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Pheochromocytoma

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https://www.readbyqxmd.com/read/28932734/spontaneous-ruptured-pheochromocytoma-an-unusual-case-report-and-literature-review
#1
Ye Seob Jee
Ruptured pheochromocytoma is a rare disease. Its mortality rate is up to 31%-50%. Proper management of ruptured pheochromocytoma remains unclear. A 44-year-old male patient visited our Emergency Department and presented with abrupt onset of left flank pain. His blood pressure was 190/140 mmHg with purse rate of 130 beats/min. CT scan showed 8.1 × 5.6-cm-sized heterogeneously mass with rupture on the left retroperitoneal space and active bleeding. His symptom of abdominal pain was aggravated. Follow-up laboratory analysis revealed elevated WBC count and decreased hemoglobin 2 hours after admission...
September 2017: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/28931294/-carney-triad
#2
L Fiala, I Kocáková, R Šimůnek, E Krejčí, I Babánková, R Šefr
Carney triad is a synchronous or metachronous association of gastric gastrointestinal stromal tumors (GIST), pulmonary chondroma and extra-adrenal paraganglioma. The majority of patients have only one or two components of the triad, all three tumors being found in only about 2% of the patients at the time of the first diagnosis. The most common combination is gastric and pulmonary tumors. We report a case of Carney triad which was diagnosed at Masaryk Memorial Cancer Institute. A 57-year-old female patient with a history of gastric resection for leiomyosarcoma at the age of 14 and with an unclear pulmonary lesion evident on chest X-ray since as early as 2003...
2017: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/28929040/cardiovascular-dysfunction-presenting-as-autonomic-dysreflexia-in-a-patient-with-spinal-cord-injury
#3
Ahmed H Qavi, Salman Assad, Wardha Shabbir, Maryam Kundi, Maham Habib, Sumbal Babar, Mehr Zahid
Autonomic dysreflexia (AD) is a medical emergency that is characterized by hypertension as an autonomic response to noxious stimuli in patients with a history of spinal cord injury at the level of T6 or above. We present the case of a 31-year-old Caucasian male with a history of spinal cord injury at the level of C3-C4, with symptoms described as recurring episodes of hypertension with flushing and sweating above the level of the lesion for the past five to six years. His symptoms are triggered by bowel distention, excitement, a bumpy car ride, or a simple turning of the neck to the left...
July 10, 2017: Curēus
https://www.readbyqxmd.com/read/28928232/sdhd-ablation-promotes-thyroid-tumorigenesis-by-inducing-a-stem-like-phenotype
#4
Amruta Ashtekar, Danielle Huk, Alexa Magner, Krista La Perle, Xiaoli Zhang, Jose Piruat, Jose Lopez-Barneo, Sissy Jhiang, Lawrence Kirschner
Mutations in genes encoding enzymes in the tricarboxylic acid cycle (TCA, also known as the Krebs cycle) have been implicated as causative genetic lesions in a number of human cancers, including renal cell cancers, glioblastomas, and pheochromocytomas. In recent studies, missense mutations in the Succinate dehydrogenase (SDH) complex have also been proposed to cause differentiated thyroid cancer. In order to gain mechanistic insight into this process, we generated mice lacking the SDH subunit D (SDHD) in the thyroid...
September 19, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28925400/the-hif-and-other-quandaries-in-vhl-disease
#5
REVIEW
D Tarade, M Ohh
Mutations in VHL underlie von Hippel-Lindau (VHL) disease, a hereditary cancer syndrome with several subtypes depending on the risk of developing certain combination of classic features, such as clear cell renal cell carcinoma (ccRCC), hemangioblastoma and pheochromocytoma. Although numerous potential substrates and functions of pVHL have been described over the past decade, the best-defined role of pVHL has remained as the negative regulator of the heterodimeric hypoxia-inducible factor (HIF) transcription factor via the oxygen-dependent ubiquitin-mediated degradation of HIF-α subunit...
