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https://www.readbyqxmd.com/read/28550366/minimally-invasive-resection-of-adrenal-masses-in-infants-and-children-results-of-a-european-multi-center-survey
#1
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnaki, Piergiorgio Gamba
BACKGROUND: Minimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis. METHOD: Pediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welch's t-test, X-square, Fisher tests, multiple regression model)...
May 26, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28546721/a-rare-presentation-of-infantile-virilization-secondary-to-malignant-etiology
#2
V Dasarwar Nagesh, D Ramkrishnan Santosh, N Datla Sravya
Adrenocortical tumor is a rare malignancy (1-2/million) in children with a heterogenous presentation and generally poor prognosis. We report two cases of adrenocortical carcinoma who presented primarily with virilization along with cushingoid features and hypertension. Both children were managed with surgical resection of tumor, steroid replacement, and antihypertensives.
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28544861/social-buffering-of-stress-in-development-a-career-perspective
#3
Megan R Gunnar
This review provides a broad overview of my research group's work on social buffering in human development in the context of the field. Much of the focus is on social buffering of the hypothalamic-pituitary-adrenocortical (HPA) system, one of the two major arms of the mammalian stress system. This focus reflects the centrality of the HPA system in research on social buffering in the fields of developmental psychobiology and developmental science. However, buffering of the cardiovascular and autonomic nervous system is also discussed...
May 2017: Perspectives on Psychological Science: a Journal of the Association for Psychological Science
https://www.readbyqxmd.com/read/28540670/open-adrenalectomy-in-the-era-of-laparoscopic-surgery-a-review
#4
REVIEW
Giovanni Taffurelli, Claudio Ricci, Riccardo Casadei, Saverio Selva, Francesco Minni
Open adrenalectomy still plays an important role in adrenal surgery. A review of the current literature has been carried out to discuss the main indication to open adrenalectomy with regards to the main adrenal pathologies. The authors underlined the role of open adrenalectomy on the basis of personal experience and a literature review. Indication to open adrenalectomy for adrenal cysts, myelolipomas, pheochromocytomas, metastases, adrenocortical carcinomas and tumour recurrences were analysed and discussed...
May 24, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28540407/a-new-mutation-site-in-the-aire-gene-causes-autoimmune-polyendocrine-syndrome-type-1
#5
Wufei Zhu, Zhen Hu, Xiangyu Liao, Xing Chen, Wenrong Huang, Yu Zhong, Zhaoyang Zeng
Autoimmune polyendocrine syndrome type 1 (APS-1, OMIM 2403000) is a rare autosomal recessive disease that is caused by autoimmune regulator (AIRE). The main symptoms of APS-1 are chronic mucocutaneous candidiasis, autoimmune adrenocortical insufficiency (Addison's disease) and hypoparathyroidism. We collected APS-1 cases and analysed them. The AIRE genes of the patient and his family members were sequenced to identify whether the APS-1 patient had an AIRE mutation. We discovered a mutation site (c.206A>C) that had never before been reported in the AIRE gene located in exon 2 of the AIRE gene...
May 24, 2017: Immunogenetics
https://www.readbyqxmd.com/read/28536510/influence-of-pre-reproductive-maternal-enrichment-on-coping-response-to-stress-and-expression-of-c-fos-and-glucocorticoid-receptors-in-adolescent-offspring
#6
Debora Cutuli, Erica Berretta, Greta Pasqualini, Paola De Bartolo, Paola Caporali, Daniela Laricchiuta, Patricia Sampedro-Piquero, Francesca Gelfo, Matteo Pesoli, Francesca Foti, Azucena Begega, Laura Petrosini
Environmental enrichment (EE) is an experimental setting broadly used for investigating the effects of complex social, cognitive, and sensorimotor stimulations on brain structure and function. Recent studies point out that parental EE experience, even occurring in the pre-reproductive phase, affects neural development and behavioral trajectories of the offspring. In the present study we investigated the influences of pre-reproductive EE of female rats on maternal behavior and adolescent male offspring's coping response to an inescapable stressful situation after chronic social isolation...
