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Richter's Syndrome

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https://www.readbyqxmd.com/read/29922281/alpha-1-antitrypsin-inhibits-atp-mediated-release-of-interleukin-1%C3%AE-via-cd36-and-nicotinic-acetylcholine-receptors
#1
Kathrin Siebers, Bijan Fink, Anna Zakrzewicz, Alisa Agné, Katrin Richter, Sebastian Konzok, Andreas Hecker, Sven Zukunft, Mira Küllmar, Jochen Klein, J Michael McIntosh, Thomas Timm, Katherina Sewald, Winfried Padberg, Nupur Aggarwal, Walee Chamulitrat, Sentot Santoso, Wendy Xia, Sabina Janciauskiene, Veronika Grau
While interleukin (IL)-1β is a potent pro-inflammatory cytokine involved in host defense, high levels can cause life-threatening sterile inflammation including systemic inflammatory response syndrome. Hence, the control of IL-1β secretion is of outstanding biomedical importance. In response to a first inflammatory stimulus such as lipopolysaccharide, pro-IL-1β is synthesized as a cytoplasmic inactive pro-form. Extracellular ATP originating from injured cells is a prototypical second signal for inflammasome-dependent maturation and release of IL-1β...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29915970/comparison-of-surgical-outcomes-of-trabeculectomy-ahmed-shunt-and-baerveldt-shunt-in-uveitic-glaucoma
#2
Audrey Chow, Bruce Burkemper, Rohit Varma, Damien C Rodger, Narsing Rao, Grace M Richter
BACKGROUND: Uveitis is defined as a collection of syndromes involving intraocular inflammation which can lead to pain, tissue damage, and vision loss. Ophthalmic surgery in uveitis patients can be challenging due to inflammation-induced fibrosis and scarring. Trabeculectomy and implantation of glaucoma drainage devices (aqueous shunts) have been used in surgical management of uveitic glaucoma, however there is a paucity of literature examining the comparative results of these entities in this unique setting...
June 18, 2018: Journal of Ophthalmic Inflammation and Infection
https://www.readbyqxmd.com/read/29891884/expanding-the-clinical-and-molecular-findings-in-rasa1-capillary-malformation-arteriovenous-malformation
#3
Whitney L Wooderchak-Donahue, Peter Johnson, Jamie McDonald, Francine Blei, Alejandro Berenstein, Michelle Sorscher, Jennifer Mayer, Angela E Scheuerle, Tracey Lewis, J Fredrik Grimmer, Gresham T Richter, Marcie A Steeves, Angela E Lin, David A Stevenson, Pinar Bayrak-Toydemir
RASA1-related disorders are vascular malformation syndromes characterized by hereditary capillary malformations (CM) with or without arteriovenous malformations (AVM), arteriovenous fistulas (AVF), or Parkes Weber syndrome. The number of cases reported is relatively small; and while the main clinical features are CMs and AVMs/AVFs, the broader phenotypic spectrum caused by variants in the RASA1 gene is still being defined. Here, we report the clinical and molecular findings in 69 unrelated cases with a RASA1 variant identified at ARUP Laboratories...
June 11, 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29876601/-prevalence-of-sleep-related-breathing-disorders-of-inpatients-with-psychiatric-disorders
#4
M Behr, J Acker, S Cohrs, M Deuschle, H Danker-Hopfe, R Göder, C Norra, K Richter, D Riemann, C Schilling, H-G Weeß, T C Wetter, L M Wollenburg, T Pollmächer
BACKGROUND: Sleep-related breathing disorders seriously impair well-being and increase the risk for relevant somatic and psychiatric disorders. Moreover, risk factors for sleep-related breathing disorders are highly prevalent in psychiatric patients. The aim of this study was for the first time in Germany to study the prevalence of obstructive sleep apnea syndrome (OSAS) as the most common form of sleep-related breathing disorder in patients with psychiatric disorders. METHODS: In 10 psychiatric hospitals in Germany and 1 hospital in Switzerland, a total of 249 inpatients underwent an 8‑channel sleep polygraphy to investigate the prevalence of sleep apnea in this group of patients...
