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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
#1
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
#2
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
#3
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
#4
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
#5
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 28, 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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/29520844/children-with-sickle-cell-anemia-with-normal-tcd-and-without-silent-infarcts-have-a-low-incidence-of-new-strokes
#10
Lori C Jordan, Dionna O Roberts Williams, Mark J Rodeghier, Brittany Covert, Maria R Ponisio, James F Casella, Robert C McKinstry, Michael J Noetzel, Fenella J Kirkham, Emily R Meier, Beng Fuh, Melissa McNaull, Sharada Sarnaik, Suvankar Majumdar, Timothy L McCavit, Michael R DeBaun
In a prospective cohort study, we tested the hypothesis that children with sickle cell anemia (SCA) with normal transcranial Doppler ultrasound (TCD) velocities and without silent cerebral infarcts (SCIs) would have a lower incidence rate of new neurological events (strokes, seizures or transient ischemic attacks) compared to children with normal TCD measurements and SCIs, not receiving regular blood transfusions. Non-randomized participants from the Silent Cerebral Infarct Transfusion (SIT) Trial who had screening magnetic resonance imaging (MRI) of the brain and normal TCD measurements were included...
March 9, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29519371/mortality-in-children-adolescents-and-adults-with-sickle-cell-anemia-in-rio-de-janeiro-brazil
#11
Clarisse Lopes de Castro Lobo, Emilia Matos do Nascimento, Leonardo José Carvalho de Jesus, Thiago Gotelip de Freitas, Jocemir Ronaldo Lugon, Samir K Ballas
OBJECTIVE: To determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. METHODS: The number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population. RESULTS: The overall number of deaths was 281 patients with a mortality rate of 16...
January 2018: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/29504121/transcranial-doppler-velocity-among-jamaican-children-with-sickle-cell-anaemia-determining-the-significance-of-haematological-values-and-nutrition
#12
Angela E Rankine-Mullings, Nadine Morrison-Levy, Deanne Soares, Karen Aldred, Lesley King, Susanna Ali, Jennifer M Knight-Madden, Margaret Wisdom-Phipps, Robert J Adams, Russell E Ware, Marvin Reid
This study investigated the association of nutritional and haematological variables with maximum time-averaged mean velocity (TAMV) measured by transcranial Doppler (TCD) velocity and the agreement of classification between two protocols. TCD categories included: normal (<170 cm/s), conditional (170-199 cm/s) and abnormal (≥200 cm/s) based on TAMV in distal internal carotid artery (dICA), middle cerebral artery (MCA), internal carotid bifurcation, anterior and posterior cerebral arteries. Of 358 children with sickle cell anaemia (SCA) examined, the mean age (±standard deviation) was 7·4 ± 2·7 years; 13·1% and 6·7% had conditional and abnormal velocities, respectively...
March 5, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29500287/regional-oxygen-extraction-predicts-border-zone-vulnerability-to-stroke-in-sickle-cell-disease
#13
Melanie E Fields, Kristin P Guilliams, Dustin K Ragan, Michael M Binkley, Cihat Eldeniz, Yasheng Chen, Monica L Hulbert, Robert C McKinstry, Joshua S Shimony, Katie D Vo, Allan Doctor, Hongyu An, Andria L Ford, Jin-Moo Lee
OBJECTIVE: To determine mechanisms underlying regional vulnerability to infarction in sickle cell disease (SCD) by measuring voxel-wise cerebral blood flow (CBF), oxygen extraction fraction (OEF), and cerebral metabolic rate of oxygen utilization (CMRO2 ) in children with SCD. METHODS: Participants underwent brain MRIs to measure voxel-based CBF, OEF, and CMRO2 . An infarct heat map was created from an independent pediatric SCD cohort with silent infarcts and compared to prospectively obtained OEF maps...
March 2, 2018: Neurology
https://www.readbyqxmd.com/read/29488622/predictive-validity-of-developmental-screening-in-young-children-with-sickle-cell-disease-a-longitudinal-follow-up-study
#14
Jeffrey Schatz, Alyssa M Schlenz, Kelsey E Smith, Carla W Roberts
AIM: To assess the predictive validity of developmental screenings in children with sickle cell disease (SCD) for academic outcomes and stroke risk. METHOD: Parent questionnaires and medical record data were collected for a cohort receiving developmental screenings between September 2004 and May 2008 as toddlers or early school age. Screening outcomes were dichotomized (positive, negative) by a priori criteria. Questionnaires assessed school and social functioning, services received, and quality of life...
February 28, 2018: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/29478253/blood-vessel-occlusion-with-erythrocyte-aggregates-causes-burn-injury-progression-microvasculature-dilation-as-a-possible-therapy
#15
Richard A F Clark, Justine Fenner, Arielle Sasson, Steve A McClain, Adam J Singer, Marcia G Tonnesen
Other than radiation, caustic strong alkali insults, and massive over-reactive inflammation (pyoderma gangrenosum), injury progression after trauma is usually secondary to ischemia from decreased blood vessel perfusion. Impeded blood flow contributes to injury progression in a variety of disorders including venous stasis ulcers, arterial ulcers, diabetic ulcers, flap and graft necrosis, sickle cell disease, cryoglobulinemia and other vasculopathies, vasculitis, post-myocardial infarction, and post stroke. The cause of impeded blood flow in these disorders/diseases is well known...
February 25, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29475609/moya-moya-syndrome-after-cranial-radiation-for-optic-glioma-with-nf1-case-report-and-literature-review-of-syndromic-cases
#16
P Brandicourt, L Bonnet, Y Béjot, C Drouet, T Moulin, L Thines
INTRODUCTION: Moya-Moya angiopathy is a neurovascular disease that predisposes to ischemic or hemorrhagic strokes. It is generated by a steno-occlusion of the terminal portion of the internal carotid arteries, which induces the development of abnormal neovessels in the deep regions of the brain. Some pathologies such as sickle cell disease, Down syndrome or Graves' disease may be associated with Moya-Moya angiopathy. These syndromic forms harbor several differences compared with idiopathic Moya-Moya disease...
March 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#17
Dipty Jain, Dipika Mohanty
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India...
February 19, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29431623/prevalence-and-risk-factors-for-venous-thromboembolism-in-children-with-sickle-cell-disease-an-administrative-database-study
#18
Riten Kumar, Joseph Stanek, Susan Creary, Amy Dunn, Sarah H O'Brien
A hypercoagulable state resulting in increased venous thromboembolism (VTE) has been described in adults with sickle cell disease (SCD), but similar data for children are lacking. The objective of this retrospective cohort study was to describe the rate of VTE and risk factors associated with VTE in children with SCD across tertiary-care children's hospitals in the United States between the years 2009 and 2015. We used the Pediatric Health Information System database to investigate all pediatric patients with SCD admitted to 1 of 48 participating institutions between 1 January 2009 and 30 September 2015...
February 13, 2018: Blood Advances
https://www.readbyqxmd.com/read/29415213/risk-of-exertional-heat-illnesses-associated-with-sickle-cell-trait-in-u-s-military
#19
Darrell E Singer, Celia Byrne, Ligong Chen, Stephanie Shao, Jonathan Goldsmith, David W Niebuhr
Introduction: A number of studies have found an association between sickle cell trait (SCT) and exertional heat illnesses (EHIs) including heat stroke, a potentially fatal condition. The strength of this association varied across studies, limiting the ability to quantify potential benefits of SCT-screening policies for competitive athletics and military service members. We determined the relative rate and attributable risk of developing EHI associated with being SCT positive and the EHI health care utilization...
February 5, 2018: Military Medicine
https://www.readbyqxmd.com/read/29411418/feasibility-trial-for-primary-stroke-prevention-in-children-with-sickle-cell-anemia-in-nigeria-spin-trial
#20
Najibah A Galadanci, Shehu Umar Abdullahi, Leah D Vance, Abdulkadir Musa Tabari, Shehi Ali, Raymond Belonwu, Auwal Salihu, Aisha Amal Galadanci, Binta Wudil Jibir, Halima Bello-Manga, Kathleen Neville, Fenella J Kirkham, Yu Shyr, Sharon Phillips, Brittany V Covert, Adetola A Kassim, Lori C Jordan, Muktar H Aliyu, Michael R DeBaun
No abstract text is available yet for this article.
March 2018: American Journal of Hematology
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