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Pheochromocytoma

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https://www.readbyqxmd.com/read/28339047/hypothalamo%C3%A2-hypophysial-system-in-rats-with-autotransplantation-of-the-adrenal-cortex
#1
Nae Takizawa, Susumu Tanaka, Souichi Oe, Taro Koike, Tadashi Matsuda, Hisao Yamada
Patients with bilateral pheochromocytoma often require an adrenalectomy. Autotransplantation of the adrenal cortex is an alternative therapy that could potentially be performed instead of receiving glucocorticoid replacement following adrenalectomy. Adrenal cortex autotransplantation aims to avoid the side effects of long‑term steroid treatment and adrenal insufficiency. Although the function of the hypothalamo‑hypophysial system is critical for patients who have undergone adrenal cortex autotransplantation, the details of that system, with the exception of adrenocorticotropic hormone in the subjects with adrenal autotransplantation, have been overlooked for a long time...
March 24, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28337414/pheochromocytoma-with-negative-metanephrines-a-rarity-and-the-significance-of-dopamine-secreting-tumors
#2
Michael Bozin, Alastair Lamb, Lydia Johns Putra
We describe a case of a 25-year-old female with a dopamine secreting PPGL diagnosed retrospectively with biochemical analysis. This finding resulted in change in approach to investigation and management, given their important clinical implications. There are important differences in management of dopamine secreting PPGL compared to classical noradrenaline and adrenaline-secreting PPGL. This includes the risk of peri-operative cardiovascular collapse peri-operatively with alpha/beta blockade, risk of malignancy/recurrence, and associated genetic abnormalities...
May 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28336782/functional-imaging-signature-of-patients-presenting-with-polycythemia-paraganglioma-syndromes
#3
Ingo Janssen, Clara Chi Chen, Zhenping Zhuang, Corina M Millo, Katherine Wolf, Alexander Ling, Frank I Lin, Karen T Adams, Peter Herscovitch, Richard A Feelders, Antonio T Fojo, David Taieb, Electron Kebebew, Karel Pacak
Rationale: Pheochromocytoma/paraganglioma (PPGL) syndromes associated with polycythemia have previously been described in association with mutations in the von Hippel-Lindau (VHL) gene. Recently, mutations in the prolyl hydroxylase gene (PHD) 1 and 2 and in the hypoxia-inducible factor 2 alpha (HIF2A) were also found to be associated with multiple and recurrent PPGL. Such patients also presented with PPGL and polycythemia, and later on, some presented with duodenal somatostatinoma. In additional patients presenting with PPGL and polycythemia, no further mutations have been discovered...
March 23, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28332883/precision-medicine-an-update-on-genotype-biochemical-phenotype-relationships-in-pheochromocytoma-paraganglioma-patients
#4
Garima Gupta, Karel Pacak
Pheochromocytomas and paragangliomas are rare neuroendocrine tumors known to produce and secrete high levels of circulating catecholamines and their metabolites. The biochemical characteristics of these tumors can be used to divide them into three major phenotypes. The adrenergic phenotype comprises of tumors with predominant elevations in epinephrine and metanephrine levels whereas the noradrenergic phenotype is composed of tumors with predominant elevations in norepinephrine and normetanephrine levels. The dopaminergic phenotype is a third rare group composed of tumors mainly secreting dopamine and 3-methoxytyramine...
March 23, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28332877/pentraxin-3-as-a-new-cardiovascular-marker-in-adrenal-adenomas
#5
Muhammed Kizilgul, Selvihan Beysel, Ozgur Ozcelik, Seyfullah Kan, Mahmut Apaydin, Mustafa Caliskan, Bekir Ucan, Erkam Sencar, Seyda Ozdemir, Erman Cakal
BACKGROUND: Pentraxin 3 (PTX3) is an acute-phase glycoprotein, which is increased in patients with cardiovascular disease (CVD) and considered as a predictor of CVD in the general population. Both functional and nonfunctional adrenal tumors are associated with a higher risk of cardiovascular events and mortality. We aimed to investigate plasma PTX3 levels in patients with functioning and nonfunctioning adrenal tumors and to determine its relationship with cardiovascular risk factors. MATERIAL AND METHODS: Twenty-one patients with functional adrenal tumors (11 pheochromocytomas, 9 Cushing's Syndrome and 1 primary hyperaldosteronism), 28 patients with non-functional adrenal incidentilomas, and 40 healthy controls were enrolled in the study...
