title: Vitamin B6 status of children with sickle cell disease.
abstract: OBJECTIVE
  In vitro, vitamin B(6) has antisickling properties, but the effect of vitamin B status on the health of children with sickle cell disease-SS (SCD-SS) is not well described. The purpose of this study was to assess vitamin B(6) status of children with SCD-SS ages 3 to 20 years and determine its relationship to growth, dietary intake, and disease severity.
  
  
  METHODS
  Vitamin B(6) status was assessed by serum pyridoxal 5-phosphate (PLP) concentration in subjects with SCD-SS and by urinary 4-pyridoxic acid (4-PA) concentration in other subjects with SCD-SS and healthy control children. Concentration of PLP was compared with anthropometric measures of growth and nutritional status, dietary intake, hematologic indices, and frequency of SCD-related illness.
  
  
  RESULTS
  The PLP concentration of subjects with SCD-SS was 15.6 +/- 15.2 nmol/L. Seventy-seven percent had a PLP concentration below the deficiency criterion (20 nmol/L) suggested by the Dietary Reference Intakes (1998). Controlling for alkaline phosphatase, age, and gender, PLP concentration was associated positively with weight, body mass index, and arm circumference -scores and negatively with reticulocyte count. Urinary 4-PA was lower in children with SCD-SS versus controls, although 4-PA/creatinine values did not differ between groups.
  
  
  CONCLUSIONS
  Children with SCD-SS had apparently low serum PLP concentrations in the absence of excess vitamin B(6) excretion, suggesting low vitamin B(6) status. Low serum PLP concentration was associated with indicators of poor nutritional status and may be related to increased hemolysis in children with SCD-SS.
authors.affiliation: Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399, USA.
authors.forename: Melissa C
authors.initials: MC
authors.lastname: Nelson
authors.forename: Babette S
authors.initials: BS
authors.lastname: Zemel
authors.forename: Deborah A
authors.initials: DA
authors.lastname: Kawchak
authors.forename: Elizabeth M
authors.initials: EM
authors.lastname: Barden
authors.forename: Edward A
authors.initials: EA
authors.lastname: Frongillo
authors.forename: Stephen P
authors.initials: SP
authors.lastname: Coburn
authors.forename: Kwaku
authors.initials: K
authors.lastname: Ohene-Frempong
authors.forename: Virginia A
authors.initials: VA
authors.lastname: Stallings
mesh_terms: D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase / Q000378:metabolism; D000469:Alkaline Phosphatase / Q000378:metabolism; D000755:Anemia, Sickle Cell / Q000453:epidemiology / Q000378:metabolism*; D000886:Anthropometry; D001219:Aspartate Aminotransferases / Q000378:metabolism; D015415:Biomarkers / Q000032:analysis; D002648:Child; D002664:Child Nutritional Physiological Phenomena; D002675:Child, Preschool; D004032:Diet; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009752:Nutritional Status; D015143:Philadelphia / Q000453:epidemiology; D011732:Pyridoxal Phosphate / Q000097:blood; D011735:Pyridoxic Acid / Q000652:urine; D025101:Vitamin B 6 / Q000378:metabolism*
chemical_list: D015415:Biomarkers; D011735:Pyridoxic Acid; D011732:Pyridoxal Phosphate; D025101:Vitamin B 6; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase