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https://www.readbyqxmd.com/read/29020197/sickle-cell-trait-and-heat-injury-among-u-s-army-soldiers
#1
D Alan Nelson, Patricia A Deuster, Francis G O'Connor, Lianne M Kurina
There is concern that sickle cell trait increases risk of exertional collapse, a primary cause of which is heat injury. However, no population-based studies among active individuals have addressed this, representing a critical evidence gap. We conducted a retrospective cohort study of sickle cell trait-tested African American soldiers on active-duty in the U.S. Army anytime between January 2011 and December 2014. Using Cox proportional hazards models and adjusting for demographic and medical factors, we observed no significant associations between sickle cell trait and either mild heat injury (hazard ratio (HR), 1...
August 17, 2017: American Journal of Epidemiology
https://www.readbyqxmd.com/read/28992220/blood-pressure-and-arterial-stiffness-in-kenyan-adolescents-with-the-sickle-cell-trait
#2
Anthony O Etyang, Christopher K Wandabwa, Sailoki Kapesa, Esther Muthumbi, Emily Odipo, Marylene Wamukoya, Nicholas Ngomi, Tilahun Haregu, Catherine Kyobutungi, Thomas N Williams, Johnstone Makale, Alex Macharia, J Kennedy Cruickshank, Liam Smeeth, J Anthony G Scott
The potential association between sickle cell trait (SCT) and increased arterial stiffness/blood pressure (BP) has not been evaluated in detail despite its association with stroke, sudden death and renal disease. We performed 24-hour ambulatory BP monitoring and arterial stiffness measurements in adolescents raised in a malaria free environment in Kenya.Between December 2015 and June 2016, 938 randomly selected adolescents that had been continuous residents of Nairobi from birth were invited to participate in the study...
June 12, 2017: American Journal of Epidemiology
https://www.readbyqxmd.com/read/28981541/contrasting-resting-state-fmri-abnormalities-from-sickle-and-non-sickle-anemia
#3
Julie Coloigner, Yeun Kim, Adam Bush, Soyoung Choi, Melissa C Balderrama, Thomas D Coates, Sharon H O'Neil, Natasha Lepore, John C Wood
Sickle cell disease (SCD) is a chronic blood disorder that is often associated with acute and chronic cerebrovascular complications, including strokes and impaired cognition. Using functional resting state magnetic resonance images, we performed whole-brain analysis of the amplitude of low frequency fluctuations (ALFF), to detect areas of spontaneous blood oxygenation level dependent signal across brain regions. We compared the ALFF of 20 SCD patients to that observed in 19 healthy, age and ethnicity-matched, control subjects...
2017: PloS One
https://www.readbyqxmd.com/read/28951881/state-level-implementation-of-health-and-safety-policies-to-prevent-sudden-death-and-catastrophic-injuries-within-secondary-school-athletics
#4
William M Adams, Samantha E Scarneo, Douglas J Casa
BACKGROUND: Sudden death and catastrophic injuries during sport can be attenuated with the implementation of evidence-based health and safety policies. However, the extent of the implementation of these policies within secondary school athletics is unknown. PURPOSE: To provide an assessment of the implementation of health and safety policies pertaining to the leading causes of sudden death and catastrophic injuries in sport within secondary school athletics in the United States...
September 2017: Orthopaedic Journal of Sports Medicine
https://www.readbyqxmd.com/read/28948451/a-diagnostic-approach-to-stroke-in-young-adults
#5
REVIEW
Christopher A Stack, John W Cole
Optimal diagnosis and management of stroke in young adults benefit from a multidisciplinary team, including a vascular neurology specialist. In addition to the "standard" vascular risk factors including smoking, hypertension, diabetes, and hyperlipidemia, one needs to consider alternative etiologies including substance abuse, carotid/vertebral artery dissections, and rare genetic conditions among others. Once a young patient is determined to have had a stroke, the next question a clinician should ask is why did this patient have a stroke? A "heart to head" diagnostic approach is recommended...
September 25, 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28931524/degree-of-anemia-indirect-markers-of-hemolysis-and-vascular-complications-of-sickle-cell-disease-in-africa
#6
Marie Dubert, Jacques Elion, Aissata Tolo, Dapa Aly Diallo, Saliou Diop, Ibrahima Diagne, Ibrahima Sanogo, Suzanne Belinga, Odette Guifo, Guillaume Wamba, Françoise Ngo Sack, Kouakou Boidy, Ismael Kamara, Youssouf Traore, Cheick Oumar Diakite, Valérie Gbonon, Blaise Felix Faye, Moussa Seck, Indou Deme Ly, David Chelo, Roland N'Guetta, Ibrahima Bara Diop, Bamba Gaye, Xavier Jouven, Brigitte Ranque
The hyperhemolysis paradigm that describes overlapping "hyperhemolytic-endothelial dysfunction" and "high hemoglobin-hyperviscous" subphenotypes of sickle cell disease (SCD) patients is based on North American studies. We performed a transversal study nested in the CADRE cohort to analyze the association between steady-state hemolysis and vascular complications of SCD among sub-Saharan African patients. In Mali, Cameroon, and Ivory Coast, 2,407 SCD patients (1,751 SS-Sβ(0), 495 SC and 161 Sβ(+)), aged three years old and over, were included at steady-state...
