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Acute chest syndrome (ACS) in sickle cell disease (SCD): pathogenesis and pharmacotherapies in early clinical development.
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Department of Women and Children's Health, School of Life Course and Population Sciences, Faculty of Life Sciences and Medicine, King's College London, London, UK.
Introduction: Sickle cell disease (SCD) is a monogenic disorder caused by a point mutation in the gene, leading to the production of sickle hemoglobin (HbS). Under hypoxic or acidic conditions, HbS polymerizes within erythrocytes, leading to a series of downstream events resulting in tissue ischemia. Acute chest syndrome (ACS) is a severe and often life-threatening complication of SCD and the leading cause of intensive care unit admission and mortality in children.
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Family Medicine, Aeon Medical Center, Abuja, NGA.
Hemoglobinopathy has a diverse clinical presentation and complications, and is severe among individuals with the homozygous form. It is the most common cause of chronic anemia among affected individuals. Hemoglobinopathy is an inherited blood disorder arising from mutations in globin genes and is broadly categorized into those involving structural changes that produce abnormal hemoglobin variants or defects in globin chain production.
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Division of Endocrinology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Objective: Continuous glucose monitoring (CGM) systems reduce self-monitoring burden compared with glucose meter use but have limitations when used after total pancreatectomy with islet autotransplantation (TPIAT). An example is false elevation of sensor readings after hydroxyurea (HU) administration, a medication often used after TPIAT. This study compared user experiences of CGM not affected by HU with CGM affected by HU in pediatric patients post-TPIAT.
View Article and Find Full Text PDFEffectiveness of hydroxyurea (hydroxycarbamide) in a national sickle cell disease programme in Ghana.
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Department of Health Policy Planning and Management, University of Ghana School of Public Health, College of Health Sciences, University of Ghana, Accra, Ghana.
Hydroxyurea (hydroxycarbamide; HU) has been shown to be a safe and effective drug for individuals living with sickle cell disease (SCD) in Africa. However, reports of large-scale use of HU outside of controlled trial settings are limited on the continent. Access to HU in Ghana has improved through a major public-private partnership aimed at enhancing holistic care for communities affected by SCD.
View Article and Find Full Text PDFParaspinal Extramedullary Hematopoiesis in a Transfusion-Dependent Beta-Thalassemia Patient: A Case Report.
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Faculty of Medicine, University of Jordan, Amman, JOR.
Extramedullary hematopoiesis (EMH) is a compensatory mechanism in chronic anemias, such as transfusion-dependent beta-thalassemia (TDT), most commonly affecting the liver and spleen. Paraspinal EMH is rare and may lead to spinal cord compression, resulting in neurological deficits. We present a 26-year-old male patient with longstanding TDT who developed progressive bilateral lower limb weakness, pelvic paresthesia, and acute urinary retention.
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