June 6, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29805042/mutations-in-the-epithelial-cadherin-p120-catenin-complex-cause-mendelian-non-syndromic-cleft-lip-with-or-without-cleft-palate
#5
Liza L Cox, Timothy C Cox, Lina M Moreno Uribe, Ying Zhu, Chika T Richter, Nichole Nidey, Jennifer M Standley, Mei Deng, Elizabeth Blue, Jessica X Chong, Yueqin Yang, Russ P Carstens, Deepti Anand, Salil A Lachke, Joshua D Smith, Michael O Dorschner, Bruce Bedell, Edwin Kirk, Anne V Hing, Hanka Venselaar, Luz C Valencia-Ramirez, Michael J Bamshad, Ian A Glass, Jonathan A Cooper, Eric Haan, Deborah A Nickerson, Hans van Bokhoven, Huiqing Zhou, Katy N Krahn, Michael F Buckley, Jeffrey C Murray, Andrew C Lidral, Tony Roscioli
Non-syndromic cleft lip with or without cleft palate (NS-CL/P) is one of the most common human birth defects and is generally considered a complex trait. Despite numerous loci identified by genome-wide association studies, the effect sizes of common variants are relatively small, with much of the presumed genetic contribution remaining elusive. We report exome-sequencing results in 209 people from 72 multi-affected families with pedigree structures consistent with autosomal-dominant inheritance and variable penetrance...
May 16, 2018: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29799609/validation-of-a-novel-irritant-gas-syndrome-triage-algorithm
#6
Joan M Culley, Sara Donevant, Jean Craig, Jane Richter, Abbas S Tavakoli, Erik Svendsen, Salvatore DiNardi
OBJECTIVE: Our objective was to validate a novel irritant gas syndrome agent (IGSA) triage algorithm for use in an emergency department (ED). We assessed efficiency, accuracy, and precision of our IGSA triage algorithm based on signs/symptoms of actual patients. DESIGN: After characterizing the signs/symptoms of an actual IGSA exposure event, we developed and validated the IGSA triage algorithm using a simulated computer exercise to compare the IGSA triage algorithm to the preferred hospital triage algorithm, the Emergency Severity Index (ESI)...
January 2018: American Journal of Disaster Medicine
https://www.readbyqxmd.com/read/29796007/b-chronic-lymphocytic-leukemia-showed-triple-transformation-to-diffuse-large-b-cell-cd20-negative-and-t-cell-neoplasm-during-ofatumumab-treatment-a-case-report
#7
Osamu Imataki, Makiko Uemura
Background: Chronic lymphocytic leukemia (CLL) is a mature lymphoid neoplasm currently categorized as an indolent type of malignant lymphoma. CLL progresses slowly over years, but it eventually transforms to a more aggressive lymphoma such as the diffuse large B-cell (DLBCL) type, also known as Richter's syndrome. Case presentation: We treated a 69-year-old Japanese male who was histologically diagnosed with Richter's syndrome after 6 years of CLL. His lymphadenopathy had systemically progressed for years, with lymphocyte counts of less than 10,000 cells/μL and a disease status of Rai classification stage I and Binet classification B...
2018: BMC Clinical Pathology
https://www.readbyqxmd.com/read/29745744/rapid-complete-response-to-blinatumomab-as-a-successful-bridge-to-allogeneic-stem-cell-transplantation-in-a-case-of-refractory-richter-syndrome
#8
Juan Pablo Alderuccio, Nicholas Mackrides, Jennifer R Chapman, Francisco Vega, Izidore S Lossos
No abstract text is available yet for this article.