September 20, 2017: Blood
https://www.readbyqxmd.com/read/28925802/higher-prevalence-of-spontaneous-cerebral-vasculopathy-and-cerebral-infarcts-in-a-mouse-model-of-sickle-cell-disease
#7
Hyacinth I Hyacinth, Courtney L Sugihara, Thomas L Spencer, David R Archer, Andy Y Shih
Stroke is a dramatic complication of sickle cell disease (SCD), which is associated with cerebral vasculopathies including moya moya, intravascular thrombi, cerebral hyperemia, and increased vessel tortuosity. The spontaneous occurrence of these pathologies in the sickle cell mouse model has not been described. Here, we studied Townes humanized sickle cell and age-matched control mice that were 13 months old. We used in vivo two-photon microscopy to assess blood flow dynamics, vascular topology, and evidence of cerebral vasculopathy...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28912365/d-dimer-in-african-americans-whole-genome-sequence-analysis-and-relationship-to-cardiovascular-disease-risk-in-the-jackson-heart-study
#8
Laura M Raffield, Neil A Zakai, Qing Duan, Cecelia Laurie, Joshua D Smith, Marguerite R Irvin, Margaret F Doyle, Rakhi P Naik, Ci Song, Ani W Manichaikul, Yongmei Liu, Peter Durda, Jerome I Rotter, Nancy S Jenny, Stephen S Rich, James G Wilson, Andrew D Johnson, Adolfo Correa, Yun Li, Deborah A Nickerson, Kenneth Rice, Ethan M Lange, Mary Cushman, Leslie A Lange, Alex P Reiner
OBJECTIVE: Plasma levels of the fibrinogen degradation product D-dimer are higher among African Americans (AAs) compared with those of European ancestry and higher among women compared with men. Among AAs, little is known of the genetic architecture of D-dimer or the relationship of D-dimer to incident cardiovascular disease. APPROACH AND RESULTS: We measured baseline D-dimer in 4163 AAs aged 21 to 93 years from the prospective JHS (Jackson Heart Study) cohort and assessed association with incident cardiovascular disease events...
September 14, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28894559/pattern-of-cerebral-blood-flow-velocity-using-transcranial-doppler-ultrasonography-in-children-with-sickle-cell-disorder-in-lagos-state-nigeria
#9
Motunrayo Oluwabukola Adekunle, Adeola Barakat Animasahun, Ijeoma Nnenna Diaku-Akinwumi, Olisamedua Fidelis Njokanma
Cerebrovascular accident (CVA) is a common, devastating neurological complication of sickle cell disorder (SCD) with a high recurrent and mortality rate. The Stroke Prevention Trial in Sickle Cell Anaemia study (STOP) recommends routine screening with transcranial Doppler ultrasonography in children aged two to sixteen years with SCD. The present study assessed cerebral blood flow velocities of children with SCD in accordance with the recommendation of routine screening by the STOP study. METHODS: Transcranial Doppler ultrasonography was done for children with SCD that attended Sickle Cell Foundation, Nigeria between July and November 2015...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28882446/relationship-between-mixed-donor-recipient-chimerism-and-disease-recurrence-after-hematopoietic-cell-transplantation-for-sickle-cell-disease
#10
Allistair Abraham, Matthew Hsieh, Mary Eapen, Courtney Fitzhugh, Jeanette Carreras, Daniel Keesler, Gregory Guilcher, Naynesh Kamani, Mark C Walters, Jaap J Boelens, John Tisdale, Shalini Shenoy
Mixed donor chimerism after 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 and at 1 and 2 years after transplantation. Recurrent disease was defined as recurrence of vaso-occlusive crises, acute chest syndrome, stroke, and/or HbS levels > 50%...
September 4, 2017: Biology of Blood and Marrow Transplantation
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
#11
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/28864674/neurologic-complications-of-sickle-cell-disease-in-africa-a-systematic-review-and-meta-analysis
#12
REVIEW
Jean Jacques Noubiap, Michel K Mengnjo, Nicolas Nicastro, Joseph Kamtchum-Tatuene
OBJECTIVE: To summarize prevalence data on the neurologic complications of sickle cell disease (SCD) in Africa. METHODS: We searched EMBASE, PubMed, and African Index Medicus to identify all relevant articles published from inception to May 31, 2016. Each study was reviewed for methodologic quality. A random-effects model was used to estimate the prevalence of neurologic complications of SCD across studies. RESULTS: Thirty-one studies were included...