March 23, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28331462/a-case-of-malignant-pheochromocytoma-presenting-7-years-after-the-initial-surgery
#6
Larsa Al-Omaishi, Jonathan Babin, Ralph L Corsetti
BACKGROUND: Pheochromocytoma (PHEO) is a rare tumor of the adrenal medulla and sympathetic ganglion that produces the catecholamines norepinephrine and epinephrine. Traditionally, approximately 10% of PHEOs were thought to be malignant, but recent developments in PHEO research have noted that specific genetic mutations are associated with higher risk of metastatic spread. CASE REPORT: We report the case of a 71-year-old female who presented with abdominal pain in September 2009 when she was 64 years old...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28327598/global-dna-methylation-analysis-identifies-two-discrete-clusters-of-pheochromocytoma-with-distinct-genomic-and-genetic-alterations
#7
Samuel Backman, Rajani Maharjan, Alberto Falk-Delgado, Joakim Crona, Kenko Cupisti, Peter Stålberg, Per Hellman, Peyman Björklund
Pheochromocytomas and paragangliomas (PPGLs) are rare and frequently heritable neural-crest derived tumours arising from the adrenal medulla or extra-adrenal chromaffin cells respectively. The majority of PPGL tumours are benign and do not recur with distant metastases. However, a sizeable fraction of these tumours secrete vasoactive catecholamines into the circulation causing a variety of symptoms including hypertension, palpitations and diaphoresis. The genetic landscape of PPGL has been well characterized and more than a dozen genes have been described as recurrently mutated...
March 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28324715/assessment-of-clinical-and-radiologic-differences-between-small-and-large-adrenal-pheochromocytomas
#8
Dong Won Kim, Seong Kuk Yoon, Sang Hyeon Kim, Eun Ju Kang, Hee Jin Kwon
OBJECTIVE: To evaluate differences in clinical and radiologic features of small and large pheochromocytomas. MATERIALS AND METHODS: This study included 39 patients with adrenal pheochromocytomas. Several clinical and radiologic features were statistically analyzed and compared between small and large pheochromocytomas. RESULTS: No significant differences were found in clinical features between them. Small pheochromocytomas had more relatively homogeneous attenuation although large pheochromocytomas had more cystic or necrotic changes...
March 10, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28324046/characteristics-of-pediatric-vs-adult-pheochromocytomas-and-paragangliomas
#9
Christina Pamporaki, Barbora Hamplova, Mirko Peitzsch, Aleksander Prejbisz, Felix Beuschlein, Henri J L M Timmers, Martin Fassnacht, Barbara Klink, Maya Lodish, Constantine A Stratakis, Angela Huebner, Stephanie Fliedner, Mercedes Robledo, Richard O Sinnott, Andrzej Januszewicz, Karel Pacak, Graeme Eisenhofer
Context: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared to adults. Hereditary PPGLs can be separated into two groups: cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways respectively. Objective: To identify differences in presentation of PPGLs between children and adults. Design: A retrospective cross-sectional clinical study...
January 31, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28323957/risk-profile-of-the-ret-a883f-germline-mutation-an-international-collaborative-study
#10
Jes Sloth Mathiesen, Mouhammed Amir Habra, John Howard Duncan Bassett, Sirazum Mubin Choudhury, Sabapathy Prakash Balasubramanian, Trevor A Howlett, Bruce G Robinson, Anne-Paule Gimenez-Roqueplo, Frederic Castinetti, Peter Vestergaard, Karin Frank-Raue
Context: The A883F germline mutation of the REarranged during Transfection proto-oncogene causes multiple endocrine neoplasia 2B. In the revised American Thyroid Association (ATA) guidelines for the management of medullary thyroid carcinoma (MTC) the A883F mutation has been reclassified from the highest to high risk level, although no well-defined risk profile for this mutation exists. Objective: To create a risk profile for the A883F mutation for appropriate classification in the ATA risk levels...
