Search Results - "Patel, Niren"
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Characteristics of sickle cell patients with frequent emergency department visits and hospitalizations
Published in PloS one (22-02-2021)“…Vaso-occlusive episodes (VOEs) are a hallmark of sickle cell disease (SCD), and account for >90% of health care encounters for this patient population. The…”
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Hydroxyurea differentially modulates activator and repressors of γ-globin gene in erythroblasts of responsive and non-responsive patients with sickle cell disease in correlation with Index of Hydroxyurea Responsiveness
Published in Haematologica (Roma) (01-12-2017)“…Hydroxyurea (HU), the first of two drugs approved by the US Food and Drug Administration for treating patients with sickle cell disease (SCD), produces…”
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Two New γ Chain Variants: Hb F-Augusta GA [Gγ59(E3)Lys → Arg; HBG2: c.179A > G] and Hb F-Port Royal-II [Aγ125(H3)Glu → Ala; HBG1: c.377A > C]
Published in Hemoglobin (01-10-2014)“…Abstract The total number of hemoglobin (Hb) variants so far reported to the HbVar database is 1598 (April 9 2014) and 130 of them are fetal Hb variants. Fetal…”
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Proteomics of Plasma from Patients with Sickle Cell Disease with and without Chronic Pain: A Pilot Biomarker Discovery Study
Published in Blood (15-11-2022)Get full text
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Downregulation of Cytoskeletal Protein Kazrin in Lung Endothelial Cells of Preclinical Sickle Cell Disease Mouse Model - Essential Role of Endothelial Barrier Function
Published in Blood (13-11-2019)“…Background: Lung capillary barrier dysfunction is common to all causes of acute chest syndrome (ACS), a leading cause of morbidity and mortality in individuals…”
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Preclinical and Clinical Use of AB1, a DNMT1 Protein Depleter, to Upregulate Fetal Hemoglobin in Townes Sickle Cell Disease (SCD) Mice and Patients with SCD
Published in Blood (02-11-2023)“…Background: Sickle Cell Disease (SCD) is an inherited hematologic disorder characterized by lifelong hemolysis, vaso-occlusion and severe complications…”
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Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia—TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial
Published in The Lancet (British edition) (13-02-2016)“…Summary Background For children with sickle cell anaemia and high transcranial doppler (TCD) flow velocities, regular blood transfusions can effectively…”
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Initial Results in a Phase 1b Trial of PB-04 in Sickle Cell Disease Demonstrate Fetal Hemoglobin Induction, Additive Activity with Hydroxyurea, and Improved Red Blood Cell Sickling Parameters
Published in Blood (02-11-2023)“…Extensive evidence has shown fetal hemoglobin (HbF) is a major modulator of sickle cell disease (SCD) phenotype. Clinical benefit with any increment of HbF was…”
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ETA Receptor Blockade and Vascular Function in Patients with Sickle Cell Disease
Published in Blood (05-11-2020)“…Endothelin-1 (ET1) is a potent vasoconstrictor, mitogen, proinflammatory mediator, and a mediator of nociception, whose synthesis is increased by hypoxia,…”
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Erythroferrone (ERFE) and Hepcidin Levels in Sickle Cell Disease with and without Transfusional Iron Overload
Published in Blood (08-12-2017)“…Transfusional iron (Fe) overload is not rare among patients with sickle cell disease (SCD) and can lead to significant morbidity and even mortality. We…”
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Correlation of Serum Ferritin Levels with Liver Iron Concentration By MRI Measurement in Sickle Cell Patients with Transfusional Iron Overload
Published in Blood (29-11-2018)“…Background: Recent reports from the thalassemia literature suggest that serum ferritin may be a poor and possibly misleading measure of total iron store in…”
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Regulation of iron homeostasis through the erythroferrone‐hepcidin axis in sickle cell disease
Published in British journal of haematology (01-06-2020)“…Summary Sickle cell disease (SCD) has a distinct pattern of transfusional iron overload (IO) when compared to transfusion‐dependent β‐thalassaemia major (TDT)…”
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Survival of transfused red blood cells from a donor with alpha‐thalassemia trait in a recipient with sickle cell disease
Published in Transfusion (Philadelphia, Pa.) (01-06-2024)“…Background Post‐transfusion survival of donor red blood cells (RBCs) is important for effective chronic transfusion therapy in conditions including sickle cell…”
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Characteristics and potential biomarkers of adult sickle cell patients with chronic pain
Published in European journal of haematology (01-10-2020)“…Objectives In this study, we investigated the evolution of chronic pain in sickle cell patients (SCD) as an age‐dependent phenomenon and studied the frequency…”
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Characteristics and Potential Biomarkers of Adult Sickle Cell Patients with Chronic Pain
Published in Blood (29-11-2018)“…A paradigm shift is occurring in our understanding of pain in patients with sickle cell disease (SCD). Vaso-occlusive episodes (VOEs) are crises of acute…”
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Coinheritance of Alpha-3.7 Deletion and Hb Questembert [131alpha-2 Tct > CCT](Ser131>Pro)] in an African American Patient Results in an Alpha Thalassemia Phenotype
Published in Blood (03-12-2015)“…Patients with Hemoglobin Questembert carry a Ser131Pro mutation in one of their alpha globin chains encoded by a TCT-CCT mutation of one of their alpha 2…”
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Characteristics and Potential Biomarkers for Chronic Pain in Patients with Sickle Cell Disease
Published in Blood (03-12-2015)“…Vaso-occlusive episodes (VOE) or pain crises are a hallmark of sickle cell disease (SCD), with increasing recognition that a significant portion of SCD…”
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Analytic Characteristics and Performance of Novel Immunoassay Point-of-Care Tests for Early Diagnosis of Sickle Cell Disease: A Systematic Review
Published in Point of care (01-09-2020)Get full text
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Diagnostic Challenges in a Low a2 β-Thalassemia Case
Published in Blood (06-12-2014)“…Inheritance of two β-thal alleles results in transfusion dependent anemia, growth retardation, and skeletal abnormalities (β-thal major), whereas…”
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Characteristics of Sickle Cell Patients with Frequent ED Visits and Hospitalizations: Demographic, Clinical, Laboratory and Psycho-Social Aspects
Published in Blood (02-12-2016)“…Vaso-occlusive episodes (VOE), are considered a hallmark of sickle cell disease (SCD), and account for >90% of health care encounters for this patient…”
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