【摘要】：目的觀察磷酸肌酸鈉治療Leber遺傳性視神經(jīng)病變(LHON)的有效性。設(shè)計(jì)回顧性病例系列。研究對(duì)象2014年1月至2016年1月武漢同濟(jì)醫(yī)院眼科確診的LHON患者19例。根據(jù)患者是否同意住院治療分為兩組,10例住院者為治療組,9例門診觀察未做治療者為對(duì)照組。方法對(duì)治療組的10例LHON患者進(jìn)行磷酸肌酸鈉治療,每日2次,每次1 g,加入250 ml生理鹽水中靜脈滴注,共7天。觀察患者治療前后的視力、視野變化。對(duì)照組的9例患者未治療。19例患者隨訪觀察1年。主要指標(biāo)視力、視野。結(jié)果與治療前視力相比,治療7天后,治療組患者中3例患者(1號(hào)右眼,6號(hào)右眼,7號(hào)左眼)視力提高,分別提高0.3 LogMAR、0.4 LogMAR、0.7 LogMAR。隨訪1年后,1號(hào)右眼視力下降,6號(hào)和7號(hào)視力無(wú)變化。3例患者中14484位點(diǎn)突變的有2例(6號(hào)和7號(hào)),11778位點(diǎn)突變的有1例(1號(hào));另外7例視力無(wú)顯著改變。10例患者的視野(VFI和MD)治療前后無(wú)顯著差異(P=0.167,0.20)。未經(jīng)治療的9例患者隨訪觀察1年,視力無(wú)變化。結(jié)論磷酸肌酸鈉治療Leber遺傳性視神經(jīng)病變或許對(duì)14484位點(diǎn)突變患者的視力提高或穩(wěn)定有一定的療效。
[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.
【作者單位】： 華中科技大學(xué)同濟(jì)醫(yī)學(xué)院附屬同濟(jì)醫(yī)院眼科;鄂州市第五醫(yī)院藥劑科;鄂州市第五醫(yī)院超聲科;
【分類號(hào)】：R774.6

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