March 17, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28321067/the-histological-features-of-a-myocardial-biopsy-specimen-in-a-patient-in-the-acute-phase-of-reversible-catecholamine-induced-cardiomyopathy-due-to-pheochromocytoma
#11
Miyuki Miura, Hiroaki Kawano, Takeo Yoshida, Yuki Yamagata, Tomoo Nakata, Seiji Koga, Satoshi Ikeda, Kan Kageyama, Kuniko Abe, Koji Maemura
A 63-year-old Japanese woman with an adrenal tumor was transferred to our hospital due to cardiogenic shock. Right and left ventriculography showed severe hypokinesis of the middle segment and the apex in both ventricles, and an endomyocardial biopsy demonstrated a small number of necrotic myocytes and cellular infiltration. She was diagnosed with pheochromocytoma and quickly recovered after treatment with an α-blocker. The functional disability of both the right and left ventricles with less myocardial damage due to an excessive level of catecholamine seemed to be related to the early recovery the present patient with catecholamine-induced cardiomyopathy due to pheochromocytoma...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28319498/fdopa-patterns-in-adrenal-glands-a-pictorial-essay
#12
Aurélie Moreau, Anne Laure Giraudet, David Kryza, Françoise Borson-Chazot, Claire Bournaud-Salinas, Thomas Mognetti, Jean-Christophe Lifante, Patrick Combemale, Francesco Giammarile, Claire Houzard
F-FDOPA is a well-established tool to explore pheochromocytomas. It tends to replace I-MIBG scan in metastatic pheochromocytomas, multiple endocrine neoplasia type 2-related tumors, succinate dehydrogenase [ubiquinone] iron-sulfur subunit-negative tumors, and succinate dehydrogenase[ZERO WIDTH SPACE]-positive lesions. To our knowledge, no study has characterized physiological and pathological adrenal glands with F-FDOPA from a quantitative point of view. We report the features of different normal and pathological adrenal glands with F-FDOPA...
March 17, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28300276/reassessing-the-clinical-spectrum-associated-with-hereditary-leiomyomatosis-and-renal-cell-carcinoma-syndrome-in-french-fh-mutation-carriers
#13
Marie Muller, Sophie Ferlicot, Marine Guillaud-Bataille, Gwénaël Le Teuff, Catherine Genestie, Sophie Deveaux, Abdelhamid Slama, Nicolas Poulalhon, Bernard Escudier, Laurence Albiges, Nadem Soufir, Marie-Françoise Avril, Betty Gardie, Carolina Saldana, Yves Allory, Anne-Paule Gimenez-Roqueplo, Brigitte Bressac-de Paillerets, Stéphane Richard, Patrick R Benusiglio
We addressed uncertainties regarding Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) by exploring all French cases, representing the largest series to date. Fumarate Hydratase (FH) germline testing was performed with Sanger sequencing and qPCR/MLPA. Enzyme activity was measured when necessary. We carried out whenever possible a pathology review of RCC and S-(2-succino)-cysteine (2SC)/fumarate hydratase immunohistochemistry. We estimated survival using non-parametric Kaplan-Meier. There were 182 cases from 114 families...
March 16, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28291009/a-family-with-von-hippel-lindau-syndrome-the-findings-of-indium-111-somatostatin-receptor-scintigraphy-iodine-123-metaiodobenzylguanidine-scintigraphy-and-single-photon-emission-computerized-tomography
#14
Pelin Arıcan, Berna Okudan Tekin, Seniha Naldöken, Rıza Şefizade, Dilek Berker
Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant hereditary familial disorder characterized by development of malignant and benign neoplasms. Differential diagnosis of the adrenal and pancreatic masses are difficult in patients with VHLS. Iodine-123 metaiodobenzylguanidine (I-123 MIBG) and indium-111 somatostatin receptor scintigraphies (In-111 SRS) have important roles in the differential diagnosis of adrenal and pancreatic masses in those patients. In this case report, we present the findings of I-123 MIBG single-photon emission computerized tomography (SPECT/CT) and In-111 SRS SPECT/CT in three members of a family with VHLS...
February 5, 2017: Molecular Imaging and Radionuclide Therapy
https://www.readbyqxmd.com/read/28288049/catecholamine-induced-chest-pain-mimicking-infarction-due-to-an-mibg-negative-and-dopa-positive-succinate-dehydrogenase-syndrome-subunit-b-related-pheochromocytoma
#15
Alberto Mazza, Alice Ferretti, Antonella Paola Sacco, Domenico Rubello, Patrick M Colletti
This 16-year-old boy presented with acute retrosternal pain possibly representing acute myocardial infarction. Cardiac enzymes were within reference ranges. There were marked increases in metanephrine to 3299 μg/24 h (reference, <400 μg/24 h), normetanephrine to 1309 μg/24 h (reference, 0-390 μg/24 h), and chromogranin A to 1605 ng/mL (reference, 0-150 μg/24 h). An incidental left adrenal mass was found during CTPA performed to exclude pulmonary embolism. I-MIBG scintigraphy was negative, and genetic screening detected SDHB (succinate dehydrogenase syndrome subunit B) gene mutation...
