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https://www.readbyqxmd.com/read/28913922/a-clinically-meaningful-fetal-hemoglobin-threshold-for-children-with-sickle-cell-anemia-during-hydroxyurea-therapy
#1
Jeremie H Estepp, Matthew P Smeltzer, Guolian Kang, Chen Li, Winfred C Wang, Christina Abrams, Banu Aygun, Russell E Ware, Kerri Nottage, Jane S Hankins
Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose-dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. HUSTLE (NCT00305175) is a prospective observational study with a primary goal of describing the long-term clinical effects of hydroxyurea escalated to maximal tolerated dose (MTD) in children with SCA...
September 14, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28902183/patient-characteristics-affect-the-response-to-ketamine-and-opioids-during-the-treatment-of-vaso-occlusive-episode-related-pain-in-sickle-cell-disease
#2
Raissa Nobrega, Kathy A Sheehy, Caroline Lippold, Amy L Rice, Julia C Finkel, Zenaide M N Quezado
BackgroundN-methyl-D-aspartate receptor activation has been implicated in the pathobiology of inflammatory, nociceptive and neuropathic pain, opioid tolerance, opioid-induced hyperalgesia, and central sensitization. Some of those mechanisms underlie sickle cell disease(SCD)-associated pain.MethodsWe conducted an exploratory cohort study of SCD patients who during vaso-occlusive episodes (VOEs) received subanesthetic doses of the N-methyl-D-aspartate receptor antagonist, ketamine, as an adjunct to opioids. We sought to identify predictors of changes in pain scores and of the percentage of ketamine infusions associated with meaningful changes (≥20% reduction) in pain and opioid consumption...
September 13, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28891545/protein-c-and-antithrombin-levels-in-patients-with-sickle-cell-anemia-in-ahmadu-bello-university-teaching-hospital-zaria-nigeria
#3
I U Kusfa, A I Mamman, S M Aminu, A Hassan, H M Muktar
BACKGROUND: Alterations in the components of hemostasis, namely platelet function, the procoagulant, anticoagulant, and the fibrinolytic systems, are observed in sickle cell anemia (SCA) and are in favor of a procoagulant phenotype. Therefore, study of protein C and antithrombin (AT) levels in patients with SCA in steady state may be used in the treatment and/or prevention of SCA-related thrombotic complications. We studied the changes of these naturally occurring anticoagulants in patients with SCA attending the sickle cell clinic in Ahmadu Bello University Teaching Hospital, Zaria...
August 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28888622/the-utility-of-thromboelastography-and-thrombin-generation-in-assessing-the-prothrombotic-state-of-adults-with-sickle-cell-disease
#4
Marije Wijnberge, Kiran Parmar, Rachel Kesse-Adu, Jo Howard, Alexander T Cohen, Beverley J Hunt
INTRODUCTION: Previous studies have suggested a chronic hypercoagulable state in SCD, and that thrombosis also plays a role in the pathophysiology of sickle cell vaso-occlusive pain crises (VOC). Studies looking at thrombin generation have produced conflicting results. In this study we aimed to assess and compare whole blood thromboelastography (TEG) and plasma Calibrated Automated Thrombogram (CAT) in SCD versus healthy controls and in four different SCD subgroups. MATERIALS AND METHODS: In this prospective observational study, TEG and 1pM TF activated CAT assays were performed in citrated blood samples from 77 adult (18-66years old) SCD patients (HbSS and HbSB) and 22 healthy (HbAA) ethnically-matched controls...
September 6, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28882446/relationship-between-mixed-donor-recipient-chimerism-and-disease-recurrence-following-hematopoietic-cell-transplantation-for-sickle-cell-disease
#5
Allistair Abraham, Matthew Hsieh, Mary Eapen, Courtney Fitzhugh, Jeanette Carreras, Daniel Kessler, Gregory Guilcher, Naynesh Kamani, Mark C Walters, Jaap J Boelens, John Tisdale, Shalini Shenoy
Mixed donor chimerism following hematopoietic cell transplantation for sickle cell disease (SCD) can result in resolution of disease symptoms but symptoms recur when donor chimerism is critically low. The relationship between chimerism, hemoglobin S (HbS) level and symptomatic disease was correlated retrospectively in 95 patients who had chimerism reports available at day 100, 1 and 2 years after transplantation. Recurrent disease was defined as recurrence of vaso-occlusive crises, acute chest syndrome, or stroke, and/or HbS level >50%...
