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ectopic ACTH

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https://www.readbyqxmd.com/read/27903577/adrenal-cushing-syndrome-with-detectable-acth-from-an-unexpected-source
#1
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/27879325/cushing-syndrome-in-a-child-due-to-pro-opiomelanocortin-pomc-secretion-from-a-yolk-sac-tumor
#2
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/27855236/vandetanib-induces-a-marked-anti-tumor-effect-and-amelioration-of-ectopic-cushing-s-syndrome-in-a-medullary-thyroid-carcinoma-patient
#3
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/27746436/metastatic-pancreatic-neuroendocrine-tumor-that-progressed-to-ectopic-adrenocorticotropic-hormone-acth-syndrome-with-growth-hormone-releasing-hormone-ghrh-production
#4
Rie Tadokoro, Shotaro Sato, Fumiko Otsuka, Makoto Ueno, Shinichi Ohkawa, Hideki Katakami, Matsuo Taniyama, Shoichiro Nagasaka
The patient was a 61-year-old woman who had a well-differentiated pancreatic neuroendocrine tumor (PNET) with lymph node metastasis. After 15 months of octreotide treatment, glucose control deteriorated and pigmentation of the tongue and moon face developed, leading to the diagnosis of ectopic adrenocorticotropic hormone (ACTH) syndrome. An abnormal secretion of growth hormone (GH) was identified, and the plasma growth hormone-releasing hormone (GHRH) level was elevated. A tumor biopsy specimen positively immunostained for ACTH and GHRH...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27725464/ectopic-adrenocorticotropic-hormone-syndrome-caused-by-neuroendocrine-carcinoma-of-the-colon
#5
Kazuyo Fujimoto, Takatoshi Nakashima, Kazunari Sasaki, Kenichi Hayashi, Masao Hanafusa, Shiei Yoshida, Satoshi Myojo, Shun-Ichi Yoshida, Shigeaki Sawai, Nobuya Sano
A 48-year-old woman with a history of autoimmune hemolytic anemia and taking long-term corticosteroid therapy presented with a 3-month history of general fatigue, abdominal distension, and pigmentation. A computed tomography scan of the abdomen showed a tumor in the sigmoid colon and multiple metastatic nodules in the liver. A colonoscopy revealed an obstructing mass with the presence of an irregular ulcer in the sigmoid colon. Following biopsy and histopathological analysis, the patient was diagnosed with neuroendocrine carcinoma (NEC) of the colon...
2016: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/27699708/frequency-of-cushing-s-syndrome-due-to-acth-secreting-adrenal-medullary-lesions-a-retrospective-study-over-10-years-from-a-single-center
#6
Henrik Falhammar, Jan Calissendorff, Charlotte Höybye
Cushing's syndrome due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions has occasionally been described. We retrospectively reviewed all 164 cases of Cushing's syndrome and 77 cases of pheochromocytomas during 10 years. Of all cases with Cushing's syndrome, only two cases (1.2 %) were due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions (one case of pheochromocytoma and one case of adrenal medullary hyperplasia). Of all pheochromocytomas only the above-mentioned case (1...
October 3, 2016: Endocrine
https://www.readbyqxmd.com/read/27695589/ectopic-cushing-s-syndrome-due-to-retroperitoneal-acth-producing-paragangliomas
#7
Fan Chen, Xiangyu Wang, Yang Wang, Hui Meng, Xinguo Hou, Yaofeng Zhu, Wei Gao, Xuewen Jiang, Shouzhen Chen, Zhaocun Zhang, Zhichuan Zou, Tianyi He, Yue Yang, Kejia Zhu, Yong Wang, Yaxiao Liu, Jianfeng Cui, Benkang Shi, Gang Yin
Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH)...
September 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/27562041/-etiology-analysis-of-522-hospitalized-cases-with-cushing-syndrome
#8
L L Li, J T Dou, G Q Yang, W J Gu, Z H Lü, Y M Mu
OBJECTIVE: To summarize the characteristics of the etiology distribution of Cushing syndrome (CS) patients in Chinese PLA general hospital during the past 20 years. METHODS: All data of CS patients admitted to Chinese PLA General Hospital between 1995 and 2014 were collected. The sex ratio of patients, visiting age, final diagnosis were retrospectively analyzed. RESULTS: From January 1995 to December 2014, 522 patients with CS were admitted, of which, 134 (25...
August 16, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27547198/acth-antagonists
#9
REVIEW
Adrian John Clark, Rachel Forfar, Mashal Hussain, Jeff Jerman, Ed McIver, Debra Taylor, Li Chan
Adrenocorticotropin (ACTH) acts via a highly selective receptor that is a member of the melanocortin receptor subfamily of type 1 G protein-coupled receptors. The ACTH receptor, also known as the melanocortin 2 receptor (MC2R), is unusual in that it is absolutely dependent on a small accessory protein, melanocortin receptor accessory protein (MRAP) for cell surface expression and function. ACTH is the only known naturally occurring agonist for this receptor. This lack of redundancy and high degree of ligand specificity suggests that antagonism of this receptor could provide a useful therapeutic aid and a potential investigational tool...
