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Richter's Syndrome

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https://www.readbyqxmd.com/read/28433046/-the-risks-of-retroperitoneoscopic-adrenalectomy
#1
P Zonča, M Peteja, P Vávra, V Richter, P Ostruszka
INTRODUCTION: Minimally invasive adrenalectomy has become the gold standard for surgery of the suprarenal gland. Retroperitoneoscopic adrenalectomy with dorsal approach is preferred. The aim of our case report is to discuss potential complications that may arise from retroperitoneoscopic adrenalectomy, specifically an intra-operative injury of the inferior vena cava. CASE REPORT: A 47-year-old male patient was admitted to undergo elective adrenalectomy on the right side...
2017: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/28404637/surfactant-inhibits-atp-induced-release-of-interleukin-1%C3%AE-via-nicotinic-acetylcholine-receptors
#2
Soeren Backhaus, Anna Zakrzewicz, Katrin Richter, Jelena Damm, Sigrid Wilker, Gabriele Fuchs-Moll, Mira Kuellmar, Andreas Hecker, Ivan Manzini, Clemens Ruppert, J Michael McIntosh, Winfried Padberg, Veronika Grau
Interleukin-1β (IL-1β) is a potent pro-inflammatory cytokine of innate immunity involved in host defense. High systemic IL-1β; levels, however, cause life-threatening inflammatory diseases including systemic inflammatory response syndrome. In response to various danger signals, pro-IL-1β; is synthesized and stays in the cytoplasm unless a second signal such as extracellular ATP activates the inflammasome, which enables processing and release of mature IL-1β. As pulmonary surfactant is known for its anti-inflammatory properties, we hypothesize that surfactant inhibits ATP-induced release of IL-1β...
April 12, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28363626/validating-signs-and-symptoms-from-an-actual-mass-casualty-incident-to-characterize-an-irritant-gas-syndrome-agent-igsa-exposure-a-first-step-in-the-development-of-a-novel-igsa-triage-algorithm
#3
Joan M Culley, Jane Richter, Sara Donevant, Abbas Tavakoli, Jean Craig, Salvatore DiNardi
INTRODUCTION: Chemical exposures can pose a significant threat to life. Rapid assessment by first responders/emergency nurses is required to reduce death and disability. Currently, no informatics tools for irritant gas syndrome agents (IGSA) exposures exist to process victims efficiently, continuously monitor for latent signs/symptoms, or make triage recommendations. This study describes the first step in developing ED informatics tools for chemical incidents: validation of signs/symptoms that characterize an IGSA syndrome...
March 28, 2017: Journal of Emergency Nursing: JEN: Official Publication of the Emergency Department Nurses Association
https://www.readbyqxmd.com/read/28342715/prediction-of-neonatal-respiratory-morbidity-by-quantitative-ultrasound-lung-texture-analysis-a-multicenter-study
#4
Montse Palacio, Elisenda Bonet-Carne, Teresa Cobo, Alvaro Perez-Moreno, Joan Sabrià, Jute Richter, Marian Kacerovsky, Bo Jacobsson, Raúl A García-Posada, Fernando Bugatto, Ramon Santisteve, Àngels Vives, Mauro Parra-Cordero, Edgar Hernandez-Andrade, José Luis Bartha, Pilar Carretero-Lucena, Kai Lit Tan, Rogelio Cruz-Martínez, Minke Burke, Suseela Vavilala, Igor Iruretagoyena, Juan Luis Delgado, Mauro Schenone, Josep Vilanova, Francesc Botet, George S H Yeo, Jon Hyett, Jan Deprest, Roberto Romero, Eduard Gratacos
BACKGROUND: Prediction of neonatal respiratory morbidity may be useful to plan delivery in complicated pregnancies. The limited predictive performance of the current diagnostic tests together with the risks of an invasive procedure limits the use of fetal lung maturity assessment. OBJECTIVE: The objective of the study was to evaluate the performance of quantitative ultrasound texture analysis (quantusFLM) to predict neonatal respiratory morbidity in preterm and early-term (<39...
March 23, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28303898/circulating-tumour-dna-reflects-treatment-response-and-clonal-evolution-in-chronic-lymphocytic-leukaemia
#5
Paul Yeh, Tane Hunter, Devbarna Sinha, Sarah Ftouni, Elise Wallach, Damian Jiang, Yih-Chih Chan, Stephen Q Wong, Maria Joao Silva, Ravikiran Vedururu, Kenneth Doig, Enid Lam, Gisela Mir Arnau, Timothy Semple, Meaghan Wall, Andjelija Zivanovic, Rishu Agarwal, Pasquale Petrone, Kate Jones, David Westerman, Piers Blombery, John F Seymour, Anthony T Papenfuss, Mark A Dawson, Constantine S Tam, Sarah-Jane Dawson
Several novel therapeutics are poised to change the natural history of chronic lymphocytic leukaemia (CLL) and the increasing use of these therapies has highlighted limitations of traditional disease monitoring methods. Here we demonstrate that circulating tumour DNA (ctDNA) is readily detectable in patients with CLL. Importantly, ctDNA does not simply mirror the genomic information contained within circulating malignant lymphocytes but instead parallels changes across different disease compartments following treatment with novel therapies...
