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Cushing disease

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https://www.readbyqxmd.com/read/29161691/failure-to-thrive-in-the-context-of-carney-complex
#1
Amit Tirosh, Adi Auerbach, Belen Bonella, Phaedon D Zavras, Elena Belyavskaya, Charalampos Lyssikatos, Karen Meir, Ram Weiss, Hagit Daum, Maya B Lodish, David Gillis, Constantine A Stratakis
BACKGROUND/AIMS: Carney complex (CNC) is a rare syndrome associated with multiple tumors and several other unique manifestations. We describe the clinical, genetic, and laboratory findings in a cohort of patients with CNC and failure to thrive (FTT). METHODS: A retrospective case series of pediatric patients with CNC presenting with FTT. RESULTS: We describe a patient with infantile Cushing syndrome (CS) who presented with severe FTT and liver disease; the patient was subsequently diagnosed with CNC...
November 21, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29161416/combined-ovarian-and-adrenal-venous-sampling-in-the-localization-of-acth-independent-ectopic-cushing-s-syndrome
#2
Shi Chen, Ran Li, Xiaobo Zhang, Lin Lu, Ji Li, Hui Pan, Huijuan Zhu
Context: Cushing's syndrome is rarely caused by the secretion of cortisol from the ovarian tumors. It is important to determine whether the ovarian tumor is capable of secreting cortisol or not in clinical decision making. Selective ovarian and adrenal venous sampling has not been reported previously in the localization of ACTH-independent ectopic Cushing's syndrome. Case description: Herein, we presented a case of 40-year-old Chinese woman who has been manifested with weight gain, hirsutism, hypertension and menstrual disorder over a period of 6 months...
November 16, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29159047/voxel-based-comparison-of-brain-glucose-metabolism-between-patients-with-cushing-s-disease-and-healthy-subjects
#3
Shuai Liu, Yinyan Wang, Kaibin Xu, Fan Ping, Fang Li, Renzhi Wang, Xin Cheng
Cognitive impairment and psychiatric symptoms are common in patients with Cushing's disease (CD) owing to elevated levels of glucocorticoids. Molecular neuroimaging methods may help to detect changes in the brain of patients with CD. The aim of this study was to investigate the characteristics of brain metabolism and its association with serum cortisol level in CD. We compared brain metabolism, as measured using [(18)F]-fluorodeoxyglucose positron emission tomography (FDG PET), between 92 patients with CD and 118 normal subjects on a voxel-wise basis...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29158903/severe-cushing-s-syndrome-and-bilateral-pulmonary-nodules-beyond-ectopic-acth
#4
Carlos Tavares Bello, Emma van der Poest Clement, Richard Feelders
Cushing's syndrome is a rare disease that results from prolonged exposure to supraphysiological levels of glucocorticoids. Severe and rapidly progressive cases are often, but not exclusively, attributable to ectopic ACTH secretion. Extreme hypercortisolism usually has florid metabolic consequences and is associated with an increased infectious and thrombotic risk. The authors report on a case of a 51-year-old male that presented with severe Cushing's syndrome secondary to an ACTH-secreting pituitary macroadenoma, whose diagnostic workup was affected by concurrent subclinical multifocal pulmonary infectious nodules...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29154455/translational-evidence-of-prothrombotic-and-inflammatory-endothelial-damage-in-cushing-syndrome-after-remission
#5
Gloria Aranda, Rebeca Fernandez-Ruiz, Marta Palomo, Mónica Romo, Mireia Mora, Irene Halperin, Gregori Casals, Joaquim Enseñat, Oscar Vidal, Maribel Diaz-Ricart, Felicia A Hanzu
OBJECTIVE: Sustained evidence from observational studies indicates that after remission of Cushing Syndrome (CS) a cardiovascular risk phenotype persists. Here we performed a translational study in active CS and CS in remission to evaluate the subclinical cardiometabolic burden and to explore the direct proinflammatory and prothrombotic potential of their sera on the endothelium in an in vitro translational atherothrombotic cell model. PATIENTS: Cross-sectional study...
