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JOURNAL ARTICLE
Camilo Jimenez, Mouhammed Amir Habra, Matthew T Campbell, Gina Tamsen, Damaris Cruz-Goldberg, James Long, Roland Bassett, Robert Dantzer, Vania Balderrama-Brondani, Jeena Varghese, Yang Lu
BACKGROUND: Metastatic phaeochromocytomas and paragangliomas (MPPGs) are orphan diseases. Up to 50% of MPPGs are associated with germline pathogenic variants of the SDHB gene. These tumours and many non-familial MPPGs exhibit a phenotype that is characterised by abnormal angiogenesis. We aimed to assess the activity and safety of cabozantinib, an antiangiogenic multi-tyrosine kinase inhibitor, in patients with MPPGs. METHODS: The Natalie Trial is a single-arm, phase 2 clinical trial being conducted at The University of Texas MD Anderson Cancer Center (Houston, TX, USA)...
April 9, 2024: Lancet Oncology
#2
REVIEW
Alessandra Bracigliano, Antonella Lucia Marretta, Luigi Pio Guerrera, Roberto Simioli, Ottavia Clemente, Vincenza Granata, Anita Minopoli, Giuseppina Della Vittoria Scarpati, Fernanda Picozzi, Lucia Cannella, Antonio Pizzolorusso, Francesca Di Gennaro, Roberto Tafuto, Maria Rosaria Sarno, Ernesta Cavalcanti, Dario Ribera, Salvatore Tafuto
Pheochromocytomas (PCCs) and Paragangliomas (PGLs), commonly known as PPGLs to include both entities, are rare neuroendocrine tumors that may arise in the context of hereditary syndromes or be sporadic. However, even among sporadic PPGLs, identifiable somatic alterations in at least one of the known susceptibility genes can be detected. Therefore, about 3/4 of all PPGL patients can be assigned to one of the three molecular clusters that have been identified in the last years with difference in the underlying pathogenetic mechanisms, biochemical phenotype, metastatic potential, and prognosis...
March 8, 2024: Pharmaceuticals
#3
JOURNAL ARTICLE
David Killock
No abstract text is available yet for this article.
March 8, 2024: Nature Reviews. Clinical Oncology
#4
JOURNAL ARTICLE
Julie Ann Tarling, Rajeev Kumar, Louise J Ward, Christopher Boot, W S Wassif
Phaeochromocytomas and paragangliomas are rare catecholamine-producing neuroendocrine tumours which can potentially cause catastrophic crises with high morbidity and mortality. This best practice article considers the causes and presentation of such tumours, screening and diagnostic tests, management of these patients and consideration of family members at risk.
March 7, 2024: Journal of Clinical Pathology
#5
JOURNAL ARTICLE
Eric Baudin, Bernard Goichot, Alfredo Berruti, Julien Hadoux, Salma Moalla, Sandrine Laboureau, Svenja Nölting, Christelle de la Fouchardière, Tina Kienitz, Timo Deutschbein, Stefania Zovato, Laurence Amar, Magalie Haissaguerre, Henri Timmers, Patricia Niccoli, Antongiulio Faggiano, Moussa Angokai, Livia Lamartina, Florina Luca, Deborah Cosentini, Stefanie Hahner, Felix Beuschlein, Marie Attard, Matthieu Texier, Martin Fassnacht
BACKGROUND: No randomised controlled trial has ever been done in patients with metastatic phaeochromocytomas and paragangliomas. Preclinical and first clinical evidence suggested beneficial effects of sunitinib. We aimed to evaluate the safety and efficacy of sunitinib in patients with metastatic phaeochromocytomas and paragangliomas. METHODS: FIRSTMAPPP is a multicentre, international, randomised, placebo-controlled, double-blind, phase 2 trial done at 14 academic centres across four European countries...
February 22, 2024: Lancet
#6
JOURNAL ARTICLE
Mitsuhide Naruse, William F Young
No abstract text is available yet for this article.
March 16, 2024: Lancet
#7
JOURNAL ARTICLE
A Bojoga, S P Balasubramanian, R Mihai
INTRODUCTION: There is wide variability in the perioperative management of phaeochromocytoma and paraganglioma (PPGL) in different centres. This study aimed to summarise the management of PPGLs as reported in the United Kingdom Registry for Endocrine and Thyroid Surgery (UKRETS) database and to determine current perioperative management of PPGLs by surveying UK clinicians. METHODS: Data recorded on UKRETS from 2005 to 2021 were subjected to descriptive analyses...
February 16, 2024: Annals of the Royal College of Surgeons of England
#8
Charles John Nhungo, Jabu Abbysinia Mavundla, Geofrey Chiloleti, Gabriel Mtaturu, Charles Mkony, Ali Mwanga
INTRODUCTION AND IMPORTANCE: Phaeochromocytomas are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and are known as intra-adrenal paragangliomas. On the contrary, paragangliomas are non-epithelial neuroendocrine neoplasms that produce other peptide hormones such as norepinephrine, epinephrine, and dopamine. In a setting with limited resources, such a condition may result in inaccurate diagnosis and treatment, which may lose a patient life if left untreated...
