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Pyridoxal 5'-phosphate levels in children with sickle cell disease
1Department of Pediatrics, Emory University School of Medicine, Atlanta, GA 30322.
Insights
Low plasma pyridoxal 5'-phosphate (PLP) levels are common in black children, regardless of sickle cell disease (SCD) status. This finding suggests a broader prevalence of vitamin B6 deficiency in this demographic.
Area of Science:
- Biochemistry
- Hematology
- Nutritional Science
Background:
- Pyridoxal 5 -phosphate (PLP), the active coenzyme of vitamin B6, exhibits antisickling properties in vitro.
- Previous studies indicated low plasma PLP levels in adults with sickle cell anemia.
- The prevalence of low PLP in children with sickle cell disease (SCD) was previously unknown.
Purpose of the Study:
- To determine the prevalence of low plasma PLP levels in asymptomatic, nontransfused children with SCD.
- To compare PLP levels in children with SCD to control groups of black and white children, and black adults.
Main Methods:
- Plasma PLP levels were measured using a radioenzymatic technique.
- Study included 55 children with SCD and three control groups (black children, white children, black adults).
- Statistical comparisons were made between the groups.
Main Results:
- No significant difference in plasma PLP was found between black children with SCD and black control children.
- Both black groups (SCD and control children) had significantly lower plasma PLP levels compared to white control children.
- These findings suggest a high prevalence of low PLP levels in black children, irrespective of SCD.
Conclusions:
- Low plasma PLP levels are prevalent in black children, including those with sickle cell disease.
- The observed low PLP levels in black children may indicate a broader nutritional deficiency.
- Further investigation into vitamin B6 status in this population is warranted.
Abstract:
Pyridoxal 5'-phosphate (PLP), the major coenzyme form of vitamin B6, is known to have antisickling properties in vitro. Recently, low plasma PLP levels were reported in a group of adults with sickle cell anemia. We measured the plasma PLP levels in a group of 55 asymptomatic nontransfused children with sickle cell diseases (SCD) to determine the prevalence of low plasma PLP levels in this population. Comparative studies were made with the measurement of PLP in three other groups serving as controls: Group A (black children, n = 36); Group B (white children, n = 37); and Group C (black adults, n = 13). PLP was measured directly in plasma by a radioenzymatic technique. The results of these comparisons showed that there was no statistically significant difference in plasma PLP of black children with SCD (10.7 +/- 10.0 ng/ml) as compared with black control children (group A, 9.0 +/- 12.3 ng/ml). The low plasma levels PLP in these two groups were significantly lower than that of the plasma PLP of white control children (group B, 15.85 +/- 15.92 ng/ml). This data suggest that a high prevalence of low PLP levels exists in black children seen at Grady Memorial Hospital, both with and without SCD.