scholarly journals Drag-reducing polymers improve hepatic vaso-occlusion in SCD mice

2020 ◽  
Vol 4 (18) ◽  
pp. 4333-4336
Author(s):  
Dan Crompton ◽  
Ravi Vats ◽  
Tirthadipa Pradhan-Sundd ◽  
Prithu Sundd ◽  
Marina V. Kameneva
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Key Points ◽  
Drag Reducing Polymer

Key Points Nanomolar concentrations of drag-reducing polymer (DRP) reduce vaso-occlusion in the liver of sickle cell disease (SCD) mice. The potential for DRP as a rheology-based treatment/therapy for SCD warrants further study.

scholarly journals Gene-centric association study of acute chest syndrome and painful crisis in sickle cell disease patients

Blood ◽  
2013 ◽  
Vol 122 (3) ◽  
pp. 434-442 ◽  
Author(s):  
Geneviève Galarneau ◽  
Sean Coady ◽  
Melanie E. Garrett ◽  
Neal Jeffries ◽  
Mona Puggal ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Association Study ◽  
Sickle Cell ◽  
Genome Wide Association Study ◽  
Inflammatory Responses ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Genome Wide ◽  
Key Points ◽  
Painful Crisis

Key Points Using genome-wide association study, we found the first replicated genetic association with acute chest syndrome in sickle cell disease patients. The locus identified includes COMMD7, a gene highly expressed in the lung that interacts with NFκB to control inflammatory responses.

scholarly journals Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease

Blood ◽  
2014 ◽  
Vol 123 (11) ◽  
pp. 1747-1756 ◽  
Author(s):  
Erica M. Sparkenbaugh ◽  
Pichika Chantrathammachart ◽  
Jacqueline Mickelson ◽  
Joanne van Ryn ◽  
Robert P. Hebbel ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Mouse Model ◽  
Sickle Cell ◽  
Vascular Inflammation ◽  
Vascular Pathology ◽  
Cell Disease ◽  
Potential Target ◽  
Key Points ◽  
Thrombotic Complications ◽  
Differential Contribution

Key Points Inhibition of FXa or thrombin might be considered to reduce thrombotic complications and vascular inflammation in sickle cell patients. PAR-2 could be a potential target to inhibit vascular pathology associated with sickle cell disease.

scholarly journals HO-1hi patrolling monocytes protect against vaso-occlusion in sickle cell disease

Blood ◽  
2018 ◽  
Vol 131 (14) ◽  
pp. 1600-1610 ◽  
Author(s):  
Yunfeng Liu ◽  
Fangmiao Jing ◽  
Woelsung Yi ◽  
Avital Mendelson ◽  
Patricia Shi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Key Points ◽  
Patrolling Monocytes ◽  
Lower Frequencies

Key Points SCD patients with a recent VOC episode have lower frequencies and numbers of HO-1hi patrolling monocytes. Heme-driven SCD vaso-occlusion is exacerbated in mice lacking patrolling monocytes and reversed following patrolling monocyte transfer.

scholarly journals Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study

2018 ◽  
Vol 2 (3) ◽  
pp. 285-291 ◽  
Author(s):  
Riten Kumar ◽  
Joseph Stanek ◽  
Susan Creary ◽  
Amy Dunn ◽  
Sarah H. O’Brien
Keyword(s):  
Risk Factors ◽  
Retrospective Study ◽  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Administrative Database ◽  
Similar Data ◽  
Cell Disease ◽  
Database Study ◽  
Key Points

Key Points Adults with SCD have an increased incidence of VTE, but similar data in children are lacking. In this 7-year, multicenter retrospective study, 1.7% of children with SCD developed VTE.

scholarly journals Acid sphingomyelinase is activated in sickle cell erythrocytes and contributes to inflammatory microparticle generation in SCD

