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Cushings disease

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https://www.readbyqxmd.com/read/29343284/extensive-armc5-genetic-variance-in-primary-bilateral-macronodular-adrenal-hyperplasia-that-started-with-exophthalmos-a-case-report
#1
Ping Jin, Muhammad Usman Janjua, Qin Zhang, Chang-Sheng Dong, Youbo Yang, Zhao-Hui Mo
BACKGROUND: Primary bilateral macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome characterized by the presence of bilateral secretory adrenal nodules. Recent studies have shown that primary bilateral macronodular adrenal hyperplasia is caused by combined germline and somatic mutations of the ARMC5 gene. Exophthalmos is an underappreciated sign of Cushing's syndrome. CASE PRESENTATION: A 52-year-old Chinese woman with progressively worsening bilateral proptosis presented to our hospital...
January 18, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29330227/earlier-post-operative-hypocortisolemia-may-predict-durable-remission-from-cushing-s-disease
#2
Natasha Ironside, Gregoire Chatain, David Asuzu, Sarah Benzo, Maya Lodish, Susmeeta Sharma, Lynnette Nieman, Constantine Stratakis, Russell Lonser, Prashant Chittiboina
CONTEXT: Achievement of hypocortisolemia following transsphenoidal surgery (TSS) for Cushing's disease (CD) is associated with successful adenoma resection. However, up to one third of these patients recur. OBJECTIVE: We assessed whether delay in reaching post-operative cortisol nadir may delineate those at risk of recurrence for CD following TSS. METHODS: A retrospective review of of 257 patients who received 291 TSS procedures for CD at NIH, between 2003 and 2016...
January 12, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29329418/variability-of-late-night-salivary-cortisol-in-cushing-disease-a-prospective-study
#3
Zahrae Sandouk, Philip Johnston, Dustin Bunch, Sihe Wang, James Bena, Amir Hamrahian, Laurence Kennedy
Background: The frequency of variable hormonogenesis in patients with Cushing disease (CD) but without cyclical symptoms is unclear. Aim: To assess the frequency of variable hormonogenesis in patients presenting with Cushing disease. Methods: Over a 6-month period patients with confirmed or suspected Cushing disease provided late-night salivary samples for up to 42 consecutive nights. Results: Of 19 patients confirmed to have CD 16 provided at least seven consecutive salivary samples and 13 provided at least 21; these 16 patients are the subjects of this report...
January 10, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29325527/single-center-experience-with-laparoscopic-adrenalectomy-on-a-large-clinical-series
#4
Giovanni Conzo, Claudio Gambardella, Giancarlo Candela, Alessandro Sanguinetti, Andrea Polistena, Guglielmo Clarizia, Renato Patrone, Francesco Di Capua, Chiara Offi, Mario Musella, Sergio Iorio, Giseppe Bellastella, Daniela Pasquali, Annamaria De Bellis, Antonio Sinisi, Nicola Avenia
BACKGROUND: Laparoscopic adrenalectomy is considered the gold standard technique for the treatment of benign small and medium size adrenal masses (<6 cm), due to low morbidity rate, short hospitalization and patient rapid recovery. The aim of our study is to analyse the feasibility and efficiency of this surgical approach in a broad spectrum of adrenal gland pathologies. METHODS: Pre-operative, intra-operative and post-operative data from 126 patients undergone laparoscopic adrenalectomy between January 2003 and December 2015 were retrospectively collected and reviewed...
January 11, 2018: BMC Surgery
https://www.readbyqxmd.com/read/29319131/temozolomide-therapy-for-aggressive-pituitary-crooke-s-cells-corticotropinoma-causing-cushing-s-disease-a-case-report-with-literature-review
#5
Aleksandra Gilis-Januszewska, Małgorzata Wilusz, Jacek Pantofliński, Renata Turek-Jabrocka, Grzegorz Sokołowski, Anna Sowa-Staszczak, Łukasz Kluczyński, Dorota Pach, Grzegorz Zieliński, Alicja Hubalewska-Dydejczyk
AbstractContext: Aggressive pituitary tumours causing Cushing's Disease are very rare, difficult to treat, and usually resistant to conventional therapy. There is growing evidence for the use of temozolomide (TZM), an alkylating chemotherapeutic agent, as first line chemotherapy in tumours resistant to repeated neurosurgery, radiotherapy and adrenalectomy. OBJECTIVE: To present the response to TMZ in a rare case of an aggressive pituitary tumour in the course of Cushing's Disease and to review the literature referring to similar cases...
