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Condition: Anemia
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New | phs002386.v1.p1 | Optimizing Primary Stroke Prevention in Children with Sickle Cell Anemia (STOP II)
Optimizing Primary Stroke Prevention in Children with Sickle Cell Anemia (STOP II)  (study page |release notes)
Source: dbGaP, the database of Genotypes and Phenotypes - March 26, 2021 Category: Genetics & Stem Cells Tags: StudyRelease Source Type: research

Impact of anemia on acute ischemic stroke outcomes: A systematic review of the literature
DiscussionThis data suggests that the contemporary thresholds for treating anemia in AIS patients may be inadequate because anemia is strongly associated with poor outcomes (e.g., mRS>2 or mortality) and increased LOS in AIS patients. The current generalized Hb threshold for transfusion (7 g/dL) is also used in AIS patients, however, a more aggressive transfusion parameter should be further explored based on these findings. Further studies are required to confirm these findings and to determine if a more liberal RBCT threshold will result in clinical benefits.
Source: PLoS One - January 5, 2023 Category: Biomedical Science Authors: Ansh Desai Source Type: research

Polymorphisms and gene expression of metalloproteinases and their inhibitors associated with cerebral ischemic stroke in young patients with sickle cell anemia
CONCLUSION: The findings of this study, the SNPs, and expression provide initial support for understanding the role of MMPs-TIMPs in the pathophysiology of SCA in young patients.PMID:36720795 | DOI:10.1007/s11033-023-08262-2
Source: Molecular Biology Reports - January 31, 2023 Category: Molecular Biology Authors: Ó do Kleyton Palmeira Ana Karla da Silva Freire D ébora Nascimento de Nóbrega Roberta Dos Santos Souza Isabela Cristina Cordeiro Farias Taciana Furtado de Mendon ça Belmont Andreia Soares da Silva Gabriela da Silva Arcanjo Aderson da Silva Araujo Ana Source Type: research

Associations of Anemia With Outcomes in Patients With Spontaneous Intracerebral Hemorrhage: A Meta-Analysis
Conclusions: Anemia on admission was associated with higher mortality and an increased risk of poor outcome in patients with ICH. However, the results were limited by the high heterogeneity of included studies. Prospective, multi-center or population-based, large sample cohort studies are needed in the future. Introduction Intracerebral hemorrhage (ICH) is the second most common cause of stroke and a highly lethal disease (1), which still lacks effective therapeutic interventions (2, 3). Although age, baseline ICH volume and neurological status on admission are well-known predictors of outcome of ICH (4), none of t...
Source: Frontiers in Neurology - April 24, 2019 Category: Neurology Source Type: research

Outcomes in Children with Severe Vasculopathy from Sickle Cell Anemia after Treatment with Chronic Transfusion, Surgical Revascularization and/or Allogenic Hematopoetic Stem Cell Transplantation
Conclusions: The risk for cerebral infarction and/or vasculopathy progression after initiation of treatment with either chronic transfusion, HSCT or EDAS is still a major concern. Our data suggest HSCT and surgical revascularization with chronic transfusion provide the greatest benefit in reducing stroke risk and HSCT reduces risk for progression of a severe vasculopathy. Additional, large population studies are needed to clarify the risk.DisclosuresMajumdar: NIMHD: Research Funding. Campbell: Functional Fluitics: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Johnson, C. W., Majumdar, S., Campbell, A. D., Magge, S., Darbari, D. S., Webb, J., Diab, Y. A., Nickel, R. S., Speller-Brown, B., Carpenter, J., Abraham, A. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research

Association of alpha-thalassemia, TNF-alpha (-308G>A) and VCAM-1 (c.1238G>C) gene polymorphisms with cerebrovascular disease in a newborn cohort of 411 children with sickle cell anemia.
This study found no association between VCAM1 c.1238G>C and stroke. An association between stroke and TNF-α -308A allele has been suggested. Our results have confirmed the protective role of HBA deletion against stroke and CVD. PMID: 25175566 [PubMed - as supplied by publisher]
Source: Blood Cells, Molecules and Diseases - August 27, 2014 Category: Hematology Authors: Belisário AR, Nogueira FL, Rodrigues RS, Toledo NE, Cattabriga AL, Velloso-Rodrigues C, Duarte FO, Silva CM, Viana MB Tags: Blood Cells Mol Dis Source Type: research

