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View article: Sex and genotype specificities of microvasculature and muscle remodeling in sub-Saharan sickle cell trait and disease
Sex and genotype specificities of microvasculature and muscle remodeling in sub-Saharan sickle cell trait and disease Open
Sickle cell disease (SCD) is associated with microvascular and muscle remodeling as well as reduced exercise tolerance. However, SCD repercussions on microvasculature and muscle in women remain unknown. This study aimed to compare (1) musc…
View article: Hydroxyurea is associated with later onset of acute splenic sequestration crisis in sickle cell disease: Lessons from the European Sickle Cell Disease Cohort—Hydroxyurea (<scp>ESCORT‐HU</scp>) study
Hydroxyurea is associated with later onset of acute splenic sequestration crisis in sickle cell disease: Lessons from the European Sickle Cell Disease Cohort—Hydroxyurea (<span>ESCORT‐HU</span>) study Open
Acute splenic sequestration crisis (ASSC) is a potentially life‐threatening complication of sickle cell disease (SCD), typically occurring in young patients under 5 years of age, with a median age at first episode of less than 2 years. Bec…
View article: Fetal Hemoglobin Decrease during Voxelotor Treatment
Fetal Hemoglobin Decrease during Voxelotor Treatment Open
Sickle cell disease (SCD) is an inherited disorder caused by a point mutation in the β-globin gene leading to the formation of hemoglobin S (HbS). The polymerization of deoxygenated HbS is the first event in the molecular pathogenesis of S…
View article: Genetic reversal of the globin switch concurrently modulates both fetal and sickle hemoglobin and reduces red cell sickling
Genetic reversal of the globin switch concurrently modulates both fetal and sickle hemoglobin and reduces red cell sickling Open
We previously reported initial clinical results of post-transcriptional gene silencing of BCL11A expression (NCT 03282656) reversing the fetal to adult hemoglobin switch. A goal of this approach is to increase fetal hemoglobin (HbF) expres…
View article: P1473: CLINICALLY RELEVANT HEMOGLOBIN RESPONSE IN ADULTS WITH PYRUVATE KINASE DEFICIENCY TREATED WITH MITAPIVAT – A SUB-ANALYSIS OF THE ACTIVATE TRIAL
P1473: CLINICALLY RELEVANT HEMOGLOBIN RESPONSE IN ADULTS WITH PYRUVATE KINASE DEFICIENCY TREATED WITH MITAPIVAT – A SUB-ANALYSIS OF THE ACTIVATE TRIAL Open
Topic: 28. Enzymopathies, membranopathies and other anemias Background: Mitapivat, an oral, allosteric activator of pyruvate kinase (PK), is approved by the US FDA for the treatment of hemolytic anemia in adults with PK deficiency and by t…
View article: Characterization of genetic variants in the <i>EGLN1/PHD2</i> gene identified in a European collection of patients with erythrocytosis
Characterization of genetic variants in the <i>EGLN1/PHD2</i> gene identified in a European collection of patients with erythrocytosis Open
Hereditary erythrocytosis is a rare hematologic disorder characterized by an excess of red blood cell production. Here we describe a European collaborative study involving a collection of 2,160 patients with erythrocytosis sequenced in ten…
View article: Outcomes of Pregnancy in Sickle Cell Disease Patients: Results from the Prospective ESCORT-HU Cohort Study
Outcomes of Pregnancy in Sickle Cell Disease Patients: Results from the Prospective ESCORT-HU Cohort Study Open
Sickle cell disease (SCD) refers to a group of inherited hemoglobin disorders in which sickle red blood cells display altered deformability, leading to a significant burden of acute and chronic complications, such as vaso-occlusive pain cr…
View article: Comprehensive <i>in silico</i> and functional studies for classification of <i>EPAS1/HIF2A</i> genetic variants identified in patients with erythrocytosis
Comprehensive <i>in silico</i> and functional studies for classification of <i>EPAS1/HIF2A</i> genetic variants identified in patients with erythrocytosis Open
Gain-of-function mutations in the EPAS1/HIF2A gene have been identified in patients with hereditary erythrocytosis that can be associated with the development of paraganglioma, pheochromocytoma and somatostatinoma. In the present study, we…
View article: Variation and impact of polygenic hematologic traits in monogenic sickle cell disease
Variation and impact of polygenic hematologic traits in monogenic sickle cell disease Open
Several of the complications observed in sickle cell disease (SCD) are influenced by variation in hematologic traits (HT), such as fetal hemoglobin (HbF) level and neutrophil count. Previous large-scale genome-wide association studies carr…
View article: P1544: BONE MINERAL DENSITY REMAINS STABLE IN PYRUVATE KINASE DEFICIENCY PATIENTS RECEIVING LONG-TERM TREATMENT WITH MITAPIVAT
P1544: BONE MINERAL DENSITY REMAINS STABLE IN PYRUVATE KINASE DEFICIENCY PATIENTS RECEIVING LONG-TERM TREATMENT WITH MITAPIVAT Open
Background: Hereditary pyruvate kinase (PK) deficiency results in chronic hemolysis that may lead to reduced bone mineral density (BMD). Mitapivat is an oral activator of PK in red blood cells (RBC) that improves hemoglobin concentration a…
View article: Mitapivat versus Placebo for Pyruvate Kinase Deficiency
Mitapivat versus Placebo for Pyruvate Kinase Deficiency Open
In patients with pyruvate kinase deficiency, mitapivat significantly increased the hemoglobin level, decreased hemolysis, and improved patient-reported outcomes. No new safety signals were identified in the patients who received mitapivat.…
View article: Variation and impact of polygenic hematological traits in monogenic sickle cell disease
Variation and impact of polygenic hematological traits in monogenic sickle cell disease Open
Several complications observed in sickle cell disease (SCD) are influenced by variation in hematological traits (HT), such as fetal hemoglobin (HbF) level and neutrophil count. Previous large-scale genome-wide association studies carried o…
View article: Lower Muscle and Blood Lactate Accumulation in Sickle Cell Trait Carriers in Response to Short High-Intensity Exercise
Lower Muscle and Blood Lactate Accumulation in Sickle Cell Trait Carriers in Response to Short High-Intensity Exercise Open
It remains unclear whether sickle cell trait (SCT) should be considered a risk factor during intense physical activity. By triggering the polymerization-sickling-vaso-occlusion cascade, lactate accumulation-associated acidosis in response …
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VINITI (All-Russian Institute of Science & Technological Information) • Web of Science ® (Thomson ISI).For submission instructions, subscription and all other information visit: www.wileyonlinelibrary.com/ajh.Wiley's Corporate Citizenship …
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View article: Alpha haemoglobin‐stabilising protein concentration in the red blood cells of patients with sickle cell anaemia with and without hydroxycarbamide treatment
Alpha haemoglobin‐stabilising protein concentration in the red blood cells of patients with sickle cell anaemia with and without hydroxycarbamide treatment Open
Summary Alpha haemoglobin‐stabilising protein (AHSP) is a key chaperone synthesised in red blood cell (RBC) precursors. Many studies have reported AHSP as a potential biomarker of various diseases. AHSP gene expression has been studied in …
View article: Epidemiology and disease burden of sickle cell disease in France: A descriptive study based on a French nationwide claim database
Epidemiology and disease burden of sickle cell disease in France: A descriptive study based on a French nationwide claim database Open
Context Sickle cell disease (SCD) is a severe hematological disorder. The most common acute complication of SCD is vaso-occlusive crisis (VOC), but SCD is a systemic disease potentially involving all organs. SCD prevalence estimates rely m…
View article: <scp>Real‐Life</scp> experience with hydroxyurea in patients with sickle cell disease: Results from the prospective <scp>ESCORT‐HU</scp> cohort study
<span>Real‐Life</span> experience with hydroxyurea in patients with sickle cell disease: Results from the prospective <span>ESCORT‐HU</span> cohort study Open
Several controlled studies have evidenced good efficacy and short‐term and mid‐term safety profiles for hydroxyurea (HU), which has become the cornerstone for prevention of sickle‐cell disease (SCD)‐related vaso‐occlusive crises. However, …
View article: Cardiovascular phenotypes predict clinical outcomes in sickle cell disease: An echocardiography‐based cluster analysis
Cardiovascular phenotypes predict clinical outcomes in sickle cell disease: An echocardiography‐based cluster analysis Open
This study sought to link cardiac phenotypes in homozygous Sickle Cell Disease (SCD) patients with clinical profiles and outcomes using cluster analysis. We analyzed data of 379 patients included in the French Etendard Cohort. A cluster an…
View article: Impact of pre‐eclampsia on renal outcome in sickle cell disease patients
Impact of pre‐eclampsia on renal outcome in sickle cell disease patients Open
Summary The long‐term consequences of pre‐eclampsia (PrE) for renal function have never been determined in patients with sickle cell disease (SCD). Between 2008 and 2015, we screened 306 pregnancies in women with SCD and identified 40 with…
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10,651 (The Americas), US$11,067 (Rest of World), €7,145(Europe), £5,652 (UK).Prices are exclusive of tax.Asia-Pacific GST,
View article: Transcranial color‐coded duplex sonography reliably identifies intracranial vasculopathy in adult patients with sickle cell disease
Transcranial color‐coded duplex sonography reliably identifies intracranial vasculopathy in adult patients with sickle cell disease Open
In order to prevent stroke, screening for disease‐related intracranial vasculopathy using Doppler ultrasound is recommended in sickle‐cell disease (SCD) children. How to screen such vasculopathy in adults remains largely unknown. The objec…
View article: Whole‐blood CCR7 expression and chemoattraction in red blood cell alloimmunization
Whole‐blood CCR7 expression and chemoattraction in red blood cell alloimmunization Open
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View article: Whole-blood phenotyping to assess alloimmunization status in transfused sickle cell disease patients
Whole-blood phenotyping to assess alloimmunization status in transfused sickle cell disease patients Open
It is essential to limit hemolytic transfusion reactions in polytransfused individuals, and the prevention of alloimmunization is a key solution. CD4+ T lymphocyte (TL) markers, particularly follicular T helper (Tfh) cells, may differentia…