keyword
MENU ▼
Read by QxMD icon Read
search

Evan's syndrome

keyword
https://www.readbyqxmd.com/read/28937520/evans-syndrome-as-first-manifestation-of-primary-immunodeficiency-in-clinical-practice
#1
Libny Martínez-Valdez, Angela Deyà-Martínez, María T Giner, Rubén Berrueco, Ana Esteve-Solé, Manel Juan, Ana M Plaza-Martín, Laia Alsina
BACKGROUND: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID)...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28930962/attenuation-of-hemorrhage-associated-lung-injury-by-adjuvant-treatment-with-c23-an-oligopeptide-derived-from-cold-inducible-rna-binding-protein
#2
Fangming Zhang, Weng-Lang Yang, Max Brenner, Ping Wang
BACKGROUND: Hemorrhagic shock (HS) is an important cause of mortality. HS is associated with an elevated incidence of acute lung injury and acute respiratory distress syndrome, significantly contributing to HS morbidity and mortality. Cold-inducible RNA-binding protein (CIRP) is released into the circulation during HS and can cause lung injury. C23 is a CIRP-derived oligopeptide that binds with high affinity to the CIRP receptor and inhibits CIRP-induced phagocyte secretion of TNF-α...
October 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28925590/analytic-methods-for-evaluating-patterns-of-multiple-congenital-anomalies-in-birth-defect-registries
#3
REVIEW
A J Agopian, Jane A Evans, Philip J Lupo
BACKGROUND: It is estimated that 20 to 30% of infants with birth defects have two or more birth defects. Among these infants with multiple congenital anomalies (MCA), co-occurring anomalies may represent either chance (i.e., unrelated etiologies) or pathogenically associated patterns of anomalies. While some MCA patterns have been recognized and described (e.g., known syndromes), others have not been identified or characterized. Elucidating these patterns may result in a better understanding of the etiologies of these MCAs...
September 19, 2017: Birth defects research
https://www.readbyqxmd.com/read/28918841/epidural-abscess-in-lemierre-s-syndrome
#4
Christina M Meade, Valeria D Cantos, Hachem Nasri, Mara Serbanescu, Evan J Anderson
No abstract text is available yet for this article.
September 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28918392/cnvs-affecting-cancer-predisposing-genes-cpgs-detected-as-incidental-findings-in-routine-germline-diagnostic-chromosomal-microarray-cma-testing
#5
Josie Innes, Lisa Reali, Jill Clayton-Smith, Georgina Hall, Derek Hk Lim, George J Burghel, Kim French, Unzela Khan, Daniel Walker, Fiona Lalloo, D Gareth R Evans, Dominic McMullan, Eamonn R Maher, Emma R Woodward
BACKGROUND: Identification of CNVs through chromosomal microarray (CMA) testing is the first-line investigation in individuals with learning difficulties/congenital abnormalities. Although recognised that CMA testing may identify CNVs encompassing a cancer predisposition gene (CPG), limited information is available on the frequency and nature of such results. METHODS: We investigated CNV gains and losses affecting 39 CPGs in 3366 pilot index case individuals undergoing CMA testing, and then studied an extended cohort (n=10 454) for CNV losses at 105 CPGs and CNV gains at 9 proto-oncogenes implicated in inherited cancer susceptibility...
September 16, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28916678/gucy2c-signaling-opposes-the-acute-radiation-induced-gi-syndrome
#6
Peng Li, Evan Wuthrick, Jeff A Rappaport, Crystal Kraft, Jieru E Lin, Glen Marszalowicz, Adam E Snook, Tingting Zhan, Terry M Hyslop, Scott A Waldman
High doses of ionizing radiation induce acute damage to epithelial cells of the gastrointestinal (GI) tract, mediating toxicities restricting the therapeutic efficacy of radiation in cancer and morbidity and mortality in nuclear disasters. No approved prophylaxis or therapy exists for these toxicities, in part reflecting an incomplete understanding of mechanisms contributing to the acute radiation-induced GI syndrome (RIGS). Guanylate cyclase C (GUCY2C) and its hormones guanylin and uroguanylin have recently emerged as one paracrine axis defending intestinal mucosal integrity against mutational, chemical, and inflammatory injury...
