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Elevated adenosine signaling via adenosine A2B receptor induces normal and sickle erythrocyte sphingosine kinase 1 activity
Erythrocyte possesses high sphingosine kinase 1 (SphK1) activity and is the major cell type supplying plasma sphingosine-1-phosphate, a signaling lipid regulating multiple physiological and pathological functions. Recent studies revealed that erythrocyte SphK1 activity is upregulated in sickle cell disease (SCD) and contributes to sickling and disease progression. However, how erythrocyte SphK1 activity is regulated remains unknown. Here we report that adenosine induces SphK1 activity in human and mouse sickle and normal erythrocytes in vitro. Next, using 4 adenosine receptor-deficient mice and pharmacological approaches, ...
Source: Blood - March 5, 2015 Category: Hematology Authors: Sun, K., Zhang, Y., Bogdanov, M. V., Wu, H., Song, A., Li, J., Dowhan, W., Idowu, M., Juneja, H. S., Molina, J. G., Blackburn, M. R., Kellems, R. E., Xia, Y. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research
How I treat and manage strokes in sickle cell disease
Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ~11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by...
Source: Blood - May 28, 2015 Category: Hematology Authors: Kassim, A. A., Galadanci, N. A., Pruthi, S., DeBaun, M. R. Tags: Sickle Cell Disease, How I Treat, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Clinical Trials and Observations Source Type: research
Degree of anemia, indirect markers of hemolysis, and vascular complications of sickle cell disease in Africa
In conclusion, severe anemia at steady state in SCD patients living in West and Central Africa is associated with elevated TRV, microalbuminuria, and leg ulcers, but these vascular complications are not independently associated with indirect markers of increased hemolysis. Other mechanisms leading to anemia, including malnutrition and infectious diseases, may also play a role in the development of SCD vasculopathy.
Source: Blood - November 16, 2017 Category: Hematology Authors: Dubert, M., Elion, J., Tolo, A., Diallo, D. A., Diop, S., Diagne, I., Sanogo, I., Belinga, S., Guifo, O., Wamba, G., Ngo Sack, F., Boidy, K., Kamara, I., Traore, Y., Diakite, C. O., Gbonon, V., Faye, B. F., Seck, M., Deme Ly, I., Chelo, D., NGuetta, R., D Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research
Rational modification of vanillin derivatives to stereospecifically destabilize sickle hemoglobin polymer formation
Increasing the affinity of hemoglobin for oxygen represents a feasible and promising therapeutic approach for sickle cell disease by mitigating the primary pathophysiological event, i.e. the hypoxia-induced polymerization of sickle hemoglobin (Hb S) and the concomitant erythrocyte sickling. Investigations on a novel synthetic antisickling agent, SAJ-310, with improved and sustained antisickling activity have previously been reported. To further enhance the biological effects of SAJ-310, a structure-based approach was employed to modify this compound to specifically inhibit Hb S polymer formation through interactions which ...
Source: Acta Crystallographica Section D - October 2, 2018 Category: Biochemistry Authors: Deshpande, T.M. Pagare, P.P. Ghatge, M.S. Chen, Q. Musayev, F.N. Venitz, J. Zhang, Y. Abdulmalik, O. Safo, M.K. Tags: sickle cell disease hemoglobin relaxed state aromatic aldehydes antisickling oxygen equilibrium crystal structure polymerization F-helix research papers Source Type: research
Microfluidic Methods to Advance Mechanistic Understanding and Translational Research in Sickle Cell Disease
Transl Res. 2022 Mar 27:S1931-5244(22)00065-2. doi: 10.1016/j.trsl.2022.03.010. Online ahead of print.ABSTRACTSickle cell disease (SCD) is caused by a single point mutation in the β-globin gene of hemoglobin, which produces an altered sickle hemoglobin (HbS). The ability of HbS to polymerize under deoxygenated conditions gives rise to chronic hemolysis, oxidative stress, inflammation, and vaso-occlusion. Herein, we review recent findings using microfluidic technologies that have elucidated mechanisms of oxygen-dependent and -independent induction of HbS polymerization and how these mechanisms elicit the biophysical and in...