March 10, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28285811/-composite-pheochromocytoma-a-rare-adrenal-tumor
#16
Gwladys Robinet, Nathalie Rioux-Leclercq, Andréa Manunta, Romain Mathieu, Frédérique Tissier, Benoit Peyronnet, Solène-Florence Kammerer-Jacquet
INTRODUCTION: Composite pheochromocytoma is a rare tumor of the adrenal medulla composed of pheochromocytoma and neuroblastic tumor. We report the case of a composite pheochromocytoma detected in a patient with neurofibromatosis type 1. CASE REPORT: A 61-year-old male patient presented occasional sweats with palpitation and moderate high blood pressure. Urinary catecholamine level was increased. CT scan showed a heterogeneous tumor limited to the adrenal gland. Histologically, the tumor showed two components: pheochromocytoma and ganglioneuroma and was diagnosed as a composite pheochromocytoma...
March 9, 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/28281874/protective-effect-of-medicinal-fungus-xylaria-nigripes-mycelia-extracts-against-hydrogen-peroxide-induced-apoptosis-in-pc12-cells
#17
Rupesh D Divate, Pei-Ming Wang, Chiun-Chuang Wang, Su-Tze Chou, Chen-Tien Chang, Yun-Chin Chung
Xylaria nigripes ( XN) is a medicinal fungus that was used traditionally as a diuretic, nerve tonic, and for treating insomnia and trauma. In this study, we elucidated possible mechanisms of neuroprotective effects of XN mycelia extracts. XN mycelia were produced by fermentation. Hot water extract and 70% ethanol extract of XN mycelia were evaluated on hydrogen peroxide (H2O2)-induced apoptosis in PC12, a rat pheochromocytoma cell line. Both XN extracts effectively protected PC12 cells against H2O2-induced cell damage by inhibiting release of lactate dehydrogenase, decreasing DNA damage, restoring mitochondrial membrane potential, and arresting abnormal apoptosis through upregulation of Bcl-2 and downregulation of Bax and caspase 3...
March 2017: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/28276947/assessment-of-depression-anxiety-quality-of-life-and-coping-in-long-standing-multiple-endocrine-neoplasia-type-2-patients
#18
Karine Candido Rodrigues, Rodrigo Almeida Toledo, Flavia Lima Coutinho, Adriana Bezerra Nunes, Rui M B Maciel, Ana Oliveira Hoff, Marcos C Tavares, Sergio P A Toledo, Delmar Muniz Lourenço
BACKGROUND: Scarce data on psychological harm in multiple endocrine neoplasia type 2 (MEN2) are available. OBJECTIVES: To assess anxiety, depression, quality of life and coping in long-standing MEN2 patients. PATIENTS AND METHODS: Forty-three adults (age ≥ 18 y) with clinical and genetic diagnosis of MEN2 and long-term follow-up (10.6±8.2 years; 1-33). A cross-sectional study with qualitative and quantitative psychological assessment using semi-directed interviews and HADS, EORTC QLQ C30 and MINI-MAC scales...
March 9, 2017: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/28274858/neurotoxicity-induced-by-methamphetamine-heroin-combination-in-pc12-cells
#19
Xiang Tian, Qin Ru, Qi Xiong, Kai Yue, Lin Chen, Baomiao Ma, Weimin Gan, Yuanren Si, Huqiao Xiao, Chaoying Li
Simultaneous administration of psychostimulants and opioids is a major drug abuse problem worldwide. The combination of psychostimulants and opioids produces more serious effects than either drug alone and is responsible for numerous deaths. In recent years, owing to its increased use, methamphetamine (METH), a psychostimulant, has become a popular choice for use in combination with opioids, especially heroin. However, little is known about the neurotoxicity of METH/heroin combination. The aims of this study were to evaluate whether METH/heroin combination was more neurotoxic than either drug alone and analyze the possible neurotoxic mechanisms using rat pheochromocytoma (PC12) cells...
March 6, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28273562/identification-of-dibenzyl-imidazolidine-and-triazole-acetamide-derivatives-through-virtual-screening-targeting-amyloid-beta-aggregation-and-neurotoxicity-in-pc12%C3%A2-cells
#20
Sukanya Das, Scott D Smid
Aggregation and neurotoxicity of amyloid β (Aβ) protein is a hallmark characteristic of Alzheimer's disease (AD). In this study we compared the anti-aggregatory and neuroprotective effects of five synthetic compounds against Aβ protein; four of which possessed a five membered heterocycle ring scaffold (two dibenzyl phenyl imidazolidines and two triazole sulfanyl acetamides) and one with a fused five membered heterocycle (benzoxazole) ring, selected thorough virtual screening from ZINC database. Molecular docking of their optimized structures was used to study Aβ binding characteristics...
April 21, 2017: European Journal of Medicinal Chemistry
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