September 18, 2017: Oncogene
https://www.readbyqxmd.com/read/28925363/generation-of-an-induced-pluripotent-stem-cell-line-from-a-patient-with-hereditary-multiple-endocrine-neoplasia-2b-men2b-syndrome-with-highest-risk-ret-mutation
#6
A Bennaceur-Griscelli, J Hadoux, O Féraud, P Opolon, D Divers, E Gobbo, M Schlumberger, F Griscelli, A G Turhan
Multiple Endocrine Neoplasia Type 2B (MEN2B) is a cancer-predisposing syndrome that affects patients with germline RET mutations. The clinical spectrum of the syndrome includes medullary thyroid carcinoma (MTC) and pheochromocytoma. Currently, there is no satisfactory model recapitulating all the features of the disease especially at the level of stem cells. We generated induced pluripotent stem cells (iPSCs) from a patient with RET mutation at codon 918 who developed pheochromocytoma and MTC. These iPSC had normal karyotype, harboured the RET(M918T) mutation and expressed pluripotency hallmarks...
August 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28924001/diagnosis-of-endocrine-disease-sdhx-mutations-beyond-pheochromocytomas-and-paragangliomas
#7
Massimo Mannelli, Letizia Canu, Tonino Ercolino, Elena Rapizzi, Serena Martinelli, Gabriele Parenti, Giuseppina De Filpo, Gabriella Nesi
Mutations in one of the five genes encoding the succinate dehydrogenase (SDHx) or mitochondrial complex II cause the corresponding family syndromes characterized by the occurrence of pheochromocytomas (PHEO) and paragangliomas (PGL). Recently, other solid growths, such as gastrointestinal stromal tumors (GISTs), renal cell carcinomas (RCCs) and pituitary adenomas (PAs) have been associated with these syndromes. In the absence of prospective studies assessing their frequency, at present, their occurrence seems too infrequent to suggest systematic screening for SDHx mutation carriers...
September 18, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28919049/perioperative-outcomes-of-syndromic-paraganglioma-and-pheochromocytoma-resection-in-patients-with-von-hippel-lindau-disease-multiple-endocrine-neoplasia-type-2-or-neurofibromatosis-type-1
#8
James J Butz, Qi Yan, Travis J McKenzie, Toby N Weingarten, Alexandre N Cavalcante, Irina Bancos, William F Young, Darrell R Schroeder, David P Martin, Juraj Sprung
BACKGROUND: Pheochromocytoma and/or paraganglioma associated with neurofibromatosis type 1, multiple endocrine neoplasia type 2A, and von Hippel-Lindau disease have different catecholamine biochemical phenotypes. We examined perioperative outcomes of pheochromocytoma/paraganglioma resection in 3 syndromic forms. METHODS: Retrospective review of patients undergoing resection of syndromic pheochromocytoma/paraganglioma from 2000 through 2016. RESULTS: Eighty-one patients underwent pheochromocytoma/paraganglioma resection (multiple endocrine neoplasia type 2A, n = 36; neurofibromatosis type 1, n = 26; von Hippel-Lindau disease, n = 19)...
September 14, 2017: Surgery
https://www.readbyqxmd.com/read/28918451/quantitative-18-f-dopa-pet-ct-in-pheochromocytoma-the-relationship-between-tumor-secretion-and-its-biochemical-phenotype
#9
Vincent Amodru, Carole Guerin, Sarkis Delcourt, Pauline Romanet, Anderson Loundou, Bruna Viana, Thierry Brue, Frédéric Castinetti, Frédéric Sebag, Karel Pacak, David Taïeb
INTRODUCTION: (18)F-FDOPA illustrates the properties of uptake and storage of catecholamines in pheochromocytomas (PHEOs). Until now, the relationship between (18)F-FDOPA quantitative parameters and a PHEO secretory profile has not been specifically evaluated. MATERIALS AND METHODS: Fifty-six patients (56% females, median age: 47.5 yrs) with non-metastatic PHEO, evaluated by (18)F-FDOPA PET/CT, were included in this retrospective study. Forty-five patients had negative genetic testing (80...
September 16, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28916965/factors-affecting-operative-efficiency-and-post-operative-convalescence-in-laparoendoscopic-single-site-less-adrenalectomy
#10
Yao-Chou Tsai, Chung-Hsien Chen, Ya-Hui Hu, Leay-Kiaw Er, Che-Hsiung Wu, Shih-Chieh Chueh, Victor Chia-Hsiang Lin
BACKGROUND: Laparoendoscopic single-site (LESS) adrenalectomy is a novel challenging technique which is still under clinical evaluation. Initial reports have revealed its superiority in patient convalescence. In addition, it has been reported that some patient or anatomic factors might affect the ergonomics of LESS adrenalectomy. The aim of this study is to investigate the possible factors that might affect procedural efficiency and patient convalescence in LESS adrenalectomy. METHODS: Between October 2009 and July 2015, 105 consecutive adult patients with benign adrenal tumors, who underwent LESS retroperitoneal adrenalectomy were enrolled in this study...
