Sickle cell disease (SCD) is the most common inherited haemoglobinopathy wordwide, with the highest prevalence in sub-Saharan Africa. Due to the lack of national strategies and scarcity of diagnostic tools in resource-limited settings, the disease may be significantly underdiagnosed. We carried out a 6-month retrospective review of paediatric admissions in a district hospital in northern Sierra Leone. Our aim was to identify patients with severe anaemia, defined as Hb < 7 g/dl, and further analyse the records of those tested for SCD. Of the 273 patients identified, only 24.5% had had an Emmel test, among which 34.3% were positive. Furthermore, only 17% of patients with a positive Emmel test were discharged on prophylactic antibiotics. Our study shows that increased awareness of SCD symptoms is required in high-burden areas without established screening programmes. In addition, the creation or strengthening of follow-up programmes for SCD patients is essential for disease control.
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http://dx.doi.org/10.1093/omcr/omz061 | DOI Listing |
Asian J Transfus Sci
September 2022
Department of Zoology, CCS HAU, Hisar, Haryana, India.
Context: Hemoglobinopathies are the most common heterogeneous group of monogenetic disorder in the world and its prevalence varies with geographical regions. India is developing country and many studies show a significant burden of hemoglobinopathies in India.
Aims: The aim of the present study was to check the prevalence of various hemoglobinopathies in anemic subjects using high-performance liquid chromatography (HPLC) method in Pune region which has multiple ethnic population groups from all parts of India.
Asian J Transfus Sci
December 2022
Department of Transfusion Medicine, IMS and SUM Hospital, Siksha 'O' Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India.
Red cell exchange is important to treat acutely ill sickle cell patients, but it is time-consuming. An automated red cell exchange technique using cell separators developed by different manufacturers helps in removal of sickled hemoglobin and improving blood viscosity. The use of these cell separators permits automated red cell exchange to be performed safely and smoothly with the isovolemic hemodilution.
View Article and Find Full Text PDFGenet Med Open
September 2024
St. Luke's Health System, Boise, ID.
Transfusion
January 2025
Department of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.
Background: Prior studies have evaluated transfusion recipient variables impacting red blood cell (RBC) alloimmunization, but few focused on potentially modifiable blood donor or blood component variables.
Study Design And Methods: Data from the Recipient Epidemiology and Donor Evaluation Study (REDS)-III, which links donor, component, and patient data in an integrated database, were accessed. For any given RBC unit with sufficient blood donor and component data, we determined if the transfusion recipient experienced a new RBC alloimmunization event ("case") within 16 weeks of the transfusion or not ("control").
JAMA Netw Open
January 2025
Department of Neurology, Washington University in St Louis School of Medicine, St Louis, Missouri.
Importance: Both sickle cell anemia (SCA) and socioeconomic status have been associated with altered brain structure and cognitive disability, yet precise mechanisms underlying these associations are unclear.
Objective: To determine whether brains of individuals with and without SCA appear older than chronological age and if brain age modeling using brain age gap (BAG) can estimate cognitive outcomes and mediate the association of socioeconomic status and disease with these outcomes.
Design, Setting, And Participants: In this cross-sectional study of 230 adults with and without SCA, individuals underwent brain magnetic resonance imaging (MRI) and cognitive assessment.
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