March 17, 2017: Nature Communications
https://www.readbyqxmd.com/read/28295527/factors-predicting-survival-in-chronic-lymphocytic-leukemia-patients-developing-richter-syndrome-transformation-into-hodgkin-lymphoma
#6
Francesca Romana Mauro, Piero Galieni, Alessandra Tedeschi, Luca Laurenti, Giovanni Del Poeta, Gianluigi Reda, Marina Motta, Alessandro Gozzetti, Roberta Murru, Maria Denise Caputo, Melissa Campanelli, Anna Maria Frustaci, Idanna Innocenti, Sara Raponi, Anna Guarini, Fortunato Morabito, Robin Foà, Massimo Gentile
We hereby report the clinical and biologic features of 33 of 4680 (0.7%) patients with chronic lymphocytic leukemia (CLL), managed at 10 Italian centers, who developed Hodgkin lymphoma (HL), a rare variant of Richter syndrome. The median age at CLL and at HL diagnosis were 61 years (range 41-80) and 70 years (range 46-82), respectively, with a median interval from CLL to the diagnosis of HL of 90 months (range 0-258). In 3 cases, CLL and HL were diagnosed simultaneously. Hl was characterized by advanced stage in 79% of cases, International Prognostic Score (IPS) ≥4 in 50%, extranodal involvement in 39%, B symptoms in 70%...
March 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28289555/gender-differences-in-post-traumatic-stress
#7
Maria Grazia Modena, Daniele Pettorelli, Giulia Lauria, Elisa Giubertoni, Erminio Mauro, Valentina Martinotti
Acute stress can trigger cardiovascular events and disease. The earthquake is an "ideal" natural experiment for acute and chronic stress, with impact mainly on the cardiovascular system. On May 20th and 29th, 2012, two earthquakes of magnitude 5.9° to 6.4° on the Richter scale, hit the province of Modena and Reggio Emilia, an area of the north-center of Italy never considered at seismic risk. The purpose of our study was to assess whether there were gender-specific differences in stress-induced incidence of cardiovascular events and age of patients who arrived at the Emergency Departments (ED) of the three main teaching hospitals of the University of Modena and Reggio Emilia...
2017: BioResearch Open Access
https://www.readbyqxmd.com/read/28263223/hypereosinophilic-syndrome-after-liver-transplantation-a-case-report-and-a-review-of-the-literature
#8
Wiebke Aulbert, Robin Kobbe, Christian Breuer, Andrea Briem-Richter, Hansjörg Schäfer, Florian Brinkert, Anne Dettmar, Markus Josef Kemper, Enke Grabhorn
Persistently elevated eosinophil granulocytes in the peripheral blood in children is challenging because of a complex diagnosis especially after solid organ transplantation and can lead to difficulties in finding an underlying causative factor.We report a 12-year-old boy who developed severe hypereosinophilia 11 years after liver transplantation due to biliary atresia. Accompanying symptoms were recurrent fever, fatigue, elevated liver enzymes, abdominal pain, and significant weight loss. After exclusion of secondary causes of eosinophilia, an idiopathic hypereosinophilic syndrome (I-HES) was diagnosed...
May 2017: Transplantation
https://www.readbyqxmd.com/read/28262089/subsyndromal-delirium-in-the-intensive-care-setting-phenomenological-characteristics-and-discrimination-of-subsyndromal-delirium-versus-no-and-full-syndromal-delirium
#9
Soenke Boettger, David Garcia Nuñez, Rafael Meyer, André Richter, Maria Schubert, Josef Jenewein
OBJECTIVE: Similar to delirium, its subsyndromal form has been recognized as the cause of diverse adverse outcomes. Nonetheless, the nature of this subsyndromal delirium remains vastly understudied. Therefore, in the following, we evaluate the phenomenological characteristics of this syndrome versus no and full-syndromal delirium. METHOD: In this prospective cohort study, we evaluated the Delirium Rating Scale-Revised, 1998 (DRS-R-98) versus the Diagnostic and Statistical Manual of Mental Disorders, 4th ed...
