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Acute splenic sequestration in homozygous sickle cell disease: natural history and management
Insights
Sickle cell disease (SCD) patients frequently experience acute splenic sequestration (ASS) attacks. Early diagnosis and improved medical care significantly reduced ASS fatality rates in children with SCD.
Area of Science:
- Pediatric Hematology
- Sickle Cell Disease Research
- Clinical Pediatrics
Background:
- Homozygous sickle cell disease (SCD) poses significant health risks to children.
- Acute splenic sequestration (ASS) is a serious complication in young children with SCD.
- Understanding ASS incidence, risk factors, and outcomes is crucial for effective management.
Purpose of the Study:
- To analyze the incidence, clinical course, and outcomes of acute splenic sequestration (ASS) in a cohort of children with homozygous sickle cell disease.
- To identify factors influencing ASS attacks and fatalities.
- To evaluate the impact of an educational program on ASS diagnosis and outcomes.
Main Methods:
- Longitudinal cohort study of 308 children with homozygous SCD diagnosed at birth.
- Analysis of 132 clinically significant ASS events over a 10-year period.
- Survival curve analysis for time to first ASS attack; assessment of recurrence rates, associated symptoms, and protective factors like fetal hemoglobin levels.
Main Results:
- 89 children experienced 132 ASS attacks; age at first attack ranged from 3 months to 6 years.
- Cumulative probability of first ASS attack by age 5 was 0.297; 13 events were fatal, primarily during the first attack.
- Recurrences affected 49% of survivors; high fetal hemoglobin levels showed a protective effect.
- An educational program increased ASS incidence reporting but drastically reduced fatality rates from 29.4 to 3.1 per 100 events.
Conclusions:
- Acute splenic sequestration (ASS) is a common and potentially fatal complication in young children with sickle cell disease (SCD).
- While pneumococcal vaccine and penicillin prophylaxis did not prevent ASS, high fetal hemoglobin levels offered protection.
- Parental education and improved medical management led to earlier detection and a significant reduction in ASS-related mortality, highlighting the importance of timely intervention.
Abstract:
Of a cohort of 308 children with homozygous sickle cell disease diagnosed at birth, 89 experienced 132 clinically significant attacks of acute splenic sequestration (ASS) over a 10-year period. The age at first attack ranged from 3 months to 6 years. Survival curve analysis of the interval until first attack indicated a cumulative probability of 0.225 by 2 years, and 0.265 by 3 years, and 0.297 by 5 years of age. Thirteen events were fatal, 11 during the first attack, and all before transfusion could be instituted. Recurrences occurred in 49% of survivors of the first attacks, and there were diminishing intervals between subsequent events. Respiratory symptoms were associated with 52 of 132 events, but bacterial isolates on blood culture were less frequent, and ASS was not prevented by pneumococcal vaccine or penicillin prophylaxis. A high fetal hemoglobin level protected against attacks of ASS. A parental education program aimed at early diagnosis of ASS was followed by an increase in the incidence rate for ASS from a mean of 4.6 per 100 patient-years to 11.3 per 100 patient-years, probably reflecting increased awareness of the complication. During the same periods, the fatality rate fell from 29.4 per 100 events to 3.1 per 100 events. The improvement in outcome is likely to have resulted from improvement in medical management and earlier detection of ASS.