Gene-Addition/Editing Therapy in Sickle Cell Disease
Presse Med. 2023 Nov 22:104214. doi: 10.1016/j.lpm.2023.104214. Online ahead of print.ABSTRACTGene therapy is an innovative strategy that offers potential cure for patients with sickle cell disease, and no appropriate donor for transplant consideration. While we await long term data from these clinical trials, we remain optimistic that gene therapy will become a standard of care for curative treatment in sickle cell disease. As gene therapy becomes a standard of treatment in sickle cell disease, we must also acknowledge the potential for financial burden to patients. We also must acknowledge the prevalence of sickle cell d...
Source: Presse Medicale - November 24, 2023 Category: General Medicine Authors: Galia Pollock Olivier Negre Jean-Antoine Ribeil Source Type: research
The liver in sickle cell disease
Presse Med. 2023 Nov 17:104212. doi: 10.1016/j.lpm.2023.104212. Online ahead of print.ABSTRACTLiver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt re...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Christophe Duvoux Lorraine Blaise Jean-Jacques Matimbo Francky Mubenga Norbert Ngongang Monika Hurtova Alexis Laurent Julien Calderaro Edouard Reizine Alain Luciani Anoosha Habibi Dora Bachir Geoffroy Vole Justine Gellen-Dautremer Vincent Leroy Eric Leves Source Type: research
The Growing Challenge of RBC Alloimmunization in the Management of Patients with Sickle Cell Disease
Presse Med. 2023 Nov 17:104211. doi: 10.1016/j.lpm.2023.104211. Online ahead of print.ABSTRACTRBC transfusion remains a cornerstone in the treatment of sickle cell disease (SCD). However, as with many interventions, transfusion of RBCs is not without risk. Allogeneic RBC exposure can result in the development of alloantibodies, which can make it difficult to find compatible RBCs for future transfusion and increases the likelihood of life-threatening complications. The development of RBC alloantibodies occurs when a patient's immune system produces alloantibodies against foreign alloantigens present on allogeneic RBCs. Desp...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Daniel Y Chang Zakary Wankier Connie M Arthur Sean R Stowell Source Type: research
The liver in sickle cell disease
Presse Med. 2023 Nov 17:104212. doi: 10.1016/j.lpm.2023.104212. Online ahead of print.ABSTRACTLiver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt re...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Christophe Duvoux Lorraine Blaise Jean-Jacques Matimbo Francky Mubenga Norbert Ngongang Monika Hurtova Alexis Laurent Julien Calderaro Edouard Reizine Alain Luciani Anoosha Habibi Dora Bachir Geoffroy Vole Justine Gellen-Dautremer Vincent Leroy Eric Leves Source Type: research
The Growing Challenge of RBC Alloimmunization in the Management of Patients with Sickle Cell Disease
Presse Med. 2023 Nov 17:104211. doi: 10.1016/j.lpm.2023.104211. Online ahead of print.ABSTRACTRBC transfusion remains a cornerstone in the treatment of sickle cell disease (SCD). However, as with many interventions, transfusion of RBCs is not without risk. Allogeneic RBC exposure can result in the development of alloantibodies, which can make it difficult to find compatible RBCs for future transfusion and increases the likelihood of life-threatening complications. The development of RBC alloantibodies occurs when a patient's immune system produces alloantibodies against foreign alloantigens present on allogeneic RBCs. Desp...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Daniel Y Chang Zakary Wankier Connie M Arthur Sean R Stowell Source Type: research
The liver in sickle cell disease
Presse Med. 2023 Nov 17:104212. doi: 10.1016/j.lpm.2023.104212. Online ahead of print.ABSTRACTLiver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt re...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Christophe Duvoux Lorraine Blaise Jean-Jacques Matimbo Francky Mubenga Norbert Ngongang Monika Hurtova Alexis Laurent Julien Calderaro Edouard Reizine Alain Luciani Anoosha Habibi Dora Bachir Geoffroy Vole Justine Gellen-Dautremer Vincent Leroy Eric Leves Source Type: research
The Growing Challenge of RBC Alloimmunization in the Management of Patients with Sickle Cell Disease
Presse Med. 2023 Nov 17:104211. doi: 10.1016/j.lpm.2023.104211. Online ahead of print.ABSTRACTRBC transfusion remains a cornerstone in the treatment of sickle cell disease (SCD). However, as with many interventions, transfusion of RBCs is not without risk. Allogeneic RBC exposure can result in the development of alloantibodies, which can make it difficult to find compatible RBCs for future transfusion and increases the likelihood of life-threatening complications. The development of RBC alloantibodies occurs when a patient's immune system produces alloantibodies against foreign alloantigens present on allogeneic RBCs. Desp...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Daniel Y Chang Zakary Wankier Connie M Arthur Sean R Stowell Source Type: research
The liver in sickle cell disease
