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中国髓鞘少突胶质细胞糖蛋白抗体阳性儿童视神经炎的临床特征和预后。

Clinical characteristics and prognosis of myelin oligodendrocyte glycoprotein antibody-seropositive paediatric optic neuritis in China.

机构信息

Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.

Joint Shantou International Eye Center, Shantou University and Chinese University of Hong Kong, Shantou, China.

出版信息

Br J Ophthalmol. 2019 Jun;103(6):831-836. doi: 10.1136/bjophthalmol-2018-312399. Epub 2018 Jul 26.

Abstract

BACKGROUND/AIMS: To investigate clinical characteristics and prognosis of paediatric optic neuritis (PON) in patients seropositive for myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China.

METHODS

Children displaying initial onset of optic neuritis (ON) were recruited from the Neuro-ophthalmology Department in the Chinese People's Liberation Army General Hospital from January 2016 to August 2017. They were assigned into three groups based on antibody status: MOG-Ab-seropositive ON (MOG-ON), aquaporin-4 antibody-seropositive ON (AQP4-ON) and double seronegative ON (seronegative-ON).

RESULTS

Totally 48 patients were assessed, including 25 MOG-ON (52.1%), 7 AQP4-ON (14.6%) and 16 seronegative-ON (33.3%). The MOG-ON and seronegative-ON cohorts had equal ratios of female/male, but the AQP4-ON cohort was predominantly females (100%). The patients with MOG-ON were significantly younger at onset compared with the AQP4-ON group. Of the MOG-ON eyes, 97.6% had good recovery of visual acuity (VA) (≥0.5) compared with33.3% of AQP4-ON eyes (p<0.001). However, there was no significant difference compared with the seronegative-ON eyes (82.6%, p0.052). Two children in the MOG-ON group ended up being diagnosed with acute disseminated encephalomyelitis, while only one patient in the AQP4-ON group developed neuromyelitis optica during follow-up. Patients with MOG-ON had thicker peripapillary retinal nerve fibre layers overall and in the superior and inferior quadrants than in patients with AQP4-ON (p0.005, p0.002 and p0.024, respectively). In addition, the macular ganglion cell-inner plexiform in MOG-ON eyes became significantly thicker than in AQP4-ON eyes (p=0.029). Orbital MRI revealed a larger proportion of patients with MOG-ON had intracranial optic nerve involvement than patients with seronegative-ON (51.2% vs 17.4%, p0.009).

CONCLUSION

MOG-ON was the most common PON subtype in China. MOG-ON had different clinical features including earlier age of onset, equal female/male ratio, better recovery of VA and thicker peripapillary retinal nerve fibre and macular ganglion cell-inner plexiform layers. MOG-Abs may be a potential biomarker for determining visual prognosis with PON.

摘要

背景/目的:研究中国髓鞘少突胶质细胞糖蛋白抗体(MOG-Ab)阳性的儿科视神经炎(PON)患儿的临床特征和预后。

方法

2016 年 1 月至 2017 年 8 月,从中国人民解放军总医院神经眼科招募了首发视神经炎(ON)的患儿。根据抗体状态将其分为三组:MOG-Ab 阳性 ON(MOG-ON)、水通道蛋白 4 抗体阳性 ON(AQP4-ON)和双阴性 ON(阴性-ON)。

结果

共评估了 48 例患者,其中 25 例为 MOG-ON(52.1%),7 例为 AQP4-ON(14.6%),16 例为阴性-ON(33.3%)。MOG-ON 和阴性-ON 两组的男女比例相等,但 AQP4-ON 组均为女性(100%)。MOG-ON 患者的发病年龄明显低于 AQP4-ON 组。MOG-ON 眼的视力(VA)恢复良好(≥0.5)的比例为 97.6%,而 AQP4-ON 眼为 33.3%(p<0.001)。然而,与阴性-ON 眼相比,差异无统计学意义(82.6%,p0.052)。MOG-ON 组有 2 例患儿最终被诊断为急性播散性脑脊髓炎,而 AQP4-ON 组只有 1 例患者在随访中发展为视神经脊髓炎。MOG-ON 患者的视盘周围视网膜神经纤维层(pRNFL)整体及上、下象限均明显厚于 AQP4-ON 患者(p0.005,p0.002 和 p0.024)。此外,MOG-ON 眼的黄斑神经节细胞-内丛状层厚度明显大于 AQP4-ON 眼(p=0.029)。眼眶 MRI 显示,MOG-ON 患者颅内视神经受累的比例明显高于阴性-ON 患者(51.2%比 17.4%,p0.009)。

结论

MOG-ON 是中国最常见的 PON 亚型。MOG-ON 的临床特征不同,包括发病年龄更早、男女比例相等、VA 恢复更好、pRNFL 和黄斑神经节细胞-内丛状层更厚。MOG-Abs 可能是 PON 视功能预后的潜在标志物。

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