Syk inhibition suppresses NLRP3 inflammasome activation in platelets from sickle cell mice leading to decreased platelet secretion, aggregation, spreading, and in vitro thrombus formation
Platelets have a crucial function in mediating vascular inflammation and thrombosis, which play an important role in sickle cell disease [1]. A key inflammatory mechanism identified in platelets involves the pattern recognition receptor nucleotide-binding domain leucine-rich repeat containing protein 3 (NLRP3) and its adaptor, apoptosis-associated speck-like protein containing a caspase recruitment domain (ASC), which control caspase-1 activation within inflammasome complexes [2]. Activation of the platelet NLRP3 inflammasome promotes platelet aggregation, thrombosis, and vascular inflammation [2 –5]. (Source: Thrombosis Research)
Source: Thrombosis Research - March 21, 2024 Category: Hematology Authors: Sebastian Vogel, Sayuri Kamimura, Meghann L. Smith, Luis E.F. Almeida, Xizhong Cui, Christian A. Combs, Zenaide M.N. Quezado Tags: Letter to the Editors-in-Chief Source Type: research
Post-transfusion biotin-labeled red blood cell survival studies in pediatric sickle cell disease with antibodies of uncertain significance
DISCUSSION: Biotinylation of allogenic RBC can be used to assess the possible effects of RBC antibodies on transfusion survival in individual cases, particularly when it is uncertain if the detected antibodies may result in hemolysis. In the cases presented here, neither WAA nor B-RBC antibodies were associated with significant shortening of B-RBC survival in individuals with SCD.PMID:38506450 | DOI:10.1111/trf.17800 (Source: Transfusion)
Source: Transfusion - March 20, 2024 Category: Hematology Authors: Marianne E M Yee Patricia E Zerra James W McCoy Mischa L Covington Sean R Stowell Clinton H Joiner Christopher M Lough Bhaveshkumar B Delvadia Cassandra D Josephson John D Roback Ross M Fasano Source Type: research
Using the daily rate of rise in hemoglobin S to manage RBC depletion/exchange treatment in sickle cell disease
DISCUSSION: The RoR in HbS is a relatively consistent parameter for individual patients that is unaffected by medication use or procedural Hct targets and may be useful in determining intervals between procedures.PMID:38506484 | DOI:10.1111/trf.17797 (Source: Transfusion)
Source: Transfusion - March 20, 2024 Category: Hematology Authors: Kai Rogers Mouaz Alsawas James Chapman Annette J Schlueter Charles Michael Knudson Source Type: research
Ex vivo culture resting time impacts transplantation outcomes of genome-edited human hematopoietic stem and progenitor cells in xenograft mouse models
Cytotherapy. 2024 Feb 24:S1465-3249(24)00058-6. doi: 10.1016/j.jcyt.2024.02.011. Online ahead of print.ABSTRACTEx vivo resting culture is a standard procedure following genome editing in hematopoietic stem and progenitor cells (HSPCs). However, prolonged culture may critically affect cell viability and stem cell function. We investigated whether varying durations of culture resting times impact the engraftment efficiency of human CD34+ HSPCs edited at the BCL11A enhancer, a key regulator in the expression of fetal hemoglobin. We employed electroporation to introduce CRISPR-Cas9 components for BCL11A enhancer editing and co...