September 4, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28877143/stigma-and-pain-in-adolescents-hospitalized-for-sickle-cell-vaso-occlusive-pain-episodes
#6
Sarah R Martin, Lindsey L Cohen, Ifigenia Mougianis, Anya Griffin, Soumitri Sil, Carlton Dampier
OBJECTIVES: Sickle cell disease (SCD) pain and hospitalizations increase during adolescence and adolescents with SCD may be at-risk for experiencing health-related stigma, which may result in poor health outcomes. This study examined relations among health-related stigma, pain interference, social support, quality of life (QOL), and hospital outcomes (i.e., loneliness, pain reduction, and length of stay [LOS]) in adolescents hospitalized with SCD pain. METHODS: 12- to 18-year-old adolescents (N=92) hospitalized with SCD pain completed measures of stigma, pain interference, social support, QOL, and state loneliness...
September 1, 2017: Clinical Journal of Pain
https://www.readbyqxmd.com/read/28868627/hydroxyurea-prescription-availability-and-use-for-children-with-sickle-cell-disease-in-italy-results-of-a-national-multicenter-survey
#7
Raffaella Colombatti, Giovanni Palazzi, Nicoletta Masera, Lucia Dora Notarangelo, Elisa Bonetti, Piera Samperi, Angelica Barone, Silverio Perrotta, Elena Facchini, Maurizio Miano, Giovanni Carlo Del Vecchio, Maria Elena Guerzoni, Paola Corti, Federica Menzato, Simone Cesaro, Maddalena Casale, Paolo Rigano, Gian Luca Forni, Giovanna Russo, Laura Sainati
BACKGROUND: The number of patients with sickle cell disease (SCD) has increased in Italy in the past decade due to immigration. In spite of the established efficacy of hydroxyurea (HU) in childhood, population-based data regarding its prescription and effectiveness come mainly from studies performed in adults or outside Europe. POPULATION AND METHODS: The Hydroxyurea in SCD: A Large Nation-wide Cohort Study from Italy was a retrospective cohort study of adult and pediatric patients with SCD attending 32 centers...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28868518/associations-of-%C3%AE-thalassemia-and-bcl11a-with-stroke-in-nigerian-united-states-and-united-kingdom-sickle-cell-anemia-cohorts
#8
Santosh L Saraf, Titilola S Akingbola, Binal N Shah, Chinedu A Ezekekwu, Omowunmi Sonubi, Xu Zhang, Lewis L Hsu, Mark T Gladwin, Roberto F Machado, Richard S Cooper, Victor R Gordeuk, Bamidele O Tayo
Alpha-thalassemia and the BCL11A rs1427407 T allele are commonly observed in sickle cell anemia (SCA) patients and are associated with reduced hemolysis and higher hemoglobin F levels, respectively. We investigated whether a high-risk genetic profile, defined as SCA patients who did not inherit either α-thalassemia or the BCL11A rs1427407 T allele, had stronger associations with clinical and laboratory variables than the individual genetic components in the University of Ibadan cohort (n=249). We then replicated our findings in SCA cohorts from the University of Illinois at Chicago (UIC)(n=260) and Walk-Treatment of Pulmonary Hypertension and Sickle cell disease with Sildenafil Therapy (Walk-PHaSST)(n=387)...
