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https://www.readbyqxmd.com/read/29467229/management-of-endocrine-disease-can-we-cure-cushing-s-disease
#1
Xavier Bertagna
One of today's challenges in endocrinology is the treatment of Cushing's disease: Whereas pituitary surgery has the potential to "cure" the patient and restore a completely normal pituitary adrenal axis, there are immediate failures and late recurrences that will ultimately require alternate therapeutic approaches. Their high number is in direct correlation with their serious limitations and they all appear to be "default options". This "personal view" tries to shed some light on the inescapable difficulties of the current treatments of Cushing's disease, and to provide some optimistic view for the future where the pituitary adenoma should be the "reasonable obsession" of a successful therapeutist…...
February 21, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29460202/clinicopathological-significance-of-baseline-t2-weighted-signal-intensity-in-functional-pituitary-adenomas
#2
Sema Ciftci Dogansen, Gulsah Yenidunya Yalin, Seher Tanrikulu, Sakin Tekin, Nihan Nizam, Bilge Bilgic, Serra Sencer, Sema Yarman
PURPOSE: To assess baseline T2-weighted signal intensity (T2-WSI) of functional pituitary adenomas (FPA), and to investigate the relationship of baseline T2-WSI with clinical features, histopathological granulation patterns, and response to treatment in patients with acromegaly, prolactinoma and Cushing's disease (CD). METHODS: Somatotroph adenomas (n = 87), prolactinomas (n = 78) and corticotroph adenomas (n = 29) were included in the study. Baseline T2-WSI findings (grouped as hypo-, iso- and hyperintense) were compared with hormone levels, tumor diameter, granulation patterns and response to treatment...
February 19, 2018: Pituitary
https://www.readbyqxmd.com/read/29458220/incidence-of-autoimmune-and-related-disorders-after-resolution-of-endogenous-cushing-syndrome-in-children
#3
Christina Tatsi, Meg Keil, Charalampos Lyssikatos, Elena Belyavskaya, Constantine A Stratakis, Maya B Lodish
Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here we investigated the presence of autoimmune and related diseases that developed after cure of endogenous Cushing syndrome (CS) in children. We identified 129 children who were diagnosed and successfully treated for endogenous CS at the National Institutes of Health from 1997 until 2017, and who were followed for at least 6 months after treatment...
February 19, 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29456353/the-utility-of-preoperative-acth-cortisol-ratio-for-the-diagnosis-and-prognosis-of-cushing-s-disease
#4
Alev Selek, Berrin Cetinarslan, Zeynep Canturk, Ilhan Tarkun, Ozlem Zeynep Akyay, Burak Cabuk, Savas Ceylan
Purpose: Cushing's syndrome (CS) is a rare disease having diagnostic difficulties. Many diagnostic tests have been defined but none of these are diagnostic alone. Determination of the cause is another problem which sometimes requires more sophisticated and invasive procedures. Therefore, we aimed to evaluate the utility of pretreatment plasma adrenocorticotropic hormone (ACTH)/cortisol ratios in patients with confirmed endogenous CS for the diagnosis and differential diagnosis of CS. Materials and Methods: This retrospective evaluation included 145 patients with the diagnosis of CS, 119 patients with Cushing's disease (CD), and 26 patients with ACTH-independent CS (AICS), in a university hospital...
January 2018: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29442480/cushing-s-disease-major-difficulties-in-diagnosis-and-management-during-pregnancy
#5
Francisca Caimari, Rosa Corcoy, Susan M Webb
Pregnancy in women with a diagnosis of Cushing' syndrome (CS) is an extremely rare event and its diagnosis and treatment are a real medical challenge. During pregnancy, the hypothalamus-pituitary-adrenal axis undergoes major changes leading to a significant increase in plasma cortisol levels throughout gestation. The difficulties in diagnosis are related to the resemblance of symptoms of CS and those of pregnancy, and to the complex interpretation of the screening tests. Moreover, the diagnostic work up in the postnatal period may be difficult in the first weeks postpartum...
