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View article: P1438: L-GLUTAMINE: A NOVEL AND EFFECTIVE THERAPEUTIC TOOL FOR VOC REDUCTION IN SCD
P1438: L-GLUTAMINE: A NOVEL AND EFFECTIVE THERAPEUTIC TOOL FOR VOC REDUCTION IN SCD Open
Topic: 26. Sickle cell disease Background: L-glutamine plays an important role in the defense against oxidative stress, which is a contributor to the pathophysiology of sickle cell disease (SCD). Our metabolomic study also showed that L-gl…
View article: Deregulated JAK3 mediates growth advantage and hemophagocytosis in extranodal nasal-type natural killer/T-cell lymphoma
Deregulated JAK3 mediates growth advantage and hemophagocytosis in extranodal nasal-type natural killer/T-cell lymphoma Open
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View article: Drepadom - Establishment of Home Care Services and Hospitalizations for Sickle Cell Disease Patients As Standard Care Since the Covid-19 Pandemic
Drepadom - Establishment of Home Care Services and Hospitalizations for Sickle Cell Disease Patients As Standard Care Since the Covid-19 Pandemic Open
INTRODUCTION Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cause of patients' emergency room (ER) admissions and hospitalizations. Acute chest syndrome (ACS) is a life-threatening complication that can oc…
View article: Deficient mitophagy pathways in sickle cell disease
Deficient mitophagy pathways in sickle cell disease Open
Summary Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS‐mito + ) in their mature red blood cells, while 60% …
View article: Downregulation of Mitochondrial TSPO Inhibits Mitophagy and Reduces Enucleation During Human Terminal Erythropoiesis
Downregulation of Mitochondrial TSPO Inhibits Mitophagy and Reduces Enucleation During Human Terminal Erythropoiesis Open
Translocator protein (TSPO) and voltage dependent anion channels (VDAC) are two proteins forming a macromolecular complex in the outer mitochondrial membrane that is involved in pleiotropic functions. Specifically, these proteins were desc…
View article: Effects of sphingolipids overload on red blood cell properties in Gaucher disease
Effects of sphingolipids overload on red blood cell properties in Gaucher disease Open
Gaucher disease (GD) is a genetic disease with mutations in the GBA gene that encodes glucocerebrosidase causing complications such as anaemia and bone disease. GD is characterized by accumulation of the sphingolipids (SL) glucosylceramide…
View article: Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors
Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors Open
Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between …