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Recurrent infections in homozygous sickle cell disease

S A Magnus1, I R Hambleton, F Moosdeen

  • 1The Medical Research Council Laboratories, University of the West Indies, Kingston 7, Jamaica.

Archives of Disease in Childhood
|May 20, 1999
PubMed

Insights

Patients with sickle cell disease (SCD) experience invasive bacterial infections, with Streptococcus pneumoniae and Salmonella being common. Recurrent infections suggest a subgroup of SCD patients may have increased susceptibility.

Area of Science:

  • Pediatrics
  • Infectious Diseases
  • Hematology

Background:

  • Homozygous sickle cell disease (SS) significantly increases susceptibility to invasive bacterial infections.
  • Understanding the specific pathogens and their age-related incidence is crucial for managing SS disease complications.

Purpose of the Study:

  • To characterize the epidemiology of invasive bacterial infections in patients with homozygous sickle cell disease.
  • To identify age-specific patterns and common pathogens associated with infection in this population.
  • To investigate the implications for recurrent infections and patient susceptibility.

Main Methods:

  • Retrospective analysis of 214 invasive bacterial infection episodes in 176 patients with SS disease.
  • Identification of causative pathogens including Streptococcus pneumoniae, Salmonella spp., Haemophilus influenzae type b, Escherichia coli, and Klebsiella spp.
  • Examination of pathogen incidence based on patient age and sex, with a focus on recurrent infections.

Main Results:

  • Streptococcus pneumoniae and Haemophilus influenzae type b were most common in children under 5.
  • Salmonella spp. incidence increased linearly with age.
  • Escherichia coli infections predominantly affected older girls, and recurrent infection rates were significantly higher in a subgroup of patients.

Conclusions:

  • Invasive bacterial infections in SS disease show distinct age-related pathogen patterns.
  • A subset of SS disease patients exhibits heightened susceptibility to recurrent infections.
  • Early and aggressive investigation for further infection is recommended for patients with previous bacteremia.

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