title: Pyridoxal 5'-phosphate levels in children with sickle cell disease.
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.
authors.affiliation: Department of Pediatrics, Emory University School of Medicine, Atlanta, GA 30322.
authors.forename: L
authors.initials: L
authors.lastname: Flores
authors.forename: R
authors.initials: R
authors.lastname: Pais
authors.forename: I
authors.initials: I
authors.lastname: Buchanan
authors.forename: D
authors.initials: D
authors.lastname: Arnelle
authors.forename: V M
authors.initials: VM
authors.lastname: Camp
authors.forename: M
authors.initials: M
authors.lastname: Kutner
authors.forename: B A
authors.initials: BA
authors.lastname: Faraj
authors.forename: J
authors.initials: J
authors.lastname: Eckman
authors.forename: A
authors.initials: A
authors.lastname: Ragab
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000188:drug therapy / Q000235:genetics; D044383:Black People; D002648:Child; D002675:Child, Preschool; D006454:Hemoglobins / Q000235:genetics; D006801:Humans; D007223:Infant; D010641:Phenotype; D011732:Pyridoxal Phosphate / Q000097:blood*; D011736:Pyridoxine / Q000627:therapeutic use; D012016:Reference Values; D044465:White People
chemical_list: D006454:Hemoglobins; D011732:Pyridoxal Phosphate; D011736:Pyridoxine