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Sickle cell cerebral vasculopathy

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https://www.readbyqxmd.com/read/28837721/visual-function-in-asymptomatic-patients-with-homozygous-sickle-cell-disease-and-temporal-macular-atrophy
#1
Gilles C Martin, Charlotte Dénier, Olivia Zambrowski, David Grévent, Lenaïc Bruère, Valentine Brousse, Mariane de Montalembert, Dominique Brémond-Gignac, Matthieu P Robert
Importance: Temporal macular involvement in sickle cell disease can now easily be detected by optical coherence tomography (OCT). However, while recent studies have demonstrated its high prevalence, little is known about its potential consequences on visual function. Objective: To assess the visual function of patients with sickle cell disease with no visual symptoms despite temporal macular atrophy. Design, Setting, and Participants: This retrospective case series included data collection and explorations made in a single referral center for sickle cell disease in 2016...
August 24, 2017: JAMA Ophthalmology
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
#2
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...
August 15, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28747064/graph-lasso-based-test-for-evaluating-functional-brain-connectivity-in-sickle-cell-disease
#3
Julie Coloigner, Ronald Phlypo, Thomas D Coates, Nastasha 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...
July 26, 2017: Brain Connectivity
https://www.readbyqxmd.com/read/28670947/genetic-background-of-the-sickle-cell-disease-pediatric-population-of-dakar-senegal-and-characterization-of-a-novel-frameshift-%C3%AE-thalassemia-mutation-hbb-c-265_266del-p-leu89glufs-2
#4
Fatou Gueye Tall, Cyril Martin, El Hadji Malick Ndour, Indou Déme Ly, Céline Renoux, Louis Chillotti, Nicolas Veyrenche, Philippe Connes, Papa Madieye Gueye, Rokhaya Ndiaye Diallo, Philippe Lacan, Ibrahima Diagne, Pape Amadou Diop, Aynina Cissé, Philomène Lopez Sall, Philippe Joly
Sickle cell disease is a genetic disorder with a large variability in the pattern and severity of clinical manifestations. Different genetic modulators have been identified but very few epidemiologic data are available on these modifier genes in Senegal. This study aimed to determine their prevalence in a Senegalese sickle cell disease pediatric population. The following genetic parameters were genotyped in 295 sickle cell disease children of the Dakar pediatric hospital: sickle cell disease genotype [β(S)/β(S) (HBB: c...
March 2017: Hemoglobin
https://www.readbyqxmd.com/read/28579187/functional-mri-bold-response-in-sickle-mice-with-hyperalgesia
#5
Ying Wang, Xiao Wang, Wei Chen, Kalpna Gupta, Xiao-Hong Zhu
Patients with sickle cell anemia (SCA) have abnormal hemoglobin (sickle hemoglobin S) leading to the crystallization of hemoglobin chains in red blood cells (RBCs), which assume sickle shape and display reduced flexibility. Sickle RBCs (sRBCs) adhere to vessel walls and block blood flow, thus preventing oxygen delivery to the tissues leading to vaso-occlusive crises (VOC), acute pain and organ damage. SCA patients often have chronic pain that can be attributed to inflammation, vasculopathy, neuropathy, ischemia-reperfusion injury and organ damage...
June 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28362411/continuous-manual-exchange-transfusion-for-patients-with-sickle-cell-disease-an-efficient-method-to-avoid-iron-overload
#6
Bérengère Koehl, Florence Missud, Laurent Holvoet, Ghislaine Ithier, Oliver Sakalian-Black, Zinedine Haouari, Emmanuelle Lesprit, André Baruchel, Malika Benkerrou
Children with sickle cell anemia (SCA) may be at risk of cerebral vasculopathy and strokes, which can be prevented by chronic transfusion programs. Repeated transfusions of packed red blood cells (PRBCs) is currently the simplest and most used technique for chronic transfusion programs. However, iron overload is one of the major side effects of this therapy. More developed methods exist, notably the apheresis of RBC (erythrapheresis), which is currently the safest and most efficient method. However, it is costly, complicated, and cannot be implemented everywhere, nor is it suitable for all patients...
