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https://www.readbyqxmd.com/read/27922495/toxic-and-endocrine-myopathies
#1
Hans D Katzberg, Charles D Kassardjian
PURPOSE OF REVIEW: This article discusses the clinical features, pathophysiology, and management of toxic and endocrine myopathies. RECENT FINDINGS: Early detection and expeditious correction of metabolic disturbances in endocrinopathies such as Cushing syndrome, thyroid and parathyroid diseases, and acromegaly can minimize and prevent neurologic complications including myopathy. Recently proposed mechanisms of injury in patients with critical illness myopathy include inhibition of protein synthesis, mitochondrial dysfunction, disruption of the ubiquitin-proteasome system, oxidative stress, and disruption of intramuscular calcium homeostasis, which can cause a myosin-loss myopathy...
December 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27919009/successful-percutaneous-ct-guided-microwave-ablation-of-adrenal-gland-for-ectopic-cushing-syndrome
#2
Rebecca Zener, Andrew Zaleski, Stan H Van Uum, Daryl K Gray, Amol Mujoomdar
Adrenocorticotropic hormone production by pancreatic neuroendocrine tumor (PNET) is rare and results in hyperstimulation of the adrenal gland to produce ectopic Cushing syndrome. Our case showcases the safety and effectiveness of percutaneous CT-guided microwave ablation of the adrenal gland in a 49-year-old female with PNET and hepatic metastases who presented with ectopic Cushing syndrome despite surgical resection of the primary pancreatic tumor and left adrenal gland. Prior to ablation, the right adrenal gland measured 4...
November 29, 2016: Clinical Imaging
https://www.readbyqxmd.com/read/27903577/adrenal-cushing-syndrome-with-detectable-acth-from-an-unexpected-source
#3
Thinzar M Lwin, Nehal Galal, Shweta Gera, Jennifer L Marti
Mixed corticomedullary adrenal tumours (MCMT) are rare. We describe the second reported case of a male patient presenting with hypertension and Cushing syndrome with MCMT. A man aged 48 years presented with hypertension and signs of Cushing syndrome. 24-hour urine cortisol was elevated, with detectable adrenocorticotropic hormone (ACTH). A high-dose dexamethasone suppression test indicated an adrenal or ectopic Cushing syndrome. Plasma metanephrines were normal. A 3 cm left adrenal mass was identified without potential ectopic sources of ACTH on imaging...
November 30, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27901643/bilateral-testicular-tumors-resulting-in-recurrent-cushing-s-syndrome-after-bilateral-adrenalectomy
#4
Troy Puar, Manon Engels, Antonius E van Herwaarden, Fred C G J Sweep, Christina Hulsbergen-van de Kaa, Karin Kamphuis-van Ulzen, Vasileios Chortis, Wiebke Arlt, Nike Stikkelbroeck, Hedi L Claahsen-van der Grinten, Ad R M M Hermus
CONTEXT: Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing's disease is extremely rare. PATIENT: We present a rare case of a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing's disease with complete clinical resolution. Cushingoid features recurred 12 years later, along with bilateral testicular enlargement. Hormonal tests confirmed ACTH-dependent Cushing's. Surgical resection of the testicular tumors led to clinical and biochemical remission...
November 30, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27882768/-adrenocortical-oncocytoma-presenting-as-cushing%C3%A2-s-syndrome-in-pregnancy-with-spontaneous-postpartum-uterine-rupture
#5
M Kotoulová, I Mikysková, J Dušková, J Vláčil, M Dvořák, M Halaška
OBJECTIVE: The description of rare case of the Cushings syndrome in pregnancy resulting in the spontaneous rupture of uterus. DESIGNS: A case report. SETTING: Department of Gynecology and Obstetrics, 1st Medical Faculty of Charles University and Hospital Na Bulovce. CASE REPORT: The authors report the case of a 33 year old woman, who was admitted to the clinic in 30th week of pregnancy for elevated blood pressure and hypokalemia...
