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Phaeochromocytoma

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https://www.readbyqxmd.com/read/28808335/direct-electrochemical-measurement-of-metanephrines-in-spot-urine-samples-for-the-diagnosis-of-phaeochromocytomas
#1
Zheng-Hu Shi, Xiao-Qing Zhang, Qian-Na Zhen, Ming Zuo, Gang Tian, Yi-Fan He, Min Ding
Metanephrines (MNs) were suggested as a potential first-line biochemical index for the diagnosis of phaeochromocytomas (PHEO). In this study, we developed a simple electrochemical method for the quantitative measurement of MNs in spot urine samples. As MNs contain a hydroxyphenyl group, they could be oxidized at a certain potential to quinines, which could be further detected by the differential pulse voltammetry (DPV) method using unmodified screen-printed carbon electrode (SPCE). Meanwhile, the solid phase extraction (SPE) technique was used to eliminate the matrix effect in the samples...
August 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28746746/outcomes-of-patients-with-metastatic-phaeochromocytoma-and-paraganglioma-a-systematic-review-and-meta-analysis
#2
Oksana Hamidi, William F Young, Lucinda Gruber, John Smestad, Qi Yan, Oscar J Ponce, Larry Prokop, M Hassan Murad, Irina Bancos
OBJECTIVE: The outcomes of patients with metastatic phaeochromocytoma (PHEO) and paraganglioma (PGL) are unclear. We performed a systematic review and meta-analysis of baseline characteristics and mortality rates of patients with metastatic PHEO and PGL (PPGL). DESIGN: Ovid Medline In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, Scopus, Web of Science, and references of key articles were searched from inception to 2016...
July 26, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28693800/review-of-sequencing-platforms-and-their-applications-in-phaeochromocytoma-and-paragangliomas
#3
REVIEW
Suja Pillai, Vinod Gopalan, Alfred King-Yin Lam
Genetic testing is recommended for patients with phaeochromocytoma (PCC) and paraganglioma (PGL) because of their genetic heterogeneity and heritability. Due to the large number of susceptibility genes associated with PCC/PGL, next-generation sequencing (NGS) technology is ideally suited for carrying out genetic screening of these individuals. New generations of DNA sequencing technologies facilitate the development of comprehensive genetic testing in PCC/PGL at a lower cost. Whole-exome sequencing and targeted NGS are the preferred methods for screening of PCC/PGL, both having precise mutation detection methods and low costs...
August 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28680468/a-rare-cause-of-hypertension-in-pregnancy-phaeochromocytoma
#4
Sonali Shah, Lindsay Edwards, Andrew Robinson, Amy Crosthwaite, Christine Houlihan, Kathy Paizis
A 26-year-old primigravida at 35 weeks' gestation was transferred to our institution from a regional hospital for management of presumed preeclampsia. Due to the labile nature of her hypertension, further investigation was undertaken which revealed a right-sided phaeochromocytoma. Alpha blockade was commenced, and an uncomplicated elective caesarean delivery was performed at 38 weeks' gestation under spinal anaesthetic. The patient underwent an elective right laparoscopic adrenalectomy six weeks post-partum...
June 2017: Obstetric Medicine
https://www.readbyqxmd.com/read/28651811/bilateral-juxtapapillary-retinal-capillary-haemangioma-usefulness-of-aflibercept-in-the-management-of-its-complications
#5
R Campos Polo, C Rubio Sánchez, D M García Guisado, M J Díaz Luque
CASE REPORT: A 45 year-old man with a history of adrenal phaeochromocytoma presented with a subretinal juxtapapillary haemorrhage on his left eye and a small asymptomatic vascular tumour in the contralateral eye. With the mentioned findings, the patient was diagnosed with bilateral retinal capillary haemangioma in the context of a von Hippel Lindau disease. Intravitreal aflibercept was prescribed, with a good outcome of the disease. DISCUSSION: Many treatments have been proposed for the management of juxtapapillary retinal capillary haemangioma with variable results...