September 15, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28916336/renal-tubular-and-adrenal-medullary-tumors-in-the-2-year-rat-study-with-canagliflozin-confirmed-to-be-secondary-to-carbohydrate-glucose-malabsorption-in-the-15-month-mechanistic-rat-study
#11
Sandra De Jonghe, Mark D Johnson, Rao N V S Mamidi, Petra Vinken, Bianca Feyen, Godelieve Lammens, Jim Proctor
During preclinical development of canagliflozin, an SGLT2 inhibitor, treatment-related pheochromocytomas, renal tubular tumors (RTT), and testicular Leydig cell tumors were reported in the 2-year rat toxicology study. In a previous 6-month rat mechanistic study, feeding a glucose free diet prevented canagliflozin effects on carbohydrate malabsorption as well as the increase in cell proliferation in adrenal medulla and kidneys, implicating carbohydrate malabsorption as the mechanism for tumor formation. In this chronic study male Sprague-Dawley rats were dosed orally with canagliflozin at high dose-levels (65 or 100 mg/kg/day) for 15 months and received either a standard diet or a glucose-free diet...
September 12, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28915965/can-cerebellin-and-renalase-measurements-contribute-to-the-elimination-of-false-positive-results-in-pheochromocytoma-and-paraganglioma-diagnoses
#12
EDITORIAL
Suleyman Aydin
No abstract text is available yet for this article.
September 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28904863/chimeric-zhhh-neuroglobin-acts-as-a-cell-membrane-penetrating-inducer-of-neurite-outgrowth
#13
Nozomu Takahashi, Wataru Onozuka, Seiji Watanabe, Keisuke Wakasugi
Neuroglobin (Ngb) is a heme protein expressed in the vertebrate brain. We previously engineered a chimeric Ngb protein, in which module M1 of human Ngb is replaced by that of zebrafish Ngb, and showed that the chimeric ZHHH Ngb has a cell membrane-penetrating activity similar to that of zebrafish Ngb and also rescues cells from death caused by hypoxia/reoxygenation as does human Ngb. Recently, it was reported that overexpression of mammalian Ngb in neuronal cells induces neurite outgrowth. In this study, we performed neurite outgrowth assays of chimeric Ngb using rat pheochromocytoma PC12 cells...
September 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28904354/identification-and-characterization-of-the-novel-reversible-and-selective-cathepsin-x-inhibitors
#14
Urša Pečar Fonović, Ana Mitrović, Damijan Knez, Tanja Jakoš, Anja Pišlar, Boris Brus, Bojan Doljak, Jure Stojan, Simon Žakelj, Jurij Trontelj, Stanislav Gobec, Janko Kos
Cathepsin X is a cysteine peptidase involved in the progression of cancer and neurodegenerative diseases. Targeting this enzyme with selective inhibitors opens a new possibility for intervention in several therapeutic areas. In this study triazole-based reversible and selective inhibitors of cathepsin X have been identified. Their selectivity and binding is enhanced when the 2,3-dihydrobenzo[b][1,4]dioxine moiety is present as the R(1) substituent. Of a series of selected triazole-benzodioxine derivatives, compound 22 is the most potent inhibitor of cathepsin X carboxypeptidase activity (Ki = 2...
September 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28902732/multimodality-radionuclide-imaging-in-a-patient-with-hereditary-paraganglioma-pheochromocytoma-syndrome
#15
So Y Yim, Valeria M Moncayo, Francisco J Pasquel, Raghuveer K Halkar
Hereditary paraganglioma (PGL)-pheochromocytoma (PCC) syndrome is a genetic disorder caused by a mutation of the tumor suppressor gene SDHD that results in a predisposition for head and neck PGLs and PCCs. We present a case of a 33-year-old woman where F-FDG PET/CT showed areas of increased uptake in both the adrenal and cervical regions, consistent with PCCs and PGLs, respectively. Further imaging revealed that PCCs were I-MIBG avid, whereas the PGLs were In-octreotide avid. This demonstrates the varying sensitivities of different imaging modalities in regard to neuroendocrine tumors and the potential for treatment using multiple targeted therapies...