March 6, 2017: Palliative & Supportive Care
https://www.readbyqxmd.com/read/28257934/randall-sakai-chronic-social-stress-and-the-research-tradition-of-curt-richter
#10
REVIEW
Gerard P Smith, Gary J Schwartz
This paper describes Randall Sakai's professional career from graduate school at the University of Pennsylvania, through postdoctoral work at Rockefeller University, and to being an independent investigator at the University of Cincinnati. He was fortunate in having Alan Epstein, Bruce McEwen, and Eliot Stellar as mentors. Early in Sakai's graduate work, Epstein and Stellar introduced him to Curt Richter, the legendary investigator at Johns Hopkins. This early introduction to Richter and his tradition of research was crucial for Sakai's scientific development...
February 28, 2017: Physiology & Behavior
https://www.readbyqxmd.com/read/28203174/richter-s-syndrome-with-hypercalcemia-induced-by-tumor-associated-production-of-parathyroid-hormone-related-peptide
#11
Naoki Watanabe, Hajime Yasuda, Soji Morishita, Yasuo Aota, Junichi Tomomatsu, Masaru Tanaka, Akimichi Ohsaka, Norio Komatsu
Humoral hypercalcemia due to parathyroid hormone-related peptide (PTHrP) elevation is a well-known complication of various malignancies, but the situation is rare concerning hematological malignancies except for adult T-cell leukemia/lymphoma. We report a case of Richter's syndrome with humoral hypercalcemia, and demonstrate by reverse transcription polymerase chain reaction (RT-PCR) that peripheral blood PTHrP levels were 2,500-fold higher compared to healthy controls. PTHrP production by tumor cells in chronic lymphocytic leukemia (CLL) and Richter's syndrome has been previously demonstrated by nonquantitative methods such as immunohistochemistry and northern blot analysis, but this is the first report using the RT-PCR method...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28185672/cariprazine-versus-risperidone-monotherapy-for-treatment-of-predominant-negative-symptoms-in-patients-with-schizophrenia-a-randomised-double-blind-controlled-trial
#12
György Németh, István Laszlovszky, Pál Czobor, Erzsébet Szalai, Balázs Szatmári, Judit Harsányi, Ágota Barabássy, Marc Debelle, Suresh Durgam, István Bitter, Stephen Marder, W Wolfgang Fleischhacker
BACKGROUND: Although predominant negative symptoms of schizophrenia can be severe enough to cause persistent impairment, effective treatment options are lacking. We aimed to assess the new generation antipsychotic cariprazine in adult patients with predominant negative symptoms. METHODS: In this randomised, double-blind, phase 3b trial, we enrolled adults aged 18-65 years with long-term (>2 year), stable schizophrenia and predominant negative symptoms (>6 months) at 66 study centres (mainly hospitals and university clinics, with a small number of private practices) in 11 European countries...
February 7, 2017: Lancet
https://www.readbyqxmd.com/read/28181942/successful-management-of-a-patient-with-possible-mast-cell-activation-syndrome-undergoing-pulmonary-embolectomy-a-case-report
#13
Ellen W Richter, Kai-Ling Hsu, Vanessa Moll
We report the successful perioperative management of a patient with presumed mastocytosis undergoing pulmonary embolectomy. Postoperatively the patient went into vasodilatory shock, which was partly attributed to mast cell mediator release. H1- and H2-antagonists, steroids, and a single dose of methylene blue were given with improvement of hemodynamics. The patient was weaned off vasoactive substances and extubated by postoperative day 2. We discuss the perioperative management of patients with mastocytosis, briefly review the literature concerning anesthetic management for cardiac surgery in patients with this disorder, and discuss our patient's alternative but related diagnosis of idiopathic mast cell activation syndrome...
February 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28103472/microsatellite-expansion-diseases-repeat-toxicity-found-in-translation
#14
Fen-Biao Gao, Joel D Richter
Sellier et al. (2017) show that translation of expanded CGG repeats in fragile X-associated tremor/ataxia syndrome is initiated at an upstream ACG near-cognate start codon. The resulting polyglycine-containing protein, but not repeat RNA, is pathogenic by disrupting the nuclear lamina.
January 18, 2017: Neuron
https://www.readbyqxmd.com/read/28095146/phase-i-first-in-human-study-of-venetoclax-in-patients-with-relapsed-or-refractory-non-hodgkin-lymphoma
#15
Matthew S Davids, Andrew W Roberts, John F Seymour, John M Pagel, Brad S Kahl, William G Wierda, Soham Puvvada, Thomas J Kipps, Mary Ann Anderson, Ahmed Hamed Salem, Martin Dunbar, Ming Zhu, Franklin Peale, Jeremy A Ross, Lori Gressick, Monali Desai, Su Young Kim, Maria Verdugo, Rod A Humerickhouse, Gary B Gordon, John F Gerecitano
Purpose B-cell leukemia/lymphoma-2 (BCL-2) overexpression is common in many non-Hodgkin lymphoma (NHL) subtypes. A phase I trial in patients with NHL was conducted to determine safety, pharmacokinetics, and efficacy of venetoclax, a selective, potent, orally bioavailable BCL-2 inhibitor. Patients and Methods A total of 106 patients with relapsed or refractory NHL received venetoclax once daily until progressive disease or unacceptable toxicity at target doses from 200 to 1,200 mg in dose-escalation and safety expansion cohorts...
