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Condition: Sickle Cell Anemia

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Total 1318 results found since Jan 2013.

Association Between Patent Foramen Ovale and Overt Ischemic Stroke in Children With Sickle Cell Disease
This study investigates the association between PFO and overt ischemic stroke in the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study cohort of 5,247 children with SCA of whom 1,414 had at least one clinical non-contrast transthoracic echocardiogram. Presence of PFO was taken from the clinical report. Further, we assessed the association between PFO and other clinical and hemolytic factors in children with SCA such as history of abnormal sickle stroke screen [elevated Transcranial Doppler ultrasound (TCD) velocity] and patient's baseline hemoglobin. In 642 children for ...
Source: Frontiers in Neurology - December 13, 2021 Category: Neurology Source Type: research

Genetic mapping and exome sequencing identify 2 mutations associated with stroke protection in pediatric patients with sickle cell anemia
Stroke is a devastating complication of sickle cell anemia (SCA), occurring in 11% of patients before age 20 years. Previous studies of sibling pairs have demonstrated a genetic component to the development of cerebrovascular disease in SCA, but few candidate genetic modifiers have been validated as having a substantial effect on stroke risk. We performed an unbiased whole-genome search for genetic modifiers of stroke risk in SCA. Genome-wide association studies were performed using genotype data from single-nucleotide polymorphism arrays, whereas a pooled DNA approach was used to perform whole-exome sequencing. In combina...
Source: Blood - April 18, 2013 Category: Hematology Authors: Flanagan, J. M., Sheehan, V., Linder, H., Howard, T. A., Wang, Y.-D., Hoppe, C. C., Aygun, B., Adams, R. J., Neale, G. A., Ware, R. E. Tags: Pediatric Hematology, Red Cells, Iron, and Erythropoiesis Source Type: research

Stroke in sickle cell anemia patients: A need for multidisciplinary approaches
Abstract: Sickle cell anemia (SCA) is an autosomal recessive disorder, with Mendelian inheritance pattern, caused by a missense mutation in the β-polypeptide chain of the hemoglobin B. SCA preferentially affects populations in countries where malaria was/is present (e.g. Africa, USA, Brazil). Thereby, in USA, the incidence of SCA is relatively high, around 1/500, and the prevalence is about 1/1000. In Brazil, SCA represents a major public health problem with an incidence ranging from 1/2000 to 1/600 depending on the regions. Homozygotic patients present more severe medical conditions and reduced life expectancy than heter...
Source: Atherosclerosis - June 10, 2013 Category: Cardiology Authors: Farid Menaa Tags: Reviews Source Type: research

Transcranial Doppler ultrasound in children with stroke and cerebrovascular disorders
This article summarizes the basic physics and variables used during TCD, recent pediatric data published on the use of TCD in stroke and cerebrovascular disorders and how it may impact diagnosis and management, and some issues to be resolved so that TCD can be put into clinical practice. Recent findings: In sickle cell disease in children, TCD is the gold standard stroke prediction tool. Recent data suggest that TCD may provide important information in ischemic stroke because of other childhood arteriopathies such as moyamoya syndrome, transient or focal cerebral arteriopathy, and genetic/syndromic causes. TCD may also det...
Source: Current Opinion in Pediatrics - November 10, 2015 Category: Pediatrics Tags: NEUROLOGY: Edited by Robert C. Tasker Source Type: research

Glucose‐6‐Phosphate Dehydrogenase Deficiency in Brazilian Children With Sickle Cell Anemia is not Associated With Clinical Ischemic Stroke or High‐Risk Transcranial Doppler
ConclusionsOur study demonstrated that G6PD molecular deficiency was not associated either with clinical ischemic stroke or high‐risk TCD. Similarly, we found no associations between G6PD enzyme activity and stroke or high‐risk TCD. Small sample size precludes definitive conclusions.
Source: Pediatric Blood and Cancer - February 3, 2016 Category: Cancer & Oncology Authors: André Rolim Belisário, Rahyssa Rodrigues Sales, Nayara Evelin Toledo, Cibele Velloso‐Rodrigues, Célia Maria Silva, Marcos Borato Viana Tags: Research Article Source Type: research

The Role of Hydroxyurea to Prevent Silent Stroke in Sickle Cell Disease: Systematic Review and Meta-Analysis
Chronic blood transfusions are standard of care for stroke prevention in sickle cell disease. We evaluated hydroxyurea's efficacy in preventing silent stroke. We searched for randomized controlled trials (RCTs) and observational studies on Pubmed, CENTRAL, Embase, and Web of Science without using language/time limits. Eligible studies compared hydroxyurea with transfusions or observation to prevent silent stroke. This systematic review adheres to the Cochrane guidelines. Data were pooled using random effects model using STATA to perform meta-analysis. Methodological quality of RCTs was investigated using the Cochrane risk ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Hasson, C., Veling, L., Rico, J. F., Mhaskar, R. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

The association between obstructive sleep apnea and stroke in sickle-cell disease children
ConclusionPatients who had OSA had a higher prevalence of stroke compared to non-OSA patients by 16% with almost three times higher odds. The difference was statistically significant in bivariant but not multivariant analysis. The rate of hospitalization, emergency visit, and blood transfusion were not affected by OSA status. Screening for OSA in high-risk patients such as SCD children and early management could prevent the risk of SCD complications.
Source: European Archives of Oto-Rhino-Laryngology - January 29, 2022 Category: ENT & OMF Source Type: research

Revascularization Is Associated With a Reduced Stroke Risk in Patients With Sickle Cell–Associated Moyamoya Syndrome
CONCLUSION: The results of this study demonstrate that revascularization is associated with a significant reduction in stroke risk, both relative to prerevascularization rates and compared with medical management. According to these findings, surgical revascularization offers a safe and durable preventative therapy for stroke and should be pursued aggressively in this patient population.
Source: Neurosurgery - March 24, 2022 Category: Neurosurgery Tags: Research—Human—Clinical Studies: Cerebrovascular Source Type: research

Transient Ischemic Attack Requiring Hospitalization of Children in the United States: Kids' Inpatient Database 2003 to 2009 Brief Reports
Conclusions— Recognized risk factors for TIA, including sickle cell disease, congenital heart disease, moyamoya, recent stroke, and migraine, were present in <60% of children. Pediatric admissions for ischemic stroke were 5-fold more common than for TIA. Further study is required to understand the risk of stroke after TIA in children to guide appropriate evaluation and treatment.
Source: Stroke - February 24, 2014 Category: Neurology Authors: Adil, M. M., Qureshi, A. I., Beslow, L. A., Jordan, L. C. Tags: Transient Ischemic Attacks Brief Reports Source Type: research

High mortality among children with sickle cell anemia and overt stroke who discontinue blood transfusion after transition to an adult program.
CONCLUSION: Efforts must be made to maintain adequate chronic simple or exchange blood transfusion for children with SS and stroke after transition to adult care. PMID: 26593779 [PubMed - as supplied by publisher]
Source: Transfusion - November 23, 2015 Category: Hematology Authors: McLaughlin JF, Ballas SK Tags: Transfusion Source Type: research

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research