Source: Cell Research - March 30, 2022 Category: Cytology Authors: Melissa Azul Eudorah F Vital Wilbur A Lam David K Wood Joan D Beckman Source Type: research
MASP-2 and MASP-3 inhibitors block complement activation, inflammation, and microvascular stasis in a murine model of vaso-occlusion in sickle cell disease
Transl Res. 2022 Jul 22:S1931-5244(22)00152-9. doi: 10.1016/j.trsl.2022.06.018. Online ahead of print.ABSTRACTPatients with sickle cell disease (SCD) have ongoing hemolysis that promotes endothelial injury, complement activation, inflammation, vaso-occlusion, ischemia-reperfusion pathophysiology, and pain. Complement activation markers are increased in SCD in steady-state and further increased during vaso-occlusive crisis (VOC). However, the mechanisms driving complement activation in SCD have not been completely elucidated. Ischemia-reperfusion and heme released from hemoglobin during hemolysis, events that characterize S...
Source: Cell Research - July 25, 2022 Category: Cytology Authors: John D Belcher Julia Nguyen Chunsheng Chen Fuad Abdulla Ruan Conglin Zalaya K Ivy Jason Cummings Thomas Dudler Gregory M Vercellotti Source Type: research
Treatment of sickle cell disease - options and perspective
Am J Blood Res. 2023 Apr 15;13(2):61-70. eCollection 2023.ABSTRACTSickle Cell Disease (SCD) is one of the most inherited hematologic diseases affecting humans. Clinically, there is a progressive multiorgan failure and increased mortality in severe cases. The highest prevalence is in West Africa, India, the Mediterranean region, and Middle East countries. Hydroxyurea was the primary drug available for SCD and remains first-line therapy for patients with SCD. Three additional drug therapies, L-glutamine, Voxelotor, and Crizanlizumab, have been approved as adjunctive agents. However, none of these treatments are curative. Eff...
Source: Cell Research - May 22, 2023 Category: Cytology Authors: Loubna Abdel-Hadi Yendry Ventura Carmenate Yandy Marx Castillo-Aleman Samira Sheikh Aya Zakaria John Phillips Source Type: research
Pain management in adult patients with sickle cell disease in the emergency department: how does current practice compare with existing standards of care?
CONCLUSION: Existing recommendations are that opioid analgesia be provided within 30 min of triage for patients with SCD and VOEs. Our data show this target is rarely met, even in a department in which SCD VOEs are a common presenting concern. The association of earlier opioid analgesia with order set use and administration prior to physician assessment highlights potential avenues for improving time to analgesia.PMID:37661245 | DOI:10.1007/s43678-023-00579-y
Source: Pain Physician - September 3, 2023 Category: Anesthesiology Authors: Markus Gulilat Lanre Tunji-Ajayi Serena Thompson Marie-Pascale Poku Ruth Appiah-Boateng Nia Navarro Hasan Sheikh Jennifer Hulme Jennifer Bryan University Health Network Emergency Department Sickle Cell Working Group Source Type: research
Gene-centric association study of acute chest syndrome and painful crisis in sickle cell disease patients
Patients with sickle cell disease (SCD) present with a wide range of clinical complications. Understanding this clinical heterogeneity offers the prospects to tailor the right treatments to the right patients and also guide the development of novel therapies. Several environmental (eg, nutrition) and nonenvironmental (eg, fetal hemoglobin levels, α-thalassemia status) factors are known to modify SCD severity. To find new genetic modifiers of SCD severity, we performed a gene-centric association study in 1514 African American participants from the Cooperative Study of Sickle Cell Disease (CSSCD) for acute chest syndro...
Source: Blood - July 18, 2013 Category: Hematology Authors: Galarneau, G., Coady, S., Garrett, M. E., Jeffries, N., Puggal, M., Paltoo, D., Soldano, K., Guasch, A., Ashley-Koch, A. E., Telen, M. J., Kutlar, A., Lettre, G., Papanicolaou, G. J. Tags: Sickle Cell Disease, Free Research Articles, Red Cells, Iron, and Erythropoiesis Source Type: research
MP4CO, a pegylated hemoglobin saturated with carbon monoxide, is a modulator of HO-1, inflammation, and vaso-occlusion in transgenic sickle mice
Transgenic sickle mice expressing βS hemoglobin have activated vascular endothelium in multiple organs that exhibits enhanced expression of NF-B and adhesion molecules and promotes microvascular stasis in sickle, but not normal, mice in response to hypoxia/reoxygenation (H/R), or heme. Induction of heme oxygenase-1 (HO-1) or administration of its products, carbon monoxide (CO) or biliverdin, inhibits microvascular stasis in sickle mice. Infusion of human hemoglobin conjugated with polyethylene glycol and saturated with CO (MP4CO) markedly induced hepatic HO-1 activity and inhibited NF-B activation and H/R-induced micr...
