Scientific Literature
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Management of the Sickle Cell Trait: An Opinion by Expert Panel Members
The number of individuals with the sickle cell trait exceeds 300 million worldwide, making sickle cell disease one of the most common monogenetic diseases globally. Because of the high frequency…
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Economic and Clinical Burden of Managing Transfusion-dependent β-Thalassemia in the United States
Aims: To describe clinical complications, treatment use, healthcare resource utilization (HCRU), and costs among patients with transfusion-dependent β-thalassemia (TDT) in the United States. Materials and methods: Merative MarketScan Databases were used to…
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Efficacy and Safety of Early-start Deferiprone in Infants and Young Children with Transfusion-dependent Beta Thalassemia: Evidence for Iron Shuttling to Transferrin in a Randomized, Double-blind, Placebo-controlled, Clinical Trial (START)
Children with transfusion‐dependent thalassemia (TDT) require regular blood transfusions that, without iron‐chelation therapy, lead to iron‐overload toxicities. Current practice delays chelation therapy (late‐start) until reaching iron overload (serum ferritin ≥1000 μg/L)…
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Burden of Aging: Health Outcomes Among Adolescents and Young Adults With Sickle Cell Disease
Although ≈95% of children with sickle cell disease (SCD) will reach adulthood in high-income settings, adolescents and young adults (AYA) face difficulties in establishing adult care and experience progression of disease…
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Αlpha-thalassemia Genotypes in Vietnam: A Report of 12,030 Pregnant Women and Their Husbands Performing Prenatal Screening for Alpha-thalassemia
BACKGROUND: Αlpha (α)-thalassemia is a global health concern, and improving screening methods is crucial for disease prevention. This study aimed to assess α-thalassemia genotypes and evaluate the effectiveness of various…
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Low-dose Warfarin May Be a Life-saving Treatment Regimen in Sickle Cell Diseases
The sickled or just hardened red blood cells (RBCs)-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures and sudden deaths even at childhood. Although RBCs suspensions and…
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Nursing Interventions to Improve Quality of Life Among Children and Adolescents with Thalassemia: A Scoping Review
Thalassemia is an inherited blood disorder affecting hemoglobin synthesis which leads to chronic hemolysis that can reduce the quality of life of children with it due to the disease and…
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Epidemiology of Clinically Significant Forms of alpha- and beta-Thalassemia: A Global Map of Evidence and Gaps
This systematic literature review assessed the global prevalence and birth prevalence of clinically significant forms of alpha- and beta-thalassemia. Embase, MEDLINE, and the Cochrane Library were searched for observational studies…
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Examining Mental Health, Education, Employment, and Pain in Sickle Cell Disease
Importance: Pain related to sickle cell disease (SCD) is complex and associated with social determinants of health. Emotional and stress-related effects of SCD impact daily quality of life and the frequency…
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Venous Thromboembolism Prophylaxis in Sickle Cell Disease: A Multicenter Cohort Study of Adolescent Inpatients
Patients with sickle cell disease (SCD) are predisposed to a hypercoagulable state. Despite the increased risk of venous thromboembolism in the SCD population, there is limited evidence available to guide…
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