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https://www.readbyqxmd.com/read/28626863/prolactin-correction-for-adequacy-of-petrosal-sinus-cannulation-may-diminish-diagnostic-accuracy-in-cushing-s-disease
#1
Sunita Mc De Sousa, Ann I McCormack, Shaun McGrath, David J Torpy
OBJECTIVE: Petrosal venous prolactin concentrations have been promoted to improve the diagnostic accuracy of inferior petrosal sinus sampling (IPSS), beyond that achieved with ACTH measurement alone, in diagnosing a pituitary ACTH source, and determining corticotrophinoma side (L/R). Our objective was to assess the effect of using prolactin to confirm adequacy of petrosal cannulation in a cohort of patients with ACTH-dependent Cushing's syndrome. DESIGN: Retrospective cohort study...
June 19, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28614036/an-individualized-approach-to-the-evaluation-of-cushing-syndrome
#2
Susmeeta T Sharma
Cushing syndrome (CS) is caused by chronic exposure to excess glucocorticoids. Early recognition and treatment of hypercortisolemia can lead to decreased morbidity and mortality. The diagnosis of CS and thereafter, establishing the cause can often be difficult, especially in patients with mild and cyclic hypercortisolism. Surgical excision of the cause of excess glucocorticoids is the optimal treatment for CS. Medical therapy (steroidogenesis inhibitors, medications that decrease adrenocorticotropic hormone [ACTH] levels or glucocorticoid antagonists) and pituitary radiotherapy may be needed as adjunctive treatment modalities in patients with residual, recurrent or metastatic disease, in preparation for surgery, or when surgery is contraindicated...
June 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28614007/ectopic-cushing-s-syndrome-a-ten-year-experience-from-a-tertiary-care-centre-in-southern-india
#3
Samantha Sathyakumar, Thomas Vizhalil Paul, Hesargatta Shyamsunder Asha, Birla Roy Gnanamuthu, M J Paul, Deepak Thomas Abraham, Simon Rajaratnam, Nihal Thomas
OBJECTIVE: Ectopic ACTH secretion is a less common cause of Cushing's syndrome, and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care centre in Southern India. METHODS: The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features which included radiological findings and PET scans, management, details of follow-up and outcomes were documented...
June 14, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28614003/biochemical-control-during-long-term-follow-up-of-230-adult-patients-with-cushing-s-disease-a-multi-center-retrospective-study
#4
Eliza B Geer, Ismat Shafiq, Murray B Gordon, Vivien Bonert, Alejandro Ayala, Ronald S Swerdloff, Laurence Katznelson, Yelena Lalazar, Ekaterina Manuylova, Karen J Pulaski-Liebert, John D Carmichael, Zeina Hannoush, Vijaya Surampudi, Michael S Broder, Dasha Cherepanov, Marianne Eagan, Jackie Lee, Qayyim Said, Maureen P Neary, Beverly Mk Biller
OBJECTIVES: Cushing's disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately-treated CD is associated with significant morbidity and elevated mortality. Multi-center data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. The study purpose was to describe the burden of illness and treatment outcomes for CD patients. METHODS: Eight pituitary centers in 4 US regions participated in this multi-center retrospective chart review study...
June 14, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28599697/paraneoplastic-cushing-syndrome-due-to-wilm-s-tumor
#5
Mahwish Faizan, Jaida Manzoor, Muhammad Saleem, Saadia Anwar, Qaiser Mehmood, Ambreen Hameed, Agha Shabbir Ali
Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28597198/long-term-treatment-of-cushing-s-disease-with-pasireotide-5-year-results-from-an-open-label-extension-study-of-a-phase-iii-trial
#6
S Petersenn, L R Salgado, J Schopohl, L Portocarrero-Ortiz, G Arnaldi, A Lacroix, C Scaroni, S Ravichandran, A Kandra, B M K Biller
BACKGROUND: Treating hypercortisolism in patients with Cushing's disease after failed surgery often requires chronic medication, underlining the need for therapies with favourable long-term efficacy and safety profiles. METHODS: In a randomised, double-blind study, 162 adult patients with persistent/recurrent or de novo Cushing's disease received pasireotide. Patients with mean urinary free cortisol at/below the upper limit of normal or clinical benefit at month 12 could continue receiving pasireotide during an open-ended, open-label phase, the outcomes of which are described herein...
June 9, 2017: Endocrine
https://www.readbyqxmd.com/read/28590513/diagnostic-accuracy-of-increased-urinary-cortisol-cortisone-ratio-to-differentiate-acth-dependent-cushing-s-syndrome
#7
Filippo Ceccato, Laura Trementino, Mattia Barbot, Giorgia Antonelli, Mario Plebani, Luca Denaro, Daniela Regazzo, Federico Rea, Anna Chiara Frigo, Carolina Concettoni, Marco Boscaro, Giorgio Arnaldi, Carla Scaroni
BACKGROUND AND AIM: Differential diagnosis between Cushing's Disease (CD) and Ectopic ACTH Syndrome (EAS) may be a pitfall for endocrinologists. The increasing use in clinical practice of chromatography and mass spectrometry improves the measurement of urinary free cortisol (UFF) and cortisone (UFE). We have recently observed that cortisol to cortisone ratio (FEr) was higher in a small series of EAS; in this paper we collected a larger number of ACTH-dependent CS to study the role of FEr to characterize the source of corticotropin secretion...