June 23, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28626127/a-case-of-retroperitoneal-paraganglioma
#6
Takao Amaike, Toshihisa Tamura, Atsuhiro Koga, Kazunori Shibao, Aiichiro Higure, Keiji Hirata
We report a surgical case of retroperitoneal paraganglioma. A paraganglioma is a catecholamine-producing tumor originating in the chromaffin cells of the sympathetic ganglion. It is a kind of pheochromocytoma which occurs on the outside of the adrenal gland. The patient was a 72 year old male with a history of hypertension and a pacemaker implantation. A mass in the ventral side of the right iliopsoas muscle was detected during a routine contrasting computed tomography (CT) examination for checking his pacemaker...
2017: Journal of UOEH
https://www.readbyqxmd.com/read/28605448/efficacy-of-peptide-receptor-radionuclide-therapy-prrt-for-functional-metastatic-paraganglioma-and-phaeochromocytoma
#7
Grace Kong, Simona Grozinsky-Glasberg, Michael S Hofman, Jason Callahan, Amichay Meirovitz, Ofra Maimon, David A Pattison, David J Gross, Rodney J Hicks
Purpose: Treatment options for unresectable Paraganglioma (PGL)/Phaeochromocytoma (PCC), especially with uncontrolled secondary hypertension (HTN) are limited. Preliminary studies with PRRT suggest efficacy but data on HTN control and survival are lacking. We assessed PRRT outcomes in such patients from 2 referral centres. Methods: 20 consecutive patients (M:F=13:7; 21-77y.o.) with high somatostatin receptor (SSTR) expression treated with 177Lu-DOTA-octreotate, 9 with radiosensitising chemotherapy, were retrospectively reviewed...
June 9, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28605116/phaeochromocytoma-in-multiple-endocrine-neoplasia-type-2-ret-codon-specific-penetrance-and-changes-in-management-during-the-last-four-decades
#8
L Mucha, G Leidig-Bruckner, K Frank-Raue, Th Bruckner, M Kroiss, F Raue
OBJECTIVES: We describe phaeochromocytoma (phaeo) penetrance in multiple endocrine neoplasia type 2 (MEN2) according to RET protooncogene-specific mutations and report changes in phaeo diagnosis and management from 1968 to 2015. DESIGN: This retrospective chart review included 309 MEN2 patients from one specialized ambulatory care centre. Phaeo patients were categorized by diagnosis date: early, 1968-1996, n=40, and recent, 1997-2015, n=45. RESULTS: Phaeochromocytoma was diagnosed in 85/309 patients with RET mutations in the following exons (phaeos/all carriers, %): exon 11 (56/120, 46...
June 12, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28548279/significant-decreases-in-blood-propofol-concentrations-during-adrenalectomy-for-phaeochromocytoma
#9
Tatsunori Watanabe, Haruhiko Hiraoka, Takuya Araki, Daisuke Nagano, Tohru Aomori, Tomonori Nakamura, Koujirou Yamamoto, Hiroshi Baba
AIM: The kinetics of propofol are influenced by cardiac output. The aim of this study was to examine changes in blood propofol concentrations during phaeochromocytoma surgery using target-controlled infusion (TCI) anaesthesia with propofol. METHODS: This is a prospective observational study. Ten patients with phaeochromocytoma who underwent unilateral adrenalectomy were included. Cardiac output was measured using an arterial pressure-based cardiac output analysis method...
May 26, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28462638/complete-laparoscopic-excision-of-a-giant-retroperitoneal-paraganglioma
#10
Zar Jawad, A B Fajardo-Puerta, D Lefroy, J Todd, P B Lim, L R Jiao
Paragangliomas (or 'extra-adrenal phaeochromocytomas') are rare tumours arising from neural crest cells. They occur most commonly in the abdomen along the paraspinal sympathetic ganglion. The malignancy rate can be up to 35% and surgical resection is the recommended treatment. While laparoscopic excision of phaeochromocytomas is now well established, the overall number of cases of paragangliomas reported is much smaller owing to their rarity (even more so for giant paragangliomas of ≥8cm) and controversy remains over the completeness of excision...
