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https://www.readbyqxmd.com/read/28540626/cross-sectional-prevalence-of-pancreatic-cystic-lesions-in-patients-with-acromegaly-a-single-center-experience
#1
Yukiko Odake, Hidenori Fukuoka, Masaaki Yamamoto, Yoshifumi Arisaka, Junya Konishi, Kenichi Yoshida, Ryusaku Matsumoto, Hironori Bando, Kentaro Suda, Hitoshi Nishizawa, Genzo Iguchi, Shozo Yamada, Wataru Ogawa, Yutaka Takahashi
PURPOSE: Acromegaly is a disease associated with an increased risk for several kinds of neoplasms including colon and thyroid cancer. Although the association between acromegaly and pancreatic neoplasms has not been elucidated, it has recently been reported that GNAS gene mutations were found in 58% of intraductal papillary mucinous neoplasms (IPMNs), which are representative pancreatic cystic lesions, suggesting a link between IPMNs and acromegaly. To assess the prevalence of pancreatic cystic lesions in patients with acromegaly, we performed a retrospective cross-sectional single institute study...
May 24, 2017: Pituitary
https://www.readbyqxmd.com/read/28540625/pituitary-dysfunction-in-granulomatosis-with-polyangiitis
#2
REVIEW
Daniela Esposito, Penelope Trimpou, Dario Giugliano, Mats Dehlin, Oskar Ragnarsson
PURPOSE: Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD). METHODS: A retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed...
May 24, 2017: Pituitary
https://www.readbyqxmd.com/read/28537398/discovery-of-clinical-candidate-2-2s-6s-2-phenyl-6-hydroxyadamantan-2-yl-1-3-hydroxyazetidin-1-yl-ethanone-bms-816336-an-orally-active-novel-selective-11%C3%AE-hydroxysteroid-dehydrogenase-type-1-inhibitor
#3
Xiang-Yang Ye, Stephanie Y Chen, Shung Wu, David S Yoon, Haixia Wang, Zhenqiu Hong, Stepen P O'Connor, Jun Li, James J Li, Lawrence L Kennedy, Steven J Walker, Akbar Nayeem, Steven Sheriff, Daniel M Camac, Vidyhashankar Ramamurthy, Paul E Morin, Rachel Zebo, Joseph R Taylor, Nathan N Morgan, Randolph P Ponticiello, Thomas Harrity, Atsu Apedo, Rajasree Golla, Ramakrishna Seethala, Mengmeng Wang, Timothy W Harper, Bogdan G Sleczka, Bin He, Mark Kirby, David K Leahy, Jianqing Li, Ronald L Hanson, Zhiwei Guo, Yi-Xin Li, John D DiMarco, Raymond Scaringe, Brad D Maxwell, Frederick Moulin, Joel C Barrish, David A Gordon, Jeffrey A Robl
BMS-816336 (6n-2), a hydroxyl-substituted adamantyl acetamide, has been identified as a novel, potent inhibitor against human 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) enzyme (IC50 3.0 nM) with >10000 fold selectivity over human 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2). BMS-816336 exhibits a robust acute pharmacodynamic effects in cynomolgus monkeys (ED50 0.13 mpk) and in DIO mice. It is orally bioavailable (%F ranges from 20-72% in preclinical species) and has a predicted pharmacokinetic profile of a high peak to trough ratio and short half-life in humans...
May 24, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28536363/update-on-treatment-guideline-in-fibromyalgia-syndrome-with-focus-on-pharmacology
#4
REVIEW
Sanam Kia, Ernet Choy
Fibromyalgia syndrome (FMS) is a chronic condition with unknown aetiology. The pathophysiology of the disease is incompletely understood; despite advances in our knowledge with regards to abnormal central and peripheral pain processing, and hypothalamo-pituitary-adrenal dysfunction, there is no clear specific pathophysiological therapeutic target. The management of this complex condition has thus perplexed the medical community for many years, and several national and international guidelines have aimed to address this complexity...
