormone (ACTH)-dependent pituitary adenoma cases where magnetic resonance imaging (MRI) either gives equivocal results or fails to localize it. A 12-year-girl with cushingoid features and raised ACTH levels presented to us with two consecutive MRI's done outside showing gross change in the morphology of a left-sided pituitary adenoma. Hence, a bilateral IPSS was done which showed a higher IPS/periphery ACTH ratio on the right side as compared to left...
October 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29672894/unexpected-adverse-effect-of-metyrapone-a-case-report
#2
A Rouland, A Nguyen, C Fourmont, M Lapray, B Vergès, J M Petit, B Bouillet
Hypercortisolism, or Cushing's syndrome, is a disease which can be treated surgically or with medications such as mitotane, ketoconazole or metyrapone. However, the use of metyrapone is not common, and the adverse effects are not well known. A metyrapone overdose is usually associated with gastro-intestinal symptoms and signs of adrenal failure. To our knowledge, no other side effects have been reported in connection with an ingestion of high doses of metyrapone. This article is protected by copyright. All rights reserved...
April 19, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29664314/iran-pituitary-tumor-registry-description-of-the-program-and-initial-results
#3
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
#4
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/29650226/are-cushing-s-disease-patients-curable
#5
Xavier Bertagna
Treatment of Cushing's disease remains a challenge. Whereas pituitary surgery can "cure" the patient and restore a completely normal pituitary adrenal axis, there are immediate failures and late recurrences which ultimately require alternate therapeutic approaches. These are numerous, but so are their drawbacks, and all appear to be "default options". For the future, pituitary adenoma has to remain the "reasonable obsession" of efficient and optimistic therapists….
April 9, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29650225/an-update-on-cushing-syndrome-in-pediatrics
#6
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/29648902/diagnostic-evaluation-in-steroid-induced-myopathy-case-report-suggesting-clinical-utility-of-quantitative-muscle-ultrasonography
#7
Marco Alessandro Minetto, Cristina Caresio, Valentina D'Angelo, Fabio Lanfranco, Lucia Ghizzoni, Silvestro Roatta, Emanuela Arvat, Santosh Kesari
PURPOSE: We present a patient with adrenal Cushing's syndrome causing steroid myopathy. The purpose of the case report is to illustrate the clinical usefulness of quantitative muscle ultrasonography for the assessment of glucocorticoid-induced changes in muscle mass (MM) and structure. METHODS: Assessments of physical performance, muscle strength, MM (i.e., total body skeletal MM, appendicular skeletal MM, and thickness of lower limb muscles), and muscle structure (i...
April 12, 2018: Endocrine Research
https://www.readbyqxmd.com/read/29626059/evaluation-of-lipid-profile-and-its-relationship-with-blood-pressure-in-patients-with-cushing-s-disease
#8
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
#9
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/29623679/pregnancy-outcomes-after-exposure-to-certolizumab-pegol-updated-results-from-a-pharmacovigilance-safety-database
#10
Megan E B Clowse, Angela E Scheuerle, Christina Chambers, Anita Afzali, Alexa B Kimball, John J Cush, Maureen Cooney, Laura Shaughnessy, Mark Vanderkelen, Frauke Förger
BACKGROUND: Anti-tumor necrosis factor medications (anti-TNFs) are effective in controlling chronic inflammatory diseases, but information about their use and safety in pregnancy is limited. Consequently, anti-TNFs are often discontinued early in gestation. Certolizumab pegol (CZP), a PEGylated, Fc-free anti-TNF approved for treatment of rheumatic diseases and/or Crohn's disease, has minimal to no active placental transfer. This project provides information on pregnancy outcomes in women receiving CZP, especially those with early pregnancy exposure...
April 5, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29621813/cushing-s-syndrome-a-historic-review-of-the-treatment-strategies-and-corresponding-outcomes-in-a-single-tertiary-center-over-the-past-half-century
#11
Anelia Nankova, Maria Yaneva, Atanaska Elenkova, Dimitar Tcharaktchiev, Marin Marinov, Asen Hadzhiyanev, Tanyo Sechanov, Georgi Gantchev, Georgi Todorov, Georgi Kirilov, Krasimir Kalinov, Maria Andreeva, Sabina Zacharieva
Cushing's syndrome (CS) is associated with serious comorbidities and an increased mortality rate that could be reduced only if strict biochemical control is achieved. The aim of this study was to show the 50-year experience of a single tertiary center in the management of CS patients - the different treatment modalities used over the years and the corresponding outcomes. It was a retrospective study of a large cohort of patients from the Bulgarian CS database: 613 patients (374 with ACTH-dependent and 239 with ACTH-independent CS)...
