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Published on: February 23, 2014
Infection caused by Streptococcus pneumoniae in children with sickle cell disease: epidemiology, immunologic
W Y Wong1, G D Overturf, D R Powars
1Department of Pediatrics, University of Southern California Medical Center, Los Angeles 90033.
Insights
Children with sickle cell anemia face a high risk of Streptococcus pneumoniae infections. Current vaccines show limited efficacy, necessitating the development of alternative vaccines and improved antibiotic prophylaxis strategies.
Area of Science:
- Pediatrics
- Infectious Diseases
- Hematology
Background:
- Children with sickle cell anemia (SS genotype) under 5 years old have a 30-100 times higher incidence of invasive Streptococcus pneumoniae infections compared to healthy children.
- Splenic dysfunction is identified as the primary factor contributing to the elevated risk of pneumococcal infections in this vulnerable population.
- Potential contributing factors include abnormalities in immunologic defense mechanisms, such as complement pathways and antibody synthesis, and possible viral predispositions.
Purpose of the Study:
- To highlight the significant risk of invasive Streptococcus pneumoniae infections in young children with sickle cell anemia.
- To evaluate the efficacy of current pneumococcal vaccines and antibiotic prophylaxis in this patient group.
- To underscore the need for novel vaccine strategies and optimized prophylactic regimens.
Main Methods:
- Review of existing literature and clinical data on Streptococcus pneumoniae infections in pediatric sickle cell anemia patients.
- Analysis of the prevalence of specific pneumococcal serotypes causing invasive disease.
- Assessment of the protective efficacy of current pneumococcal vaccines and antibiotic prophylaxis.
Main Results:
- The incidence rate of invasive Streptococcus pneumoniae infection is 6.9 per 100 patient-years in children with sickle cell anemia under 5.
- Serotypes 6, 14, 18, 19, and 23 are the most prevalent and are frequently implicated in vaccine failures.
- Current pneumococcal vaccines offer only modest protection, and antibiotic prophylaxis, while effective, presents challenges in compliance and optimal duration.
Conclusions:
- There is a critical need for alternative pneumococcal vaccines tailored for children with sickle cell anemia.
- Current antibiotic prophylaxis regimens require improvement to address compliance issues and ensure complete protection.
- Further research is necessary to determine the optimal duration and strategies for antibiotic prophylaxis in this population.
Abstract:
The incidence of invasive infection due to Streptococcus pneumoniae is 6.9 infections per 100 patient-years among children with sickle cell anemia (SS genotype) who are less than 5 years of age; this rate is 30-100 times that which would be expected in a healthy population of this age. Splenic dysfunction is the major contributor to the increased risk. Postulated abnormalities of immunologic defense mechanisms, including synthesis of polyclonal IgG and IgM, the alternative complement pathway, opsonic activity, and T and B cell interaction, may also enhance risk. Preceding or concomitant viral infection is suspected of predisposing to pneumococcal infection, but no definitive data are available. The most prevalent pneumococcal serotypes causing disease in this setting include types 6, 14, 18, 19, and 23; these same serotypes are most frequently involved in "vaccine failure." Current evidence demonstrates only modest protective efficacy for contemporary pneumococcal vaccines in young patients with sickle cell anemia; thus alternative vaccines are required. Convincing evidence for a protective effect of antibiotic prophylaxis has been obtained in limited time trials. However, presently used prophylactic regimens pose problems related to compliance and provide imperfect protection; moreover, their optimal duration remains unknown.
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