May 8, 2017: Biomedicines
https://www.readbyqxmd.com/read/28535535/inflammation-is-an-important-covariate-for-the-crosstalk-of-sleep-and-the-hpa-axis-in-rheumatoid-arthritis
#5
Rainer H Straub, Jaqueline Detert, René Dziurla, Ingo Fietze, Peter-Andreas Loeschmann, Gerd R Burmester, Frank Buttgereit
Rheumatoid arthritis (RA) patients have sleep problems, and inflammation influences sleep. We demonstrated that sleep quality improves during intensified treatment with methotrexate (MTX) or etanercept (ETA). Since the hypothalamic-pituitary-adrenal (HPA) axis is involved in sleep regulation, this study investigated the interrelation between sleep parameters, inflammation as objectified by C-reactive protein (CRP), and serum cortisol and adrenocorticotropic hormone (ACTH) levels. Thirty-one eligible patients (disease activity score, DAS28CRP ≥3...
May 24, 2017: Neuroimmunomodulation
https://www.readbyqxmd.com/read/28533356/loss-of-function-mutations-in-the-cables1-gene-are-a-novel-cause-of-cushing-s-disease
#6
Laura C Hernández-Ramírez, Ryhem Gam, Nuria Valdés, Maya Lodish, Nathan Pankratz, Aurélio Balsalobre, Yves Gauthier, Fabio R Faucz, Giampaolo Trivellin, Prashant Chittiboina, John Lane, Denise M Kay, Aggeliki Dimopoulou, Stephane Gaillard, Mario Neou, Jerome Bertherat, Guillaume Assié, Chiara Villa, James L Mills, Jacques Drouin, Constantine A Stratakis
The CABLES1 cell cycle regulator participates in the adrenal-pituitary negative feedback, and its expression is reduced in corticotropinomas, pituitary tumors with a largely unexplained genetic basis. We investigated the presence of CABLES1 mutations/copy number variations (CNVs) and their associated clinical, histopathological and molecular features in patients with Cushing's disease (CD). Samples from 146 pediatric (118 germline DNA only/28 germline and tumor DNA) and 35 adult (tumor DNA) CD patients were screened for CABLES1 mutations...
May 22, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28532906/first-report-of-coexistence-of-2-ectopic-pituitary-tumors-rathke-cleft-cyst-and-silent-acth-adenoma
#7
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/28532436/vinblastine-chemotherapy-in-adult-patients-with-langerhans-cell-histiocytosis-a-multicenter-retrospective-study
#8
Abdellatif Tazi, Gwenaël Lorillon, Julien Haroche, Antoine Neel, Stéphane Dominique, Achille Aouba, Jean-David Bouaziz, Constance de Margerie-Melon, Emmanuelle Bugnet, Vincent Cottin, Thibault Comont, Christian Lavigne, Jean-Emmanuel Kahn, Jean Donadieu, Sylvie Chevret
BACKGROUND: Vinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this treatment could be extended to adults with LCH is questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% with multisystem LCH) who were treated with vinblastine + steroids as a first-line chemotherapy and followed for a median time of 83 months. The objectives were to determine the overall response rate (based on the Histiocyte Society criteria), disease reactivation rate, toxicity, permanent consequences, and survival rate corresponding to this treatment...
May 22, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28530316/-radiosurgery-for-pituitary-adenomas
#9
REVIEW
Or Cohen-Inbar
Pituitary adenomas represent one of the most common types of intracranial tumors, accounting for 10-20% of intracranial tumors. While their macroscopic appearance and anatomical location are relatively homogeneous, pituitary tumors differ widely, generating a variety of neurological and endocrine clinical sequelae. Treatment options include hormone suppressive medical therapy, microscopic or endoscopic neurosurgical resection, radiosurgery, radiation therapy, or observation depending on the biochemical profile and the clinical status of the patient...