2017: Frontiers in Behavioral Neuroscience
https://www.readbyqxmd.com/read/28533648/increased-f-fdg-uptake-in-bilateral-gynecomastia-secondary-to-feminizing-adrenal-tumor-a-rare-case-report-and-review-of-literature
#7
Priyanka Verma, Gaurav Malhotra, Sneha Kothari, Rajlaxmi Jagtap, Ramesh V Asopa
A 21 year old male who presented with painful enlargement of both the breasts and a hyperestrogenic state, was found to harbor a heterogeneous mass arising from the right adrenal on contrast enhanced Computed Tomography abdomen. The mass was hypermetabolic with no regional, nodal or distant metastases on Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography /Computed Tomography examination. Notably, substantial tracer uptake was seen in bilateral gynecomastia. The patient underwent a right adrenalectomy with the histopathology report confirming adrenocortical carcinoma...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28533088/a-ubiquitin-proteasome-inhibitor-bortezomib-suppresses-the-expression-of-cyp11b2-a-key-enzyme-of-aldosterone-synthesis-as-revealed-by-chemical-screening
#8
Ryo Ito, Ikuko Sato, Tadayuki Tsujita, Atsushi Yokoyama, Akira Sugawara
CYP11B2 is a key enzyme involved in the synthesis of the mineralocorticoid aldosterone. CYP11B2 expression in the adrenal glands is controlled by the renin-angiotensin system (RAS), and plays an important role in the maintenance of electrolyte metabolism in higher organisms. Abnormal overexpression of CYP11B2 results in the disruption of mineral balance and can lead to hypertension. Though the molecular mechanism of the regulation of CYP11B2 expression has remained elusive, we hypothesize that compounds that prevent CYP11B2 expression could represent a novel class of antihypertensive drugs...
May 19, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28523067/extension-of-adrenocortical-carcinoma-into-the-right-atrium
#9
Yulanka Castro-Dominguez, Fatima Samad, Hayder Hashim, Alfonso Waller
Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm which can produce intravascular extension into the inferior vena cava (IVC) rarely extend into the right atrium (RA). We report a case of a male patient with large ACC with extension into the IVC and RA. Computed tomography showed a large right adrenal mass with contiguous tumor thrombus extending into IVC and RA with extension to the level of tricuspid valve. Patient underwent combined cardiac and abdominal surgical intervention on cardiopulmonary bypass with removal of the mass...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28522647/prkar1a-mutation-causing-pituitary-dependent-cushing-disease-in-a-patient-with-carney-complex
#10
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/28515682/nitric-oxide-synthase-inhibitor-attenuates-the-effects-of-repeated-restraint-stress-on-synaptic-transmission-in-the-paraventricular-nucleus-of-the-rat-hypothalamus
#11
Magdalena Kusek, Anna Tokarska, Marcin Siwiec, Anna Gadek-Michalska, Bernadeta Szewczyk, Grzegorz Hess, Krzysztof Tokarski
Corticotropin-releasing hormone (CRH)-synthesizing parvocellular neuroendocrine cells (PNCs) of the hypothalamic paraventricular nucleus (PVN) play a key role in the activation of the hypothalamic-pituitary-adrenocortical (HPA) axis. Several studies have demonstrated that synaptic inputs to these cells may undergo stress-related enhancement but, on the other hand, it has been reported that exposition to the same stressor for prolonged time periods may induce a progressive reduction in the response of the HPA axis to homotypic stressors...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28514325/an-etomidate-analogue-with-less-adrenocortical-suppression-stable-hemodynamics-and-improved-behavioral-recovery-in-rats
#12
Bin Wang, Jun Yang, Jun Chen, Yi Kang, Ling-Hui Yang, Jin Liu, Wen-Sheng Zhang
BACKGROUND: ET-26 hydrochloride (ET-26HCl) is a novel etomidate analogue designed to alleviate the adrenocortical suppression caused by etomidate while retaining the rapid sedative-hypnotic onset and stable hemodynamic features of etomidate. This study compared the anesthetic effect, hemodynamic stability, and recovery profiles of ET-26HCl, etomidate, and the sedative-hypnotic drug propofol in rats. METHODS: The metabolic half-life of ET-26HCl was determined in vitro using high performance liquid chromatography analysis of samples of rat plasma and liver homogenates taken from 3 animals...
May 12, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28511989/biased-agonism-antagonism-at-the-angii-at1-receptor-implications-for-adrenal-aldosterone-production-and-cardiovascular-therapy
#13
REVIEW
Jennifer Maning, Shmuel Negussie, Michelle A Clark, Anastasios Lymperopoulos
Many of the effects of angiotensin II (AngII), including adrenocortical aldosterone release, are mediated by the AngII type 1 receptor (AT1R), a receptor with essential roles in cardiovascular homeostasis. AT1R belongs to the G protein-coupled receptor (GPCR) superfamily, mainly coupling to the Gq/11 type of G proteins. However, it also signals through βarrestins, oftentimes in parallel to eliciting G protein-dependent signaling. This has spurred infinite possibilities for cardiovascular pharmacology, since various beneficial effects are purportedly exerted by AT1R via βarrestins, unlike AT1R-induced G protein-mediated pathways that usually result in damaging cardiovascular effects, including hypertension and aldosterone elevation...