2016: Ceská Gynekologie
https://www.readbyqxmd.com/read/27879325/cushing-syndrome-in-a-child-due-to-pro-opiomelanocortin-pomc-secretion-from-a-yolk-sac-tumor
#6
Evelien Gevers, Suzanne Meredith, Pratik Shah, John Torpiano, Catherine Peters, Neil Sebire, Olga Slater, Anne White, Mehul Datani
CONTEXT: Pituitary microadenomas and adrenal tumours are the most common causes for endogenous Cushing syndrome (CS) in children. CASE DESCRIPTION: We describe a two-year old girl with Cushing syndrome due to ectopic pro-opiomelanocortin (POMC) production from an abdominal yolk sac tumor. Cortisol concentrations were elevated but adrenocorticotropic hormone (ACTH) concentrations were equivocal. The use of antibodies specifically detecting ACTH precursors revealed that plasma ACTH precursors were elevated...
November 22, 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27875378/cushing-syndrome-in-carney-complex-clinical-pathologic-and-molecular-genetic-findings-in-the-17-affected-mayo-clinic-patients
#7
Kathleen M Lowe, William F Young, Charalampos Lyssikatos, Constantine A Stratakis, J Aidan Carney
Carney complex (CNC) is a rare dominantly inherited multiorgan tumoral disorder that includes Cushing syndrome (CS). To establish the Mayo Clinic experience with the CS component, including its clinical, laboratory, and pathologic findings, we performed a retrospective search of the patient and pathologic databases of Mayo Clinic in Rochester, MN, for patients with CNC and clinical or laboratory findings of CS. Thirty-seven patients with CNC were identified. Twenty-nine had clinical, pathologic, or laboratory evidence of an adrenocortical disorder...
November 21, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27873108/flushing-in-neuro-endocrinology
#8
REVIEW
Fady Hannah-Shmouni, Constantine A Stratakis, Christian A Koch
Cutaneous flushing is a common presenting complaint in endocrine disorders. The pathophysiology of flushing involves changes in cutaneous blood flow triggered by multiple intrinsic factors that are either related to physiology or disease. Flushing can be divided into episodic or persistent causes. Episodic flushing is mediated by the release of endogenous vasoactive mediators or medications, while persistent flushing results in a fixed facial erythema with telangiectasia and cyanosis due to slow-flowing deoxygenated blood in large cutaneous blood vessels...
November 21, 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27869874/-pharmacological-treatment-in-adrenal-cushing-s-syndrome
#9
Antonio Stigliano, Lidia Cerquetti, Vincenzo Toscano
ACTH-independent adrenal Cushing's syndrome is the least common form of endogenous hypercortisolism. Recently, advances in genetics have allowed the description of several forms different to pathogenetic etiology, morphostructural characteristics and evolution towards the hypercortisolism. Alongside these, the adrenocortical carcinoma is also frequently responsible of a hypercortisolism clinical picture. The availability of steroidogenesis inhibitors, such as metyrapone and ketoconazole, provides to endocrinologist a therapeutic chance against different metabolic disorders sustained by hypercortisolism...
November 2016: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/27869719/targeted-molecular-imaging-in-adrenal-disease-an-emerging-role-for-metomidate-pet-ct
#10
REVIEW
Iosif A Mendichovszky, Andrew S Powlson, Roido Manavaki, Franklin I Aigbirhio, Heok Cheow, John R Buscombe, Mark Gurnell, Fiona J Gilbert
Adrenal lesions present a significant diagnostic burden for both radiologists and endocrinologists, especially with the increasing number of adrenal 'incidentalomas' detected on modern computed tomography (CT) or magnetic resonance imaging (MRI). A key objective is the reliable distinction of benign disease from either primary adrenal malignancy (e.g., adrenocortical carcinoma or malignant forms of pheochromocytoma/paraganglioma (PPGL)) or metastases (e.g., bronchial, renal). Benign lesions may still be associated with adverse sequelae through autonomous hormone hypersecretion (e...
