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Acute splenic sequestration crisis in sickle cell disease: early detection and treatment
R W Powell1, G L Levine, Y M Yang
1Comprehensive Sickle Cell Center, University of South Alabama College of Medicine, Mobile.
Insights
Acute splenic sequestration crisis (ASSC) in children with sickle cell disease can be life-threatening. Early detection through parental education and screening can reduce mortality and morbidity from this serious complication.
Area of Science:
- Pediatric Hematology
- Sickle Cell Disease Research
Background:
- Acute splenic sequestration crisis (ASSC) is a life-threatening complication of sickle cell disease (SCD) in children.
- It can lead to circulatory collapse due to significant blood volume loss in the spleen.
Observation:
- A study treated 12 children (5.5 months to 7 years) with ASSC over 6 years.
- Eleven patients had homozygous SCD, and one had sickle-thalassemia.
- One patient died from circulatory collapse; eight underwent splenectomy with no serious infectious complications post-surgery.
Findings:
- Splenectomy was effective in preventing recurrence in patients with major ASSC episodes.
- Three patients with minor episodes were managed conservatively without recurrence.
- An educational program for parents on spleen examination and recognizing ASSC symptoms was implemented.
Implications:
- Early detection of ASSC through neonatal screening and parental education can significantly reduce morbidity and mortality.
- Parental education on recognizing spleen enlargement and ASSC symptoms is crucial for timely intervention.
- This approach can improve outcomes for children with sickle cell disease.
Abstract:
Acute splenic sequestration crisis (ASSC) in children with various forms of sickle cell disease can result in life-threatening circulatory collapse due to the loss of circulating blood volume. Over a 6-year period we have treated 12 patients ranging in age from 5 1/2 months to 7 years presenting with acute sequestration crisis. Eleven had homozygous sickle cell disease and the other had sickle-thalassemia. One patient died of acute circulatory collapse. Eight patients underwent splenectomy after a major episode of sequestration with no serious infectious complications up to 5 years following splenectomy. Three patients with minor episodes have been followed with no recurrences. To foster early detection of this potentially lethal complication of sickle cell disease, an educational program in our Comprehensive Sickle Cell Center instructs the parents to examine the spleen and bring their child in for evaluation if the spleen enlarges. A newly developed videotape describes the common symptoms of ASSC and illustrates the technique of palpating the spleen. With early detection of sickle cell disease by neonatal screening and the educational program, the morbidity and mortality from this complication of sickle cell disease can be reduced.