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https://www.readbyqxmd.com/read/29889287/association-between-sickle-cell-trait-with-selected-chronic-medical-conditions-in-u-s-service-members
#1
David W Niebuhr, Ligong Chen, Stephanie Shao, Jonathan Goldsmith, Celia Byrne, Darrell E Singer
Introduction: Sickle cell trait (SCT), the heterozygous carrier state for hemoglobin S, is present in an estimated 1.6% of all newborns and 7.3% in black individuals in the USA. SCT has long been considered a benign condition with anticipated normal life expectancy and no increased risk for chronic diseases. The medical literature is inconclusive on the potential association between SCT and chronic medical conditions (CMC) including chronic kidney disease, venous thromboembolism, and stroke...
June 8, 2018: Military Medicine
https://www.readbyqxmd.com/read/29861814/endothelial-cell-culture-in-microfluidic-devices-for-investigating-microvascular-processes
#2
Robert G Mannino, Yongzhi Qiu, Wilbur A Lam
Numerous conditions and disease states such as sickle cell disease, malaria, thrombotic microangiopathy, and stroke significantly impact the microvasculature function and its role in disease progression. Understanding the role of cellular interactions and microvascular hemodynamic forces in the context of disease is crucial to understanding disease pathophysiology. In vivo models of microvascular disease using animal models often coupled with intravital microscopy have long been utilized to investigate microvascular phenomena...
July 2018: Biomicrofluidics
https://www.readbyqxmd.com/read/29843828/assessment-of-executive-functions-in-preschool-children-with-sickle-cell-anemia
#3
Michelle Downes, Fenella J Kirkham, Paul T Telfer, Michelle de Haan
OBJECTIVES: Children with sickle cell anemia (SCA) are commonly reported to experience executive dysfunction. However, the development of executive function (EF) in preschool-age children without stroke in this patient population has not been investigated so it is unclear when and how these deficits emerge. METHODS: This case-control study examines the feasibility of assessing the early development of executive functioning in 22 preschool children years with SCA in the domains of processing speed, working memory, attention, inhibitory control, and cognitive flexibility, as well as everyday function, in comparison to matched control children...
May 30, 2018: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/29806147/new-approaches-to-genetic-predisposition-for-hemorrhagic-stroke-in-sickle-cell-disease
#4
Robert J Adams, Rufus O Akinyemi, Mayowa O Owolabi, Dan L Lackland, Bruce Ovbiagele
No abstract text is available yet for this article.
May 27, 2018: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/29797633/annual-stroke-incidence-in-nigerian-children-with-sickle-cell-disease-and-elevated-tcd-velocities-treated-with-hydroxyurea
#5
IkeOluwa Lagunju, B J Brown, A O Oyinlade, A Asinobi, J Ibeh, A Esione, O O Sodeinde
BACKGROUND: Elevated transcranial Doppler (TCD) velocities accurately predict stroke risk in children with sickle cell disease (SCD). Chronic blood transfusion, the gold standard for primary stroke prevention, is faced with numerous challenges in Africa. Hydroxyurea (HU) has been shown to reduce elevated TCD velocities in children with SCD. AIM: To determine the effectiveness of HU in reducing the risk of primary stroke in a cohort of Nigerian children with SCD and elevated velocities treated with HU...
May 24, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29785408/stroke-prevalence-in-children-with-sickle-cell-disease-in-sub-saharan-africa-a-systematic-review-and-meta-analysis
#6
Lianna J Marks, Deogratias Munube, Philip Kasirye, Ezekiel Mupere, Zhezhen Jin, Philip LaRussa, Richard Idro, Nancy S Green
Objectives . The prevalence of stroke among children with sickle cell disease (SCD) in sub-Saharan Africa was systematically reviewed. Methods . Comprehensive searches of PubMed, Embase, and Web of Science were performed for articles published between 1980 and 2016 (English or French) reporting stroke prevalence. Using preselected inclusion criteria, titles and abstracts were screened and full-text articles were reviewed. Results . Ten full-text articles met selection criteria. Cross-sectional clinic-based data reported 2...
2018: Global Pediatric Health
https://www.readbyqxmd.com/read/29778312/biomarker-signatures-of-sickle-cell-disease-severity
#7
Mengtian Du, Sarah Van Ness, Victor Gordeuk, Sayed M Nouraie, Sergei Nekhai, Mark Gladwin, Martin H Steinberg, Paola Sebastiani
Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster analysis to discover 17 signatures of 17 common circulating biomarkers in 2320 participants of the Cooperative Study of Sickle Cell Disease, and evaluated the association of these signatures with risk for stroke, pain, leg ulceration, acute chest syndrome, avascular necrosis, seizure, death, and trend of fetal hemoglobin and hemolysis using longitudinally collected data...
May 16, 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#8
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29732592/transcranial-doppler-ultrasound-in-peninsular-arab-patients-with-sickle-cell-disease
#9
Adekunle Adekile, Meaad Hassan, Akram Asbeutah, Mohamed Al-Hinai, Omar Trad, Nayef Farhan
OBJECTIVES: Transcranial Doppler ultrasound is used to identify patients with sickle cell disease (SCD) at risk for stroke. We performed transcranial Doppler studies in patients from 4 countries in the Arabian Peninsula (Kuwait, Oman, Iraq, and United Arab Emirates) to document the prevalence of abnormal transcranial Doppler findings. METHODS: The patients were recruited from outpatient clinics and studied in a steady state. Transcranial Doppler examinations were performed with standard equipment by experienced operators...
May 6, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29710269/association-of-sickle-cell-trait-with-ischemic-stroke-among-african-americans-a-meta-analysis
#10
Hyacinth I Hyacinth, Cara L Carty, Samantha R Seals, Marguerite R Irvin, Rakhi P Naik, Gregory L Burke, Neil A Zakai, James G Wilson, Nora Franceschini, Cheryl A Winkler, Victor A David, Jeffrey B Kopp, Suzanne E Judd, Robert J Adams, W T Longstreth, Leonard Egede, Daniel T Lackland, Herman Taylor, JoAnn E Manson, Virginia Howard, Matthew Allison, Beatrice E Gee, Adolfo Correa, Monika M Safford, Donna K Arnett, George Howard, Alexander P Reiner, Mary Cushman
Importance: African Americans and individuals of African ancestry have a higher risk of stroke compared with non-Hispanic white individuals. Identifying the source of this disparity could provide an opportunity for clinical stroke risk stratification and more targeted therapy. Whether sickle cell trait (SCT) is an indicator of increased risk of ischemic stroke among African Americans is still unclear. Objective: To examine whether SCT is associated with a higher risk of incident ischemic stroke among African Americans...
April 23, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29668544/splenectomy-to-optimize-hemoglobin-s-control-in-children-with-sickle-cell-anemia-on-chronic-transfusion-therapy-for-stroke-prevention
#11
L Vandy Black, Jolie S Ramesar, Catherine H Boston
Chronic transfusion therapy with the goal of maintaining a hemoglobin (Hb) S <30% is the primary recommended treatment for children with sickle cell anemia and a history of overt stroke or abnormal transcranial Doppler examination. We report chronic hypersplenism as a cause of poor HbS% control in 3 children on chronic transfusion therapy for stroke prevention. Splenectomy resulted in a 39.77% (95% confidence interval, 34.3-45.3, P<0.0001) mean reduction in HbS% with no perioperative or infectious complications suggesting the need for additional research into splenectomy as a therapeutic option for select high-risk children to optimize transfusion therapy for stroke prevention...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29666400/the-corticosteroid-compounds-prednisolone-and-vamorolone-do-not-alter-the-nociception-phenotype-and-exacerbate-liver-injury-in-sickle-cell-mice
#12
Luis E F Almeida, Jesse M Damsker, Sarah Albani, Nina Afsar, Sayuri Kamimura, Drew Pratt, David E Kleiner, Martha Quezado, Heather Gordish-Dressman, Zenaide M N Quezado
Clinicians often hesitate prescribing corticosteroids to treat corticosteroid-responsive conditions in sickle cell disease (SCD) patients because their use can be associated with complications (increased hospital readmission, rebound pain, strokes, avascular necrosis, acute chest syndrome). Consequently, SCD patients may receive suboptimal treatment for corticosteroid-responsive conditions. We conducted a preclinical trial of dissociative (vamorolone) and conventional (prednisolone) corticosteroid compounds to evaluate their effects on nociception phenotype, inflammation, and organ dysfunction in SCD mice...