November 18, 2016: Diagnostics
https://www.readbyqxmd.com/read/27865872/thymic-hyperplasia-after-treatment-of-acth-dependent-cushing-s-syndrome-can-be-mistaken-for-a-thymic-epithelial-tumor
#11
Jaydira Del Rivero, Chul Kim, Mary Eid, Mark A Ahlman, Lynnette Nieman, Arun Rajan
No abstract text is available yet for this article.
November 16, 2016: Journal of Thoracic Oncology
https://www.readbyqxmd.com/read/27865591/outcome-of-adrenalectomy-for-subclinical-hypercortisolism-and-cushing-syndrome
#12
Marco Raffaelli, Carmela De Crea, Gerardo D'Amato, Pierpaolo Gallucci, Celestino P Lombardi, Rocco Bellantone
BACKGROUND: We compared operative and metabolic outcomes in patients with subclinical Cushing syndrome and Cushing syndrome caused by unilateral adrenal lesion, aiming to clarify the role of glucocorticoid replacement treatment in patients with subclinical Cushing syndrome after adrenalectomy. METHODS: The medical records of all the patients who underwent unilateral adrenalectomy for subclinical Cushing syndrome or Cushing syndrome were reviewed. Diagnostic criteria for subclinical Cushing syndrome were a pathologic dexamethasone suppression test plus 2 additional criteria...
November 16, 2016: Surgery
https://www.readbyqxmd.com/read/27864805/endocrine-hypertension-a-practical-approach
#13
REVIEW
Joseph M Pappachan, Harit N Buch
Elevated blood pressure resulting from few endocrine disorders (endocrine hypertension) accounts for a high proportion of cases of secondary hypertension. Although some features may be suggestive, many cases of endocrine hypertension remain silent until worked up for the disease. A majority of cases result from primary aldosteronism. Other conditions that can cause endocrine hypertension are: congenital adrenal hyperplasia, Liddle syndrome, pheochromocytomas, Cushing's syndrome, acromegaly, thyroid diseases, primary hyperparathyroidism and iatrogenic hormone manipulation...
November 19, 2016: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/27855236/vandetanib-induces-a-marked-anti-tumor-effect-and-amelioration-of-ectopic-cushing-s-syndrome-in-a-medullary-thyroid-carcinoma-patient
#14
Hashem Bseiso, Naama Lev-Cohain, David J Gross, Simona Grozinsky-Glasberg
: A 55-year-old woman diagnosed with sporadic MTC underwent total thyroidectomy 20 years ago. After the first surgery, elevated calcitonin levels in parallel with local disease persistence were noted and therefore she underwent repeated neck dissections. During follow-up, multiple foci of metastatic disease were noted in the neck and mediastinal lymph nodes, lungs and bones; however, the disease had an indolent course for a number of years, in parallel with a calcitonin doubling time of more than two years and without significant symptoms...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27828691/long-term-complete-remission-of-crooke-s-corticotropinoma-after-temozolomide-treatment
#15
Maria Kurowska, Jerzy S Tarach, Joanna Malicka, Grzegorz Zieliński, Maria Maksymowicz, Piotr Denew
INTRODUCTION: Crooke's corticotropinomas are the unique cause of Cushing's disease. The majority of them are aggressive macroadenomas, refractory to conventional therapy, with a high recurrence rate. The aim of the study was the presentation, in relation to data from the literature, of a case of a patient with ACTH-dependent Cushing's syndrome caused by recurrent Crooke's cells corticotropinoma, who achieved 33-month complete remission after treatment with temozolomide (TMZ). CASE REPORT: A 54-year-old man was diagnosed with Cushing's disease five years earlier on the basis of a typical clinical picture and hormonal tests...