May 10, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29735551/novel-richter-s-syndrome-xenograft-models-to-study-genetic-architecture-biology-and-therapy-responses
#9
Tiziana Vaisitti, Esteban Braggio, John N Allan, Francesca Arruga, Sara Serra, Alberto Zamò, Wayne Tam, Amy Chadburn, Richard R Furman, Silvia Deaglio
Richter's syndrome (RS) represents the evolution of chronic lymphocytic leukemia into an aggressive tumor, most commonly diffuse large B cell lymphoma. The lack of in vitro and in vivo models has severely hampered drug testing in a disease that is poorly responsive to common chemo-immunotherapeutic combinations as well as to novel kinase inhibitors. Here we report for the first time the establishment and genomic characterization of two patient-derived tumor xenograft models of RS, RS9737 and RS1316. RS xenografts were genetically, morphologically and phenotypically stable and similar to the corresponding primary tumor...
May 7, 2018: Cancer Research
https://www.readbyqxmd.com/read/29692342/biology-and-treatment-of-richter-syndrome
#10
Davide Rossi, Valeria Spina, Gianluca Gaidano
Richter syndrome (RS) is the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Two pathologic variants of RS are recognized, namely the diffuse large B-cell lymphoma (DLBCL) variant, and the rare Hodgkin lymphoma (HL) variant. Histologic documentation is mandatory to diagnose RS. The clinical suspicion of RS should be based on clinical signs and symptoms. Differential diagnosis between CLL progression and RS and choice of the biopsy site may take advantage of PET/CT...
April 24, 2018: Blood
https://www.readbyqxmd.com/read/29682366/whole-exome-sequencing-and-molecular-modeling-of-a-missense-variant-in-tnfaip3-that-segregates-with-disease-in-a-family-with-chronic-urticaria-and-angioedema
#11
Antoneicka L Harris, Patrick R Blackburn, John E Richter, Jennifer M Gass, Thomas R Caulfield, Ahmed N Mohammad, Paldeep S Atwal
Chronic urticaria is a common condition characterized by recurrent hives lasting several weeks or months and is usually idiopathic. Approximately half of the individuals with chronic urticaria will present with episodes of angioedema that can be severe and debilitating. In this report, we describe a 47-year-old Hispanic male who presented initially for an evaluation of chronic hives following hospitalization due to hive-induced anaphylaxis. The individual had a history significant for urticaria and angioedema beginning in his early 30s...
2018: Case Reports in Genetics
https://www.readbyqxmd.com/read/29679775/influence-of-side-effects-on-early-therapy-persistence-with-letrozole-in-post-menopausal-patients-with-early-breast-cancer-results-of-the-prospective-evaluate-tm-study
#12
N Nabieva, T Fehm, L Häberle, J de Waal, M Rezai, B Baier, G Baake, H-C Kolberg, M Guggenberger, M Warm, N Harbeck, R Wuerstlein, J-U Deuker, P Dall, B Richter, G Wachsmann, C Brucker, J W Siebers, M Popovic, T Kuhn, C Wolf, H-W Vollert, G-P Breitbach, W Janni, R Landthaler, A Kohls, D Rezek, T Noesselt, G Fischer, S Henschen, T Praetz, V Heyl, T Kühn, T Krauss, C Thomssen, A Hohn, H Tesch, C Mundhenke, A Hein, C C Hack, K Schmidt, E Belleville, S Y Brucker, S Kümmel, M W Beckmann, D Wallwiener, P Hadji, P A Fasching
BACKGROUND: Endocrine treatment (ET) with an aromatase inhibitor (AI) is the treatment of choice in post-menopausal patients with hormone receptor-positive early breast cancer (EBC). However, adverse events (AEs) often lead to treatment discontinuation. This analysis aimed to identify side-effects that lead to patients failing to persist with letrozole treatment. PATIENTS AND METHODS: Post-menopausal hormone receptor-positive EBC patients starting ET with letrozole were enroled in EvAluate-TM, a non-interventional study...