September 15, 2017: Cancer Research
https://www.readbyqxmd.com/read/28904807/the-return-of-a-former-foe-syphilis-with-antiphospholipid-syndrome-as-a-cause-of-acute-stroke
#7
Daniela Noakes, Katherine Evans, Rohan Pathansali
This article highlights a rare complication of syphilis infection and the importance of including syphilis and antiphospholipid antibody testing in the acute stroke screen..
September 2017: JRSM Open
https://www.readbyqxmd.com/read/28892549/pediatric-obstructive-sleep-apnea-in-high-risk-populations-clinical-implications
#8
Mai El Mallah, Evan Bailey, Michelle Trivedi, Ted Kremer, Lawrence M Rhein
Certain common medical conditions are associated with a higher risk of pediatric obstructive sleep apnea (OSA). A lower threshold for screening is therefore indicated for such patient cohorts. In this article, we briefly discuss the high prevalence of OSA in children born prematurely, and in those with Down syndrome, craniofacial disorders, and neuromuscular disorders. Primary care providers should have an increased index of suspicion for OSA in these children, considering the neurocognitive disability that occurs in these high-risk groups when OSA is left untreated...
September 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28890943/trial-of-amiloride-in-type-2-diabetes-with-proteinuria
#9
Mark L Unruh, V Shane Pankratz, John E Demko, Evan C Ray, Rebecca P Hughey, Thomas R Kleyman
INTRODUCTION: Renal Na(+) retention and extracellular fluid volume expansion are hallmarks of nephrotic syndrome, which occurs even in the absence of activation of hormones that stimulate renal Na(+) transporters. Plasmin-dependent activation of the epithelial Na(+) channel (ENaC) has been proposed to have a role in renal Na(+) retention in the setting of nephrotic syndrome. We hypothesized that the ENaC inhibitor amiloride would be an effective therapeutic agent in inducing a natriuresis and lowering blood pressure in individuals with macroscopic proteinuria...
September 2017: KI Reports
https://www.readbyqxmd.com/read/28887897/opportunistic-invasive-cutaneous-fungal-infections-associated-with-administration-of-cyclosporine-to-dogs-with-immune-mediated-disease
#10
B B McAtee, K J Cummings, A K Cook, J A Lidbury, J C Heseltine, M D Willard
BACKGROUND: Opportunistic invasive fungal infections (OIFIs) occur in dogs administered immunosuppressive medications. However, the epidemiology of OIFIs among dogs undergoing immunosuppressive treatment is poorly understood. The aims of this study were to (1) estimate the incidence of OIFIs among dogs diagnosed with certain immune-mediated diseases and treated with immunosuppressive drugs, and (2) determine if administration of particular drug(s) was a risk factor for OIFIs. HYPOTHESIS: Dogs receiving cyclosporine treatment (alone or as part of a multidrug protocol) are at higher risk of developing OIFIs...
September 9, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28878866/association-of-autoimmune-hepatitis-type-1-in-a-child-with-evans-syndrome
#11
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, David A Gremse
Autoimmune hepatitis (AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome (ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab...
August 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28867142/exonic-mosaic-mutations-contribute-risk-for-autism-spectrum-disorder
#12
Deidre R Krupp, Rebecca A Barnard, Yannis Duffourd, Sara A Evans, Ryan M Mulqueen, Raphael Bernier, Jean-Baptiste Rivière, Eric Fombonne, Brian J O'Roak
Genetic risk factors for autism spectrum disorder (ASD) have yet to be fully elucidated. Postzygotic mosaic mutations (PMMs) have been implicated in several neurodevelopmental disorders and overgrowth syndromes. By leveraging whole-exome sequencing data on a large family-based ASD cohort, the Simons Simplex Collection, we systematically evaluated the potential role of PMMs in autism risk. Initial re-evaluation of published single-nucleotide variant (SNV) de novo mutations showed evidence consistent with putative PMMs for 11% of mutations...