November 20, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29145000/proopiomelanocortin-processing-and-prohormone-convertase-1-level-in-dogs-with-pituitary-corticotroph-tumors
#6
G Benchekroun, P de Fornel-Thibaud, D Rosenberg
Preliminary data suggest that prohormone convertase 1 (PC1/3) protein expression and proopiomelanocortin (POMC) processing are altered in large corticotroph tumors. The aim of this study was to characterize the levels of ACTH precursors and PC1/3 protein in small and large corticotroph tumors of dogs with Cushing's disease. Pituitary tumors of dogs with Cushing's disease were collected postmortem 30 min to 12h after natural death or euthanasia, and classified as small or large. POMC, pro-ACTH, and PC1/3 were detected by Western blotting...
October 13, 2017: Domestic Animal Endocrinology
https://www.readbyqxmd.com/read/29143885/consensus-driven-in-hospital-cortisol-assessment-after-acth-secreting-pituitary-adenoma-resection
#7
Yana Stolyarov, James Mirocha, Adam N Mamelak, Anat Ben-Shlomo
PURPOSE: Remission from Cushing disease (CD) after pituitary adenoma resection may be predicted by a postoperative reduction in serum cortisol level. A 2008 consensus statement recommends assessing morning cortisol levels during the first postoperative week, and replacing glucocorticoid (GC) if cortisol nadir of < 2 or < 5 µg/dL is achieved. We sought to evaluate adherence to consensus recommendations following adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma resection at our tertiary medical center, and assess time to cortisol nadir to better define the window for assessment and intervention...
November 16, 2017: Pituitary
https://www.readbyqxmd.com/read/29143179/diagnostic-work-up-in-steroid-myopathy
#8
REVIEW
Marco Alessandro Minetto, Valentina D'Angelo, Emanuela Arvat, Santosh Kesari
INTRODUCTION: Steroid myopathy is a well-known sign of endogenous Cushing's syndrome as well as a side effect of glucocorticoid administration. The clinical finding of muscle weakness and the clinical inspection of the muscle size are the most commonly used diagnostic tools, sometimes in combination with needle electromyography, but there are no means to detect the myopathy before the appearance of clinical or electrodiagnostic signs. Until now, no guidelines have been produced for a disease-specific evaluation of muscle impairment in patients with Cushing's syndrome...
November 15, 2017: Endocrine
https://www.readbyqxmd.com/read/29142029/routine-use-of-quantitative-disease-activity-measurements-among-us-rheumatologists-implications-for-treat-to-target-management-strategies-in-rheumatoid-arthritis
#9
Jeffrey R Curtis, Lang Chen, Maria I Danila, Kenneth G Saag, Kathy L Parham, John J Cush
OBJECTIVE: The aim of our study was to examine why real-world practices and attitudes regarding quantitative measurements of rheumatoid arthritis (RA) have received limited attention. METHODS: An e-mail survey asked US rheumatologists to self-report on their use of quantitative measurements (metric). RESULTS: Among 439 respondents, metric rheumatologists (58%) were more likely to be in group practice and to use tumor necrosis factor inhibitors...
November 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/29134965/depression-is-associated-with-more-aggressive-inflammatory-bowel-disease
#10
Bharati Kochar, Edward L Barnes, Millie D Long, Kelly C Cushing, Joseph Galanko, Christopher F Martin, Laura E Raffals, Robert S Sandler
OBJECTIVES: Depression is prevalent in inflammatory bowel disease (IBD) patients. The impact of depression on IBD is not well-studied. It is unknown how providers should assess depression. METHODS: We used data from the Sinai-Helmsley Alliance for Research Excellence cohort, to assess methods of diagnosing depression and effects of baseline depression on disease activity at follow-up. A patient health questionnaire (PHQ-8) score ≥5 was consistent with mild depression...
