Passerieux Emilie, Hayot Maurice, Jaussent Audrey, Carnac Gilles, Gouzi Fares, Pillard Fabien, Picot Marie-Christine, Böcker Koen, Hugon Gerald, Pincemail Joel, Defraigne Jean O, Verrips Theo, Mercier Jacques, Laoudj-Chenivesse Dalila
University of Montpellier 1 and 2, INSERM Unit 1046, Montpellier, France.
University of Montpellier 1 and 2, INSERM Unit 1046, Montpellier, France and Department of Clinical Physiology, University Hospital, Montpellier, France.
Free Radic Biol Med. 2015 Apr;81:158-69. doi: 10.1016/j.freeradbiomed.2014.09.014. Epub 2014 Sep 20.
Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant disease characterized by progressive weakness and atrophy of specific skeletal muscles. As growing evidence suggests that oxidative stress may contribute to FSHD pathology, antioxidants that might modulate or delay oxidative insults could help in maintaining FSHD muscle function. Our primary objective was to test whether oral administration of vitamin C, vitamin E, zinc gluconate, and selenomethionine could improve the physical performance of patients with FSHD. Adult patients with FSHD (n=53) were enrolled at Montpellier University Hospital (France) in a randomized, double-blind, placebo-controlled pilot clinical trial. Patients were randomly assigned to receive 500 mg vitamin C, 400mg vitamin E, 25mg zinc gluconate and 200 μg selenomethionine (n=26), or matching placebo (n=27) once a day for 17 weeks. Primary outcomes were changes in the two-minute walking test (2-MWT), maximal voluntary contraction, and endurance limit time of the dominant and nondominant quadriceps (MVCQD, MVCQND, TlimQD, and TlimQND, respectively) after 17 weeks of treatment. Secondary outcomes were changes in the antioxidant status and oxidative stress markers. Although 2-MWT, MVCQ, and TlimQ were all significantly improved in the supplemented group at the end of the treatment compared to baseline, only MVCQ and TlimQ variations were significantly different between groups (MVCQD: P=0.011; MVCQND: P=0.004; TlimQD: P=0.028; TlimQND: P=0.011). Similarly, the vitamin C (P<0.001), vitamin E as α-tocopherol (P<0.001), vitamin C/vitamin E ratio (P=0.017), vitamin E γ/α ratio (P=0.022) and lipid peroxides (P<0.001) variations were significantly different between groups. In conclusion, vitamin E, vitamin C, zinc, and selenium supplementation has no significant effect on the 2-MWT, but improves MVCQ and TlimQ of both quadriceps by enhancing the antioxidant defenses and reducing oxidative stress. This trial was registered at clinicaltrials.gov (number: NCT01596803).
面肩肱型肌营养不良症（FSHD）是一种常染色体显性疾病，其特征为特定骨骼肌的进行性肌无力和萎缩。越来越多的证据表明氧化应激可能导致FSHD病理改变，因此，可能调节或延缓氧化损伤的抗氧化剂或许有助于维持FSHD患者的肌肉功能。我们的主要目的是测试口服维生素C、维生素E、葡萄糖酸锌和硒代蛋氨酸是否能改善FSHD患者的身体机能。法国蒙彼利埃大学医院招募了53例成年FSHD患者，进行一项随机、双盲、安慰剂对照的临床试验。患者被随机分配，一组每天服用500毫克维生素C、400毫克维生素E、25毫克葡萄糖酸锌和200微克硒代蛋氨酸（n = 26），另一组服用匹配的安慰剂（n = 27），为期17周。主要观察指标为治疗17周后两分钟步行试验（2-MWT）、最大自主收缩以及优势和非优势股四头肌的耐力极限时间（分别为MVCQD、MVCQND、TlimQD和TlimQND）的变化。次要观察指标为抗氧化状态和氧化应激标志物的变化。尽管与基线相比，补充组在治疗结束时2-MWT、MVCQ和TlimQ均有显著改善，但两组之间只有MVCQ和TlimQ的变化存在显著差异（MVCQD：P = 0.011；MVCQND：P = 0.004；TlimQD：P = 0.028；TlimQND：P = 0.011）。同样，两组之间维生素C（P < 0.001）、α-生育酚形式的维生素E（P < 0.001）、维生素C/维生素E比值（P = 0.017）、维生素Eγ/α比值（P = 0.022）和脂质过氧化物（P < 0.001）的变化也存在显著差异。总之，补充维生素E、维生素C、锌和硒对2-MWT没有显著影响，但通过增强抗氧化防御和降低氧化应激，改善了双侧股四头肌的MVCQ和TlimQ。该试验已在clinicaltrials.gov注册（编号：NCT01596803）。