Source: Cancer Control - March 20, 2024 Category: Cancer & Oncology Authors: Selami Demirci Muhammad B N Khan Gabriela Hinojosa Anh Le Alexis Leonard Khaled Essawi Bjorg Gudmundsdottir Xiong Liu Jing Zeng Zaina Inam Rebecca Chu Naoya Uchida Daisuke Araki Evan London Henna Butt Stacy A Maitland Daniel E Bauer Scot A Wolfe Andre Lar Source Type: research
Using the daily rate of rise in hemoglobin S to manage RBC depletion/exchange treatment in sickle cell disease
DISCUSSION: The RoR in HbS is a relatively consistent parameter for individual patients that is unaffected by medication use or procedural Hct targets and may be useful in determining intervals between procedures.PMID:38506484 | DOI:10.1111/trf.17797 (Source: Transfusion)
Source: Transfusion - March 20, 2024 Category: Hematology Authors: Kai Rogers Mouaz Alsawas James Chapman Annette J Schlueter Charles Michael Knudson Source Type: research
Post-transfusion biotin-labeled red blood cell survival studies in pediatric sickle cell disease with antibodies of uncertain significance
DISCUSSION: Biotinylation of allogenic RBC can be used to assess the possible effects of RBC antibodies on transfusion survival in individual cases, particularly when it is uncertain if the detected antibodies may result in hemolysis. In the cases presented here, neither WAA nor B-RBC antibodies were associated with significant shortening of B-RBC survival in individuals with SCD.PMID:38506450 | DOI:10.1111/trf.17800 (Source: Transfusion)
Source: Transfusion - March 20, 2024 Category: Hematology Authors: Marianne E M Yee Patricia E Zerra James W McCoy Mischa L Covington Sean R Stowell Clinton H Joiner Christopher M Lough Bhaveshkumar B Delvadia Cassandra D Josephson John D Roback Ross M Fasano Source Type: research
Effect of Unmetabolized Folic Acid on Immunoinflammatory Markers in Sickle Cell Disease Patients Taking Folic Acid Supplementation
AbstractFolic acid (FA) supplementation in sickle cell disease (SCD) patients lead to accumulation of unmetabolized folic acid (UMFA) which might influence the level of cytokines and NK cell activity and thus trigger the crisis event. The aim of the study was to investigate the effect of UMFA levels on immuno-inflammatory markers in SCD patients taking FA supplementation. The cross-sectional study was conducted on 60 HbSS confirmed SCD cases with 22 crisis and 38 cases at steady state of 15 –40 years age group. Serum FA, 5-Methyl Tetrahydrofolate (5-MTHF), Dihydrofolate reductase, Interleukin-6 (IL-6), Highly sensitive ...
Source: Indian Journal of Clinical Biochemistry - March 20, 2024 Category: Biochemistry Source Type: research
Must the capacitous young person also be competent to provide consent for treatment and research?
Must the capacitous young person, in addition, be competent to provide consent for treatment? Drs Davies, Fisher and Birchley propose that this situation has been reached in the common law; and that the lack of legal clarity extends to medical research and guidance. And so it would, since as they point out, ‘capacity’ and ‘competence’ are not synonymous notions. A judgement, In Re X, handed down in January 20211 concerned a Gillick2 competent girl aged 15 years, soon to be 16. She was seeking to refuse blood transfusion for her latest sickle cell crisis. The Gillick case was decided on the basis tha...
Source: Archives of Disease in Childhood - March 19, 2024 Category: Pediatrics Authors: Wheeler, R. Tags: Editorial Source Type: research
Whiteout: a social history of sickle cell disease in Ontario, Canada
Volume 34, Issue 1, February 2024, Page 1-11 . (Source: Critical Public Health)
Source: Critical Public Health - March 19, 2024 Category: International Medicine & Public Health Authors: Sinthu Srikanthana Red Blood Cell Disorders Clinic, University Health Network, Toronto, Canadab Youth Research and Evaluation eXchange, School of Social Work, York University, Toronto, Canada Source Type: research
Sickle cell trait in S ão Tomé e Príncipe: a population-based prevalence study in women of reproductive age