March 14, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28293402/treosulfan-based-conditioning-regimen-in-sibling-and-alternative-donor-hematopoietic-stem-cell-transplantation-for-children-with-sickle-cell-disease
#7
Antonio Marzollo, Elisabetta Calore, Manuela Tumino, Marta Pillon, Maria Vittoria Gazzola, Roberta Destro, Raffaella Colombatti, Piero Marson, Tiziana Tison, Anna Colpo, Chiara Mainardi, Maria Gabelli, Maria Paola Boaro, Sara Rossin, Aurora Strano, Nadia Quaglia, Federica Menzato, Giuseppe Basso, Laura Sainati, Chiara Messina
BACKGROUND AND OBJECTIVES: Lack of suitable donors and regimen related toxicity are major barriers for hematopoietic stem cell transplantation (HSCT) in patients with sickle cell disease (SCD). The aim of the study is the assessment of efficacy and toxicity of Treosulfan-based conditioning regimen for SCD also when alternative donors such as mismatched unrelated donor and haploidentical donor are employed. METHODS: We report our single-center experience: 11 patients with SCD received HSCT with a Treosulfan/Thiotepa/Fludarabine/Anti-thymoglobulin conditioning regimen between 2010 and 2015...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28270343/comprehensive-neuropsychological-evaluation-of-children-and-adolescents-with-sickle-cell-anemia-a-hospital-based-sample
#8
Samantha Nunes, Nayara Argollo, Marivania Mota, Camilo Vieira, Eduardo Pondé de Sena
BACKGROUND: Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia. METHODS: The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28159432/large-vessel-vasculopathy-in-children-with-sickle-cell-disease-a-magnetic-resonance-imaging-study-of-infarct-topography-and-focal-atrophy
#9
Kristin P Guilliams, Melanie E Fields, Dustin K Ragan, Yasheng Chen, Cihat Eldeniz, Monica L Hulbert, Michael M Binkley, James N Rhodes, Joshua S Shimony, Robert C McKinstry, Katie D Vo, Hongyu An, Jin-Moo Lee, Andria L Ford
BACKGROUND: Large-vessel vasculopathy (LVV) increases stroke risk in pediatric sickle cell disease beyond the baseline elevated stroke risk in this vulnerable population. The mechanisms underlying this added risk and its unique impact on the developing brain are not established. METHODS: We analyzed magnetic resonance imaging and angiography scans of 66 children with sickle cell disease and infarcts by infarct density heatmaps and Jacobian determinants, a metric utilized to delineate focal volume change, to investigate if infarct location, volume, frequency, and cerebral atrophy differed among hemispheres with and without LVV...
April 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/27814292/hemorheological-alterations-in-sickle-cell-anemia-and-their-clinical-consequences-the-role-of-genetic-modulators
#10
Marisa Silva, Sofia Vargas, Andreia Coelho, Alexandra Dias, Teresa Ferreira, Anabela Morais, Raquel Maia, Paula Kjöllerström, João Lavinha, Paula Faustino
Sickle cell anemia (SCA) is an autosomal recessive disease caused by the HBB:c.20A>T mutation that leads to hemoglobin S synthesis. The disease presents with high clinical heterogeneity characterized by chronic hemolysis, recurrent episodes of vaso-oclusion and infection. This work aimed to characterize by in silico studies some genetic modulators of severe hemolysis and stroke risk in children with SCA, and understand their consequences at the hemorheological level.Association studies were performed between hemolysis biomarkers as well as the degree of cerebral vasculopathy and the inheritance of several polymorphic regions in genes related with vascular cell adhesion and vascular tonus in pediatric SCA patients...
2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27619954/expanding-treatment-for-existing-neurological-disease-extend-an-open-label-phase-ii-clinical-trial-of-hydroxyurea-treatment-in-sickle-cell-anemia
#11
Angela E Rankine-Mullings, Courtney R Little, Marvin E Reid, Deanne P Soares, Carolyn Taylor-Bryan, Jennifer M Knight-Madden, Susan E Stuber, Asha V Badaloo, Karen Aldred, Margaret E Wisdom-Phipps, Teresa Latham, Russell E Ware
BACKGROUND: Cerebral vasculopathy in sickle cell anemia (SCA) begins in childhood and features intracranial arterial stenosis with high risk of ischemic stroke. Stroke risk can be reduced by transcranial doppler (TCD) screening and chronic transfusion therapy; however, this approach is impractical in many developing countries. Accumulating evidence supports the use of hydroxyurea for the prevention and treatment of cerebrovascular disease in children with SCA. Recently we reported that hydroxyurea significantly reduced the conversion from conditional TCD velocities to abnormal velocities; whether hydroxyurea can be used for children with newly diagnosed severe cerebrovascular disease in place of starting transfusion therapy remains unknown...