May 13, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/28508690/gene-polymorphisms-associated-with-temperament
#14
Xiaoyan Qiu, Graeme B Martin, Dominique Blache
When individuals are exposed to stressful environmental challenges, the response varies widely in one or more of three components: psychology, behavior and physiology. This variability among individuals can be defined as temperament. In recent years, an increasing large body of evidence suggests that the dimensions of temperament, as well as personality, psychological disorders and behavioral traits, are influenced by genetic factors, and much of the variation appears to involve variation in genes or gene polymorphisms in the hypothalamic-pituitary-adrenocortical (HPA) axis and the behavior-controlling neurotransmitter networks...
May 16, 2017: Journal of Neurogenetics
https://www.readbyqxmd.com/read/28501574/androgen-production-in-pediatric-adrenocortical-tumors-may-occur-via-both-the-classic-and-or-the-alternative-backdoor-pathway
#15
Nesa Marti, Jana Malikova, José A Galván, Maude Aebischer, Marco Janner, Zdenek Sumnik, Barbora Obermannova, Genevieve Escher, Aurel Perren, Christa E Flück
Children with adrenocortical tumors (ACTs) often present with virilization due to high tumoral androgen production, with dihydrotestosterone (DHT) as most potent androgen. Recent work revealed two pathways for DHT biosynthesis, the classic and the backdoor pathway. Usage of alternate routes for DHT production has been reported in castration-resistant prostate cancer, CAH and PCOS. To assess whether the backdoor pathway may contribute to the virilization of pediatric ACTs, we investigated seven children suffering from androgen producing tumors using steroid profiling and immunohistochemical expression studies...
May 10, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28499267/a-novel-germline-tp53-mutation-p-pro190arg-detected-in-a-patient-with-lung-and-bilateral-breast-cancers
#16
Małgorzata Krześniak, Dorota Butkiewicz, Jadwiga Rachtan, Iwona Matuszczyk, Ewa Grzybowska, Marek Rusin
PURPOSE: Li-Fraumeni syndrome (LFS) is a rare genetic disease with strong predispositions to multiple early-onset neoplasms, mostly sarcomas, breast cancers, brain tumors and adrenocortical carcinomas (LFS core cancers). In most LFS families the germline mutations of TP53 tumor suppressor gene were found. Lung cancer does not belong to the core cancers of LFS, however its higher incidence is observed in families with TP53 mutations. Our aim was to search for TP53 mutations in female lung cancer patients whose clinico-demographic characteristics suggested a probable genetic predisposition to the disease...
May 9, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28497219/robot-assisted-adrenalectomy-indications-and-drawbacks
#17
C Nomine-Criqui, A Germain, A Ayav, L Bresler, L Brunaud
Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers...
May 12, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28476230/genetics-of-adrenocortical-development-and-tumors
#18
REVIEW
Maya Lodish
This article links the understanding of developmental physiology of the adrenal cortex to adrenocortical tumor formation. Many molecular mechanisms that lead to formation of adrenocortical tumors have been discovered via next-generation sequencing approaches. The most frequently mutated genes in adrenocortical tumors are also factors in normal adrenal development and homeostasis, including those that alter the p53 and Wnt/β-catenin pathways. In addition, dysregulated protein kinase A signaling and ARMC5 mutations have been identified as key mediators of adrenocortical tumorigenesis...
June 2017: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/28474562/stress-neuropeptides-and-gastric-mucosa
#19
Klara Gyires, Ágnes Fehér
Exposure of the organism to a hostile stimulus results in a series of coordinated reactions that aim to avoid the aversive effect and maintain or restore homeostasis of the organism. In response to noxious stimuli corticotropin-releasing factor (CRF), the primary mediator of stress responses is released from the paraventricular nucleus resulting in activation of the hypothalamic-pituitary-adrenocortical axis and coordination of the endocrine, autonomic, behavioral and immune responses to stress. Several other neuropeptides, released in a coordinated way are also involved in the regulation of the stress response...
November 18, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28471969/long-term-recurrence-free-survival-of-adrenocortical-cancer-extending-into-the-inferior-vena-cava-and-right-atrium-case-report-and-literature-review
#20
Abdulaziz Alghulayqah, Naif Alghasab, Tarik Amin, Nora Alkahtani, Rafif Farhat, Ali S Alzahrani
CONTEXT: Adrenocortical cancer (ACC) is rare but frequently fatal malignancy. Tumor extension into the inferior vena cava signifies an advanced stage (stage III) of the disease and is frequently associated with high risk of recurrence and short-term survival. OBJECTIVE: To present the surgical and medical management of an unusual case of ACC with IVC invasion up to the right atrium. He has the longest reported tumor-free survival of such a situation. We also reviewed and summarized the literature of similar cases...
May 2017: Medicine (Baltimore)
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