June 2018: European Journal of Cancer
https://www.readbyqxmd.com/read/29642561/%C3%AE-nicotinamide-adenine-dinucleotide-%C3%AE-nad-inhibits-atp-dependent-il-1%C3%AE-release-from-human-monocytic-cells
#13
Sebastian Daniel Hiller, Sarah Heldmann, Katrin Richter, Innokentij Jurastow, Mira Küllmar, Andreas Hecker, Sigrid Wilker, Gabriele Fuchs-Moll, Ivan Manzini, Günther Schmalzing, Wolfgang Kummer, Winfried Padberg, J Michael McIntosh, Jelena Damm, Anna Zakrzewicz, Veronika Grau
While interleukin-1β (IL-1β) is a potent pro-inflammatory cytokine essential for host defense, high systemic levels cause life-threatening inflammatory syndromes. ATP, a stimulus of IL-1β maturation, is released from damaged cells along with β-nicotinamide adenine dinucleotide (β-NAD). Here, we tested the hypothesis that β-NAD controls ATP-signaling and, hence, IL-1β release. Lipopolysaccharide-primed monocytic U937 cells and primary human mononuclear leukocytes were stimulated with 2'(3')- O -(4-benzoyl-benzoyl)ATP trieethylammonium salt (BzATP), a P2X7 receptor agonist, in the presence or absence of β-NAD...
April 10, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29625968/2018-update-of-the-eular-recommendations-for-the-management-of-beh%C3%A3-et-s-syndrome
#14
Gulen Hatemi, Robin Christensen, Dongsik Bang, Bahram Bodaghi, Aykut Ferhat Celik, Farida Fortune, Julien Gaudric, Ahmet Gul, Ina Kötter, Pietro Leccese, Alfred Mahr, Robert Moots, Yesim Ozguler, Jutta Richter, David Saadoun, Carlo Salvarani, Francesco Scuderi, Petros P Sfikakis, Aksel Siva, Miles Stanford, Ilknur Tugal-Tutkun, Richard West, Sebahattin Yurdakul, Ignazio Olivieri, Hasan Yazici
Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in the light of these new data under the auspices of the European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs. A task force was formed that included BS experts from different specialties including internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, along with a methodologist, a health professional, two patients and two fellows in charge of the systematic literature search...
June 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29568782/proteinuria-hypoalbuminemia-and-chronic-lymphocytic-leukemia-an-unusual-trio
#15
William Wung, Shubha Ananthakrishnan, Brian A Jonas
Chronic lymphocytic leukemia (CLL) is a chronic, progressive lymphoproliferative disorder characterized by a monoclonal population of functionally incompetent lymphocytes. Renal involvement is rare and poorly described. A 57-year-old male with no prior medical history was diagnosed with CLL and followed with a watch and wait approach. He was referred to our institution several months later due to concern for Richter's transformation to diffuse large B-cell lymphoma. A positron emission tomography/computed tomography scan showed no evidence of diffuse large B-cell lymphoma; however, the patient was noted to have hypoalbuminemia, nephrotic range proteinuria, an acute left renal vein thrombus, and a right pulmonary embolus...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29484684/clinical-pathological-and-biological-characterization-of-richter-syndrome-developing-after-ibrutinib-treatment-for-relapsed-chronic-lymphocytic-leukemia
#16
Idanna Innocenti, Davide Rossi, Giulio Trapè, Francesco Autore, Luigi Maria Larocca, Vincenzo Gomes, Michaela Cerri, Paolo Falcucci, Simona Sica, Gianluca Gaidano, Luca Laurenti
Richter syndrome, a transformation of chronic lymphocytic leukemia (CLL) into a diffuse large B-cell lymphoma, is a rare complication of patients treated with chemo-immunotherapy. Richter syndrome might be both clonally related or unrelated to the underlying CLL and often showed mutations of the TP53 and NOTCH1 genes. Recently, ibrutinib was approved for patients with relapsed/refractory CLL or for untreated CLL patients with del 17p or TP53 mutation. The clinical picture, pathology, and genetics of Richter transformation after IBR treatment are largely unknown...