September 12, 2016: JMIR Research Protocols
https://www.readbyqxmd.com/read/27321952/unusual-association-between-lysinuric-protein-intolerance-and-moyamoya-vasculopathy
#12
Valérie Ghilain, Elsa Wiame, Edward Fomekong, Marie-Françoise Vincent, Dana Dumitriu, Marie-Cécile Nassogne
INTRODUCTION: Lysinuric protein intolerance (LPI) is a form of inherited aminoaciduria caused by a deficiency in the cationic amino acid transport process on the basolateral membrane of enterocytes and renal tubular cells. Clinical signs include gastrointestinal symptoms, failure to thrive, hepatosplenomegaly, osteoporosis, episodes of coma, intellectual deficiency, lung and renal involvement, bone marrow abnormalities, as well as altered immune response. Moyamoya disease is a cerebrovascular disorder predisposing sufferers to stroke through progressive stenosis of the intracranial internal carotid arteries and their proximal branches...
September 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/27106860/reduction-in-overt-and-silent-stroke-recurrence-rate-following-cerebral-revascularization-surgery-in-children-with-sickle-cell-disease-and-severe-cerebral-vasculopathy
#13
Erin M Hall, Jeffrey Leonard, Jodi L Smith, Kristin P Guilliams, Michael Binkley, Robert J Fallon, Monica L Hulbert
BACKGROUND: Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. METHODS: This was a retrospective cohort study of all children with SCD and moyamoya treated at two children's hospitals. Clinical events and imaging studies were reviewed...
August 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/26823369/non-invasive-imaging-of-oxygen-extraction-fraction-in-adults-with-sickle-cell-anaemia
#14
Lori C Jordan, Melissa C Gindville, Allison O Scott, Meher R Juttukonda, Megan K Strother, Adetola A Kassim, Sheau-Chiann Chen, Hanzhang Lu, Sumit Pruthi, Yu Shyr, Manus J Donahue
Sickle cell anaemia is a monogenetic disorder with a high incidence of stroke. While stroke screening procedures exist for children with sickle cell anaemia, no accepted screening procedures exist for assessing stroke risk in adults. The purpose of this study is to use novel magnetic resonance imaging methods to evaluate physiological relationships between oxygen extraction fraction, cerebral blood flow, and clinical markers of cerebrovascular impairment in adults with sickle cell anaemia. The specific goal is to determine to what extent elevated oxygen extraction fraction may be uniquely present in patients with higher levels of clinical impairment and therefore may represent a candidate biomarker of stroke risk...
March 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/26130796/evaluation-of-intracranial-cerebral-blood-flow-velocities-in-splenectomised-and-non-splenectomised-patients-with-%C3%AE-thalassemia-intermedia-using-transcranial-doppler-sonography
#15
Aikaterini Kanavaki, Antonis Kattamis, Polyxeni Delaporta, Ioannis Papassotiriou, Konstantinos Spengos
BACKGROUND: A high incidence of clinically-silent cerebral ischemic events has been reported in splenectomised patients with β-thalassemia intermedia (βTI). These could be due to cerebral large-vessel disease. Based on the example of sickle cell disease, we applied transcranial Doppler sonography (TCD) to evaluate cerebral vessels velocity as a possible indicator of cerebral vasculopathy. PATIENTS AND METHODS: In our study, we included 17 splenectomised and 13 non-splenectomised (control group) patients with βTI...
