儿童血浆和脑脊液中的维生素B6
Vitamin B6 in plasma and cerebrospinal fluid of children.
作者信息
Albersen Monique, Bosma Marjolein, Jans Judith J M, Hofstede Floris C, van Hasselt Peter M, de Sain-van der Velden Monique G M, Visser Gepke, Verhoeven-Duif Nanda M
机构信息
Department of Medical Genetics, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
Department of Paediatric Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.
出版信息
PLoS One. 2015 Mar 11;10(3):e0120972. doi: 10.1371/journal.pone.0120972. eCollection 2015.
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.
MATERIALS AND 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.
CONCLUSION
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.
背景
在过去几年中，维生素B6在大脑发育和功能中的重要作用已得到认可，并且已确定导致功能性维生素B6缺乏的遗传代谢紊乱。然而，关于儿童体内维生素B6各成分的数据却很稀少。
材料与方法
采用超高效液相色谱-串联质谱法测定了70名智障儿童同时采集的血浆和脑脊液（CSF）中维生素B6各成分的浓度。出于伦理原因，无法从健康儿童获取脑脊液样本。研究了性别、年龄、癫痫以及抗癫痫药物治疗的影响。
结果
血浆中维生素B6各成分的组成（磷酸吡哆醛（PLP）> 吡哆酸 > 吡哆醛（PL））与脑脊液中的不同（PL > PLP > 吡哆酸 > 吡哆胺）。在血浆和脑脊液内以及两者之间，维生素B6各成分之间存在强相关性。抗癫痫药物治疗导致脑脊液中PL和PLP浓度降低。
结论
我们提供了智障（±癫痫）儿童血浆和脑脊液中所有维生素B6各成分的浓度，这些浓度可用于研究与维生素B6代谢相关的已知和新型疾病，以及监测维生素B6治疗的生化效果。
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