September 7, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28852188/lipidomic-signatures-of-nonhuman-primates-with-radiation-induced-hematopoietic-syndrome
#13
Evan L Pannkuk, Evagelia C Laiakis, Vijay K Singh, Albert J Fornace
Concern over potential exposures of ionizing radiation (IR) to large populations has emphasized the need for rapid and reliable methods of biodosimetry to determine absorbed dose and required triage. Lipidomics has emerged as a powerful technique for large-scale lipid identification and quantification. Indirect effects from IR exposure generate reactive oxygen species (ROS) through water hydrolysis and may subsequently damage cellular lipids. Thus, rapid identification of specific affected lipid molecules represents possible targets for biodosimetry...
August 29, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28841841/isolated-non-traumatic-non-aneurysmal-convexal-subarachnoid-hemorrhage-in-a-patient-with-evans-syndrome
#14
Anna Misyail Abdul Rashid, Mohamad Syafeeq Faeez Md Noh
BACKGROUND: Non-traumatic, spontaneous subarachnoid hemorrhage occurs in approximately 85% of cases where there is a ruptured saccular aneurysm. An additional 10% of cases arise from non-aneurysmal peri-mesencephalic hemorrhages. CASE PRESENTATION: We report a rare case of a young female, with underlying Evans syndrome, who was initially thought to have non-hemorrhagic stroke, eventually diagnosed having isolated non-traumatic, non-aneurysmal convexal subarachnoid haemorrhage...
August 25, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28811186/identification-and-characterization-of-small-molecule-inhibitors-of-porcine-reproductive-and-respiratory-syndrome-virus
#15
Alyssa B Evans, Pengfei Dong, Hyelee Loyd, Jianqiang Zhang, George A Kraus, Susan Carpenter
Porcine reproductive and respiratory syndrome virus (PRRSV) is the etiological agent of PRRS, an economically significant disease of swine worldwide. PRRSV is poorly controlled by the currently available vaccines, and alternative control strategies are needed to help prevent the continual circulation of the virus. Previously, we developed a synthetic route for the natural compound atractylodinol and demonstrated anti-PRRSV activity in vitro. However, the synthetic route was inefficient and the yield was poor...
August 12, 2017: Antiviral Research
https://www.readbyqxmd.com/read/28807233/overcoming-obstacles-in-the-design-of-cancer-anorexia-weight-loss-trials
#16
REVIEW
Jennifer G Le-Rademacher, Jeffrey Crawford, William J Evans, Aminah Jatoi
Most advanced cancer patients suffer loss of appetite (anorexia) and loss of weight. Despite the fact that cancer anorexia and weight loss are associated with a poor prognosis and detract from quality of life, no interventions have been demonstrated to palliate this syndrome in its entirety, particularly in patients with treatment-refractory malignancies. Recently, two registration trials - one with anamorelin and another with enobosarm - failed to reach their primary endpoints, thus raising questions. Were both these agents ineffective? Alternatively, did study design issues compromise the ability of these trials to identify effective agents? Thus, this review is timely insofar it serves as an introduction to study design, offers guidance on how to test promising agents for cancer anorexia/weight loss, and provides advice for overcoming trial design obstacles...