Blood ◽  
2014 ◽  
Vol 124 (12) ◽  
pp. 1941-1950 ◽  
Author(s):  
Anthony O. Awojoodu ◽  
Philip M. Keegan ◽  
Alicia R. Lane ◽  
Yuying Zhang ◽  
Kevin R. Lynch ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acid Sphingomyelinase ◽  
Sphingolipid Metabolism ◽  
Monocyte Adhesion ◽  
Cell Disease ◽  
Key Points

Key Points Alterations in RBC membranes contribute to dysregulated sphingolipid metabolism in sickle cell disease (SCD). Increased RBC-derived MP production enhances monocyte adhesion and activation in SCD.

scholarly journals g(HbF): a genetic model of fetal hemoglobin in sickle cell disease

2018 ◽  
Vol 2 (3) ◽  
pp. 235-239 ◽  
Author(s):  
Kate Gardner ◽  
Tony Fulford ◽  
Nicholas Silver ◽  
Helen Rooks ◽  
Nikolaos Angelis ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Studies ◽  
Genetic Model ◽  
Fetal Hemoglobin ◽  
Genetic Component ◽  
Cell Disease ◽  
Quantitative Variable ◽  
Key Points ◽  
Variable G

Key Points The 3 established HbF genetic loci can be summarized into 1 quantitative variable, g(HbF), in SCD and influence markers of SCD severity. g(HbF) provides a quantitative marker for the genetic component of HbF% variability, potentially useful in genetic and clinical studies in SCD.

scholarly journals P-selectin–deficient mice to study pathophysiology of sickle cell disease

2020 ◽  
Vol 4 (2) ◽  
pp. 266-273 ◽  
Author(s):  
Margaret F. Bennewitz ◽  
Egemen Tutuncuoglu ◽  
Shweta Gudapati ◽  
Tomasz Brzoska ◽  
Simon C. Watkins ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Key Points ◽  
Deficient Mice

Key Points P-selectin–deficient SCD mice are protected from lung vaso-occlusion. P-selectin–deficient SCD mice will be useful in assessing the benefits of anti–P-selectin therapy in diverse complications of SCD.

scholarly journals Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease

Blood ◽  
2014 ◽  
Vol 123 (24) ◽  
pp. 3818-3827 ◽  
Author(s):  
Grace Chen ◽  
Dachuan Zhang ◽  
Tobias A. Fuchs ◽  
Deepa Manwani ◽  
Denisa D. Wagner ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Proinflammatory Cytokines ◽  
Neutrophil Extracellular Traps ◽  
Cell Disease ◽  
Extracellular Traps ◽  
Key Points

Key Points NETs are present and pathogenic in sickle cell disease. Plasma heme and proinflammatory cytokines collaborate to activate release of NETs.

scholarly journals Myelodysplastic syndrome unrelated to lentiviral vector in a patient treated with gene therapy for sickle cell disease

2020 ◽  
Vol 4 (9) ◽  
pp. 2058-2063
Author(s):  
Matthew M. Hsieh ◽  
Melissa Bonner ◽  
Francis John Pierciey ◽  
Naoya Uchida ◽  
James Rottman ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Myelodysplastic Syndrome ◽  
Sickle Cell ◽  
Lentiviral Vector ◽  
Cell Disease ◽  
Novel Treatments ◽  
Key Points

Key Points Ability to accurately attribute adverse events post–gene therapy is required to describe the benefit-risk of these novel treatments. A SCD patient developed myelodysplastic syndrome post-LentiGlobin treatment; we show how insertional oncogenesis was excluded as the cause.

High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors

Blood ◽  
2013 ◽  
Vol 122 (6) ◽  
pp. 1062-1071 ◽  
Author(s):  
Stella T. Chou ◽  
Tannoa Jackson ◽  
Sunitha Vege ◽  
Kim Smith-Whitley ◽  
David F. Friedman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Key Points ◽  
High Prevalence ◽  
Transfusion Reactions

Key Points Rh serologic phenotype–matched transfusions from minority donors do not prevent all Rh alloimmunization in patients with SCD. Variant RH genes are common in patients with SCD and contribute to Rh alloimmunization and transfusion reactions.

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