April 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/28867378/autologous-fat-transplants-to-deliver-glitazone-and-adiponectin-for-vasculoprotection
#9
William G Sanders, Huan Li, Ilya Zhuplatov, Yuxia He, Seong-Eun Kim, Alfred K Cheung, Jayant Agarwal, Christi M Terry
The insulin sensitizing glitazone drugs, rosiglitazone (ROS) and pioglitazone (PGZ) both have anti-proliferative and anti-inflammatory effects and induce adipose tissue (fat) to produce the vaso-protective protein adiponectin. Stenosis due to intimal hyperplasia development often occurs after placement of arteriovenous synthetic grafts used for hemodialysis. This work was performed to characterize the in vitro and in vivo effects of ROS or PGZ incorporation in fat and to determine if fat/PGZ depots could decrease vascular hyperplasia development in a porcine model of hemodialysis arteriovenous graft stenosis...
September 1, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28863145/associations-between-endothelial-dysfunction-and-clinical-and-laboratory-parameters-in-children-and-adolescents-with-sickle-cell-anemia
#10
Rozana Santos Teixeira, Regina Terse-Ramos, Tatiane Anunciação Ferreira, Vinícius Ramos Machado, Marya Izadora Perdiz, Isa Menezes Lyra, Valma Lopes Nascimento, Ney Boa-Sorte, Bruno B Andrade, Ana Marice Ladeia
BACKGROUND: Hematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA. METHODS: This study included 40 children and adolescents with stable SCA as well as 25 healthy children; aged 6-18 years...
2017: PloS One
https://www.readbyqxmd.com/read/28853040/state-of-the-art-management-of-acute-vaso-occlusive-pain-in-sickle-cell-disease
#11
REVIEW
Latika Puri, Kerri A Nottage, Jane S Hankins, Doralina L Anghelescu
Acute vaso-occlusive crisis (VOC) is a hallmark of sickle cell disease (SCD). Multiple complex pathophysiological processes can result in pain during a VOC. Despite significant improvements in the understanding and management of SCD, little progress has been made in the management of pain in SCD, although new treatments are being explored. Opioids and non-steroidal anti-inflammatory drugs (NSAIDs) remain the mainstay of treatment of VOC pain, but new classes of drugs are being tested to prevent and treat acute pain...
August 29, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28852484/sickle-cell-disease-and-albuminuria-recent-advances-in-our-understanding-of-sickle-cell-nephropathy
#12
Vincent Audard, Pablo Bartolucci, Thomas Stehlé
Albuminuria is considered to be a relevant biomarker for the detection of early glomerular damage in patients with sickle cell disease (SCD). Improvements in our understanding of the pathophysiological processes and molecular mechanisms underlying albuminuria are required, because increasing numbers of patients with SCD are developing chronic kidney disease. The early recognition of sickle cell nephropathy (SCN) and studies of the natural course of this emerging renal disease are therefore crucial, together with identification of the associated clinical and biological risk factors, to make it possible to initiate kidney-protective therapy at early stages of renal impairment...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28837214/combined-genotypes-of-the-mbl2-gene-related-to-low-mannose-binding-lectin-levels-are-associated-with-vaso-occlusive-events-in-children-with-sickle-cell-anemia
#13
Fernanda Silva Medeiros, Taciana Furtado de Mendonça, Katiuscia Araújo de Miranda Lopes, Laís Medeiros da Câmara França, Andreia Soares da Silva, Luydson Richardson Silva Vasconcelos, Maria do Carmo Valgueiro Costa de Oliveira, Ana Cláudia Mendonça Dos Anjos, Betânia Lucena Domingues Hatzlhofer, Marcos André Cavalcanti Bezerra, Aderson da Silva Araújo, Patrícia Moura, Maria do Socorro de Mendonça Cavalcanti
Sickle cell anemia (SCA) presents heterogenous clinical manifestations that cannot be explained solely by alterations to hemoglobin (Hb); other components such as endothelial adhesion, thrombosis and inflammation may be involved. The mannose-binding lectin (MBL) has an important role in innate immunity and inflammatory diseases. In this report, we describe an association between MBL2 polymorphism related to low production of serum MBL and the frequency of vasoocclusive events (FVOE) in children ≤ 5 years old with SCA (p = 0...