May 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28445897/adrenal-vein-catecholamine-levels-and-ratios-reference-intervals-derived-from-patients-with-primary-aldosteronism
#11
Candy W C Sze, Samuel Matthew O'Toole, Roger Kent Tirador, Scott A Akker, Matthew Matson, Leslie Perry, Maralyn Rose Druce, Tanja Dekkers, Jaap Deinum, Jacques W M Lenders, Graeme Eisenhofer, William Martyn Drake
Phaeochromocytoma localisation is generally reliably achieved with modern imaging techniques, particularly in sporadic cases. On occasion, however, there can be diagnostic doubt due to the presence of bilateral adrenal abnormalities, particularly in patients with mutations in genes predisposing them to the development of multiple phaeochromocytomas. In such cases, surgical intervention is ideally limited to large or functional lesions due to the long-term consequences associated with hypoadrenalism. Adrenal venous sampling (AVS) for catecholamines has been used in this situation to guide surgery, although there are few data available to support diagnostic thresholds...
April 26, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28429830/establishment-and-evaluation-of-a-novel-biomarker-based-nomogram-for-malignant-phaeochromocytomas-and-paragangliomas
#12
Xu Zhong, Lei Ye, TingWei Su, Jing Xie, Weiwei Zhou, Yiran Jiang, Lei Jiang, Guang Ning, Weiqing Wang
OBJECTIVE: No single histological or molecular marker is diagnostic for malignant phaeochromocytomas and paragangliomas (PPGLs). This study aimed to establish and evaluate a prognostic nomogram to improve the prediction of metastatic probability in individual PPGL patients. METHODS: Three hundred and 47 consecutive PPGL patients from January 2002 through December 2014 were randomly divided into a training set (n=208) and a validation set (n=139). A multivariate logistic regression analysis of selected prognostic features was performed, and a nomogram to predict metastasis was constructed...
August 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28385489/developmental-neurotoxicity-of-different-pesticides-in-pc-12-cells-in-vitro
#13
COMPARATIVE STUDY
Verena Christen, Manuel Rusconi, Pierre Crettaz, Karl Fent
The detection of developmental neurotoxicity (DNT) of chemicals has high relevance for protection of human health. However, DNT of many pesticides is only little known. Furthermore, validated in vitro systems for assessment of DNT are not well established. Here we employed the rat phaeochromocytoma cell line PC-12 to evaluate DNT of 18 frequently used pesticides of different classes, including neonicotinoids, pyrethroids, organophosphates, organochlorines, as well as quaternary ammonium compounds, the organic compound used in pesticides, piperonyl butoxide, as well as the insect repellent diethyltoluamide (DEET)...
June 15, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28369925/cousins-not-twins-intratumoural-and-intertumoural-heterogeneity-in-syndromic-neuroendocrine-tumours
#14
Aidan Flynn, Trisha Dwight, Diana Benn, Siddhartha Deb, Andrew J Colebatch, Stephen Fox, Jessica Harris, Emma L Duncan, Bruce Robinson, Annette Hogg, Jason Ellul, Henry To, Cuong Duong, Julie A Miller, Christopher Yates, Paul James, Alison Trainer, Anthony J Gill, Roderick Clifton-Bligh, Rodney J Hicks, Richard W Tothill
Hereditary endocrine neoplasias, including phaeochromocytoma/paraganglioma and medullary thyroid cancer, are caused by autosomal dominant mutations in several familial cancer genes. A common feature of these diseases is the presentation of multiple primary tumours, or multifocal disease representing independent tumour clones that have arisen from the same initiating genetic lesion, but have undergone independent clonal evolution. Such tumours provide an opportunity to discover common cooperative changes required for tumourigenesis, while controlling for the genetic background of the individual...
March 31, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28331569/an-unusual-diagnosis-for-persistent%C3%A2-diarrhoea-and-vomiting
#15
James Nott, Asad Khan, Ravi Madhotra, George MacFaul, Kamran Rostami
Identifying the etiology of chronic diarrhoea might be challenging in some patients, and before a diagnosis is made these patients may spend a substantial length of time with unresolved symptoms leading to uncertainty and anxiety that is severely impairing their life quality. A 45-year-old female was referred by her general practitioner with a 5-year history of increasingly frequent episodes of cyclical diarrhoea, vomiting, abdominal pain and intermittent palpitations. Contrast CT Abdomen/Pelvis revealed a 36x33x46 mm mass in the aorto caval region of her retro-peritoneum, just above the bifurcation...