January 2017: Harefuah
https://www.readbyqxmd.com/read/28530019/men1-in-children-and-adolescents-data-from-patients-of-a-regional-referral-center-for-hereditary-endocrine-tumors
#10
Letizia Vannucci, Francesca Marini, Francesca Giusti, Simone Ciuffi, Francesco Tonelli, Maria Luisa Brandi
PURPOSE: To retrospectively evaluate the age of onset of MEN1-associated lesions in a group of affected children and adolescents and to compare the clinical features of our series with the evidence derived from the literature. METHODS: The study population consisted of 22 Italian children and adolescents (age 6-31 years at the time of the inclusion in this study) all with a clinical and/or a genetic diagnosis of MEN1 performed before the age of 16 who have been followed-up regularly from 1998 to 2016 at the Regional Referral Center for Hereditary Endocrine Tumors...
May 22, 2017: Endocrine
https://www.readbyqxmd.com/read/28529722/recent-advances-in-understanding-cushing-disease-resistance-to-glucocorticoid-negative-feedback-and-somatic-usp8-mutations
#11
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
https://www.readbyqxmd.com/read/28527578/tr2-and-tr4-orphan-nuclear-receptors-an-overview
#12
Shin-Jen Lin, Dong-Rong Yang, Guosheng Yang, Chang-Yi Lin, Hong-Chiang Chang, Gonghui Li, Chawnshang Chang
Testicular nuclear receptors 2 and 4 (TR2, TR4), also known as NR2C1 and NR2C2, belong to the nuclear receptor superfamily and were first cloned in 1989 and 1994, respectively. Although classified as orphan receptors, several natural molecules, their metabolites, and synthetic compounds including polyunsaturated fatty acids (PUFAs), PUFA metabolites 13-hydroxyoctadecadienoic acid, 15-hydroxyeicosatetraenoic acid, and the antidiabetic drug thiazolidinediones can transactivate TR4. Importantly, many of these ligands/activators can also transactivate peroxisome proliferator-activated receptor gamma (PPARγ), also known as NR1C3 nuclear receptor...
2017: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/28527569/estrogen-hormone-biology
#13
Katherine J Hamilton, Sylvia C Hewitt, Yukitomo Arao, Kenneth S Korach
The hormone estrogen is involved in both female and male reproduction, as well as numerous other biological systems including the neuroendocrine, vascular, skeletal, and immune systems. Therefore, it is also implicated in many different diseases and conditions such as infertility, obesity, osteoporosis, endometriosis, and a variety of cancers. Estrogen works through its two distinct nuclear receptors, estrogen receptor alpha (ERα) and estrogen receptor beta (ERβ). Various transcriptional regulation mechanisms have been identified as the mode of action for estrogen, mainly the classical mechanism with direct DNA binding but also a nongenomic mode of action and one using tethered or indirect binding...
2017: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/28523537/prolactinoma-management-predictors-of-remission-and-recurrence-after-dopamine-agonists-withdrawal
#14
Margarida Teixeira, Pedro Souteiro, Davide Carvalho
OBJECTIVE: Prolactinomas are the most common functional pituitary tumour. Dopamine agonists (DA) are its principal treatment. The criteria that should guide therapy withdrawal and the factors that influence disease remission or relapse are not yet fully established. Our purpose is to evaluate the proportion of patients who attempted DA withdrawal, and to identify the factors that influence clinicians to try it. In addition, we aim to study the factors that are involved in prolactinoma remission/relapse after therapy withdrawal...
May 18, 2017: Pituitary
https://www.readbyqxmd.com/read/28522647/prkar1a-mutation-causing-pituitary-dependent-cushing-disease-in-a-patient-with-carney-complex
#15
Florian W Kiefer, Yvonne Winhofer, Donato Iacovazzo, Marta Korbonits, Stefan Wolfsberger, Engelbert Knosp, Franz Trautinger, Romana Höftberger, Michael Krebs, Anton Luger, Alois Gessl
CONTEXT: Carney complex (CNC) is an autosomal dominant condition caused, in most cases, by an inactivating mutation of the PRKAR1A gene, which encodes for the type 1 alpha regulatory subunit of protein kinase A. CNC is characterized by the occurrence of endocrine overactivity, myxomas and typical skin manifestations. Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine disease observed in CNC. CASE DESCRIPTION: Here we describe the first case of a patient with CNC and adrenocorticotropic hormone (ACTH)-dependent Cushing disease due to a pituitary corticotroph adenoma...