February 13, 2018: Minerva Endocrinologica
https://www.readbyqxmd.com/read/29440131/diagnostic-accuracy-and-comparison-of-bipss-in-response-to-lysine-vasopressin-and-hcrh
#6
Kush Dev Singh Jarial, Anil Bhansali, Vivek Gupta, Paramjeet Singh, Kanchan K Mukherjee, Akhilesh Sharma, Rakesh Vasishta, Suja P Sukumar, Naresh Sachdeva, Rama Walia
CONTEXT: Bilateral inferior petrosal sinus sampling (BIPSS) using hCRH is currently considered the 'gold standard' test for the differential diagnosis of ACTH-dependent Cushing's syndrome (CS). Vasopressin is more potent than CRH to stimulate ACTH secretion as shown in animal studies, however; no comparative data of its use is available during BIPSS. OBJECTIVE: To study the diagnostic accuracy and comparison of hCRH and lysine vasopressin (LVP) stimulation during BIPSS...
February 12, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29432944/expression-of-mmp-9-pttg-hmga2-and-ki-67-in-acth-secreting-pituitary-tumors-and-their-association-with-tumor-recurrence
#7
Xiaohai Liu, Ming Feng, Ye Zhang, Congxin Dai, Bowen Sun, Xinjie Bao, Kan Deng, Yong Yao, Renzhi Wang
OBJECTIVE: MMP-9, PTTG, and HMGA2 play important roles in the tumorigenesis of adrenocorticotrophic hormone (ACTH)-secreting pituitary tumors, but their associations with tumor recurrence after transsphenoidal adenomectomy remain unclear. The aim of the study was to investigate the immunohistochemical expression profiles of MMP-9, PTTG, HMGA2, and Ki-67 in recurrent and non-recurrent ACTH-secreting pituitary tumors and to identify their associations with tumor behavior and recurrence status...
February 9, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29426273/testosterone-and-cortisol-secreting-oncocytic-adrenocortical-adenoma-in-the-pediatric-age-group
#8
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29421453/adrenal-axis-insufficiency-following-endoscopic-transsphenoidal-resection-of-pituitary-adenomas
#9
Abdulrazag Ajlan, Khadeejah A Almufawez, Abdulrahman Albakr, Laurence Katznelson, Griffith R Harsh
INTRODUCTION: Hormonal insufficiency of one or more pituitary axes can appear after pituitary surgery. Adrenal axis impairment after surgery can lead to serious consequences if not identified and treated. OBJECTIVE: Assess early and late post-operative adrenal insufficiency (AI) and identify the risk factors predicting their occurrence after endoscopic transsphenoidal resection of pituitary adenomas. METHOD: Retrospective review identified 176 pituitary adenomas resected using an endoscopic transsphenoidal approach...
February 5, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29406000/neonatal-cushing-syndrome-a-rare-but-potentially-devastating-disease
#10
REVIEW
Christina Tatsi, Constantine A Stratakis
Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. More rarely, neonatal CS presents as part of other syndromes such as McCune-Albright syndrome or Beckwith-Wiedemann syndrome...
March 2018: Clinics in Perinatology
https://www.readbyqxmd.com/read/29396758/insulin-sensitivity-and-secretion-and-adipokine-profile-in-patients-with-cushing-s-disease-treated-with-pasireotide
#11
V Guarnotta, G Pizzolanti, A Ciresi, C Giordano
PURPOSE: To evaluate the effect of pasireotide on β-cell and adipose function in patients with Cushing's disease (CD). METHODS: Clinical and hormonal parameters, insulin secretion evaluated by HOMA-β and by the area under the curve (AUC2h) of C-peptide during a mixed meal tolerance test and insulin sensitivity, evaluated by the euglycaemic hyperinsulinaemic clamp, were evaluated in 12 patients with active CD, before and after 6 and 12 months of pasireotide. In addition, a panel of adipokines including leptin (Ob), leptin/leptin receptor ratio (Ob/Ob-R ratio), adiponectin, resistin, visfatin, adipocyte fatty acid binding protein (AFABP) and non-esterified fatty acids (NEFAs) was evaluated at baseline and after 12 months of pasireotide...