July 2015: In Vivo
https://www.readbyqxmd.com/read/26072930/g6pd-deficiency-and-absence-of-%C3%AE-thalassemia-increase-the-risk-for-cerebral-vasculopathy-in-children-with-sickle-cell-anemia
#16
Philippe Joly, Nathalie Garnier, Kamila Kebaili, Céline Renoux, Arthur Dony, Nathalie Cheikh, Cécile Renard, Antony Ceraulo, Daniela Cuzzubbo, Corinne Pondarré, Cyril Martin, Vincent Pialoux, Alain Francina, Yves Bertrand, Philippe Connes
The aim of this study was to test the association between hematological/genetic factors and cerebral vasculopathy in children with sickle cell anemia (SCA). A group with cerebral vasculopathy (VASC) was composed of children who had stroke (n = 6), silent infarct (n = 11), or an abnormal transcranial Doppler (n = 5). Eighty-four patients had neither positive history of stroke or silent infarct, nor abnormal transcranial Doppler (NORM group). An intermediate group (COND; n = 15) was composed of SCA children with a conditional transcranial Doppler...
April 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/25944412/how-i-manage-cerebral-vasculopathy-in-children-with-sickle-cell-disease
#17
REVIEW
Valentine Brousse, Manoelle Kossorotoff, Mariane de Montalembert
Sickle cell disease induces specific brain alterations that involve both the macrocirculation and the microcirculation. The main overt neurovascular complications in children are infarctive stroke, transient ischaemic attack and cerebral haemorrhage. Silent cerebral infarction, cognitive dysfunction and recurrent headache are also common. Cerebrovascular disease selectively affects children with the HbSS or HbS-β(0) genotypes (i.e. sickle cell anaemia). The incidence of stroke peaks between 2 and 5 years of age (1·02/100 patient-years) and increases with the severity of the anaemia...
September 2015: British Journal of Haematology
https://www.readbyqxmd.com/read/25729487/transcranial-doppler-screening-in-sickle-cell-disease-the-implications-of-using-peak-systolic-criteria
#18
Lena N Naffaa, Yasmeen K Tandon, Neville Irani
AIM: To compare time average maximum mean velocity (TAMV) and peak systolic velocity (PSV) criteria of Trans Cranial Doppler (TCD) in their ability to predict abnormalities on magnetic resonance imaging (MRI)/magnetic resonance angiogram (MRA) in patients with sickle cell disease. METHODS: A retrospective evaluation was performed of the outcomes in all patients with a Transcranial Doppler examination at our institution since the implementation of the hospital picture archiving and communication system (PACS) system in January 2003 through December 2012...
February 28, 2015: World Journal of Radiology
https://www.readbyqxmd.com/read/25636211/-screening-of-cerebral-vasculopathy-in-sickle-cell-anemia-children-using-transcranial-doppler
#19
A Dorie, A Guindo, Y S Saro, B A Touré, B Fané, A K Dembelé, D A Diallo
Cerebral vasculopathy exposes patients to a high risk of stroke, a major complication of sickle cell disease (SCD) associated with a high risk of death and disability. Transcranial doppler (TCD) ultrasonography used to identify SCD patients at risk of stroke may contribute to significantly reducing morbidity and mortality in these patients by indicating appropriate treatment. From March 2008 to February 2013, we conducted systematic screening for cerebral vasculopathy using TCD in 572 SCD patients (including 375 SS, 144 SC, 26 S/β(0), and 27 S/β(+) thalassemia patients) aged 1-17 years in a comprehensive center for follow-up and research on sickle cell disease in Bamako, Mali...
March 2015: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/25510140/-chronic-complications-of-sickle-cell-disease
#20
Pablo Bartolucci, François Lionnet
Chronic complications of sickle cell disease take an increasing role in the management of patients due to their morbidity and mortality impact. The prevalence of chronic organ damages increases as the age of patients followed in France. Few organs seem unaffected by the disease. The natural history of chronic complication is highly variable from one patient to another, and the distribution of those manifestations throughout life, is different depending on their nature and pathophysiology. Thus we can, for example, distinguish SS patients presenting a "hyperhemolytic" phenotype associated with dense red blood cells that have a high risk of vascular complications including kidney disease, pulmonary hypertension, leg ulcers and priapism, from SC patients with high hemoglobin levels, who have a higher risk of retinopathy, osteonecrosis and sensory syndrome, probably related to hyperviscosity...
October 2014: La Revue du Praticien
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