April 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29614637/inflammation-in-sickle-cell-disease
#13
Nicola Conran, John D Belcher
The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant pathophysiological consequences that result in hemolytic events and the induction of the inflammatory processes that ultimately lead to vaso-occlusion. In addition to their role in the initiation of the acute painful vaso-occlusive episodes that are characteristic of SCD, inflammatory processes are also key components of many of the complications of the disease including autosplenectomy, acute chest syndrome, pulmonary hypertension, leg ulcers, nephropathy and stroke...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614631/simple-chronic-transfusion-therapy-a-crucial-therapeutic-option-for-sickle-cell-disease-improves-but-does-not-normalize-blood-rheology-what-should-be-our-goals-for-transfusion-therapy
#14
Jon A Detterich
Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614629/genetic-modifiers-of-severity-in-sickle-cell-disease
#15
Alicia K Chang, Carly C Ginter Summarell, Parendi T Birdie, Vivien A Sheehan
Sickle cell disease (SCD) is one of the most common single disease disorders world-wide. It is remarkable for its clinical heterogeneity, even among individuals with identical genotypes. Some individuals experience morbidity and mortality in early childhood, while others have a relatively mild course, and normal or near normal life expectancy. Many clinical complications are associated with SCD; most notably frequent pain episodes, stroke, acute chest syndrome, avascular necrosis, nephropathy, retinopathy and pulmonary hypertension...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29590102/common-%C3%AE-globin-variants-modify-hematologic-and-other-clinical-phenotypes-in-sickle-cell-trait-and-disease
#16
Laura M Raffield, Jacob C Ulirsch, Rakhi P Naik, Samuel Lessard, Robert E Handsaker, Deepti Jain, Hyun M Kang, Nathan Pankratz, Paul L Auer, Erik L Bao, Joshua D Smith, Leslie A Lange, Ethan M Lange, Yun Li, Timothy A Thornton, Bessie A Young, Goncalo R Abecasis, Cathy C Laurie, Deborah A Nickerson, Steven A McCarroll, Adolfo Correa, James G Wilson, Guillaume Lettre, Vijay G Sankaran, Alex P Reiner
Co-inheritance of α-thalassemia has a significant protective effect on the severity of complications of sickle cell disease (SCD), including stroke. However, little information exists on the association and interactions for the common African ancestral α-thalassemia mutation (-α3.7 deletion) and β-globin traits (HbS trait [SCT] and HbC trait) on important clinical phenotypes such as red blood cell parameters, anemia, and chronic kidney disease (CKD). In a community-based cohort of 2,916 African Americans from the Jackson Heart Study, we confirmed the expected associations between SCT, HbC trait, and the -α3...
March 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29574950/automated-rbc-exchange-compared-to-manual-exchange-transfusion-for-children-with-sickle-cell-disease-is-cost-effective-and-reduces-iron-overload
#17
Laurence Dedeken, Phu Quoc Lê, Laurence Rozen, Hanane El Kenz, Sophie Huybrechts, Christine Devalck, Safiatou Diallo, Catherine Heijmans, Alina Ferster
BACKGROUND: Chronic transfusion in sickle cell disease (SCD) remains the gold standard therapy for stroke prevention and for patients with severe disease despite adequate hydroxyurea treatment. The aim of our study was to assess the safety and efficacy of automated red blood cell exchange (aRBX) in patients with SCD previously treated with manual exchange transfusion (MET). Costs related to transfusion and chelation overtime were evaluated. STUDY DESIGN AND METHODS: Beginning in January 2012, children with SCD who weighed 30 kg or more on MET could switch to aRBX...
March 25, 2018: Transfusion
https://www.readbyqxmd.com/read/29545349/proteomic-analysis-of-plasma-from-children-with-sickle-cell-anemia-and-silent-cerebral-infarction
#18
Sanjay Tewari, George Renney, John Brewin, Kate Gardner, Fenella Kirkham, Baba Inusa, James E Barrett, Stephan Menzel, Swee Lay Thein, Malcolm Ward, David C Rees
Silent cerebral infarction is the commonest neurological abnormality in children with sickle cell anemia, affecting 30-40% 14 year olds. There are no known biomarkers to identify children with silent cerebral infarcts and the pathological basis is also unknown. We used an unbiased proteomic discovery approach to identify plasma proteins differing in concentration between children with and without silent cerebral infarcts. Clinical parameters and plasma samples were analysed from 51 children (mean age 11.8 years, range 6-18) with sickle cell anemia (HbSS)...
March 15, 2018: Haematologica
https://www.readbyqxmd.com/read/29542687/sickle-cell-disease
#19
REVIEW
Gregory J Kato, Frédéric B Piel, Clarice D Reid, Marilyn H Gaston, Kwaku Ohene-Frempong, Lakshmanan Krishnamurti, Wally R Smith, Julie A Panepinto, David J Weatherall, Fernando F Costa, Elliott P Vichinsky
Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system...
March 15, 2018: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29524230/transfusion-transmitted-malaria-masquerading-as-sickle-cell-crisis-with-multisystem-organ-failure
#20
Cheryl L Maier, Phillip J Gross, Christina L Dean, Satheesh Chonat, Andrew Ip, Morgan McLemore, Fuad El Rassi, Sean R Stowell, Cassandra D Josephson, Ross M Fasano
BACKGROUND: Fever accompanying vaso-occlusive crisis is a common presentation in patients with sickle cell disease (SCD) and carries a broad differential diagnosis. Here, we report a case of transfusion-transmitted malaria in a patient with SCD presenting with acute vaso-occlusive crisis and rapidly decompensating to multisystem organ failure (MSOF). CASE REPORT: An 18-year-old African American male with SCD was admitted after multiple days of fever and severe generalized body pain...
March 9, 2018: Transfusion
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