February 2, 2018: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/29390296/carney-complex-with-prkar1a-gene-mutation-a-case-report-and-literature-review
#12
Qiuli Liu, Dali Tong, Gaolei Liu, Yuting Yi, Dianzheng Zhang, Jun Zhang, Yao Zhang, Zaoming Huang, Yaoming Li, Rongrong Chen, Yanfang Guan, Xin Yi, Jun Jiang
RATIONALE: Carney complex (CNC) is a multiple neoplasia syndrome with autosomal dominant inheritance. CNC is characterized by the presence of myxomas, spotty skin pigmentation, and endocrine overactivity. No direct correlation has been established between disease-causing mutations and phenotype. PATIENT CONCERNS: A 16-year-old boy was admitted because of excessive weight gain over 3 years and purple striae for 1 year. Physical examination revealed Cushingoid features and spotty skin pigmentation on his face, lip, and sclera...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29384929/case-report-of-a-bilateral-adrenal-myelolipoma-associated-with-cushing-disease
#13
Se Yoon Park, Mi Kyung Kwak, Hye Jeong Kim, Hyeong Kyu Park, Kyo-Il Suh, Myung Hi Yoo, So Young Jin, Sumi Yun, Dong Won Byun
RATIONALE: Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive. PATIENT CONCERNS: During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Abdominal computed tomography revealed a well-defined, heterogeneously enhanced bilateral adrenal mass, suggesting a myelolipoma...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29383677/pasireotide-treatment-reduces-cardiometabolic-risk-in-cushing-s-disease-patients-an-italian-multicenter-study
#14
A Albani, F Ferraù, A Ciresi, R Pivonello, C Scaroni, D Iacuaniello, M Zilio, V Guarnotta, A Alibrandi, E Messina, M Boscaro, C Giordano, A Colao, S Cannavo
PURPOSE: Patients with Cushing's disease (CD) experience metabolic alterations leading to increased cardiovascular mortality. Recently, the visceral adiposity index (VAI) has been proposed as a marker of visceral adipose tissue dysfunction (ATD) and of the related cardiometabolic risk. We aimed to evaluate the impact of 12-month pasireotide treatment on cardiometabolic risk in CD patients. METHODS: This is a multicentre, prospective, and observational study. Sixteen CD patients, referred to the Endocrine Units of the University Hospitals of Messina, Napoli, Padova, and Palermo (Italy), successfully treated with pasireotide for 12 month have been enrolled...
January 30, 2018: Endocrine
https://www.readbyqxmd.com/read/29375882/idiopathic-intracranial-hypertension-as-a-presenting-sign-of-adrenal-insufficiency
#15
Sandra Shenouda, Khaled Al-Farawi, Jenna Dolan, Susan L Flesher
Idiopathic intracranial hypertension is a diagnosis of exclusion defined by elevated intracranial pressure without mass lesions or hydrocephalus. Causes of idiopathic intracranial hypertension include obesity, vitamin derangements, antibiotics, corticosteroids, and autoimmune disorders. Cushing's disease and Addison's disease have been associated with idiopathic intracranial hypertension. Secondary adrenal insufficiency following withdrawal of inhaled corticosteroids has been found to be a relatively common phenomenon...
2018: SAGE open medical case reports
https://www.readbyqxmd.com/read/29355797/cyberknife-radiosurgery-in-the-multimodal-management-of-patients-with-cushing-s-disease
#16
Justin M Moore, Elisa Sala, Alvaro Amorin, Hector Martinez, Aprotim C Bhowmik, Steven D Chang, Scott G Soltys, Griffith R Harsh, Laurence Katznelson
OBJECTIVE: Surgery is the primary treatment for Cushing's disease (CD). However, when surgery is not successful at normalizing hypercortisolism, adjuvant radiation, such as stereotactic radiosurgery (SRS), may be useful to improving biochemical control. METHODS: We completed a retrospectively cohort study of consecutively treated patients with CyberKnife (CK) radiosurgery for active CD at Stanford Hospital and Clinics. RESULTS: As first line treatment, all patients underwent transsphenoidal surgery with histological demonstration of an adrenocorticotropic hormone (ACTH) producing pituitary adenoma...
January 16, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29343284/extensive-armc5-genetic-variance-in-primary-bilateral-macronodular-adrenal-hyperplasia-that-started-with-exophthalmos-a-case-report
#17
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
#18
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
#19
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
#20
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
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