Association of alpha-thalassemia, TNF-alpha (-308G>A) and VCAM-1 (c.1238G>C) gene polymorphisms with cerebrovascular disease in a newborn cohort of 411 children with sickle cell anemia
This study found no association between VCAM1 c.1238G>C and stroke. An association between stroke and TNF-α -308A allele has been suggested. Our results have confirmed the protective role of HBA deletion against stroke and CVD.
Source: Blood Cells, Molecules, and Diseases - January 17, 2015 Category: Hematology Source Type: research

SOD3-Mimetic As a Complement for Genetic Therapy in Sickle Cell Disease
Conclusions:The present results support development of SanFlow, delivered through continuous infusion, for anemic SCD children to prevent the development of blood transfusion dependency in order to avoid stroke and painful vaso-occlusive crisis (VOC). These results also demonstrated that SanFlow can be used safely and effectively in the elimination of serious painful vaso-occlusive crisis and protect silent and major strokes. Clinical trials of SanFlow in SCD children, prior to their transfusion dependence, as well as in transfusion-dependent teenagers and adults with SCD patients are warranted. By extension, ß-Thala...
Source: Blood - November 21, 2018 Category: Hematology Authors: Hsia, C. J., Ma, L. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster II Source Type: research

Cerebral vasculopathy in pediatric sickle-cell anemia.
Abstract In children with sickle-cell anemia, cerebral vasculopathy is a frequent and severe complication. It is attributed not only to erythrocyte sickling but also to multiple physiological modifications associated with sickle-cell anemia: platelet and leukocyte activation, endothelial injury and remodeling, coagulation activation, hemolysis and subsequent chronic inflammation, impaired vasomotricity, etc. Intracranial large-vessel remodeling leads to clinical cerebral infarction, whereas microvascular injury and impaired vasoreactivity lead to so-called silent infarcts, which are actually associated with impair...
Source: Archives de Pediatrie - March 12, 2014 Category: Pediatrics Authors: Kossorotoff M, Grevent D, de Montalembert M Tags: Arch Pediatr Source Type: research

Non-invasive imaging of oxygen extraction fraction in adults with sickle cell anaemia
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 le...
Source: Brain - February 25, 2016 Category: Neurology Authors: Jordan, L. C., Gindville, M. C., Scott, A. O., Juttukonda, M. R., Strother, M. K., Kassim, A. A., Chen, S.-C., Lu, H., Pruthi, S., Shyr, Y., Donahue, M. J. Tags: Original Articles Source Type: research

How I manage sickle cell patients with high transcranial doppler results
Summary 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. In a subgroup of p...
Source: British Journal of Haematology - August 2, 2017 Category: Hematology Authors: John Brewin, Banu Kaya, Subarna Chakravorty Tags: Review Source Type: research

Developing a risk-based composite neurologic outcome for a trial of hydroxyurea in young children with sickle cell disease.
CONCLUSION: This set of studies provides the rationale, justification, and validation for the use of a weighted composite outcome and confirms the need for the phase III HU Prevent study. Surveys of investigators in multi-center studies can provide the basis of clinically meaningful outcomes that foster the translation of study results into practice while increasing the efficiency of a study. PMID: 30426764 [PubMed - as supplied by publisher]
Source: Clinical Trials - November 14, 2018 Category: Research Authors: Casella JF, Adams RJ, Brambilla DJ, Strouse JJ, Maier P, Dlugash R, Avadhani R, Vermillion K, Tonascia J, Voeks JH, Hanley DF, Thompson RE, Lehmann HP Tags: Clin Trials Source Type: research

Relationship between Hemolytic Index Early in Life with Development of Abnormal Transcranial Doppler Velocities in Pediatric Patients with Sickle Cell Anemia
Conclusions: This retrospective study noted an increased risk for development of an abnormal or conditional TCD with an increased HI. The addition of hemoglobin to the HI to form HHI showed an elevated risk for abnormal or conditional TCD to the same degree as HI itself. These results could offer an additional tool in the evaluation of the pediatric SCA population, although these results would benefit from a prospective look. Further look into a possible association between HI and other SCD related complications such as stroke and acute chest syndrome could provide further information for the treating team for the overall ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Goubeaux, D. L., Sherman, A., Kalpatthi, R., Woods, G. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research