January 31, 2024: International Journal of Surgery Case Reports
#9
JOURNAL ARTICLE
Qiao He, Zhengkun Zhang, Linqi Zhang, Bing Zhang, Yali Long, Yuying Zhang, Zhihong Liao, Zhihao Zha, Xiangsong Zhang
PURPOSE: To compare the detection ability of 68 Ga-labelled DOTA-l-Nal3-octreotide ([68 Ga]Ga-DOTA-NOC) and 6-[18 F]fluoro-L-3,4-dihydroxyphenylalanine ([18 F]DOPA) in patients with phaeochromocytomas and paragangliomas (PPGLs) of different origins and gene mutations, such as germline succinate dehydrogenase complex genes (SDHx). METHODS: Eighty-five patients with histopathologically confirmed PPGLs who underwent both [68 Ga]Ga-DOTA-NOC and [18 F]DOPA PET/CT from March 2017 to June 2023 were enrolled in this retrospective study...
February 1, 2024: European Journal of Nuclear Medicine and Molecular Imaging
#10
JOURNAL ARTICLE
John Staton Laws Iii, Scott D Smid
Phytochemicals are often promoted generally as antioxidants and demonstrate variable levels of reactive oxygen species (ROS) sequestration in vitro, which attributes to their neuroprotective bioactivity. Sesquiterpenes from cannabis and essential oils may demonstrate bifunctional properties towards cellular oxidative stress, possessing pro-oxidant activities by generating ROS or scavenging ROS directly. Sesquiterpenes can also oxidize forming sesquiterpene oxides, however the relative contribution they make to the bioactivity or cytotoxicity of complex botanical extracts more generally is unclear, while selected cannabis-prevalent terpenes such as β-caryophyllene may also activate cannabinoid receptors as part of their biological activity...
2024: Current research in toxicology
#11
J Kang, J Cheon, H Yoon, N Kim, S Heo
An 11-year-old spayed female, Persian cat was referred to the Jeonbuk Animal Medical Center for evaluation of a 2-month history of lethargy and anorexia. Physical examination revealed tachycardia and hypotension. Abdominal imaging via sonography and CT identified a right adrenal gland mass causing severe deviation and compression of the caudal vena cava. After stabilising the blood pressure and heart rate through positive inotropes and fluid therapy, right adrenalectomy was performed. Surgery confirmed the adrenal gland mass was severely compressing the caudal vena cava...
January 3, 2024: Journal of Small Animal Practice
#12
JOURNAL ARTICLE
Harald Groeben, Bente J Nottebaum, Aarne Feldheiser, Steffen Buch, Piero F Alesina, Martin K Walz
INTRODUCTION: Guidelines for the treatment of catecholamine-producing tumours strictly recommend starting ß-receptor blocking medication only after α-receptor blockade has been established. This recommendation is supported only by non-surgical case reports. However, in clinical practice ß-receptor blockade is often started before the diagnosis of a phaeochromocytoma is made. As we routinely treat patients with catecholamine-producing tumours without α-receptor blockade, our aim was to evaluate haemodynamic changes in such patients with and without ß-receptor blockade...
December 2023: BJA Open
#13
JOURNAL ARTICLE
Prodromos Chatzikyriakou, Dimitria Brempou, Mark Quinn, Lauren Fishbein, Roberta Noberini, Ioannis N Anastopoulos, Nicola Tufton, Eugenie S Lim, Rupert Obholzer, Johnathan G Hubbard, Mufaddal Moonim, Tiziana Bonaldi, Katherine L Nathanson, Louise Izatt, Rebecca J Oakey
BACKGROUND: Phaeochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours. Pathogenic variants have been identified in more than 15 susceptibility genes; associated tumours are grouped into three Clusters, reinforced by their transcriptional profiles. Cluster 1A PPGLs have pathogenic variants affecting enzymes of the tricarboxylic acid cycle, including succinate dehydrogenase. Within inherited PPGLs, these are the most common. PPGL tumours are known to undergo epigenetic reprograming, and here, we report on global histone post-translational modifications and DNA methylation levels, alongside clinical phenotypes...
December 20, 2023: Clinical Epigenetics
#14
REVIEW
David Taïeb, Svenja Nölting, Nancy D Perrier, Martin Fassnacht, Jorge A Carrasquillo, Ashley B Grossman, Roderick Clifton-Bligh, George B Wanna, Zachary G Schwam, Laurence Amar, Isabelle Bourdeau, Ruth T Casey, Joakim Crona, Cheri L Deal, Jaydira Del Rivero, Quan-Yang Duh, Graeme Eisenhofer, Tito Fojo, Hans K Ghayee, Anne-Paule Gimenez-Roqueplo, Antony J Gill, Rodney Hicks, Alessio Imperiale, Abhishek Jha, Michiel N Kerstens, Ronald R de Krijger, André Lacroix, Ivica Lazurova, Frank I Lin, Charlotte Lussey-Lepoutre, Eamonn R Maher, Ozgur Mete, Mitsuhide Naruse, Naris Nilubol, Mercedes Robledo, Frédéric Sebag, Nalini S Shah, Akiyo Tanabe, Geoffrey B Thompson, Henri J L M Timmers, Jiri Widimsky, William J Young, Leah Meuter, Jacques W M Lenders, Karel Pacak
Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time...