Presse Med. 2023 Nov 17:104212. doi: 10.1016/j.lpm.2023.104212. Online ahead of print.ABSTRACTLiver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt re...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Christophe Duvoux Lorraine Blaise Jean-Jacques Matimbo Francky Mubenga Norbert Ngongang Monika Hurtova Alexis Laurent Julien Calderaro Edouard Reizine Alain Luciani Anoosha Habibi Dora Bachir Geoffroy Vole Justine Gellen-Dautremer Vincent Leroy Eric Leves Source Type: research
The Growing Challenge of RBC Alloimmunization in the Management of Patients with Sickle Cell Disease
Presse Med. 2023 Nov 17:104211. doi: 10.1016/j.lpm.2023.104211. Online ahead of print.ABSTRACTRBC transfusion remains a cornerstone in the treatment of sickle cell disease (SCD). However, as with many interventions, transfusion of RBCs is not without risk. Allogeneic RBC exposure can result in the development of alloantibodies, which can make it difficult to find compatible RBCs for future transfusion and increases the likelihood of life-threatening complications. The development of RBC alloantibodies occurs when a patient's immune system produces alloantibodies against foreign alloantigens present on allogeneic RBCs. Desp...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Daniel Y Chang Zakary Wankier Connie M Arthur Sean R Stowell Source Type: research
The liver in sickle cell disease
Presse Med. 2023 Nov 17:104212. doi: 10.1016/j.lpm.2023.104212. Online ahead of print.ABSTRACTLiver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt re...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Christophe Duvoux Lorraine Blaise Jean-Jacques Matimbo Francky Mubenga Norbert Ngongang Monika Hurtova Alexis Laurent Julien Calderaro Edouard Reizine Alain Luciani Anoosha Habibi Dora Bachir Geoffroy Vole Justine Gellen-Dautremer Vincent Leroy Eric Leves Source Type: research
The Growing Challenge of RBC Alloimmunization in the Management of Patients with Sickle Cell Disease
Presse Med. 2023 Nov 17:104211. doi: 10.1016/j.lpm.2023.104211. Online ahead of print.ABSTRACTRBC transfusion remains a cornerstone in the treatment of sickle cell disease (SCD). However, as with many interventions, transfusion of RBCs is not without risk. Allogeneic RBC exposure can result in the development of alloantibodies, which can make it difficult to find compatible RBCs for future transfusion and increases the likelihood of life-threatening complications. The development of RBC alloantibodies occurs when a patient's immune system produces alloantibodies against foreign alloantigens present on allogeneic RBCs. Desp...
Source: Presse Medicale - November 19, 2023 Category: General Medicine Authors: Daniel Y Chang Zakary Wankier Connie M Arthur Sean R Stowell Source Type: research
Screening for cognitive impairment in adults with sickle cell disease: a systematic review and meta-analysis
We report a pooled prevalence of 38% [14-62%] of suspected cognitive impairment. We discuss the relative benefits and limitations of the different screening tools to help clinicians select an adapted approach tailored to their specific patients' needs. Further studies are needed to establish and validate cognitive screening strategies in patients with diverse cultural and educational backgrounds.PMID:37979834 | DOI:10.1016/j.lpm.2023.104207 (Source: Presse Medicale)
Source: Presse Medicale - November 18, 2023 Category: General Medicine Authors: Maryline Couette Justine Roy Damien Oudin Doglioni Olena Bereznyakova Christian Stapf Gregory Jacquin Val érie Fraïle Philippe Desmarais Sara-Maude Desforges Lahoud Touma B énédicte Nauche Pablo Bartolucci Kevin H M Kuo St éphanie Forté Source Type: research
Main Nose-Throat-Ears, and ophthalmic features in Sickle Cell Disease Children
Presse Med. 2023 Nov 16:104210. doi: 10.1016/j.lpm.2023.104210. Online ahead of print.NO ABSTRACTPMID:37979835 | DOI:10.1016/j.lpm.2023.104210 (Source: Presse Medicale)
Source: Presse Medicale - November 18, 2023 Category: General Medicine Authors: L éon Tshilolo Joseph Kelekele Source Type: research
Presentation of polymyalgia rheumatica and large joint polyarthritis in the spectrum of hyper-IgG4-related disease: A case report and literature review
Presse Med. 2023 Nov 16:104206. doi: 10.1016/j.lpm.2023.104206. Online ahead of print.NO ABSTRACTPMID:37979836 | DOI:10.1016/j.lpm.2023.104206 (Source: Presse Medicale)
Source: Presse Medicale - November 18, 2023 Category: General Medicine Authors: Remi Roeland Marine Trenec Samuel Deshayes Hubert de Boysson Kevin Prigent Achille Aouba Source Type: research
Complement involvement in sickle cell disease
Presse Med. 2023 Nov 14:104205. doi: 10.1016/j.lpm.2023.104205. Online ahead of print.ABSTRACTSickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. This review is centered on exploring the role of the inflammatory complement system in the pathophysiology of SCD. Our goal is to offer a comprehensive summary of the existing evidence regarding complement activation ...
Source: Presse Medicale - November 16, 2023 Category: General Medicine Authors: Marie-Sophie Meuleman Lubka T Roumenina Anne Grunenwald Source Type: research
