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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

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

Can ‘toxic’ bilirubin treat a variety of illnesses?
Generations of medical and biology students have been instilled with a dim view of bilirubin. Spawned when the body trashes old red blood cells, the molecule is harmful refuse and a sign of illness. High blood levels cause jaundice, which turns the eyes and skin yellow and can signal liver trouble. Newborns can’t process the compound, and although high levels normally subside, a persistent surplus can cause brain damage. Yet later this year up to 40 healthy Australian volunteers may begin receiving infusions of the supposedly good-for-nothing molecule. They will be participating in a phase 1 safety trial, sponsored ...
Source: ScienceNOW - June 8, 2023 Category: Science Source Type: news

McConnell ’ s Bid to Downplay Freezes Undermined by History of Politicians Lying About Their Health
After Senate Minority Leader Mitch McConnell froze during a press conference this month, the Kentucky Republican’s second such episode this summer, his office released a note from the Capitol physician intended to calm those worried about his ability to continue at his job. Dr. Brian Monahan told McConnell in the letter that there was “no evidence that you have a seizure disorder or that you experienced a stroke, TIA or movement disorder such as Parkinson’s disease.” Monahan suggested the episodes may be related to the Leader’s concussion in March or to dehydration.  [time-brightcove n...
Source: TIME: Health - September 11, 2023 Category: Consumer Health News Authors: Mini Racker Tags: Uncategorized Congress Source Type: news

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

Migration of Bone Marrow‐Derived Cells Into the Central Nervous System in Models of Neurodegeneration
This study confirms that BMDC infiltrate the CNS in animal models for stroke and Alzheimer's disease and that peripheral cells can be targeted to treat affected regions of the CNS. J. Comp. Neurol. 521:3863–3876, 2013. © 2013 Wiley Periodicals, Inc.
Source: The Journal of Comparative Neurology - October 7, 2013 Category: Neurology Authors: Antoine Lampron, Pedro M. Pimentel‐Coelho, Serge Rivest Tags: Cover Image Source Type: research

Health Status, Late Effects and Long Term Survivorship of Allogeneic Bone Marrow Transplantation: A Retrospective Study
ConclusionThis study shows that allo‐HSCT recipients are living with high burdens of chronic diseases that warrant lifelong surveillance and engagement with healthcare. Structured, multi‐disciplinary care as recommended by published guidelines for allo‐HSCT survivors may reduce long term effects and improve their outcomes.
Source: Internal Medicine Journal - November 1, 2013 Category: Internal Medicine Authors: Grace Gifford, Joycelyn Sim, Annabel Horne, David Ma Tags: Original Article Source Type: research

Health status, late effects and long‐term survivorship of allogeneic bone marrow transplantation: a retrospective study
ConclusionThis study shows that allo‐HSCT patients are living with high burdens of chronic diseases that warrant lifelong surveillance and engagement with healthcare. Structured, multi‐disciplinary care as recommended by published guidelines for allo‐HSCT survivors may reduce long‐term effects and improve their outcomes.
Source: Internal Medicine Journal - February 17, 2014 Category: Internal Medicine Authors: G. Gifford, J. Sim, A. Horne, D. Ma Tags: Original Article Source Type: research

The impact of Ly6Clow monocytes after cerebral hypoxia-ischemia in adult mice
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Source: Journal of Cerebral Blood Flow - April 30, 2014 Category: Neurology Authors: Jean-Philippe MichaudPedro Moreno Pimentel-CoelhoYannick TremblaySerge Rivest Tags: blood bone marrow transplantation innate immunity ischemia Ly6Clow monocytes stroke Source Type: research

Light chain amyloidosis: Experience in a tertiary hospital: 2005–2013
Conclusions Cardiac involvement is the major determinant of prognosis. Yield of involved organ biopsy is high (100% heart biopsies). Antineoplastic treatment with bortezomib and/or autologous bone marrow transplantation achieves hematological responses with improvements in organ impairment.
Source: Revista Clinica Espanola - November 27, 2014 Category: Internal Medicine Source Type: research

Context-Dependent Effects of SOCS3 in Angiotensin II-Induced Vascular Dysfunction and Hypertension in Mice: Mechanisms and Role of Bone Marrow-Derived Cells.
We examined direct effects of Ang II on carotid arteries from SOCS3 deficient (SOCS3+/-) mice and wild-type (WT) littermates using organ culture, followed by testing endothelial function with acetylcholine (Ach). A low concentration of Ang II (1 nmol/L) did not affect Ach-induced vasodilation in WT, but reduced that of SOCS3+/- mice by ~50% (P<0.05). In relation to mechanisms, effects of Ang II in SOCS3+/- mice were prevented by inhibitors of STAT3, IL-6, NF-κB or superoxide. Systemic Ang II (1.4 mg/kg per day for 14 d) also reduced vasodilation to Ach in WT. Surprisingly, SOCS3 deficiency prevented most of the endothe...
Source: American Journal of Physiology. Heart and Circulatory Physiology - April 21, 2016 Category: Physiology Authors: Li Y, Kinzenbaw DA, Modrick ML, Pewe LL, Faraci FM Tags: Am J Physiol Heart Circ Physiol Source Type: research

Context-dependent effects of SOCS3 in angiotensin II-induced vascular dysfunction and hypertension in mice: mechanisms and role of bone marrow-derived cells
We examined direct effects of Ang II on carotid arteries from SOCS3-deficient (SOCS3+/–) mice and wild-type (WT) littermates using organ culture and then tested endothelial function with acetylcholine (ACh). A low concentration of Ang II (1 nmol/l) did not affect ACh-induced vasodilation in WT but reduced that of SOCS3+/– mice by ~50% (P < 0.05). In relation to mechanisms, effects of Ang II in SOCS3+/– mice were prevented by inhibitors of STAT3, IL-6, NF-B, or superoxide. Systemic Ang II (1.4 mg/kg per day for 14 days) also reduced vasodilation to ACh in WT. Surprisingly, SOCS3 deficiency prevented mos...
Source: AJP: Heart and Circulatory Physiology - June 30, 2016 Category: Cardiology Authors: Li, Y., Kinzenbaw, D. A., Modrick, M. L., Pewe, L. L., Faraci, F. M. Tags: VASCULAR BIOLOGY AND MICROCIRCULATION Source Type: research

Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.
Abstract Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 dev...
Source: Texas Heart Institute Journal - November 30, 2016 Category: Cardiology Authors: Iannucci GJ, Adisa OA, Oster ME, McConnell M, Mahle WT Tags: Tex Heart Inst J Source Type: research

Macrophage-Specific Expression of IL-37 in Hyperlipidemic Mice Attenuates Atherosclerosis.
This study was undertaken to elucidate the role of macrophage-expressed IL-37 in reducing the production and effects of proinflammatory cytokines, preventing foam cell formation, and reducing the development of atherosclerosis. Expression of human IL-37 was achieved with a macrophage-specific overexpression system, using the CD68 promoter in mouse primary bone marrow-derived macrophages via retroviral transduction. Macrophage IL-37 expression in vitro resulted in decreased mRNA (e.g., IL-1B, IL-6, and IL-12) and secreted protein production (e.g., IL-6, M-CSF, and ICAM-1) of key inflammatory mediators. IL-37 expression also...
Source: Journal of Immunology - October 13, 2017 Category: Allergy & Immunology Authors: McCurdy S, Baumer Y, Toulmin E, Lee BH, Boisvert WA Tags: J Immunol 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

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