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Bacteremia in sickle hemoglobinopathies.

H S Zarkowsky, D Gallagher, F M Gill

    The Journal of Pediatrics
    |October 1, 1986
    PubMed
    Summary

    Bacteremia is common in children with sickle cell disease, especially from Streptococcus pneumoniae. Early penicillin prophylaxis may reduce pneumococcal bacteremia incidence in young children with sickle cell anemia.

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    Area of Science:

    • Hematology
    • Infectious Diseases
    • Pediatrics

    Background:

    • Sickle hemoglobinopathies, including sickle cell anemia (SS) and sickle cell-hemoglobin C (SC) disease, significantly increase the risk of bacteremia.
    • Understanding age-specific incidence and causative pathogens is crucial for managing infections in these vulnerable patients.
    • Streptococcus pneumoniae has historically been a major cause of severe infections in children with sickle cell disease.

    Purpose of the Study:

    • To determine the age-specific incidence rates of bacteremia in patients with sickle cell anemia and sickle cell-hemoglobin C disease.
    • To identify the predominant pathogens causing bacteremia across different age groups.
    • To evaluate the association of specific pathogens with clinical conditions and assess the potential impact of prophylactic penicillin.

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    Main Methods:

    • Analysis of 178 bacteremia episodes from a cohort of 3451 patients with sickle hemoglobinopathies over 13,771 patient-years.
    • Calculation of age-specific incidence rates for sickle cell anemia (SS) and sickle cell-hemoglobin C (SC) disease.
    • Identification of causative pathogens and associated clinical conditions (e.g., urinary tract infection, osteomyelitis).

    Main Results:

    • Bacteremia incidence was highest in children younger than 2 years with both SS and SC disease.
    • Streptococcus pneumoniae was the predominant pathogen in patients younger than 6 years (66%), while gram-negative organisms predominated in older patients (50%).
    • Pneumococcal bacteremia in children with SS younger than 3 years had an incidence of 6.1 events/100 patient-years and a case fatality rate of 24%.

    Conclusions:

    • Bacteremia rates vary significantly with age and disease type in sickle hemoglobinopathies.
    • Pneumococcal infections remain a critical threat in young children with sickle cell anemia, with high fatality rates.
    • Retrospective analysis suggests that prophylactic penicillin may be effective in reducing pneumococcal bacteremia incidence in children under 3 years.