April 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29620167/decreased-expression-of-sfrp2-promotes-development-of-the-pituitary-corticotroph-adenoma-by-upregulating-wnt-signaling
#12
Jie Ren, Fangfang Jian, Hong Jiang, Yuhao Sun, Sijian Pan, Changwei Gu, Xiao Chen, Weiqing Wang, Guang Ning, Liuguan Bian, Qingfang Sun
Cushing's disease is primarily caused by pituitary adrenocorticotropin‑secreting adenoma. However, its pathogenesis has remained obscure. In the present study, whole transcriptome analysis was performed by RNA sequencing (RNA‑Seq) and expression of secreted frizzled‑related protein 2 (SFRP2) was decreased in corticotroph tumors compared with normal pituitary glands. Furthermore, the RNA‑Seq results were validated and the expression of SFRP2 in tumor tissues was analyzed by comparing another cohort of 23 patients with Cushing's disease and 3 normal human pituitary samples using reverse transcription‑quantitative polymerase chain reaction, western blot and immunohistochemistry staining...
April 3, 2018: International Journal of Oncology
https://www.readbyqxmd.com/read/29617644/a-transcriptional-circuit-filters-oscillating-circadian-hormonal-inputs-to-regulate-fat-cell-differentiation
#13
Zahra Bahrami-Nejad, Michael L Zhao, Stefan Tholen, Devon Hunerdosse, Karen E Tkach, Sabine van Schie, Mingyu Chung, Mary N Teruel
Glucocorticoid and other adipogenic hormones are secreted in mammals in circadian oscillations. Loss of this circadian oscillation pattern correlates with obesity in humans, raising the intriguing question of how hormone secretion dynamics affect adipocyte differentiation. Using live, single-cell imaging of the key adipogenic transcription factors CEBPB and PPARG, endogenously tagged with fluorescent proteins, we show that pulsatile circadian hormone stimuli are rejected by the adipocyte differentiation control system...
April 3, 2018: Cell Metabolism
https://www.readbyqxmd.com/read/29600293/-usp8-mutations-in-pituitary-cushing-adenomas-targeted-analysis-by-next-generation-sequencing
#14
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/29595102/pasireotide-mechanism-of-action-and-clinical-applications
#15
Nadia Sawicka-Gutaj, Maciej Owecki, Marek Ruchala
Pasireotide (SOM230) is a multi-receptor ligand somatostatin analogue (SSA) developed as the successor of the first-generation SSAs. Currently, pasireotide is recommended for the treatment of patients with Cushing's disease in whom surgery was unsuccessful, and patients with acromegaly who either remain uncontrolled after surgical therapy or in whom tumor resection is not possible. Phase II and III clinical trials have shown pasireotide efficacy in these diseases, with a similar rate of adverse events when compared with first-line SSA, although higher incidence of hyperglycemia has been observed...
March 27, 2018: Current Drug Metabolism
https://www.readbyqxmd.com/read/29594118/the-many-faces-of-primary-aldosteronism-and-cushing-syndrome-a-reflection-of-adrenocortical-tumor-heterogeneity
#16
REVIEW
Ozgur Mete, Kai Duan
Adrenal cortical tumors constitute a heterogeneous group of neoplasms with distinct clinical, morphological, and molecular features. Recent discoveries of specific genotype-phenotype correlations in adrenal cortical adenomas have transformed our understanding of their respective endocrine syndromes. Indeed, a proportion of patients with primary aldosteronism are now known to harbor adrenal cortical adenomas with heterogeneous molecular alterations ( KCNJ5, ATP1A1, ATP2B3 , and CACNA1D ) involving the calcium/calmodulin kinase signaling pathway...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29587644/a-novel-germline-armc5-mutation-in-a-patient-with-bilateral-macronodular-adrenal-hyperplasia-a-case-report
#17
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/29575160/association-of-serious-infections-with-pemphigus-and-pemphigoid-analysis-of-the-nationwide-inpatient-sample
#18
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
#19
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/29570013/value-of-pituitary-gland-mri-at-7-t-in-cushing-s-disease-and-relationship-to-inferior-petrosal-sinus-sampling-case-report
#20
Meng Law, Regina Wang, Chia-Shang J Liu, Mark S Shiroishi, John D Carmichael, William J Mack, Martin Weiss, Danny J J Wang, Arthur W Toga, Gabriel Zada
Cushing's disease is caused by adrenocorticotrophic hormone (ACTH)-secreting pituitary adenomas, which are often difficult to identify on standard 1.5-T or 3-T MRI, including dynamic contrast imaging. Inferior petrosal and cavernous sinus sampling remains the gold standard for MRI-negative Cushing's disease. The authors report on a 27-year-old woman with Cushing's disease in whom the results of standard 1.5-T and 3-T MRI, including 1.5-T dynamic contrast imaging, were negative. Inferior petrosal sinus sampling showed a high central-to-peripheral ACTH ratio (148:1) as well as a right-to-left ACTH gradient (19:1), suggesting a right-sided pituitary microadenoma...
March 23, 2018: Journal of Neurosurgery
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