磷酸肌酸钠治疗Leber遗传性视神经病变的初步临床观察

发布时间：2019-04-20 18:04

【摘要】：目的观察磷酸肌酸钠治疗Leber遗传性视神经病变(LHON)的有效性。设计回顾性病例系列。研究对象2014年1月至2016年1月武汉同济医院眼科确诊的LHON患者19例。根据患者是否同意住院治疗分为两组,10例住院者为治疗组,9例门诊观察未做治疗者为对照组。方法对治疗组的10例LHON患者进行磷酸肌酸钠治疗,每日2次,每次1 g,加入250 ml生理盐水中静脉滴注,共7天。观察患者治疗前后的视力、视野变化。对照组的9例患者未治疗。19例患者随访观察1年。主要指标视力、视野。结果与治疗前视力相比,治疗7天后,治疗组患者中3例患者(1号右眼,6号右眼,7号左眼)视力提高,分别提高0.3 LogMAR、0.4 LogMAR、0.7 LogMAR。随访1年后,1号右眼视力下降,6号和7号视力无变化。3例患者中14484位点突变的有2例(6号和7号),11778位点突变的有1例(1号);另外7例视力无显著改变。10例患者的视野(VFI和MD)治疗前后无显著差异(P=0.167,0.20)。未经治疗的9例患者随访观察1年,视力无变化。结论磷酸肌酸钠治疗Leber遗传性视神经病变或许对14484位点突变患者的视力提高或稳定有一定的疗效。
[Abstract]:Objective to observe the efficacy of sodium phosphate in the treatment of Leber hereditary optic neuropathy (LHON). Design retrospective case series. From January 2014 to January 2016, 19 LHON patients were diagnosed in Wuhan Tongji Hospital. The patients were divided into two groups according to whether they agreed to be treated in hospital, 10 inpatients as treatment group and 9 out-patient as control group. Methods Ten LHON patients in the treatment group were treated with sodium phosphate for 7 days, twice a day, 1 g each time, injected intravenously with 250 ml normal saline for 7 days. The visual acuity and visual field before and after treatment were observed. In the control group, 9 patients were untreated and 19 patients were followed up for 1 year. Main outcome measures: visual acuity, visual field. Results after 7 days of treatment, the visual acuity of 3 patients (right eye No. 1, right eye No. 6, left eye No. 7) in the treatment group was improved by 0.3 LogMAR,0.4 LogMAR,0.7 LogMAR. respectively. After 1-year follow-up, the visual acuity of No. 1 right eye decreased, and the visual acuity of No. 6 and No. 7 did not change. Of the 3 patients, 2 (6 and 7) had 14484 point mutation and 1 (1) had 11778 point mutation. The visual field (VFI) and the visual field (MD) of 10 patients had no significant difference before and after treatment (P < 0. 167, 0. 20). There was no change in visual acuity in 9 patients who had not been treated for 1 year. Conclusion the treatment of Leber's hereditary optic neuropathy with sodium phosphate may be effective in improving or stabilizing the visual acuity of the patients with point mutation at position 14484.
【作者单位】：华中科技大学同济医学院附属同济医院眼科;鄂州市第五医院药剂科;鄂州市第五医院超声科;
【分类号】：R774.6

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