September 11, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28902384/-multiple-endocrine-neoplasia
#16
Ludwig Schaaf, Friedhelm Raue
Multiple endocrine neoplasia type 1 and 2 are hereditary cancer syndromes. They are characterized by the occurrence of many benign and malignant tumor types, in MEN1 parathyroid tumors, pituitary tumors, and pancreas tumors, in MEN2 medullary thyroid carcinoma, pheochromocytoma, and parathyroid tumors. The autosomal dominant inherited tumor syndromes are caused by mutations in the MEN1 gene, a tumor suppressor gene, and mutations in the RET gene, an activated oncogene, in MEN2. The clinical expression of the different tumors can vary within and between families, with a good genotype-phenotype correlation in MEN2...
September 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28901374/ferulic-acid-exerts-neuroprotective-effects-against-cerebral-ischemia-reperfusion-induced-injury-via-antioxidant-and-anti-apoptotic-mechanisms-in%C3%A2-vitro-and-in%C3%A2-vivo
#17
Zhongkun Ren, Rongping Zhang, Yuanyuan Li, Yu Li, Zhiyong Yang, Hui Yang
Ferulic acid (FA) is a derivative of cinnamic acid. It is used in the treatment of heart head blood-vessel disease and exerts protective effects against hypoxia/ischemia-induced cell injury in the brain. This study investigated the potential neuroprotective effects of FA against ischemia/reperfusion (I/R)-induced brain injury in vivo and in vitro through hematoxylin and eosin (H&E) and Nissl staining assays, flow cytometry, Hoechst 33258 staining, quantitative PCR, western blot analysis and fluorescence microscopic analysis...
September 7, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28895799/protective-effects-of-bellidifolin-in-hypoxia-induced-in-pheochromocytoma-cells-pc12-and-underlying-mechanisms
#18
Zhi-Ying Zhao, Yang-Yang Gao, Li Gao, Ming Zhang, He Wang, Chun-Hong Zhang
Bellidifolin, a xanthone compound derived from plants of Gentiana species, is known to exert a variety of pharmacological activities including anti-oxidation, anti-inflammatory and antitumor actions as well as a protective effect on cerebral ischemic nerve injury. The aim of this study was to examine the protective effects of bellidifolin on nerve injury produced by hypoxia and possible underlying mechanisms using pheochromocytoma cells (PC12). Data showed that the viability of PC12 cells subjected to hypoxia resulted in a significant decrease; however; pretreatment with certain concentrations of bellidifolin (20 or 40 μmol/L) prior to hypoxia significantly increased the survival rate...
September 12, 2017: Journal of Toxicology and Environmental Health. Part A
https://www.readbyqxmd.com/read/28892307/purification-of-polysaccharide-from-artificially-cultivated-anoectochilus-roxburghii-wall-lindl-by-high-speed-counter-current-chromatography-and-its-antitumor-activity
#19
Xiaoling Yu, Shouer Lin, Jinque Zhang, Liying Huang, Hong Yao, Shaoguang Li
To establish a systematic method for the extraction, purification, characterization and antitumor activity study of polysaccharide from artificially cultivated Anoectochilus roxburghii(wall.) Lindl. (AC-ARPS). High-speed counter current chromatography with two-phase aqueous systems was successfully applied to purify AC-ARPS after one-step separation. The purity of the AC-ARPS obtained by phenol/sulfuric acid method was 95.01%. The chemical structures of AC-ARPS were identified by a series analytical methods including high-performance liquid chromatography and liquid chromatography with mass spectrometry...
September 11, 2017: Journal of Separation Science
https://www.readbyqxmd.com/read/28890865/functional-imaging-of-paragangliomas-with-an-emphasis-on-von-hippel-lindau-associated-disease-a-mini-review
#20
REVIEW
Ioannis Ilias, Georgios Meristoudis
Few reports have presented data and results on functional (i.e., nuclear medicine) imaging of paragangliomas and pheochromocytomas (PGLs/PHEOs) for von Hippel-Lindau (VHL) patients. Nuclear medicine localization modalities for chromaffin tumors can be specific or nonspecific. Specific methods make use of the expression of the human norepinephrine transporter (hNET) and vesicular monoamine transporters (VMATs) by these tumors. These permit the use of radiolabeled ligands that enter the synthesis and storage pathway of catecholamines...
2017: Journal of Kidney Cancer and VHL
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