title: Vitamin B6 in plasma and cerebrospinal fluid of children.
abstract: BACKGROUND
  Over the past years, the essential role of vitamin B6 in brain development and functioning has been recognized and genetic metabolic disorders resulting in functional vitamin B6 deficiency have been identified. However, data on B6 vitamers in children are scarce.
  
  
  METHODS
  B6 vitamer concentrations in simultaneously sampled plasma and cerebrospinal fluid (CSF) of 70 children with intellectual disability were determined by ultra performance liquid chromatography-tandem mass spectrometry. For ethical reasons, CSF samples could not be obtained from healthy children. The influence of sex, age, epilepsy and treatment with anti-epileptic drugs, were investigated.
  
  
  RESULTS
  The B6 vitamer composition of plasma (pyridoxal phosphate (PLP) > pyridoxic acid > pyridoxal (PL)) differed from that of CSF (PL > PLP > pyridoxic acid > pyridoxamine). Strong correlations were found for B6 vitamers in and between plasma and CSF. Treatment with anti-epileptic drugs resulted in decreased concentrations of PL and PLP in CSF.
  
  
  CONCLUSIONS
  We provide concentrations of all B6 vitamers in plasma and CSF of children with intellectual disability (±epilepsy), which can be used in the investigation of known and novel disorders associated with vitamin B6 metabolism as well as in monitoring of the biochemical effects of treatment with vitamin B6.
authors.affiliation: Department of Medical Genetics, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Monique
authors.initials: M
authors.lastname: Albersen
authors.affiliation: Department of Medical Genetics, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Marjolein
authors.initials: M
authors.lastname: Bosma
authors.affiliation: Department of Medical Genetics, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Judith J M
authors.initials: JJ
authors.lastname: Jans
authors.affiliation: Department of Paediatric Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Floris C
authors.initials: FC
authors.lastname: Hofstede
authors.affiliation: Department of Paediatric Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Peter M
authors.initials: PM
authors.lastname: van Hasselt
authors.affiliation: Department of Medical Genetics, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Monique G M
authors.initials: MG
authors.lastname: de Sain-van der Velden
authors.affiliation: Department of Paediatric Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Gepke
authors.initials: G
authors.lastname: Visser
authors.affiliation: Department of Medical Genetics, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
authors.forename: Nanda M
authors.initials: NM
authors.lastname: Verhoeven-Duif
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002853:Chromatography, Liquid; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000188:drug therapy* / Q000378:metabolism*; D008297:Male; D011730:Pyridoxal / Q000097:blood / Q000134:cerebrospinal fluid; D011732:Pyridoxal Phosphate / Q000097:blood / Q000134:cerebrospinal fluid; D011733:Pyridoxamine / Q000134:cerebrospinal fluid; D011735:Pyridoxic Acid / Q000097:blood / Q000134:cerebrospinal fluid; D053719:Tandem Mass Spectrometry; D025101:Vitamin B 6 / Q000097:blood* / Q000134:cerebrospinal fluid*
chemical_list: D000927:Anticonvulsants; D011730:Pyridoxal; D011735:Pyridoxic Acid; D011732:Pyridoxal Phosphate; D011733:Pyridoxamine; D025101:Vitamin B 6