Source: Blood - October 10, 2013 Category: Hematology Authors: Belcher, J. D., Young, M., Chen, C., Nguyen, J., Burhop, K., Tran, P., Vercellotti, G. M. Tags: Sickle Cell Disease, Vascular Biology Source Type: research
Somatic Growth and Lung Function in Sickle Cell Disease
Summary: Somatic growth is a key indicator of overall health and well-being with important prognostic implications in the management of chronic disease. Worldwide studies of growth in children and adults with SCD have predominantly shown delayed growth (especially in terms of body weight) that is gradual and progressive in nature. However, more recent studies have shown that a substantial number of patients with SCD have normal weight gain whereas some are even obese. Height in patients with SCD is not universally affected even among those with suboptimal weight gain, whereas some achieve the same or greater height than he...
Source: Paediatric Respiratory Reviews - November 25, 2013 Category: Respiratory Medicine Authors: Tina Catanzaro, Anastassios C. Koumbourlis Tags: Mini-Symposium: Controversies in the evaluation and treatment of sickle cell disease Source Type: research
Molecular mechanisms underlying synergistic adhesion of sickle red blood cells by hypoxia and low nitric oxide bioavailability
The molecular mechanisms by which nitric oxide (NO) bioavailability modulates the clinical expression of sickle cell disease (SCD) remain elusive. We investigated the effect of hypoxia and NO bioavailability on sickle red blood cell (sRBC) adhesion using mice deficient for endothelial NO synthase (eNOS) because their NO metabolite levels are similar to those of SCD mice but without hypoxemia. Whereas sRBC adhesion to endothelial cells in eNOS-deficient mice was synergistically upregulated at the onset of hypoxia, leukocyte adhesion was unaffected. Restoring NO metabolite levels to physiological levels markedly reduced sRBC...
Source: Blood - March 20, 2014 Category: Hematology Authors: Gutsaeva, D. R., Montero-Huerta, P., Parkerson, J. B., Yerigenahally, S. D., Ikuta, T., Head, C. A. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research
How I treat renal complications in sickle cell disease
Renal disease is one of the most frequent and severe complications experienced by patients with sickle cell disease; its prevalence is likely to increase as the patient population ages. We recommend regular monitoring for early signs of renal involvement and a low threshold for the use of hydroxyurea as preventative measures for end-stage renal disease. Once renal complications are detected, a careful assessment of the patient is required to rule out other causes of renal disease. Proteinuria and hypertension should be managed aggressively and the patient referred to a specialist nephrology center when progressive decline ...
Source: Blood - June 12, 2014 Category: Hematology Authors: Sharpe, C. C., Thein, S. L. Tags: Pediatric Hematology, Sickle Cell Disease, How I Treat, Free Research Articles, Red Cells, Iron, and Erythropoiesis Source Type: research
Reply: Practice Guideline for Pulmonary Hypertension in Sickle Cell: Direct Evidence Needed before Universal Adoption.
PMID: 25025360 [PubMed - in process]
Source: American Journal of Respiratory and Critical Care Medicine - July 15, 2014 Category: Respiratory Medicine Authors: Klings ES, Machado RF, Morris CR, Gordeuk VR, Kato GJ, Ataga KI, Castro O, Hsu L, Telen MJ, Krishnamurti L, Steinberg MH, Gladwin MT, ATS Clinical Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension in Sickle Cell Disease Committee Tags: Am J Respir Crit Care Med Source Type: research
Adverse events in cancer patients with sickle cell trait or disease: case reports
chard G. Stevens
Source: Genetics in Medicine - March 3, 2015 Category: Genetics & Stem Cells Authors: Helen SwedeBiree AndemariamDavid I. GregorioBeth A. JonesDejana BraithwaiteThomas E. RohanRichard G. Stevens Tags: cancer cancer adverse effects disparities hypoxia sickle cell disease sickle cell trait Source Type: research