2016: Endokrynologia Polska
https://www.readbyqxmd.com/read/27813054/mechanisms-of-aberrant-pka-activation-by-c%C3%AE-subunit-mutations
#16
D Calebiro, K Bathon, I Weigand
Somatic mutations in PRKACA, coding for the catalytic α subunit of protein kinase A (PKA), have been recently identified as the most frequent genetic alteration in cortisol-secreting adrenocortical adenomas, which are responsible for adrenal Cushing's syndrome. The mutations identified so far lie at the interface between the catalytic (C) and regulatory (R) subunit of PKA. Detailed functional studies of the most frequent of these mutations (L206R) as well as of another one in the same region of the C subunit (199_200insW) have revealed that these mutations cause constitutive activation of PKA and lack of regulation by cAMP...
November 3, 2016: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/27805346/mediastinal-lipomatosis-with-dyslipidemia-cause-of-dyspnea
#17
Aradhana Singh
We report a case who presented to us with dyspnea and was found to have ML in the absence of steroid use, diabetes, Cushing syndrome and obesity. This case provides an important differential diagnosis in a patient presenting with dyspnea widened mediastinum and cardiomegaly. This is probably among very few cases of ML with dyslipidemia.
November 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27803417/fibromyalgia-in-a-patient-with-cushing-s-disease-accompanied-by-central-hypothyroidism
#18
Nobumasa Ohara, Shinichi Katada, Takaho Yamada, Naomi Mezaki, Hiroshi Suzuki, Akiko Suzuki, Osamu Hanyu, Yuichiro Yoneoka, Izumi Kawachi, Takayoshi Shimohata, Akiyoshi Kakita, Masatoyo Nishizawa, Hirohito Sone
A 39-year-old woman with a 3-year history of a rounded face developed widespread myalgia. Detailed examinations revealed no disorders that could explain the pain other than concomitant Cushing's disease and central hypothyroidism. Both the hypercortisolemia and hypothyroidism completely resolved after the patient underwent surgery to treat Cushing's disease, but she continued to experience unresolved myalgia and met the diagnostic criteria for fibromyalgia. Few studies have so far investigated patients with fibromyalgia associated with Cushing's syndrome...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27789139/design-synthesis-and-evaluation-of-2s-4r-ketoconazole-sulfonamide-analogs-as-potential-treatments-for-metabolic-syndrome
#19
Benjamin E Blass, Pravin Iyer, Magid Abou-Gharbia, Wayne E Childers, John C Gordon, Mercy Ramanjulu, George Morton, Premkumar Arumugam, Joshodeep Boruwa, John Ellingboe, Sayan Mitra, Rajashekar Reddy Nimmareddy, Shalini Paliwal, Jamallamudi Rajasekhar, Savithiri Shivakumar, Pratima Srivastava, Raghuram S Tangirala, Konda Venkataramanaiah, Mahesh Yanamandra
Metabolic Syndrome, also referred to as 'Syndrome X' or 'Insulin Resistance Syndrome,' remains a major, unmet medical need despite over 30years of intense effort. Recent research suggests that there may be a causal link between this condition and abnormal glucocorticoid processing. Specifically, dysregulation of the hypothalamic-pituitary-adrenocortical (HPA) axis leads to increased systemic cortisol concentrations. Cushing' syndrome, a disorder that is also typified by a marked elevation in levels of cortisol, produces clinical symptomology that is similar to those observed in MetS, and they can be alleviated by decreasing circulating cortisol concentrations...
December 1, 2016: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/27782371/a-pharmacokinetic-model-of-a-tissue-implantable-cortisol-sensor
#20
Michael A Lee, Naveed Bakh, Gili Bisker, Emery N Brown, Michael S Strano
Cortisol is an important glucocorticoid hormone whose biochemistry influences numerous physiological and pathological processes. Moreover, it is a biomarker of interest for a number of conditions, including posttraumatic stress disorder, Cushing's syndrome, Addison's disease, and others. An implantable biosensor capable of real time monitoring of cortisol concentrations in adipose tissue may revolutionize the diagnosis and treatment of these disorders, as well as provide an invaluable research tool. Toward this end, a mathematical model, informed by the physiological literature, is developed to predict dynamic cortisol concentrations in adipose, muscle, and brain tissues, where a significant number of important processes with cortisol occur...
October 26, 2016: Advanced Healthcare Materials
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