Background: People with sickle cell disease are particularly susceptible to infection. Infants and very young children are especially vulnerable, and the Cooperative Study of Sickle Cell Disease observed an incidence rate of 10 per 100 patient years of pneumococcal septicaemia in children under the age of three. Vaccines, including customary pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic penicillin regimes may be advisable for this population.
Objectives: To assess the effects of prophylactic antibiotic regimes for preventing pneumococcal infection in children with sickle cell disease.
Search Strategy: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references identified from comprehensive electronic database searches, hand searching relevant journals and hand searching abstract books of conference proceedings. Date of the most recent search: December 2001.
Selection Criteria: All randomised or quasi-randomised controlled trials comparing prophylactic antibiotics to prevent pneumococcal infection in children with sickle cell disease with placebo, no treatment or a comparator drug.
Data Collection And Analysis: Both reviewers independently extracted data and assessed trial quality.
Main Results: Five trials were identified by the initial search, of which three trials met the inclusion criteria. All of the included trials showed a reduced risk of infection in children with sickle cell disease (SS or Sb0Thal) receiving prophylactic penicillin. For initiation of treatment the odds ratio was 0.37 (95% CI 0.16, 0.86), and for withdrawal OR= 0.49 (95% CI 0.09, 2.71). Adverse drug effects were rare and minor. Rates of pneumococcal infection were found to be relatively low in children over the age of five.
Reviewer's Conclusions: Prophylactic penicillin significantly reduces risk of pneumococcal infection in children with homozygous sickle cell disease, and is associated with minimal adverse reactions. Further research may help to determine the ideal age to safely withdraw penicillin.
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http://dx.doi.org/10.1002/14651858.CD003427 | 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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