Related Experiment Videos
Fatal pneumococcal septicemia in hemoglobin SC disease
P A Lane1, Z R Rogers, G M Woods
1Colorado Sickle Cell Treatment and Research Center, University of Colorado School of Medicine, Denver.
The Journal of Pediatrics
|June 1, 1994
Summary
Children with hemoglobin SC disease are at high risk for fatal pneumococcal septicemia, especially after age 3. Early vaccination and prompt illness investigation are crucial for prevention.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Hemoglobin SC disease (HbSC) is a sickle cell hemoglobinopathy.
- Children with sickle cell disease are susceptible to encapsulated bacterial infections.
- Functional asplenia is a known risk factor for severe infections in sickle cell disease.
Purpose of the Study:
- To report on fatal pneumococcal septicemia in children with hemoglobin SC disease.
- To highlight the risk factors and clinical presentation of severe pneumococcal infections in this population.
- To inform clinical management and preventive strategies.
Main Methods:
- Retrospective review of medical and autopsy records.
- Analysis of seven pediatric cases of fatal pneumococcal septicemia.
- Examination of clinical course, laboratory findings, and autopsy results.
Main Results:
- Seven children with HbSC disease experienced fatal pneumococcal septicemia.
- Most children were unvaccinated and untreated with prophylactic penicillin.
- Clinical course was characterized by rapid deterioration despite aggressive medical care.
- Autopsy findings included splenic congestion, splenomegaly, and adrenal hemorrhage.
Conclusions:
- Functional asplenia increases the risk of fatal pneumococcal septicemia in children with HbSC disease, particularly after age 3.
- Pneumococcal vaccination is strongly recommended for all children with HbSC disease.
- Prompt investigation of acute febrile illnesses for septicemia is essential.
- The role of prophylactic penicillin in HbSC disease remains under discussion.