2016: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/27523914/ectopic-adrenocortical-carcinoma-located-in-the-ovary
#10
Farida Chentli, Nadia Terki, Said Azzoug
AIM: Ovarian corticosteroid-producing tumors are exquisitely rare. Our aim was to describe the first case observed in our practice. CASE HISTORY: A 34-year-old female was referred for Cushing's syndrome (CS) occurring in the postpartum period. Clinical examination showed severe CS with diabetes mellitus, hypertension, and a large mass in the right lower abdomen. Biochemistry demonstrated corticotropin (ACTH)-independent CS (cortisol=1900ng/mL (n=50-250), ACTH<10pg/mL (n=20-46)) with estradiol and testosterone overproduction...
October 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27489549/role-of-acth-in-the-interactive-paracrine-regulation-of-adrenal-steroid-secretion-in-physiological-and-pathophysiological-conditions
#11
REVIEW
Hervé Lefebvre, Michaël Thomas, Céline Duparc, Jérôme Bertherat, Estelle Louiset
In the normal human adrenal gland, steroid secretion is regulated by a complex network of autocrine/paracrine interactions involving bioactive signals released by endothelial cells, nerve terminals, chromaffin cells, immunocompetent cells, and adrenocortical cells themselves. ACTH can be locally produced by medullary chromaffin cells and is, therefore, a major mediator of the corticomedullary functional interplay. Plasma ACTH also triggers the release of angiogenic and vasoactive agents from adrenocortical cells and adrenal mast cells and, thus, indirectly regulates steroid production through modulation of the adrenal blood flow...
2016: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/27464539/-comparison-of-efficacy-between-the-serum-cortisol-and-24-hour-urine-free-cortisol-in-combined-dexamethasone-suppression-test-in-the-diagnosis-of-cushing-syndrome
#12
L Lu, J H Chen, H J Zhu, A L Song, M Li, S Chen, H Pan, F Y Gong, R Z Wang, B Xing, Y Yao, M Feng, Z L Lu
OBJECTIVE: To compare the sensitivity and specificity between the 24 hour urine free cortisol (24 h UFC) and serum cortisol in dexamethasone suppression test (DST) in the diagnosis of Cushing syndrome (CS). METHODS: Combined low dose DST (LDDST) and high dose DST (HDDST) were carried out in 67 cases of CS with surgically confirmed cases in recent 3 years(from January 2011 to November 2015). The serum cortisol and 24 h UFC were collected simultaneously for each subject and the sensitivity and specificity of serum cortisol and 24 h UFC were compared...
July 19, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27454103/therapeutic-compounds-for-cushing-s-syndrome-a-patent-review-2012-2016
#13
Li Ma, Lina Yin, Qingzhong Hu
INTRODUCTION: Endogenous Cushing's syndrome (CS) is a set of disorders caused by chronic exposure to excess glucocorticoids induced by neuroendocrine tumors in pituitary, adrenals, and infrequently other sites (ectopic ACTH syndrome). Due to various comorbidities, CS patients exhibit higher risks of cardiovascular diseases and thus increased mortality. Pharmaceutical therapy is an important constituent of treatment regimen. AREAS COVERED: Patents published since 2012 are reviewed, which claim therapeutic compounds interfering with ACTH secretion and down-stream signal transduction, inhibiting cortisol biosynthesis and antagonizing glucocorticoid receptors...
July 30, 2016: Expert Opinion on Therapeutic Patents
https://www.readbyqxmd.com/read/27445975/role-of-acth-and-other-hormones-in-the-regulation-of-aldosterone-production-in-primary-aldosteronism
#14
REVIEW
Nada El Ghorayeb, Isabelle Bourdeau, André Lacroix
The major physiological regulators of aldosterone production from the adrenal zona glomerulosa are potassium and angiotensin II; other acute regulators include adrenocorticotropic hormone (ACTH) and serotonin. Their interactions with G-protein coupled hormone receptors activate cAMP/PKA pathway thereby regulating intracellular calcium flux and CYP11B2 transcription, which is the specific steroidogenic enzyme of aldosterone synthesis. In primary aldosteronism (PA), the increased production of aldosterone and resultant relative hypervolemia inhibits the renin and angiotensin system; aldosterone secretion is mostly independent from the suppressed renin-angiotensin system, but is not autonomous, as it is regulated by a diversity of other ligands of various eutopic or ectopic receptors, in addition to activation of calcium flux resulting from mutations of various ion channels...
2016: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/27387249/cushing-s-syndrome-due-to-ectopic-acth-secretion
#15
Łukasz Cieszyński, Monika Berendt-Obołończyk, Michał Szulc, Krzysztof Sworczak
Cushing's syndrome (CS) is defined as a constellation of clinical signs and symptoms occurring due to hypercortisolism. Cortisol excess may be endogenous or exogenous. The most common cause of CS is glucocorticoid therapy with supraphysiological (higher than in the case of substitution) doses used in various diseases (e.g. autoimmune). One possible CS cause is ectopic (extra-pituitary) ACTH secretion (EAS) by benign or malignant tumours. Since its first description in 1963, EAS aetiology has changed, i.e. as well as small cell lung cancer (SCLC), higher incidence in other malignancies has been reported...
2016: Endokrynologia Polska
https://www.readbyqxmd.com/read/27380554/cushing-syndrome-caused-by-the-pancreatic-neuroendocrine-tumour-and-its-pelvic-metastases
#16
M Yang, B-L Tian, Y Zhang, A-P Su, W-G Wang, P-J Yue
Cushing's syndrome caused by an ectopic tumour secreting adrenocorticotropic hormone (ACTH) is not common. Furthermore, an ACTH-secreting panreatic neoplasm is extremely rare. We present a 27-year old female patient suffering from a pancreatic neuroendocrine tumour (p-NET) with extensive pelvic metastases, which could secret ACTH and cause Cushing's syndrome. The postoperative pathologic examinations of this patient prompted pancreatic poorly differentiated neuroendocrine tumour with extensive metastases of bilateral ovarian, uterus and pelvic peritoneum...
May 15, 2016: West Indian Medical Journal
https://www.readbyqxmd.com/read/27352844/bilateral-inferior-petrosal-sinus-sampling
#17
REVIEW
Benedetta Zampetti, Erika Grossrubatscher, Paolo Dalino Ciaramella, Edoardo Boccardi, Paola Loli
Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing's syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88-100% and 67-100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism...
July 2016: Endocrine Connections
https://www.readbyqxmd.com/read/27352274/ectopic-adrenocorticotropic-hormone-secreting-pituitary-adenomas-an-underestimated-entity
#18
Ulrich J Knappe, Christian Jaspers, Desirée Buschsieweke, Wolf-Dieter Reinbold, Ali Alomari, Wolfgang Saeger, Klaus Ehlenz, W Alexander Mann, Peter Herbert Kann, Joachim Feldkamp
BACKGROUND: The diagnosis of Cushing disease is based on endocrinological parameters, with no single test being specific. In some patients, dynamic thin-slice sellar magnetic resonance imaging fails to detect a pituitary tumor. OBJECTIVE: The purpose of this study is to investigate the role of ectopic pituitary adenoma in this situation. METHODS: In a retrospective chart review, 5 patients (6%) with ectopic adenomas were identified in 83 consecutive patients undergoing transsphenoidal surgery for adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas by 1 surgeon...
June 24, 2016: Neurosurgery
https://www.readbyqxmd.com/read/27330779/neuroendocrine-carcinoma-of-the-ampulla-of-vater-causing-ectopic-adrenocorticotropic-hormone-dependent-cushing-s-syndrome
#19
Akihisa Kato, Kazuki Hayashi, Itaru Naitoh, Kyoji Seno, Yukiko Okada, Tesshin Ban, Hiromu Kondo, Yuji Nishi, Shuichiro Umemura, Yasuki Hori, Makoto Natsume, Takashi Joh
Ectopic adrenocorticotropic hormone (ACTH) is rarely secreted by neuroendocrine tumors. Although neuroendocrine tumors may occur at any site in the gastrointestinal system, they very rarely occur in the ampulla of Vater and have a poor prognosis. The present study described the first Cushing's syndrome as a result of ectopic ACTH arising from the ampulla of Vater neuroendocrine carcinoma. A 69-year-old female was admitted with clinical features of Cushing's syndrome, confirmed biochemically by hypokalemia, and elevated levels of ACTH and cortisol...
July 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27330775/primary-intracranial-neuroendocrine-tumor-with-ectopic-adrenocorticotropic-hormone-syndrome-a-rare-and-complicated-case-report-and-literature-review
#20
Hailong Liu, Mingshan Zhang, Xuan Wang, Yanming Qu, Hongwei Zhang, Chunjiang Yu
Neuroendocrine tumors (NETs) and ectopic adrenocorticotropic hormone (ACTH) syndrome are frequent in adult patients. However, primary intracranial NETs, exhibiting immunonegativity for ACTH, high serum ACTH level and treated with anterior skull base reconstruction, are rare and complicated. We herein present a case of a primary intracranial NET immunonegative for ACTH, resulting in ectopic ACTH syndrome. A 40-year-old woman presented with intermittent rhinorrhea, rapid weight gain, polydipsia, polyuria, hypertension, dimness, bilateral exophthalmus, diminution of vision in the left eye and pigmentation of the skin of the face and trunk...
July 2016: Molecular and Clinical Oncology
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