September 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28793997/effect-of-intensive-versus-standard-blood-pressure-treatment-according-to-baseline-prediabetes-status-a-post-hoc-analysis-of-a-randomized-trial
#17
Adam P Bress, Jordan B King, Kathryn E Kreider, Srinivasan Beddhu, Debra L Simmons, Alfred K Cheung, Yingying Zhang, Michael Doumas, John Nord, Mary Ellen Sweeney, Addison A Taylor, Charles Herring, William J Kostis, James Powell, Anjay Rastogi, Christianne L Roumie, Alan Wiggers, Jonathan S Williams, Reem Yunis, Athena Zias, Greg W Evans, Tom Greene, Michael V Rocco, William C Cushman, David M Reboussin, Mark N Feinglos, Vasilios Papademetriou
OBJECTIVE: To determine whether the effects of intensive (<120 mmHg) compared with standard (<140 mmHg) systolic blood pressure (SBP) treatment are different among those with prediabetes versus those with fasting normoglycemia at baseline in the Systolic Blood Pressure Intervention Trial (SPRINT). RESEARCH DESIGN AND METHODS: This was a post hoc analysis of SPRINT. SPRINT participants were categorized by prediabetes status, defined as baseline fasting serum glucose ≥100 mg/dL versus those with normoglycemia (fasting serum glucose <100 mg/dL)...
August 9, 2017: Diabetes Care
https://www.readbyqxmd.com/read/28772291/baseline-surveillance-in-li-fraumeni-syndrome-using-whole-body-magnetic-resonance-imaging-a-meta-analysis
#18
Mandy L Ballinger, Ana Best, Phuong L Mai, Payal P Khincha, Jennifer T Loud, June A Peters, Maria Isabel Achatz, Rubens Chojniak, Alexandre Balieiro da Costa, Karina Miranda Santiago, Judy Garber, Allison F O'Neill, Rosalind A Eeles, D Gareth Evans, Eveline Bleiker, Gabe S Sonke, Marielle Ruijs, Claudette Loo, Joshua Schiffman, Anne Naumer, Wendy Kohlmann, Louise C Strong, Jasmina Bojadzieva, David Malkin, Surya P Rednam, Elena M Stoffel, Erika Koeppe, Jeffrey N Weitzel, Thomas P Slavin, Bita Nehoray, Mark Robson, Michael Walsh, Lorenzo Manelli, Anita Villani, David M Thomas, Sharon A Savage
Importance: Guidelines for clinical management in Li-Fraumeni syndrome, a multiple-organ cancer predisposition condition, are limited. Whole-body magnetic resonance imaging (WBMRI) may play a role in surveillance of this high-risk population. Objective: To assess the clinical utility of WBMRI in germline TP53 mutation carriers at baseline. Data Sources: Clinical and research surveillance cohorts were identified through the Li-Fraumeni Exploration Research Consortium...
August 3, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28772289/association-of-mismatch-repair-mutation-with-age-at-cancer-onset-in-lynch-syndrome-implications-for-stratified-surveillance-strategies
#19
Neil A J Ryan, Julie Morris, Kate Green, Fiona Lalloo, Emma R Woodward, James Hill, Emma J Crosbie, D Gareth Evans
Importance: Lynch syndrome is caused by dominantly inherited germline mutations that predispose individuals to colorectal, endometrial, ovarian, and other cancers through inactivation of the cellular mismatch repair system. Lynch syndrome-associated cancers are amenable to surveillance strategies that may improve survival. The age at which surveillance should start is disputed. Objective: To determine whether mutated gene and type of mutation influence age at onset of Lynch syndrome-associated cancers...
August 3, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28765416/the-physician-s-role-in-perioperative-management-of-older-patients-undergoing-surgery
#20
Adam L Gordon, Barry J Evans, Jugdeep Dhesi
Life-sustaining and life-improving surgical interventions are increasingly available to older, frailer patients, many of whom have multimorbidity. Physicians can help support perioperative multidisciplinary teams with assessment and preoperative optimisation of physiological reserve, comorbidities and associated geriatric syndromes. Similar structured support can be useful in the postoperative period where older patients are at increased risk of delirium, medical complications, increased functional dependency and where discharge planning can prove more difficult than in younger cohorts...
July 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
keyword
keyword
89028
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"