November 14, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29134558/development-of-hyperkalemia-following-treatment-with-dapagliflozin-dapa-in-a-patient-with-type-2-diabetes-after-bilateral-adrenalectomy
#11
Daichi Miyaoka, Akihiro Tsuda, Noriyuki Hayashi, Norikazu Toi, Akiyo Yamasaki, Yuki Nagata, Shinya Nakatani, Masafumi Kurajoh, Shinsuke Yamada, Tomoaki Morioka, Yasuo Imanishi, Masanori Emoto, Masaaki Inaba
Dapagliflozin (DAPA), a sodium-glucose co-transporter 2 (SGLT2) inhibitor, is known to have a beneficial diuretic effect, in addition to a glucose-lowering effect. Although SGLT2 inhibitor has been reported, the increase of hyperkalemia in patients treated with renin-angiotensin-aldosterone system (RAAS) inhibitors, their mechanism of action is unclear. We report the first case of a type 2 diabetes (T2DM) patient with potential mineralocorticoid deficiency who developed hyperkalemia after administration of DAPA...
November 13, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/29134170/effectiveness-of-bilateral-inferior-petrosal-sinuses-sampling-in-tumor-lateralization-intraoperative-findings-and-postoperative-results
#12
Pablo Harker, Oscar Feo-Lee, Manuel Giraldo-Grueso, Juan Carlos Puentes
Object  Diagnosis and treatment of Cushing's disease (CD) is a demanding endeavor. We evaluated the effectiveness of inferior petrosal sinus sampling (IPSS) in preoperative tumor lateralization and surgical outcomes. Methods  Fifty-two consecutive patients with CD and normal magnetic resonance imaging (MRI) were enrolled between 2009 and 2015. Selective catheterization of petrosal sinuses for IPSS was performed. All patients underwent microsurgical dissection, and if a lesion was found, underwent lesion resection...
December 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29128555/combined-haploidentical-and-umbilical-cord-blood-allogeneic-stem-cell-transplantation-for-high-risk-lymphoma-and-cll
#13
Jingmei Hsu, Andrew Artz, Sebastian A Mayer, Danielle Guarner, Michael R Bishop, Ronit Reich-Slotky, Sonali M Smith, June Greenberg, Justin Kline, Rosanna Ferrante, Adrienne A Phillips, Usama Gergis, Hongtao Liu, Wendy Stock, Melissa Cushing, Tsiporah B Shore, Koen van Besien
Limited studies have reported on outcomes for lymphoid malignancy patients receiving alternative donor allogeneic stem cell transplants. We have previously described combining CD34-selected haploidentical grafts with umbilical cord blood (haplo-cord) to accelerate neutrophil and platelet engraftment. Here, we examine the outcome of patients with lymphoid malignancies undergoing haplo-cord transplantation at the University of Chicago and Weill Cornell Medical College. We analyzed 42 lymphoma and CLL patients who underwent haplo-cord allogeneic stem cell transplantation...
November 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29122730/cerebral-venous-sinus-thrombosis-following-second-transsphenoidal-surgery-report-of-a-rare-complication-and-review-of-literature
#14
Zhuangzhuang Miao, Zhuo Zhang, Juan Chen, Junwen Wang, Huaqiu Zhang, Ting Lei
BACKGROUND: Cushing's disease, induced by pituitary adrenocorticotropic hormone (ACTH) secreting adenomas, is associated with high risk factors of stroke. At present, transsphenoidal surgery remains the first line of therapy. Cerebral venous sinus thrombosis (CVST) is an uncommon form of stroke with variable presentations but, to date, there have been no reports of its occurrence in Cushing's disease patients following surgery. CASE DESCRIPTION: We herein report a Cushing's disease case who suffered CVST a few days after a second transsphenoidal surgery...
November 6, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29115003/impact-of-hypercortisolism-on-skeletal-muscle-mass-and-adipose-tissue-mass-in-patients-with-adrenal-adenomas
#15
Danae A Delivanis, Nicole M Iñiguez-Ariza, Muhammad H Zeb, Michael R Moynagh, Naoki Takahashi, Travis J McKenzie, Melinda A Thomas, Charalambos Gogos, William F Young, Irina Bancos, Venetsana Kyriazopoulou
CONTEXT: Abdominal visceral adiposity and central sarcopenia are markers of increased cardiovascular risk and mortality. OBJECTIVE: To assess whether central sarcopenia and adiposity can serve as a marker of disease severity in patients with adrenal adenomas and glucocorticoid secretory autonomy. DESIGN: Retrospective cohort study. PATIENTS: Twenty-five patients with overt Cushing syndrome (CS), 48 patients with mild autonomous cortisol excess (MACE), and 32 patients with a non-functioning adrenal tumor (NFAT) were included...
