{"title":"补充维生素B6对接受D-青霉胺治疗的Wilson病患者的评价。","authors":"Justin Mbala, Abdelouahed Belmalih, Olivier Guillaud, Alain Lachaux, Eduardo Couchonnal Bedoya","doi":"10.1136/bmjgast-2023-001211","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>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 B<sub>6</sub>; therefore, vitamin supplementation is sometimes recommended, although non-consensually. The objective of our study was to evaluate the level of vitamin B<sub>6</sub> in WD patients treated with DP with and without associated supplementation.</p><p><strong>Methodology: </strong>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 B<sub>6</sub> supplementation. The level of vitamin B<sub>6</sub> was measured by the determination of pyridoxal phosphate (PLP).</p><p><strong>Results: </strong>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 B<sub>6</sub> 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.</p><p><strong>Conclusion: </strong>Long-term stable WD patients under DP treatment probably do not need vitamin B<sub>6</sub> supplementation.</p>","PeriodicalId":9235,"journal":{"name":"BMJ Open Gastroenterology","volume":null,"pages":null},"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":"{\"title\":\"Evaluation of vitamin B<sub>6</sub> 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\":\"<p><strong>Introduction: </strong>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 B<sub>6</sub>; therefore, vitamin supplementation is sometimes recommended, although non-consensually. The objective of our study was to evaluate the level of vitamin B<sub>6</sub> in WD patients treated with DP with and without associated supplementation.</p><p><strong>Methodology: </strong>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 B<sub>6</sub> supplementation. The level of vitamin B<sub>6</sub> was measured by the determination of pyridoxal phosphate (PLP).</p><p><strong>Results: </strong>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 B<sub>6</sub> 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.</p><p><strong>Conclusion: </strong>Long-term stable WD patients under DP treatment probably do not need vitamin B<sub>6</sub> supplementation.</p>\",\"PeriodicalId\":9235,\"journal\":{\"name\":\"BMJ Open Gastroenterology\",\"volume\":null,\"pages\":null},\"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}","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}
Evaluation of vitamin B6 supplementation in Wilson's disease patients treated with D-penicillamine.
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.
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.
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