Related Experiment Videos
Plasma protein C levels in children with sickle cell disease
1Department of Pediatrics, School of Medicine, Health Sciences Center, SUNY, Stony Brook.
Insights
Children with sickle cell disease (SCD) have lower protein C (PC) levels, which decrease further during vasoocclusive crisis (VOC). Lower PC may increase thrombosis risk in SCD patients.
Area of Science:
- Hematology
- Pediatric Medicine
- Thrombosis Research
Background:
- Thrombotic phenomena are key in sickle cell disease (SCD) manifestations.
- Protein C (PC) is a crucial anticoagulant protein.
Purpose of the Study:
- To investigate protein C (PC) levels in children with sickle cell disease (SCD) during steady state and vasoocclusive crisis (VOC).
- To assess the relationship between PC levels and thrombotic risk in pediatric SCD.
Main Methods:
- Protein C (PC) levels were measured in 32 children with SCD (steady state and VOC) and in healthy controls.
- PC levels were compared across different clinical states and with control groups.
Main Results:
- Children with SCD exhibited significantly lower PC levels during steady state compared to controls.
- PC levels markedly decreased during VOC in SCD patients, increasing with clinical improvement.
- Significantly lower PC levels were observed in SCD patients compared to controls.
Conclusions:
- Decreased PC levels in SCD may result from increased consumption and/or reduced production due to altered liver function.
- Lowered PC levels are postulated to elevate the risk of thrombosis in pediatric sickle cell disease.
- Protein C deficiency is a potential contributing factor to thrombotic events in SCD.
Abstract:
Since thrombotic phenomena have important roles in the vasoocclusive manifestations in sickle cell disease (SCD), protein C (PC) was determined in 32 children with SCD during steady state and vasoocclusive crisis (VOC) and in controls. Children with SCD during steady state had significantly lower PC levels as compared to controls. During VOC there was marked decrease in PC as compared to levels during steady state, and these levels increased to initial levels or higher with clinical improvement. It is postulated that the decreased levels of PC in SCD are probably secondary to increased consumption as well as decreased production because of altered liver function. This data suggests that decreased levels of PC may increase the risk of thrombosis in these patients.