Sickle Cell Disorder is Africa ’s most prevalent genetic disease. Yet, it remains a neglected condition, with high mortality under-five, and a lack of population-based studies in the region. This is the first o... (Source: BMC Public Health)
Source: BMC Public Health - March 19, 2024 Category: Consumer Health News Authors: Guilherme Queiroz, Celdidy Monteiro, Lic ínio Manco, Luís Relvas, Maria de Jesus Trovoada, Andreia Leite and Celeste Bento Tags: Research Source Type: research
Transfusing children with sickle cell disease using blood group genotyping when the pool of Black donors is limited
CONCLUSION: Although Rh-compatible donors were identified, blood units might not be available when needed and/or the extended phenotype or ABO group might not match the patient. A greater effort has to be made for the recruitment of Black donors to accommodate patients with SCD.PMID:38497419 | DOI:10.1111/trf.17778 (Source: Transfusion)
Source: Transfusion - March 18, 2024 Category: Hematology Authors: Gabriel Andr é Leiva-Torres Maude Cigna Jessica Constanzo-Yanez Maryse St-Louis Jos ée Perreault Jos ée Lavoie Genevi ève Laflamme Antoine Lewin Yves Pastore Nancy Robitaille Source Type: research
Stroke without cerebral arteriopathy in sickle cell disease children: causes and treatment
In conclusion, in a cohort of pediatric SCD patients with efficient stroke screening strategy, half of occurring ischemic strokes were related to causes other than CA. They affected a different population of SCD children and systematic long-term transfusion programs may not be necessary in these cases.PMID:38497171 | DOI:10.3324/haematol.2023.283773 (Source: Haematologica)
Source: Haematologica - March 18, 2024 Category: Hematology Authors: Sarah Liane Linguet Suzanne Verlhac Florence Missud Laurent Holvoet-Vermaut Valentine Brousse Ghislaine Ithier Alexandra Ntorkou Emmanuelle Lesprit Malika Benkerrou Mano ëlle Kossorotoff Berengere Koehl Source Type: research
Stroke without cerebral arteriopathy in sickle cell disease children: causes and treatment
In conclusion, in a cohort of pediatric SCD patients with efficient stroke screening strategy, half of occurring ischemic strokes were related to causes other than CA. They affected a different population of SCD children and systematic long-term transfusion programs may not be necessary in these cases.PMID:38497171 | DOI:10.3324/haematol.2023.283773 (Source: Haematologica)
Source: Haematologica - March 18, 2024 Category: Hematology Authors: Sarah Liane Linguet Suzanne Verlhac Florence Missud Laurent Holvoet-Vermaut Valentine Brousse Ghislaine Ithier Alexandra Ntorkou Emmanuelle Lesprit Malika Benkerrou Mano ëlle Kossorotoff Berengere Koehl Source Type: research
Stroke without cerebral arteriopathy in sickle cell disease children: causes and treatment
In conclusion, in a cohort of pediatric SCD patients with efficient stroke screening strategy, half of occurring ischemic strokes were related to causes other than CA. They affected a different population of SCD children and systematic long-term transfusion programs may not be necessary in these cases.PMID:38497171 | DOI:10.3324/haematol.2023.283773 (Source: Haematologica)
Source: Haematologica - March 18, 2024 Category: Hematology Authors: Sarah Liane Linguet Suzanne Verlhac Florence Missud Laurent Holvoet-Vermaut Valentine Brousse Ghislaine Ithier Alexandra Ntorkou Emmanuelle Lesprit Malika Benkerrou Mano ëlle Kossorotoff Berengere Koehl Source Type: research
Substance use disorder of equimolar oxygen-nitrous oxide mixture in French sickle-cell patients: results of the PHEDRE study
In many countries, nitrous oxide is used in a gas mixture (EMONO) for short-term analgesia. Cases of addiction, with significant misuse, have been reported in hospitalized patients. Patients suffering from sic... (Source: Orphanet Journal of Rare Diseases)
Source: Orphanet Journal of Rare Diseases - March 18, 2024 Category: Internal Medicine Authors: Marie G érardin, Morgane Rousselet, Marie-Laure Couec, Agathe Masseau, Aurélie Aquizerate, Nicolas Authier, Sylvie Deheul, Anne Roussin, Joelle Micallef, Samira Djezzar, Fanny Feuillet, Pascale Jolliet, Marie Grall-Bronnec and Caroline Victorri-Vigneau Tags: Research Source Type: research