July 2017: Genetics and Molecular Biology
https://www.readbyqxmd.com/read/28834951/acute-pseudophakic-cystoid-macular-edema-imaged-by-optical-coherence-tomography-angiography
#14
Mardoche Chetrit, Sophie Bonnin, Valérie Mané, Ali Erginay, Ramin Tadayoni, Alain Gaudric, Aude Couturier
PURPOSE: To study macular capillary changes and vessel density in acute pseudophakic cystoid macular edema (PCME) before and after treatment using optical coherence tomography angiography. METHODS: Retrospective observational case-control study of seven consecutive patients (eight eyes) with PCME and eight age-matched control eyes imaged with optical coherence tomography angiography (RTVue XR Avanti; Optovue, Inc, Fremont, CA) using Projection Removal Artifacts software...
August 22, 2017: Retina
https://www.readbyqxmd.com/read/28815799/characterization-of-opioid-use-in-sickle-cell-disease
#15
Jin Han, Jifang Zhou, Santosh L Saraf, Victor R Gordeuk, Gregory S Calip
PURPOSE: Opioid analgesics are commonly used to treat vaso-occlusive pain episodes in sickle cell disease (SCD), but comprehensive evidence characterizing opioid use in this patient population is limited. Our objective was to characterize opioid use patterns among SCD patients using a large nationwide database. METHODS: A large, US medical claims database was utilized to identify a cohort of 3882 SCD patients, and characteristics of opioid use were analyzed. Clinical variables including age, gender, medication use, health care utilization, and medical history were evaluated for correlations with opioid use...
August 16, 2017: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/28811304/selectin-catch-bonds-mechanotransduce-integrin-activation-and-neutrophil-arrest-on-inflamed-endothelium-under-shear-flow
#16
Vasilios A Morikis, Shannon Chase, Ted Wun, Elliot L Chaikof, John L Magnani, Scott I Simon
E-selectin extends from the plasma membrane of inflamed endothelium and serves to capture leukocytes from flowing blood via long-lived catch-bonds that support slow leukocyte rolling under shear stress. Its ligands are glycosylated with the tetrasaccharide sialyl Lewis(x) (sLe(x)), which contributes to bond affinity and specificity. E-selectin mediated rolling transmits signals into neutrophils that triggers activation of high-affinity β2-integrins necessary for transition to shear resistant adhesion and transendothelial migration...
August 15, 2017: Blood
https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#17
Laura M Bou-Maroun, Fabien Meta, Curtis J Hanba, Andrew D Campbell, Gregory A Yanik
OBJECTIVE: To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures. METHODS: Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease-specific secondary diagnoses including acute chest syndrome (ACS), vaso-occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28798247/diffuse-cerebral-oedema-from-sickle-cell-vaso-occlusive-crisis
#18
Martin D Weaver, Hayan Dayoub, Emily Damuth, Tapan Kavi
No abstract text is available yet for this article.
August 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28793119/evaluation-of-the-safety-of-hospitalized-older-adults-as-for-the-risk-of-falls
#19
Nathalia de Araújo Sarges, Maria Izabel Penha de Oliveira Santos, Emanuele Cordeiro Chaves
Objective: To evaluate the safety of hospitalized older adults as for the risk of falls according to the parameters of the Morse Fall Scale. Method: Epidemiological, cross-sectional, prospective and descriptive study with n=75. Results: Average age of 71.3 years (SD±8.2); 58.7% male; 44% with low educational level; 38.7% hospitalized for cardiovascular diseases; average hospitalization of 10 days (SD±9.38); 78.7% with comorbidities; 61.3% with the calf circumference ≥ 31 cm; 62...
July 2017: Revista Brasileira de Enfermagem
https://www.readbyqxmd.com/read/28783617/an-age-dependent-response-to-hydroxyurea-in-pediatric-sickle-cell-anemia-patients-with-alpha-thalassemia-trait
#20
Lisa Figueiredo, Kerry Morrone, Catherine Wei, Karen Ireland, Hillel W Cohen, Catherine Driscoll, Deepa Manwani
Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait...
July 31, 2017: Blood Cells, Molecules & Diseases
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