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Condition: Sickle Cell Anemia

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Total 1318 results found since Jan 2013.

Modeling the public health impact of voxelotor in the management of sickle cell disease in France
The objective of this study was to estimate the impact of voxelotor on the burden of SCD in France using a modeling approach, accounting for its anticipated adoption and diffusion over the next 5 years. We designed a sequential multi-cohort model to project and compare the cumulative incidence of SCD complications over a 20-year time horizon in a world with and without voxelotor. A distribution of patients was simulated across various levels of Hb response based on the phase 3 HO PE trial results, and relative risk reduction was adjusted using published meta-analysis results that projected risk reduction due to a 1 g/dL in...
Source: PLoS One - September 13, 2023 Category: Biomedical Science Authors: Fr édéric Galacteros Source Type: research

What Are the Complications of Sickle Cell Trait?
Discussion Sickle cell disease (SCD) is a group of inherited blood disorders where there are abnormal hemoglobin molecules and the red blood cells take on a characteristic sickled shape instead of a rounded shape. Sickled hemoglobin provides an evolutionary advantage of giving the individual increased protection against severe and cerebral malaria. The sickled shape doesn’t move through the vascular system as well and therefore is more likely to aggregate in small vessels causing an increased risk of vaso-occlusive disease, acute chest syndrome, splenic sequestration, and priapism. There is also an increased risk of ...
Source: PediatricEducation.org - September 4, 2023 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news

Cognition and education benefits of increased hemoglobin and blood oxygenation in children with sickle cell disease
by Joanna P. MacEwan, Allison A. King, Andy Nguyen, Anuj Mubayi, Irene Agodoa, Kim Smith-Whitley BackgroundAmong individuals with sickle cell disease (SCD), decreased hemoglobin is associated with lower oxygen saturation (SpO2) and increased risk of stroke, both of which are associated with lower intelligence quotient (IQ) scores. Thus, increasing hemoglobin and SpO2 in individuals with SCD may increase IQ and educational attainment. MethodsA cohort simulation model was built to determine academic performance and educational attainment based on cognitive function (measured by IQ) of a pediatric SCD cohort randomly assigned...
Source: PLoS One - August 8, 2023 Category: Biomedical Science Authors: Joanna P. MacEwan Source Type: research

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study
ConclusionAge-related cognitive trajectories of CYA-SCD may not be impaired but may progress more slowly. Longitudinal studies are required, using tests unaffected by practice. In addition to initiation of medical treatment, including measures to improve arterial oxygen content, early cognitive intervention, educational support, and delivery of extracurricular activities could support cognitive development for CYA-SCD.Graphical Abstract
Source: Frontiers in Neurology - July 25, 2023 Category: Neurology Source Type: research

Pediatric Moyamoya Revascularization Perioperative Care: A Modified Delphi Study
ConclusionsIn the absence of data supporting specific care practices before and after indirect revascularization surgery in children with moyamoya, this Delphi process defined areas of consensus among neurosurgeons, neurologists, and intensivists with moyamoya expertise. Research priorities identified include determining the role of continuous electroencephalography in postoperative moyamoya care, optimal perioperative blood pressure and hemoglobin targets, and the role of supplemental oxygen for treatment of suspected postoperative ischemia.
Source: Neurocritical Care - July 20, 2023 Category: Neurology Source Type: research

Cytomegalovirus viremia in an 11-year-old child with sickle cell disease manifested only with fever: a case report
CONCLUSION: This case highlights the need to consider CMV infection in the differential diagnosis of every case of fever of unknown origin, regardless of the patient's immune status.PMID:37427240 | PMC:PMC10328612 | DOI:10.1097/MS9.0000000000000877
Source: Annals of Medicine - July 10, 2023 Category: Internal Medicine Authors: Mahfoud Eid Abd Ghanem Elias Saloum Source Type: research

Fertility preservation in women with sickle cell disease prior to curative therapy with stem cell transplant: a case series
Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting approximately 100,000 Americans (1). Due to the pathognomonic sickling of red blood cells, individuals with SCD are at risk of venous thromboembolism, hemolytic anemia, stroke, pain crises, infection and end organ damage (2). Hematopoietic stem cell transplant (HSCT), while curative, can also lead to gonadal dysfunction and thereby ovarian insufficiency due to the associated myeloablative and radiative conditioning regimens (3).
Source: Fertility and Sterility - July 1, 2023 Category: Reproduction Medicine Authors: M. Yamasaki, P. Lindner, A. Decherney, T. Spitzer Source Type: research

Plasma exchange-A useful adjunct therapy to red cell exchange in patients with sickle cell disease and multiorgan dysfunction
CONCLUSION: The findings suggest TPE may be considered as an adjunct treatment for patients with acute complications of SCD that progress to MODS, especially in cases where there is no significant improvement following RBCx.PMID:37317564 | DOI:10.1111/trf.17448
Source: Transfusion - June 15, 2023 Category: Hematology Authors: C B Webb S G Yates R Sarode J Kim Source Type: research

MTHFR C677T Polymorphism, Plasma Homocysteine, and PDGF-AA Levels and Transcranial Doppler Velocity in Children with Sickle Cell Disease
CONCLUSION: PDGF-AA and tHcy levels could be used as predictive markers for stroke in SCD children. MTHFR Polymorphism contributes to elevated tHcy levels.PMID:37260067
Source: Indian Pediatrics - June 1, 2023 Category: Pediatrics Authors: Asmaa A Mahmoud Nahla M S Abd El Hady Mohammed S Rizk Ahmed M El-Hawwary Nagwan Y Saleh Source Type: research