2017: Gastroenterology and Hepatology From Bed to Bench
https://www.readbyqxmd.com/read/28261513/primary-clear-cell-microcystic-adenoma-of-the-sinonasal-cavity-pathological-or-fortuitous-association
#16
Rosalin Cooper, Hannah Markham, Jeffery Theaker, Adrian Bateman, David Bunyan, Matthew Sommerlad, Gillian Crawford, Diana Eccles
Primary clear cell microcystic adenoma of the sinonasal cavity is rare. It has previously been described only as a VHL-associated tumour. Von Hippel-Lindau (VHL) syndrome is an inherited cancer syndrome characterised by an elevated risk of neoplasia including clear cell renal cell carcinoma (ccRCC), haemangioblastoma, and phaeochromocytoma. We describe the second reported case of a primary clear cell microcystic adenoma of the sinonasal cavity. The 39-year-old patient with VHL syndrome had previously undergone resection and ablation of ccRCC...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28248910/high-prevalence-of-cardiac-electric-abnormalities-in-patients-with-phaeochromocytomas
#17
Elena Berra, Maria C Gonzalez, Christophe Scavée, Laurent Vroonen, Akila Bersali, Anne-Catherine Pouleur, Alexandre Persu
No abstract text is available yet for this article.
April 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28179334/potential-pitfalls-of-sdh-immunohistochemical-detection-in-paragangliomas-and-phaeochromocytomas-harbouring-germline-sdhx-gene-mutation
#18
Raffaella Santi, Elena Rapizzi, Letizia Canu, Tonino Ercolino, Gianna Baroni, Rossella Fucci, Giuseppe Costa, Massimo Mannelli, Gabriella Nesi
BACKGROUND/AIM: Germline mutations in any of the succinate dehydrogenase (SDH) genes result in destabilization of the SDH protein complex and loss of SDHB expression at immunohistochemistry. SDHA is lost together with SDHB in SDHA-mutated tumours, but its expression is retained in tumours with other SDH mutations. We investigated whether SDHA/SDHB immunohistochemistry is able to identify SDH-related tumours in a retrospective case series of phaeochromocytomas (PCCs) and paragangliomas (PGLs)...
2017: Anticancer Research
https://www.readbyqxmd.com/read/28166370/peptide-receptor-radionuclide-treatment-and-131-i-mibg-in-the-management-of-patients-with-metastatic-progressive-phaeochromocytomas-and-paragangliomas
#19
COMPARATIVE STUDY
Konstantinos Nastos, Vincent T F Cheung, Christos Toumpanakis, Shaunak Navalkissoor, Anne-Marie Quigley, Martyn Caplin, Bernard Khoo
BACKGROUND AND OBJECTIVES: Radionuclide therapy has been used to treat patients with progressive/metastatic paragangliomas (PGLs) and phaeochromocytomas (PCCs). The aim of the present study is to retrospectively compare the therapeutic outcomes of these modalities in patients with progressive/metastatic PCCs and PGLs. METHODS: Patients with progressive/metastatic PGLs and PCCs that were subjected to radionuclide treatment in our department were retrieved from our department's database for the period 1998-2013...
March 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28150464/adrenomedullin-new-perspectives-of-a-potent-peptide-hormone
#20
Ria Schönauer, Sylvia Els-Heindl, Annette G Beck-Sickinger
Adrenomedullin (ADM) is a 52-amino acid multifunctional peptide, which belongs to the calcitonin gene-related peptide (CGRP) superfamily of vasoactive peptide hormones. ADM exhibits a significant vasodilatory potential and plays a key role in various regulatory mechanisms, predominantly in the cardiovascular and lymphatic system. It exerts its effects by activation of the calcitonin receptor-like receptor associated with one of the receptor activity-modifying proteins 2 or 3. ADM was first isolated from human phaeochromocytoma in 1993...
February 2, 2017: Journal of Peptide Science: An Official Publication of the European Peptide Society
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