January 10, 2018: Endokrynologia Polska
https://www.readbyqxmd.com/read/29315507/lifestyle-factors-and-subsequent-ischemic-heart-disease-risk-after-hematopoietic-cell-transplantation
#6
Kasey J Leger, K Scott Baker, Kara L Cushing-Haugen, Mary E D Flowers, Wendy M Leisenring, Paul J Martin, Jason A Mendoza, Kerryn W Reding, Karen L Syrjala, Stephanie J Lee, Eric J Chow
BACKGROUND: The objective of this study was to evaluate whether modifiable cardiovascular risk conditions and lifestyle factors were temporally associated with an increased risk for ischemic heart disease and overall mortality in a cohort of hematopoietic cell transplantation (HCT) survivors. METHODS: HCT recipients who had survived for ≥1 year, were ≥20 years old, and had undergone transplantation between 1970 and 2010 at a transplant referral center were surveyed in 2010-2011 about cardiovascular health and lifestyle factors (n = 3833)...
January 9, 2018: Cancer
https://www.readbyqxmd.com/read/29313180/a-prospective-longitudinal-study-of-pasireotide-in-nelson-s-syndrome
#7
Eleni Daniel, Miguel Debono, Sharon Caunt, Constantine Girio-Fragkoulakis, Stephen J Walters, Scott A Akker, Ashley B Grossman, Peter J Trainer, John Newell-Price
PURPOSE: Nelson's syndrome is a challenging condition that can develop following bilateral adrenalectomy for Cushing's disease, with high circulating ACTH levels, pigmentation and an invasive pituitary tumor. There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson's syndrome. METHODS: Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1 month pasireotide 300-600 μg s...
January 8, 2018: Pituitary
https://www.readbyqxmd.com/read/29311226/outcome-of-endoscopic-vs-microsurgical-transsphenoidal-resection-for-cushing-s-disease
#8
REVIEW
Nidan Qiao
INTRODUCTION: It is unclear whether the proportions of remission and the recurrence rates differ between endoscopic transsphenoidal surgery (TS) and microscopic TS in Cushing's disease (CD); thus, we conducted a systematic review and meta-analysis to evaluate studies of endoscopic TS and microscopic TS. METHODS: We conducted a comprehensive search of PubMed to identify relevant studies. Remission and recurrence were used as outcome measures following surgical treatment of CD...
January 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29297815/serologic-response-to-canine-distemper-vaccination-in-captive-linnaeus-s-two-toed-sloths-choloepus-didactylus-after-a-fatal-canine-distemper-virus-outbreak
#9
Julie D Sheldon, Andrew C Cushing, Rebecca P Wilkes, Eman Anis, Edward J Dubovi
Canine distemper virus (CDV) affects many wild and captive, nondomestic species worldwide but has not been previously reported in Xenarthra. Paucity of information on vaccination safety and efficacy presents challenges for disease prevention in captive collections. CDV infections and subsequent mortalities in five captive Linnaeus's two-toed sloths ( Choloepus didactylus) in eastern Tennessee are reported. Clinical signs included oculonasal discharge, oral ulcerations, and diarrhea, and the diagnosis was confirmed by necropsy, histopathology, immunohistochemistry, virus isolation, and polymerase chain reaction...
December 2017: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/29289677/endocardial-fibroelastosis-in-two-related-tiger-cubs-panthera-tigris
#10
Kiira T Rodriguez, Andrew C Cushing, Carla Bernal, Ed C Ramsay, Linden E Craig, Rebecca E Gompf
Two tiger cubs (Panthera tigris) from the same litter were evaluated for suspected cardiac disease. Two cubs with a dilated cardiomyopathy phenotype were diagnosed with endocardial fibroelastosis based on necropsy and histopathologic examinations. Echocardiography revealed salient anatomic and functional aspects of this cardiac disorder. This is the first report of endocardial fibroelastosis in this species.