October 3, 2017: Neurology
https://www.readbyqxmd.com/read/28821470/design-of-the-drepagreffe-trial-a-prospective-controlled-multicenter-study-evaluating-the-benefit-of-genoidentical-hematopoietic-stem-cell-transplantation-over-chronic-transfusion-in-sickle-cell-anemia-children-detected-to-be-at-risk-of-stroke-by-transcranial
#13
Sylvie Chevret, Suzanne Verlhac, Elisabeth Ducros-Miralles, Jean-Hugues Dalle, Regis Peffault de Latour, Mariane de Montalembert, Malika Benkerrou, Corinne Pondarré, Isabelle Thuret, Corinne Guitton, Emmanuelle Lesprit, Maryse Etienne-Julan, Gisèle Elana, Jean-Pierre Vannier, Patrick Lutz, Bénédicte Neven, Claire Galambrun, Catherine Paillard, Camille Runel, Charlotte Jubert, Cécile Arnaud, Annie Kamdem, Valentine Brousse, Florence Missud, Marie Petras, Lydia Doumdo-Divialle, Claire Berger, Françoise Fréard, Olivier Taieb, Elise Drain, Monique Elmaleh, Manuela Vasile, Yacine Khelif, Myriam Bernaudin, Philippe Chadebech, France Pirenne, Gérard Socié, Françoise Bernaudin
BACKGROUND: Children with sickle cell anemia (SCA) have an 11% risk of stroke by the age of 18. Chronic transfusion applied in patients detected to be at risk by transcranial Doppler allows a significant reduction of stroke risk. However, chronic transfusion exposes to several adverse events, including alloimmunization and iron overload, and is not curative. Hematopoietic stem cell transplantation allows termination of the transfusion program, but its benefit has not been demonstrated...
November 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#14
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/28771666/how-i-manage-sickle-cell-patients-with-high-transcranial-doppler-results
#15
REVIEW
John Brewin, Banu Kaya, Subarna Chakravorty
Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non-invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. This has led to the regular use of TCD monitoring for children with SCA in order to determine stroke risk. Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non-attenders...
August 2, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28764173/clinical-and-laboratory-predictors-of-frequency-of-painful-crises-among-sickle-cell-anaemia-patients-in-nigeria
#16
Angela Ogechukwu Ugwu, Obike Godswill Ibegbulam, Theresa Ukamaka Nwagha, Anazoeze Jude Madu, Sunday Ocheni, Iheanyi Okpala
INTRODUCTION: The severity of Sickle Cell Anaemia (SCA) in terms of frequency of painful Vaso-Occlusive Crises (VOC) may be affected by clinical and haematological parameters amongst others. Elucidation of these factors in a given disease prevalent environment is necessary for prompt and effective management of patients with frequent painful VOC. AIM: This study aimed at determining the clinical and laboratory predictors of frequency of painful VOC among SCA patients in Enugu, Southeastern Nigeria...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28756377/thrombolytic-therapy-for-the-treatment-of-acute-ischaemic-stroke-in-adults-with-homozygous-sickle-cell-disease
#17
Loubna Majhadi, David Calvet, Charlotte Rosso, Pablo Bartolucci
Stroke is a significant cause of morbidity and mortality in patients with homozygous sickle cell disease (SCD). A specific large-vessel vasculopathy is often responsible for both haemorrhagic and ischaemic strokes in patients with SCD. Although intravenous thrombolysis has been considered as a therapeutic option for acute ischaemic strokes in SCD, its use remains debated because of an increased risk of spontaneous intracranial haemorrhage reported in this disease. This risk of haemorrhage is mainly supported by the presence of a Moyamoya syndrome often associated with the specific vasculopathy in patients with homozygous SCD...
July 28, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28747064/graph-lasso-based-test-for-evaluating-functional-brain-connectivity-in-sickle-cell-disease
#18
Julie Coloigner, Ronald Phlypo, Thomas D Coates, Natasha Lepore, John C Wood
Sickle cell disease (SCD) is a vascular disorder that is often associated with recurrent ischemia-reperfusion injury, anemia, vasculopathy, and strokes. These cerebral injuries are associated with neurological dysfunction, limiting the full developing potential of the patient. However, recent large studies of SCD have demonstrated that cognitive impairment occurs even in the absence of brain abnormalities on conventional magnetic resonance imaging (MRI). These observations support an emerging consensus that brain injury in SCD is diffuse and that conventional neuroimaging often underestimates the extent of injury...
September 2017: Brain Connectivity
https://www.readbyqxmd.com/read/28745855/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients-digest
#19
Sathyaseelan Subramaniam, Jennifer H Chao, Pradip Chaudhari
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 22, 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/28742875/the-significance-of-inadequate-transcranial-doppler-studies-in-children-with-sickle-cell-disease
#20
Simon Greenwood, Colin Deane, Oliver L Rees, Ben Freedman, Suresh Kumar, Naser Ben Ramadan, Sarah Wilkinson, Grant Marais, Julie Lord, Subarna Chakravorty, Susan E Height, Kate Gardner, David C Rees
Sickle cell disease (SCD) is a common cause of cerebrovascular disease in childhood. Primary stroke prevention is effective using transcranial Doppler (TCD) scans to measure intracranial blood velocities, and regular blood transfusions or hydroxycarbamide when these are abnormal. Inadequate TCD scans occur when it is not possible to measure velocities in all the main arteries. We have investigated the prevalence and significance of this in a retrospective audit of 3915 TCD scans in 1191 children, performed between 2008 and 2015...
2017: PloS One
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