February 27, 2018: Hematological Oncology
https://www.readbyqxmd.com/read/29417219/robust-identification-of-mosaic-variants-in-congenital-heart-disease
#17
Kathryn B Manheimer, Felix Richter, Lisa J Edelmann, Sunita L D'Souza, Lisong Shi, Yufeng Shen, Jason Homsy, Marko T Boskovski, Angela C Tai, Joshua Gorham, Christopher Yasso, Elizabeth Goldmuntz, Martina Brueckner, Richard P Lifton, Wendy K Chung, Christine E Seidman, J G Seidman, Bruce D Gelb
Mosaicism due to somatic mutations can cause multiple diseases including cancer, developmental and overgrowth syndromes, neurodevelopmental disorders, autoinflammatory diseases, and atrial fibrillation. With the increased use of next generation sequencing technology, multiple tools have been developed to identify low-frequency variants, specifically from matched tumor-normal tissues in cancer studies. To investigate whether mosaic variants are implicated in congenital heart disease (CHD), we developed a pipeline using the cancer somatic variant caller MuTect to identify mosaic variants in whole-exome sequencing (WES) data from a cohort of parent/affected child trios (n = 715) and a cohort of healthy individuals (n = 416)...
February 2018: Human Genetics
https://www.readbyqxmd.com/read/29378952/olt1177-a-%C3%AE-sulfonyl-nitrile-compound-safe-in-humans-inhibits-the-nlrp3-inflammasome-and-reverses-the-metabolic-cost-of-inflammation
#18
Carlo Marchetti, Benjamin Swartzwelter, Fabia Gamboni, Charles P Neff, Katrin Richter, Tania Azam, Sonia Carta, Isak Tengesdal, Travis Nemkov, Angelo D'Alessandro, Curtis Henry, Gerald S Jones, Scott A Goodrich, Joseph P St Laurent, Terry M Jones, Curtis L Scribner, Robert B Barrow, Roy D Altman, Damaris B Skouras, Marco Gattorno, Veronika Grau, Sabina Janciauskiene, Anna Rubartelli, Leo A B Joosten, Charles A Dinarello
Activation of the NLRP3 inflammasome induces maturation of IL-1β and IL-18, both validated targets for treating acute and chronic inflammatory diseases. Here, we demonstrate that OLT1177, an orally active β-sulfonyl nitrile molecule, inhibits activation of the NLRP3 inflammasome. In vitro, nanomolar concentrations of OLT1177 reduced IL-1β and IL-18 release following canonical and noncanonical NLRP3 inflammasome activation. The molecule showed no effect on the NLRC4 and AIM2 inflammasomes, suggesting specificity for NLRP3...
February 13, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29375868/vascular-surgery-of-aortic-thrombosis-in-a-dog-using-fogarty-maneuver-technical-feasibility
#19
Maartje Schwede, Olaf Richter, Michaele Alef, Tobias Theuß, Shenja Loderstedt
Aortic thromboembolism is a rare and life-threatening disease in dogs. This report aims to describe the successful surgical treatment by use of a Fogarty Thrombectomy Catheter in an 8-year-old patient. The postsurgical intensive care therapy to prevent ischemia-reperfusion syndrome is specified, despite poor outcome in our case (owner elected euthanasia).
January 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29363290/brief-report-cancer-immunotherapy-in-patients-with-preexisting-rheumatic-disease-the-mayo-clinic-experience
#20
Michael D Richter, Olga Pinkston, Lisa A Kottschade, Heidi D Finnes, Svetomir N Markovic, Uma Thanarajasingam
OBJECTIVE: To determine the risk of rheumatic disease flare and adverse effects in patients with preexisting rheumatic disease who were receiving immune checkpoint inhibitor (ICI) therapy. METHODS: A retrospective medical record review was performed to identify all patients who received ICI therapy at Mayo Clinic in Rochester, Minnesota between 2011 and 2016 (~700 patients). Those with a preexisting rheumatic disease were identified using specific diagnostic codes...
March 2018: Arthritis & Rheumatology
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