December 28, 2017: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/29288821/reduced-intensity-allogeneic-transplant-for-acute-myeloid-leukemia-and-myelodysplastic-syndrome-using-combined-cd34-selected-haploidentical-graft-and-a-single-umbilical-cord-unit-compared-to-matched-unrelated-donor-stem-cells-in-older-adults
#11
Stephanie B Tsai, Joanna Rhodes, Hongtao Liu, Tsiporah Shore, Michael Bishop, Melissa M Cushing, Usama Gergis, Lucy Godley, Justin Kline, Richard A Larson, Sebastian Mayer, Olatoyosi Odenike, Wendy Stock, Amittha Wickrema, Koen van Besien, Andrew S Artz
Haplo/cord transplantation combines an umbilical cord blood (UCB) graft with CD34-selected haploidentical cells and results in rapid hematopoietic recovery followed by durable UCB engraftment. We compared outcomes of transplants in older patients with acute myeloid leukemia (AML) or high-risk myelodysplastic syndromes (MDS) who received either HLA-matched unrelated donor cells (MUD) or haplo/cord grafts. Between 2007 and 2013, 109 adults ages 50 and older underwent similar reduced intensity conditioning (RIC) with fludarabine and melphalan and antibody-mediated T-cell depletion for AML (n=83) or high risk MDS (n=26) followed by either a MUD (n=68) or haplo/cord (n=41) graft...
December 27, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29285318/comparison-of-hydrocortisone-and-prednisone-in-the-glucocorticoid-replacement-therapy-post-adrenalectomy-of-cushing-s-syndrome
#12
Kunlong Tang, Liang Wang, Zhongyuan Yang, Yingying Sui, Liming Li, Yuting Huang, Peng Gao
Cushing's syndrome requires glucocorticoid replacement following adrenalectomy. Based on a simplified glucocorticoid therapy scheme and the peri-operative observation, we investigated its efficacy and safety up to 6 months post-adrenalectomy in this cohort study. We found the adrenocorticotropic hormone (ACTH) levels were normal post-adrenalectomy, and sufficient to stimulate the recovery of the dystrophic adrenal cortex, thus exogenous supplemental ACTH might not be necessary. Patients were grouped by oral reception of either hydrocortisone or prednisone since day 2 post-adrenalectomy...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29279458/whole-genome-sequencing-revealed-armadillo-repeat-containing-5-armc5-mutation-in-a-chinese-family-with-acth-independent-macronodular-adrenal-hyperplasia
#13
Qian Zhang, Liang Cui, Jiang-Ping Gao, Wen-Hua Yan, Nan Jin, Kang Chen, Li Zang, Jin Du, Xian-Ling Wang, Qing-Hua Guo, Guo-Qing Yang, Li-Juan Yang, Jian-Ming Ba, Wei-Jun Gu, Zhao-Hui Lv, Jing-Tao Dou, Yi-Ming Mu, Ju-Ming Lu
Primary macronodular adrenal hyperplasia (PMAH), also known in the past as bilateral macronodular adrenalhyperplasia or adrenocorticotropin (ACTH)-independent macronodular adrenal hyperplasia, is a rare type of Cushing's syndrome (CS) and is associated with bilateralenlargement of the adrenal glands. It accounts for <1% of all endogenous cases of CS. In order toidentify the pathogenic mutations in the causative gene of (AIMAH pedigrees, Whole-genome sequencing of three patients in family I was used to retrieve candidate causative genes...
December 27, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/29278511/the-adrenal-gland-microenvironment-in-health-disease-and-during-regeneration
#14
REVIEW
Waldemar Kanczkowski, Mariko Sue, Stefan R Bornstein
The adrenal gland is a key component of the stress system in the human body. Multiple direct and paracrine interactions between different cell types and their progenitors take place within the adrenal gland microenvironment. These unique interactions are supported by high vascularization and the adrenal cortex extracellular matrix. Alterations in the adrenal gland microenvironment are known to influence the progression of several pathological conditions, such as obesity and sepsis, and to be influenced by these disorders...
