Evaluation of vitamin B₆ supplementation in Wilson's disease patients treated with D-penicillamine.

IF 3.3 Q2 GASTROENTEROLOGY & HEPATOLOGY

BMJ Open Gastroenterology Pub Date : 2023-08-01 DOI:10.1136/bmjgast-2023-001211

Justin Mbala, Abdelouahed Belmalih, Olivier Guillaud, Alain Lachaux, Eduardo Couchonnal Bedoya

{"title":"Evaluation of vitamin B6 supplementation in Wilson's disease patients treated with D-penicillamine.","authors":"Justin Mbala, Abdelouahed Belmalih, Olivier Guillaud, Alain Lachaux, Eduardo Couchonnal Bedoya","doi":"10.1136/bmjgast-2023-001211","DOIUrl":null,"url":null,"abstract":"Introduction: Wilson's disease (WD) is a copper metabolism disorder characterised by a progressive accumulation of this metal mainly in the liver and the brain. Treatment is based on the removal of copper operated by the chelators, among which, D-penicillamine (DP) is prescribed as a first-line treatment in most situations. There is some evidence in linking the use of DP with a risk of vitamin B6; therefore, vitamin supplementation is sometimes recommended, although non-consensually. The objective of our study was to evaluate the level of vitamin B6 in WD patients treated with DP with and without associated supplementation.Methodology: All WD patients followed at the National Reference Centre for WD in Lyon between January 2019 and December 2020 treated with DP for more than 1 year were included and separated in two groups according to vitamin B6 supplementation. The level of vitamin B6 was measured by the determination of pyridoxal phosphate (PLP).Results: A total of 37 patients were included. Average age of 23.3±14.8 years, 15 patients with <18 years. Median duration of treatment was 51 (55.8) months. 15 patients were under vitamin B6 supplementation and 22 had interrupted it for more than 1 year. The median PLP level was significantly higher in the group with supplementation, 137.2 (86.7) nmol/L vs 64.9 (30.8) nmol/(p<0.01). No patient had a PLP level<35 nmol/L.Conclusion: Long-term stable WD patients under DP treatment probably do not need vitamin B6 supplementation.","PeriodicalId":9235,"journal":{"name":"BMJ Open Gastroenterology","volume":"10 1","pages":""},"PeriodicalIF":3.3000,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/97/35/bmjgast-2023-001211.PMC10476132.pdf","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"BMJ Open Gastroenterology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1136/bmjgast-2023-001211","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Introduction: Wilson's disease (WD) is a copper metabolism disorder characterised by a progressive accumulation of this metal mainly in the liver and the brain. Treatment is based on the removal of copper operated by the chelators, among which, D-penicillamine (DP) is prescribed as a first-line treatment in most situations. There is some evidence in linking the use of DP with a risk of vitamin B₆; therefore, vitamin supplementation is sometimes recommended, although non-consensually. The objective of our study was to evaluate the level of vitamin B₆ in WD patients treated with DP with and without associated supplementation.

Methodology: All WD patients followed at the National Reference Centre for WD in Lyon between January 2019 and December 2020 treated with DP for more than 1 year were included and separated in two groups according to vitamin B₆ supplementation. The level of vitamin B₆ was measured by the determination of pyridoxal phosphate (PLP).

Results: A total of 37 patients were included. Average age of 23.3±14.8 years, 15 patients with <18 years. Median duration of treatment was 51 (55.8) months. 15 patients were under vitamin B₆ supplementation and 22 had interrupted it for more than 1 year. The median PLP level was significantly higher in the group with supplementation, 137.2 (86.7) nmol/L vs 64.9 (30.8) nmol/(p<0.01). No patient had a PLP level<35 nmol/L.

Conclusion: Long-term stable WD patients under DP treatment probably do not need vitamin B₆ supplementation.

查看原文本刊更多论文

补充维生素B6对接受D-青霉胺治疗的Wilson病患者的评价。

引言：Wilson病（WD）是一种铜代谢紊乱，其特征是这种金属主要在肝脏和大脑中逐渐积累。治疗的基础是通过螯合剂去除铜，其中，D-青霉素（DP）在大多数情况下被规定为一线治疗。有一些证据表明DP的使用与维生素B6的风险有关；因此，有时建议补充维生素，尽管并非一致。我们研究的目的是评估服用DP和不服用相关补充剂的WD患者的维生素B6水平。方法：2019年1月至2020年12月，所有WD患者在里昂国家WD参考中心接受了为期1个月以上的DP治疗根据维生素B6的补充情况，将年份分为两组。维生素B6的水平是通过磷酸吡哆醛（PLP）的测定来测量的。结果：共纳入37例患者。平均年龄为23.3±14.8岁，15名患者服用6次补充剂，22名患者服用1次以上年补充组的中位PLP水平显著较高，分别为137.2（86.7）nmol/L和64.9（30.8）nmol/（P结论：DP治疗下的长期稳定WD患者可能不需要补充维生素B6。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

BMJ Open Gastroenterology GASTROENTEROLOGY & HEPATOLOGY-

CiteScore

5.90

自引率

3.20%

发文量

审稿时长

2 weeks

期刊介绍： BMJ Open Gastroenterology is an online-only, peer-reviewed, open access gastroenterology journal, dedicated to publishing high-quality medical research from all disciplines and therapeutic areas of gastroenterology. It is the open access companion journal of Gut and is co-owned by the British Society of Gastroenterology. The journal publishes all research study types, from study protocols to phase I trials to meta-analyses, including small or specialist studies. Publishing procedures are built around continuous publication, publishing research online as soon as the article is ready.