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 treat...
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BMJ Publishing Group
2023-08-01
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| Series: | BMJ Open Gastroenterology |
| Online Access: | https://bmjopengastro.bmj.com/content/10/1/e001211.full |
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| author | Olivier Guillaud Alain Lachaux Justin Mbala Abdelouahed Belmalih Eduardo Couchonnal Bedoya |
| author_facet | Olivier Guillaud Alain Lachaux Justin Mbala Abdelouahed Belmalih Eduardo Couchonnal Bedoya |
| author_sort | Olivier Guillaud |
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| description | 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. |
| format | Article |
| id | doaj-art-a4f75c2e3d9448a49fc9da5ad472a786 |
| institution | Kabale University |
| issn | 2054-4774 |
| language | English |
| publishDate | 2023-08-01 |
| publisher | BMJ Publishing Group |
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| series | BMJ Open Gastroenterology |
| spelling | doaj-art-a4f75c2e3d9448a49fc9da5ad472a7862025-02-02T00:55:09ZengBMJ Publishing GroupBMJ Open Gastroenterology2054-47742023-08-0110110.1136/bmjgast-2023-001211Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamineOlivier Guillaud0Alain Lachaux1Justin Mbala2Abdelouahed Belmalih3Eduardo Couchonnal Bedoya4Centre National de Référence Pour la Maladie de Wilson, Hospices Civils de Lyon, Lyon, FranceCentre National de Référence Pour la Maladie de Wilson, Hospices Civils de Lyon, Lyon, FranceDepartment of Pediatric, Kinshasa University Hospital, Kinshasa, CongoCentre National de Référence Pour la Maladie de Wilson, Hospices Civils de Lyon, Lyon, FranceCentre National de Référence Pour la Maladie de Wilson, Hospices Civils de Lyon, Lyon, FranceIntroduction 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.https://bmjopengastro.bmj.com/content/10/1/e001211.full |
| spellingShingle | Olivier Guillaud Alain Lachaux Justin Mbala Abdelouahed Belmalih Eduardo Couchonnal Bedoya Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamine BMJ Open Gastroenterology |
| title | Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamine |
| title_full | Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamine |
| title_fullStr | Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamine |
| title_full_unstemmed | Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamine |
| title_short | Evaluation of vitamin B6 supplementation in Wilson’s disease patients treated with D-penicillamine |
| title_sort | evaluation of vitamin b6 supplementation in wilson s disease patients treated with d penicillamine |
| url | https://bmjopengastro.bmj.com/content/10/1/e001211.full |
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