July 2017: Hormones: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29260252/-functional-diagnostics-in-endocrinology
#15
REVIEW
C J Auernhammer, M Reincke
When investigating many endocrinological diseases, basal laboratory parameters are not sufficient to distinguish between physiological and pathological hormone secretion. Functional diagnostics plays a decisive role in this context. Stimulation and suppression tests are used depending on whether under- or over-function needs to be diagnosed. This review article discusses selected functional tests, each of which plays an important role in current guidelines. Indications and test principles, including their performance, reliability, and limitations, are discussed...
December 19, 2017: Der Internist
https://www.readbyqxmd.com/read/29259830/iatrogenic-cushing-s-syndrome-with-subsequent-adrenal-insufficiency-in-a-patient-with-psoriasis-vulgaris-using-topical-steroids
#16
Suzan Demir Pektas, Gursoy Dogan, Nese Cinar
Iatrogenic Cushing's syndrome (ICS) is usually related to prolonged and/or high-dose oral or parenteral steroid use. Psoriasis vulgaris (PV) is chronic inflammatory disease and characterized by periods of attack and remission. Topical steroid (TS) is the first choice of treatment for localized and mild PV. The development of systemic side effects of the steroids is usually not observed after TS application. But the risk of developing ICS still exists. In the literature, there are a few adult cases who developed ICS and subsequent adrenal insufficiency associated with TS...
2017: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/29244084/the-value-of-peri-operative-levels-of-acth-dhea-and-dhea-s-and-tumor-size-in-predicting-recurrence-of-cushing-s-disease
#17
Nadine El Asmar, Aman Rajpal, Warren R Selman, Baha M Arafah
Background and Objectives: Despite the development of hypocortisolemia after corticotroph surgical adenomectomy, 15-20% patients have recurrence of Cushing's disease (CD). In this study, we investigated the impact of tumor size and the value of peri-operative assessment of ACTH and adrenal steroid levels in predicting recurrence. Methods: Peri-operatively, no glucocorticoids were administered until the serum cortisol was ≤ 3 ug/dL. Blood samples were obtained before and repeatedly after adenomectomy in 79 patients with CD...
December 13, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29241753/alopecia-and-the-metabolic-syndrome
#18
Cheryl Lie, Choon Fong Liew, Hazel H Oon
Hair loss is a common condition that affects most people at some point in their lives. It can exist as an isolated problem or with other diseases and conditions. Androgenetic alopecia (AGA) and its association with the metabolic syndrome (MetS) have received increasing interest since 1972, when the first link between cardiovascular risk factors and hair loss was raised. We have reviewed studies concerning the relationship between alopecia and MetS. Many studies have investigated the relationship among AGA and MetS and its individual components, particularly in men, where a disproportionately large number of these studies supports this association...
January 2018: Clinics in Dermatology
https://www.readbyqxmd.com/read/29223281/human-studies-on-hypothalamo-pituitary-adrenal-hpa-axis
#19
REVIEW
Kamani Liyanarachchi, Richard Ross, Miguel Debono
The daily rhythm of the hypothalamo-pituitary-adrenal (HPA) axis is regulated by the central clock in the suprachiasmatic nucleus. Cortisol, a glucocorticoid, acts as a secondary messenger between the central clock and the peripheral tissues. Changes in clock time, as seen in shift workers, alters the HPA axis and results in metabolic disturbances associated with ill health. Depression, anorexia nervosa and obstructive sleep apnoea, are associated with cortisol rhythm phase shifts and increased cortisol exposure...
October 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29219752/negative-surgical-exploration-in-patients-with-cushing-s-disease-benefit-of-two-thirds-gland-resection-on-remission-rate-and-a-review-of-the-literature
#20
Steven B Carr, Bette K Kleinschmidt-DeMasters, Janice M Kerr, Katja Kiseljak-Vassiliades, Margaret E Wierman, Kevin O Lillehei
OBJECTIVE The authors report their single-institution experience with the pathological findings, rates of remission, and complications in patients with presumed Cushing's disease (CD) who underwent a two-thirds pituitary gland resection when no adenoma was identified at the time of transsphenoidal surgery (TSS). The authors also review the literature on patients with CD, negative surgical exploration, and histological findings. METHODS This study is a retrospective analysis of cases found in neurosurgery and pathology department databases between 1989 and 2011...
December 8, 2017: Journal of Neurosurgery
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