无脉络膜症患者的纵向视力自然病程  

Longitudinal natural history study of visual acuity in choroideremia

作　　者：韩筱煦 张丁丁 李蕙[1] 邹绚[1] 睢瑞芳[1] Han Xiaoxu;Zhang Dingding;Li Hui;Zou Xuan;Sui Ruifang(Department of Ophthalmology,State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China;Medical Research Center,State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院眼科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院医研中心,北京100730

出　　处：《中华眼底病杂志》2024年第5期347-352,共6页Chinese Journal of Ocular Fundus Diseases

基　　金：国家自然科学基金(82301237);北京市自然科学基金(7224338);北京协和医院中央高水平医院临床科研专项(2022-PUMCH-B-102);中央高校基本科研业务费专项资金(3332022007)。

摘　　要：目的观察并分析无脉络膜症(CHM)患者视力进展速度以及视力双眼对称性。方法单中心回顾性纵向队列研究。2009年4月至2022年8月于北京协和医院眼科经临床和基因检测确诊的CHM患者19例38只眼纳入研究。患者均满足随访至少2次、随访间隔至少1年,且每次随访均具有双眼最佳矫正视力(BCVA)结果。BCVA记录时,将小数视力转换为最小分辨角对数(logMAR)视力。19例患者来自16个无血缘关系家系,均为男性。初诊时年龄(39.52±13.24)岁。随访次数(2.63±1.61)次。随访时间(4.95±2.68)年。共纳入50个双眼BCVA数据。基于纵向数据及横断面数据计算视力的年进展速度。采用Spearman相关性分析及Bland-Altman分析评价双眼对称性。结果基于纵向数据的视力进展速率为(0.095±0.148)logMAR单位/年;基于横断面数据的视力进展速率为(0.018±0.009)logMAR单位/年。Spearman相关性分析结果显示,双眼基线BCVA为高度对称(r=0.881,P<0.001);基于纵向数据的双眼视力进展速率的对称性为中度对称(r=0.528,P=0.020)。Bland-Altman分析结果显示,双眼间基线BCVA差异位于95%极限差(LOA)的95%可信区间(CI)内的例数为94.7%,提示双眼基线BCVA对称性好;双眼间BCVA进展速率差异位于95%LOA的95%CI内的例数为89.5%,提示双眼BCVA进展速率为中度对称。Spearman相关性分析及Bland-Altman分析所得双眼对称性结论基本一致。结论CHM患者基于纵向数据、横断面数据的视力进展速率分别为(0.095±0.148)、(0.018±0.009)logMAR单位/年;不应使用不同年龄患者的横断面数据来推断自然病程的进展速度。基线视力高度对称的双眼,其视力进展速度可能存在差异。Objective To observe and analyze the rate of visual acuity progression and binocular symmetry in patients with choroideremia(CHM).Methods A single-center retrospective longitudinal cohort study.From April 2009 to August 2022,38 eyes of 19 patients diagnosed with CHM through clinical and genetic testing at the Department of Ophthalmology,Peking Union Medical College Hospital,were included in this study.All patients underwent at least 2 follow-up visits with a minimum interval of 1 year between visits,and binocular best-corrected visual acuity(BCVA)results were recorded at each follow-up visit.Decimal visual acuity was converted into logarithm of the minimum angle of resolution(logMAR)for analysis.The patient group consisted of 19 males from 16 unrelated families.The age at initial visit was(39.52±13.24)years,with a(2.63±1.61)follow-up visits over a duration of(4.95±2.68)years.A total of 50 binocular BCVA data were included.Annual progression rate of visual acuity was calculated based on longitudinal and cross-sectional data.Spearman correlation coefficient and Bland-Altman method were used to evaluate the binocular symmetry.Results The rate of visual acuity progression was(0.095±0.148)logMAR units/year based on longitudinal data and(0.018±0.009)logMAR units/year based on cross-sectional data.The binocular symmetry for BCVA of the baseline values was strong;however,the binocular symmetry of progression rates for BCVA was moderate.Spearman correlation analysis showed that binocular symmetry in baseline BCVA was high(r=0.881,P<0.001).The symmetry of binocular vision progression rates based on longitudinal data was moderately symmetric(r=0.528,P=0.020).Bland-Altman analysis showed that 94.7%of binocular baseline BCVA differences were within 95%confidence interval(CI)of 95%limit difference(LOA),indicating good symmetry of binocular baseline BCVA.The number of binocular BCVA progression rate differences within 95%CI of 95%LOA was 89.5%,suggesting moderate symmetry in binocular BCVA progression rate.The results of S

关 键 词：无脉络膜症 视力 纵向自然病程 双眼对称性 

分 类 号：R773.4[医药卫生—眼科]