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Acute splenic sequestration crises in Saudi children with sickle cell disease
M M Salamah1, A A Mallouh, J A Hamdan
1Dhahran Health Center, Saudi Arabia.
Insights
Acute splenic sequestration crisis (ASSC) is a life-threatening complication in infants with sickle cell disease (SCD). This study found that children in Saudi Arabia experience a milder form of ASSC, differing from the severe presentation seen in Black children.
Area of Science:
- Hematology
- Pediatrics
- Genetics
Background:
- Sickle cell disease (SCD) is prevalent in Saudi Arabia's Eastern Province, affecting 1.75% of the population.
- Acute splenic sequestration crisis (ASSC) is a major cause of mortality in infants with SCD.
- The exact causes and triggers of ASSC remain unclear.
Purpose of the Study:
- To describe the clinical characteristics of acute splenic sequestration crisis (ASSC) in children with sickle cell disease (SCD) in Saudi Arabia.
- To compare the presentation of ASSC in this cohort with the classical description in Black children.
Main Methods:
- Retrospective review of 17 children diagnosed with ASSC.
- Clinical assessment of ASSC presentation and complications.
Main Results:
- All 17 children presented with a milder form of ASSC, distinct from the classical presentation.
- None of the children experienced peripheral circulatory shock.
- Complications included hypersplenism in three children and splenectomy in four due to recurrent attacks.
Conclusions:
- Children with sickle cell disease in Saudi Arabia may experience a less severe form of acute splenic sequestration crisis.
- The findings highlight regional variations in ASSC presentation and suggest further research into underlying causes and precipitants.
Abstract:
Sickle cell disease (SCD) is a common disease in the Eastern Province of Saudi Arabia. Twenty per cent of the population have sickle cell trait and 1.75% have SCD. In the first 2 years of life, infection, hand-foot syndrome and acute splenic sequestration crisis (ASSC) are the most common complications of this disease. The classical characteristics of an ASSC, the subject of this paper, are sudden and rapid enlargement of the spleen, secondary to the massive pooling of red blood cells in the splenic sinusoids in a functioning spleen. Less common minor attacks of ASSC have been described recently. An ASSC is one of the most common causes of death in infants with SCD. The underlying cause and the precipitants of attacks of ASSC remain unknown. Seventeen children with ASSC were seen. Clinically, they had minor ASSC; three developed hypersplenism and four underwent splenectomy for recurrent attacks of ASSC. None of these 17 children had the classical ASSC described in black children where peripheral circulatory shock is encountered.