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Interleukin-1 receptor inhibition reduces stroke size in a murine model of sickle cell disease.
Abstract Sickle cell disease (SCD) is associated with chronic hemolytic anemia and a heightened inflammatory state. The causal role of inflammatory pathways in stroke associated with SCD is unclear. Therefore, the hypothesis that deletion of the non-hematopoietic interleukin-1 receptor (IL-1R) pool may be beneficial in SCD was pursued. Since potential deleterious effects of IL-1R signaling in SCD could be mediated via downstream production of interleukin-6 (IL-6), the role of the non-hematopoietic IL-6 pool was also addressed. Bone marrow transplantation (BMT) from SCD to wild-type (WT) recipient mice was used to ...
Source: Haematologica - August 12, 2020 Category: Hematology Authors: Venugopal J, Wang J, Mawri J, Guo C, Eitzman D Tags: Haematologica Source Type: research

High molecular weight kininogen contributes to early mortality and kidney dysfunction in a mouse model of sickle cell disease.
CONCLUSIONS: We found elevated plasma levels of cleaved HK in sickle patients compared to healthy controls, suggesting ongoing HK activation in SCD. We used bone marrow transplantation to generate wild type and sickle cell mice on a HK-deficient background. We found that short-term HK deficiency attenuated thrombin generation and inflammation in sickle mice at steady state, which was independent of bradykinin signaling. Moreover, long-term HK deficiency attenuates kidney injury, reduces chronic inflammation, and ultimately improves of sickle mice. PMID: 32573897 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - June 22, 2020 Category: Hematology Authors: Sparkenbaugh EM, Kasztan M, Henderson MW, Ellsworth P, Davis PR, Wilson KJ, Reeves B, Key NS, Strickland S, McCrae K, Pollock DM, Pawlinski R Tags: J Thromb Haemost Source Type: research

Sickle Cell Disease Subjects Have a Distinct Abnormal Autonomic Phenotype Characterized by Peripheral Vasoconstriction With Blunted Cardiac Response to Head-Up Tilt
Conclusion We have shown that SCD subjects are much more likely than non-SCD subjects to have impaired cardiac, but intact peripheral responses to orthostatic stress induced by HUT. These abnormal responses are associated with low baseline cardiac parasympathetic activity, independent of hemoglobin level. The classification of autonomic phenotypes based on HUT response may have potential use for predicting disease severity, guiding and targeting treatments/interventions to alleviate the risk of adverse outcomes in SCD. Ethics Statement All experiments were conducted at Children’s Hospital Los Angeles (CHLA). The ...
Source: Frontiers in Physiology - April 10, 2019 Category: Physiology Source Type: research

Haploidentical Bone Marrow Transplantation with Post-Transplantation Cyclophosphamide Plus Thiotepa Improves Donor Engraftment in Patients with Sickle Cell Anemia: Results of an International Learning Collaborative
Sickle cell disease (SCD) has evolved from a life-threatening condition in early childhood to a chronic disease in adults [1,2]. Improved supportive care [3], use of hydroxyurea therapy [4], and regular blood transfusion therapy for primary [5] and secondary [6] stroke prevention have resulted in improved childhood clinical outcomes and survival. Approximately 99% of all children born with SCD are expected to live to adulthood [7,8]. The most feared complication in children with SCD is stroke [9].
Source: Biology of Blood and Marrow Transplantation - November 27, 2018 Category: Hematology Authors: Josu de la Fuente, Nathalie Dhedin, Tatsuki Koyama, Francoise Bernaudin, Mathieu Kuentz, Leena Karnik, G érard Socié, Kathryn A. Culos, Robert A. Brodsky, Michael R. DeBaun, Adetola A. Kassim Source Type: research

Haploidentical bone marrow transplant with post-transplant cyclophosphamide plus thiotepa improves donor engraftment in patients with sickle cell anemia: results of an international learning collaborative
Sickle cell disease (SCD) has evolved from a life-threatening condition in early childhood, to a chronic disease in adults [1,2]. Improved supportive care [3], use of hydroxyurea therapy [4], and regular blood transfusion therapy for primary [5] and secondary [6] stroke prevention, have resulted in improved childhood clinical outcomes and survival. Approximately 99% of all children born in high resource countries with SCD are expected to live to adulthood [7,8]. The most feared complication in children with SCD is stroke [9].
Source: Biology of Blood and Marrow Transplantation - November 27, 2018 Category: Hematology Authors: Josu de la Fuente, Nathalie Dhedin, Tatsuki Koyama, Francoise Bernaudin, Mathieu Kuentz, Leena Karnik, G érard Socié, Kathryn A. Culos, Robert A. Brodsky, Michael R. DeBaun, Adetola A. Kassim Source Type: research

Gene Addition Strategies for β-Thalassemia and Sickle Cell Anemia.
Authors: Dong AC, Rivella S Abstract Beta-thalassemia and sickle cell anemia are two of the most common diseases related to the hemoglobin protein. In these diseases, the beta-globin gene is mutated, causing severe anemia and ineffective erythropoiesis. Patients can additionally present with a number of life-threatening co-morbidities, such as stroke or spontaneous fractures. Current treatment involves transfusion and iron chelation; allogeneic bone marrow transplant is the only curative option, but is limited by the availability of matching donors and graft-versus-host disease. As these two diseases are monogenic ...
Source: Advances in Experimental Medicine and Biology - November 13, 2017 Category: Research Tags: Adv Exp Med Biol Source Type: research

Paradoxical protection from atherosclerosis and thrombosis in a mouse model of sickle cell disease
Summary Sickle cell disease (SCD) is associated with vascular complications including premature stroke. The role of atherothrombosis in these vascular complications is unclear. To determine the effect of SCD on atherosclerosis and thrombosis, mice with SCD along with controls were generated by transplantation of bone marrow from mice carrying the homozygous sickle cell mutation (Hbbhβs/hβs) or wild‐type mice (Hbb+/+) into C57BL6/J or apolipoprotein E deficient (Apoe−/−) recipient mice. At the time of sacrifice, 23–28 weeks following bone marrow transplantation, anaemia, reticulocytosis, and splenomegaly were pre...
Source: British Journal of Haematology - April 17, 2013 Category: Hematology Authors: Hui Wang, Wei Luo, Jintao Wang, Chiao Guo, Stephanie L. Wolffe, Julia Wang, Eddy B. Sun, Kori N. Bradley, Andrew D. Campbell, Daniel T. Eitzman Tags: Research Paper Source Type: research

Stem Cells for Cell-Based Therapies
The world of stem cells We know the human body comprises many cell types (e.g., blood cells, skin cells, cervical cells), but we often forget to appreciate that all of these different cell types arose from a single cell—the fertilized egg. A host of sequential, awe-inspiring events occur between the fertilization of an egg and the formation of a new individual: Embryonic stem (ES) cells are also called totipotent cells. The first steps involve making more cells by simple cell division: one cell becomes two cells; two cells become four cells, etc. Each cell of early development is undifferentiated; that is, it is...
Source: ActionBioscience - December 28, 2012 Category: Science Authors: Ali Hochberg Source Type: news