November 8, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29104610/functional-pancreatic-neuroendocrine-tumour-causing-cushing-s-syndrome-the-effect-of-chemotherapy-on-clinical-symptoms
#16
Paulo Henrique do Amor Divino, Katia Regina Marchetti, Madson Q Almeida, Rachel P Riechelmann
Background: Neuroendocrine tumours (NETs) are a heterogeneous group of diseases that can originate from any part of the gastrointestinal tract, bronchi, thyroid and pancreas. These tumours may be functioning or not depending on their ability to produce active substances, such as adrenocorticotrophic hormone (ACTH). ACTH-producing pancreatic neuroendocrine tumours are rare, with limited data about effective antitumor therapies. Case Report: A 58-year-old man with a history of type-2 diabetes mellitus and arterial hypertension was diagnosed with Cushing's syndrome (CS) secondary to an ACTH ectopic production from a well-differentiated neuroendocrine tumour of the pancreas metastatic to the liver...
2017: Ecancermedicalscience
https://www.readbyqxmd.com/read/29099758/the-hypothalamic-pituitary-axis-and-autoantibody-related-disorders
#17
REVIEW
Cristina Cocco, Carla Brancia, Giulia Corda, Gian-Luca Ferri
This review summarized different studies reporting the presence of autoantibodies reacting against cells of the pituitary (APAs) and/or hypothalamus (AHAs). Both APAs and AHAs have been revealed through immunofluorescence using different kinds of substrates. Autoantibodies against gonadotropic cells were mainly found in patients affected by cryptorchidism and hypogonadotropic hypogonadism while those against prolactin cells were found in different kinds of patients, the majority without pituitary abnormalities...
November 3, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29097132/cavernous-carotid-stenosis-following-stereotactic-radiosurgery-for-cushing-s-disease-a-rare-complication-and-review-of-the-literature
#18
Mohana Rao Patibandla, Zhiyuan Xu, David Schlesinger, Jason P Sheehan
Intracranial carotid stenosis or occlusion after SRS is an extraordinarily rare event. Because of the rarity of this event, increased awareness within the neurosurgical community is required. For those with post-radiosurgical carotid stenosis who are asymptomatic, longitudinal follow up is recommended. Management depends on whether the patient is symptomatic or asymptomatic. Those who are symptomatic may require balloon angioplasty, stenting, or bypass surgery.
October 30, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29094913/secondary-hypertension-discovering-the-underlying-cause
#19
Lesley Charles, Jean Triscott, Bonnie Dobbs
Most patients with hypertension have no clear etiology and are classified as having primary hypertension. However, 5% to 10% of these patients may have secondary hypertension, which indicates an underlying and potentially reversible cause. The prevalence and potential etiologies of secondary hypertension vary by age. The most common causes in children are renal parenchymal disease and coarctation of the aorta. In adults 65 years and older, atherosclerotic renal artery stenosis, renal failure, and hypothyroidism are common causes...
October 1, 2017: American Family Physician
https://www.readbyqxmd.com/read/29094256/efficacy-of-dexamethasone-suppression-test-during-the-diagnosis-of-primary-pigmented-nodular-adrenocortical-disease-in-chinese-adrenocorticotropic-hormone-independent-cushing-syndrome
#20
Shi Chen, Ran Li, Lin Lu, Lian Duan, Xuebin Zhang, Anli Tong, Hui Pan, Huijuan Zhu, Zhaolin Lu
OBJECTIVE: To evaluate the cut-off value of the ratio of 24 h urinary free cortisol (24 h UFC) levels post-dexamethasone to prior-dexamethasone in dexamethasone suppression test (DST) during the diagnosis of primary pigmented nodular adrenocortical disease in Chinese adrenocorticotropic hormone-independent Cushing syndrome. DESIGN: Retrospective study. PARTICIPANTS: The patients diagnosed with primary pigmented nodular adrenocortical disease (PPNAD, n = 25), bilateral macronodular adrenal hyperplasia (BMAH, n = 27), and adrenocortical adenoma (ADA, n = 84) were admitted to the Peking Union Medical College Hospital from 2001 to 2016...
November 1, 2017: Endocrine
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