December 14, 2023: Nature Reviews. Endocrinology
#15
Ano Shalomi, Ramanathan Ramesh
Phaeochromocytoma is indeed a rare and frequently misunderstood neuroendocrine tumor originating from chromaffin cells in the adrenal medulla. Its clinical presentation often includes paroxysmal hypertension, palpitations, headache, and diaphoresis, which can easily be mistaken for common medical conditions. Timely diagnosis and precise localization are paramount for ensuring the best possible outcomes for patients. In this case report, we describe an unusual presentation of phaeochromocytoma in a 36-year-old man who presented with acute myocarditis...
November 2023: Curēus
#16
JOURNAL ARTICLE
Amanda Seabrook, Anand Vasudevan, Kristen Neville, Brigitte Gerstl, Diana Benn, Janine Smith, Judy Kirk, Anthony Gill, Roderick Clifton-Bligh, Katherine Tucker
UNLABELLED: Phaeochromocytoma (PC) and paraganglioma (PGL) syndromes associated with germline pathogenic variants are associated with high morbidity and mortality. Establishing genotype-phenotype correlations within a young population is challenging due to their rare occurrence. OBJECTIVE: To describe genotype-phenotype correlations in paediatric and adolescent patients diagnosed with PC/PGL. To establish the incidence of PC/PGL in a young population and prevalence of germline pathogenic variants within this group...
December 6, 2023: Archives of Disease in Childhood
#17
REVIEW
Matthew A Nazari, Rockyb Hasan, Mark Haigney, Alireza Maghsoudi, Jacques W M Lenders, Robert M Carey, Karel Pacak
Phaeochromocytomas and paragangliomas (PPGLs) release catecholamines leading to catecholamine-induced hypertensive (CIH) crises, with blood pressure greater than or equal to 180/120 mm Hg. CIH crises can be complicated by tachyarrhythmias, hypotension, or life-threatening target organ damage while treatment remains undefined, often requiring co-management between endocrinologists and cardiologists. Furthermore, biochemical diagnosis of a PPGL as a cause of a CIH crisis can be difficult to identify or confounded by comorbid conditions, potentially resulting in misdiagnosis...
December 2023: Lancet Diabetes & Endocrinology
#18
JOURNAL ARTICLE
Shobitha Vollmer, Henrik Borg
We report an unusual case of a patient presenting with Cushing's syndrome caused by a phaeochromocytoma secreting adrenocorticotropic hormone (ACTH). The patient had a history of treatment-resistant hypertension, secondary amenorrhoea and tendency towards hypokalaemia. She had multiple signs of Cushing's syndrome, such as swelling, bruising, abdominal striae and proximal myopathy. Hypokalaemia is more common in patients with ectopic ACTH-secretion than other causes of Cushing's syndrome. Computed tomography, adrenal vein sampling and biochemistry could confirm an ACTH-secreting phaeochromocytoma...
November 7, 2023: Journal of the Royal College of Physicians of Edinburgh
#19
Rafat Chowdhury, Myuri Moorthy, Lorna Smith, Christoph A Mueller, Fiona Gong, Harriet J Rogers, Marianthi-Vasiliki Papoutsaki, Tom Syer, Giorgio Brembilla, Saurabh Singh, Adam Retter, Thomas Parry, Joey Clemente, Lucy Caselton, Hassan Jeraj, Max Bullock, Manju Mathew, Teng Teng Chung, Scott Akker, Paul Chapple, Grace A Salsbury, Alan Bainbridge, David Atkinson, David G Gadian, Umasuthan Srirangalingam, Shonit Punwani
Phaeochromocytomas (PCC) and paragangliomas (PGL), cumulatively referred to as PPGLs, are neuroendocrine tumours arising from neural crest-derived cells in the sympathetic and parasympathetic nervous systems. Predicting future tumour behaviour and the likelihood of metastatic disease remains problematic as genotype-phenotype correlations are limited, the disease has variable penetrance and, to date, no reliable molecular, cellular or histological markers have emerged. Tumour metabolism quantification can be considered as a method to delineating tumour aggressiveness by utilising hyperpolarised 13 C-MR (HP-MR)...
November 2023: BJR Case Reports
#20
JOURNAL ARTICLE
Linping Wang, Jingqi Zhang, Yue Zhao, Juan Li, Xiaoting Lu, Jing Song, Ling Zhang, Qiao Niu
BACKGROUND: Studies have shown that aluminum (Al) is one of the environmental risk factors leading to Alzheimer's disease (AD), and Al exposure can cause elevated levels of BACE1mRNA, β-secretase (BACE1), and amyloid beta (Aβ) in vivo and in vitro. Previous studies by our research group have shown that this is partly caused by the negative regulation of BACE1 by miRNA29a/b1 (miR29a/b1). Despite the observed the role of nuclear factor kappa B (NF-κB) on many miRNAs, the upstream regulation of NF-κB protein on miR29 remains poorly understood...
September 21, 2023: Journal of Trace Elements in Medicine and Biology
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