March 10, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28072810/high-frequency-spinal-cord-stimulation-for-complex-regional-pain-syndrome-a-case-report
#16
Joseph T Crapanzano, Lisa M Harrison-Bernard, Mark R Jones, Alan D Kaye, Erich O Richter, Mordeci N Potash
Complex regional pain syndrome (CRPS) is a chronic, debilitating, neuropathic pain condition which is often misdiagnosed, difficult to manage, and lacks proven methods for remission. Most available methods provide some relief to a small percentage of patients. Recent FDA approval and superiority of the Nevro Senza 10-kHz high frequency (HF10) spinal cord stimulation (SCS) therapy over traditional low-frequency spinal cord stimulation for treatment of chronic back and leg pain may provide a new interventional therapeutic option for patients suffering from CRPS...
January 2017: Pain Physician
https://www.readbyqxmd.com/read/28012536/the-impact-of-tobacco-exposure-on-development-of-otorrhea-after-myringotomy-tube-placement
#17
Jamie G Bizzell, Matthew D Cox, Annie R Wang, Gresham T Richter, Abby R Nolder
INTRODUCTION: Otorrhea is a common sequela after myringotomy with tube placement (MTP). The purpose of this study was to identify any significant relationship between passive tobacco exposure and the development of post-tympanostomy tube otorrhea. METHODS: Retrospective chart review was performed on 774 cases of patients who underwent MTP by a single surgeon at a tertiary pediatric hospital from 2012 to 2014. Patients with multiple medical comorbidities, craniofacial anomalies, syndromes and those over 12 years of age were excluded...
January 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28002286/macular-microcysts-in-schwartz-matsuo-syndrome
#18
Xuejing Chen, Grace M Richter, Joseph Caprioli, Tara A McCannel
PURPOSE: To describe a case of macular microcysts associated with advanced secondary glaucoma from Schwartz-Matsuo syndrome. METHODS: Clinical presentation, color fundus photography, optic disk photography, visual fields, and optical coherence tomography are presented. RESULTS: A 32-year-old woman presented with advanced, unilateral secondary glaucoma associated with a macula-on rhegmatogenous retinal detachment. She was diagnosed with Schwartz-Matsuo syndrome...
December 20, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27918339/irritable-bowel-syndrome-and-quality-of-life-in-women-with-fecal-incontinence
#19
Alayne D Markland, J Eric Jelovsek, David D Rahn, Lu Wang, Leah Merrin, Ashok Tuteja, Holly E Richter, Susan Meikle
OBJECTIVES: The objectives of this work were to determine the prevalence of irritable bowel syndrome (IBS) and IBS subtypes in women presenting for fecal incontinence (FI) treatment and to assess the impact of IBS on FI symptoms and quality of life (QOL). METHODS: In this multicenter prospective cohort study, women reported at least monthly solid, liquid, or mucus FI. Rome III clinical criteria defined IBS. Women also self-reported having an IBS diagnosis. Baseline questionnaires included the following: Modified Manchester Health Questionnaire, Fecal Incontinence Severity Index, Bristol Stool Scale, Pelvic Floor Distress Inventory, and the Pelvic Floor Impact Questionnaire...
May 2017: Female Pelvic Medicine & Reconstructive Surgery
https://www.readbyqxmd.com/read/27899414/sentinel-case-of-richter-transformation-from-chronic-lymphocytic-leukaemia-small-lymphocytic-lymphoma-to-cd3-diffuse-large-b-cell-lymphoma
#20
Ali Ismail, Jawed A Mallick, Dahui Qin, Mohammad O Hussaini
AIM: To report the first case of a Richter syndrome where small lymphocytic lymphoma (SLL) progressed to a CD3+ diffuse large B-cell lymphoma (DLBCL). METHODS: Macrodissection of small and large cell lymphomatous components was performed. This was followed by flow cytometric analysis along with molecular B-cell immunoglobulin (heavy and light chains) and T-cell receptor (γ and β chains) gene rearrangement studies to investigate a clonal relationship between the components...
November 29, 2016: Journal of Clinical Pathology
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