June 7, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28584167/severe-cushing-s-syndrome-due-to-small-cell-prostate-carcinoma-a-case-and-review-of-literature
#8
Marianne Elston, Veronica Crawford, Michael Swarbrick, Michael Dray, Michelle Head, John V Conaglen
Cushings syndrome (CS) due to ectopic adrenocorticotrophic hormone (ACTH) is associated with a variety of tumours most of which arise in the thorax or abdomen. Prostate carcinoma is a rare but important cause of rapidly progressive CS. To report a case of severe CS due to ACTH production from prostate neuroendocrine carcinoma and summarise previous published cases. A 71 year old man presented with profound hypokalaemia, oedema and new onset hypertension. He reported two weeks of weight gain, muscle weakness, labile mood and insomnia...
June 5, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28579289/effects-of-anti-somatostatin-agents-on-glucose-metabolism
#9
REVIEW
B Vergès
The anti-somatostatin agents used to treat acromegaly, Cushing's disease and neuroendocrine tumours also have hyperglycaemic effects. This is particularly true for pasireotide. Hyperglycaemic events are seen in 57-73% of patients with Cushing's treated with pasireotide, with a need to initiate antidiabetic treatment in about 50% of these patients. In acromegaly, treatment with pasireotide induces hyperglycaemia in 29-61% of patients. Pasireotide-induced hyperglycemia occurs early, within the first 3 months of treatment, due to a decrease in insulin secretion secondary to a fall in secretion of GLP-1 and GIP, and potentially also due to a direct inhibitory effect of pasireotide on beta cells...
June 1, 2017: Diabetes & Metabolism
https://www.readbyqxmd.com/read/28570067/lead-optimization-generates-cyp11b1-inhibitors-of-pyridylmethyl-isoxazole-type-with-improved-pharmacological-profile-for-the-treatment-of-cushing-s-disease
#10
Juliette Emmerich, Chris J van Koppen, Jens L Burkhart, Qingzhong Hu, Lorenz Siebenbürger, Carsten Boerger, Claudia Scheuer, Matthias W Laschke, Michael D Menger, Rolf W Hartmann
Cushing's disease, characterized by elevated plasma cortisol levels, can be controlled by inhibition of 11β-hydroxylase (CYP11B1). The previously identified selective and potent CYP11B1 inhibitor 5-((5-methylpyridin-3-yl)methyl)-2-phenylpyridine Ref 7 (IC50= 2 nM) exhibited promutagenic potential as well as very low oral bioavailability in rats (F = 2%) and was therefore modified to overcome these drawbacks. Successful lead optimization resulted in similarly potent and selective 5-((5-methoxypyridin-3-yl)methyl)-3-phenylisoxazole 25 (IC50 = 2 nM, 14-fold selectivity over CYP11B2), exhibiting a superior pharmacological profile with no mutagenic potential...
June 9, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28554884/ichthyosis-linearis-circumflexa-with-bamboo-hair-challenges-in-the-diagnosis-and-management
#11
Tanvi Dev, Marwaha Raman Kumar, Gomathy Sethuraman
A 15-year-old boy had persistent and refractory erythroderma since early childhood. His parents noticed polycyclic skin lesions and hair fragility around the age of 5 years. He was treated by a local untrained practitioner for more than 3 years without any significant improvement, and he developed weight gain, thinning of skin, muscle weakness and growth retardation. He was evaluated in 2015 and found to have iatrogenic Cushing's disease with severe skeletal complications and pituitary-adrenal-gonadal suppression, which persisted despite gradual withdrawal of steroids...
May 28, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28553179/peptide-receptor-radionuclide-therapy-with-177-lu-dotatate-for-metastatic-neuroendocrine-tumor-occurring-in-association-with-multiple-endocrine-neoplasia-type-1-and-cushing-s-syndrome
#12
Chinna Naik, Sandip Basu
Neuroendocrine tumor (NET) occurring in association with other endocrine syndromes forms a distinct entity. The aim was to assess the therapy response profile of the routine peptide receptor radionuclide therapy (PRRT) in this relatively uncommon but clinically challenging subgroup of patients. A retrospective analysis was undertaken from the case records from those who were treated with (177)Lu-DOTATATE for metastatic NET. In addition to assessing the therapeutic efficacy, emphasis was also given to study lesional sites and scan pattern...
April 2017: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28543178/volumetric-mri-analysis-in-patients-with-short-term-remission-of-cushing-s-disease
#13
Hong Jiang, Jie Ren, Na-Ying He, Chang Liu, Yu-Hao Sun, Fang-Fang Jian, Liu-Guan Bian, Jian-Kang Shen, Fu-Hua Yan, Si-Jan Pan, Qing-Fang Sun
OBJECTIVE: The data on patients with short-term remission of Cushing's disease (CD) might provide information that is not available from previous long-term remission studies. We aimed to investigate structural changes in the brain in these patients and to examine whether these changes were associated with clinical characteristics. DESIGN: A cross-sectional study was performed. METHODS: Thirty-four CD patients (14 with CD in short-term remission and 20 with active CD) and 34 controls matched for age, sex, and education underwent clinical evaluation and magnetic resonance imaging brain scans...
May 20, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28533356/loss-of-function-mutations-in-the-cables1-gene-are-a-novel-cause-of-cushing-s-disease
#14
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
#15
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/28530316/-radiosurgery-for-pituitary-adenomas
#16
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/28529722/recent-advances-in-understanding-cushing-disease-resistance-to-glucocorticoid-negative-feedback-and-somatic-usp8-mutations
#17
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/28522647/prkar1a-mutation-causing-pituitary-dependent-cushing-disease-in-a-patient-with-carney-complex
#18
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/28520590/role-of-tyrosine-kinase-inhibitors-in-the-treatment-of-pituitary-tumours-from-bench-to-bedside
#19
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/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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