May 18, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28520927/preoperative-stratification-of-transsphenoidal-pituitary-surgery-patients-based-on-surgical-urgency
#16
Hasan A Zaidi, Amy J Wang, David J Cote, Timothy R Smith, Daniel Prevedello, Domenico Solari, Paolo Cappabianca, Monica Quiroga, Edward R Laws
BACKGROUND: Currently, there is no prioritization scale available to distinguish those patients with pituitary tumors who require urgent surgical intervention from those who are candidates for elective treatment. OBJECTIVE: To develop a classification system that can help primary care physicians, endocrinologists, neurosurgeons, ancillary support staff, and hospital administrators identify high-priority surgical candidates. METHODS: An expert international panel of clinicians consisting of endocrinologists and neurosurgeons who are involved in the diagnosis and management of sellar disease was convened...
May 18, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28520590/role-of-tyrosine-kinase-inhibitors-in-the-treatment-of-pituitary-tumours-from-bench-to-bedside
#17
Anat Ben-Shlomo, Odelia Cooper
PURPOSE OF REVIEW: Treatment of aggressive pituitary tumours often yields suboptimal control of the tumour and confers significant morbidity. Lactotroph and corticotroph-derived tumours express ErbB receptors and ligands, and mutations in ubiquitin-specific protease 8 (USP8), which alters epidermal growth factor receptor (EGFR) degradation, have been implicated in Cushing disease pathogenesis. EGFR tyrosine kinase inhibitor (TKI) therapy has emerged as a potential new therapeutic approach for patients with aggressive prolactinomas and Cushing disease...
May 17, 2017: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/28515030/congenital-central-hypothyroidism-caused-by-a-novel-tsh-beta-subunit-gene-mutation-c-94g-a-in-two-siblings
#18
Bayram Özhan, Özlem Boz Anlaş, Bilge Sarıtepe, Burcu Albuz, Nur Gündüz Semerci
Congenital central hypothyroidism (C-CH) is a very rare disease. Alterations in genes included in pituitary development, mutations of the immunoglobulin superfamily member 1 (IGSF1) and transducin β-like protein 1(TBL1X ) can result in C-CH with multiple pituitary hormone deficiencies. However, mutations of the thyrotropin-releasing hormone receptor or TSH-beta (TSHB) gene are responsible for isolated congenital central hypothyroidism. In this paper, we present 2 patients from the same Turkish family with a novel mutation of TSHB...
May 17, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28511047/birth-weight-and-postnatal-growth-in-preterm-born-children-are-associated-with-cortisol-in-early-infancy-but-not-at-age-8-years
#19
Charlotte A Ruys, Bibian van der Voorn, Harrie N Lafeber, Monique van de Lagemaat, Joost Rotteveel, Martijn J J Finken
BACKGROUND: Preterm birth has been associated with altered hypothalamic-pituitary-adrenal (HPA-) axis activity as well as cardiometabolic diseases and neurodevelopmental impairments later in life. We assessed cortisol from term age to age 8 y in children born preterm, to explore the development of HPA-axis activity in association with intrauterine and early-postnatal growth until 6 mo. corrected age. METHODS: In 152 children born at a gestational age ≤32 wks. and/or with a birth weight ≤1,500g, random serum cortisol was assessed at term age (n=150), 3 mo...
May 4, 2017: Psychoneuroendocrinology
https://www.readbyqxmd.com/read/28510653/chronic-corticosterone-treatment-during-adolescence-has-significant-effects-on-metabolism-and-skeletal-development-in-male-c57bl6-n-mice
#20
Scott A Kinlein, Ziasmin Shahanoor, Russell D Romeo, Ilia N Karatsoreos
Glucocorticoids are potent modulators of metabolic and behavioral function. Their role as mediators in the "stress response" is well known, but arguably their primary physiological function is in the regulation of cellular and organismal metabolism. Disruption of normal glucocorticoid function is linked to metabolic disease, such as Cushing's syndrome. Glucocorticoids are also elevated in many forms of obesity, suggesting that there are bidirectional effects of these potent hormones on metabolism and metabolic function...
May 15, 2017: Endocrinology
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