Longitudinal Changes in Scotopic and Mesopic Macular Function as Assessed with Microperimetry in Patients With Stargardt Disease: SMART Study Report No. 2.
Longitudinal Changes in Scotopic and Mesopic Macular Function as Assessed with Microperimetry in Patients With Stargardt Disease: SMART Study Report No. 2.
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DOI：
10.1016/j.ajo.2021.10.014
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发表时间：
2022-04
影响因子：
4.2
通讯作者：
SMART Study Group
中科院分区：
文献类型：
--
作者：
Kong X;Ibrahim-Ahmed M;Bittencourt MG;Strauss RW;Birch DG;Cideciyan AV;Ervin AM;Ho A;Sunness JS;Audo IS;Michaelides M;Zrenner E;Sadda S;Ip MS;West S;Scholl HPN;SMART Study Group
估计和比较通过微视野测量的横截面暗视与中间视黄斑敏感性损失，并报告和比较ABCA 4基因相关性Stargardt病（STGD 1）中暗视和中间视黄斑敏感性损失的纵向比率。多中心前瞻性队列研究。从美国和欧洲的6个中心入组了127例经分子学证实的STGD 1患者，每6个月随访一次，最长持续2年。使用Nidek MP-1 S装置测量中间视觉和暗视觉条件下中央20°的黄斑敏感度。计算中心凹（2°偏心率内）和中心凹外（4°−10°偏心率）的中间视觉黄斑敏感度与正常值的平均偏差（MD），以及中心凹外暗视敏感度的MD。线性混合效应模型用于估计中间视觉和暗视觉的变化。基线间视平均偏差（mMD）和暗视MD（sMD）以及mMD和sMD的纵向变化率。基线时，所有眼睛的sMD均较大，中心凹外sMD与mMD之间的差异为10.7 dB（p<0.001）。纵向上，所有眼睛均显示出统计学显著的恶化趋势：中心凹mMD和中心凹外mMD和sMD变化率分别为0.72（95%CI：0.37至1.07）、0.86（95%CI：0.58至1.14）和1.12（95%CI：0.66至1.57）dB/年。在STGD 1中，在中心凹外，暗视黄斑功能的丧失先于且快于中间视黄斑功能的丧失。使用微视野检查的暗视和中间视黄斑敏感性为STGD 1治疗试验提供了替代的视功能结果。这项多中心研究对127名患有分子确认的ABCA-4基因突变相关Stargardt病的患者进行了长达2年的随访。研究发现，暗视黄斑功能丧失比中视黄斑功能丧失更早、更快。使用微视野检查的暗视和中间视黄斑敏感性为ABCA-4基因相关Stargardt病的治疗试验提供了可行的视功能结果。
To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losses measured by microperimetry, and to report and compare the longitudinal rates of scotopic and mesopic macular sensitivity losses in ABCA4 gene associated Stargardt Disease (STGD1). Multicenter prospective cohort study. 127 molecular confirmed STGD1 patients enrolled from 6 centers in the USA and Europe and followed every 6 months for up to 2 years. The Nidek MP-1S device was used to measure macular sensitivities of the central 20° under mesopic and scotopic conditions. The mean deviations (MD) from normal for mesopic macular sensitivity for the fovea (within 2° eccentricity) and extrafovea (4°−10° eccentricity), and the MD for scotopic sensitivity for the extrafovea were calculated. Linear mixed effects models were used to estimate mesopic and scotopic changes. Baseline mesopic mean deviation (mMD) and scotopic MD (sMD) and rates of longitudinal changes in the mMDs and sMD. At baseline, all eyes had larger sMD, and the difference between extrafoveal sMD and mMD was 10.7 dB (p<.001). Longitudinally, all eyes showed a statistically significant worsening trend: the rates of foveal mMD and extrafoveal mMD and sMD changes were 0.72 (95%CI: 0.37 to 1.07), 0.86 (95%CI: 0.58 to 1.14) and 1.12 (95%CI: 0.66 to 1.57) dB/year, respectively. In STGD1, in extrafovea, loss of scotopic macular function preceded and was faster than the loss of mesopic macular function. Scotopic and mesopic macular sensitivities using microperimetry provide alternative visual function outcomes for STGD1 treatment trials. This multicenter study followed 127 patients with molecularly confirmed ABCA-4 gene mutation associated Stargardt disease for up to 2 years. The study found that scotopic macular function loss was earlier and faster than mesopic macular function loss. Scotopic and mesopic macular sensitivities using microperimetry provide viable visual function outcomes for treatment trials of ABCA-4 gene associated Stargardt disease.
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影响因子：
13.7
作者：
Kong, Xiangrong;Strauss, Rupert W.;Scholl, Hendrik P. N.
通讯作者：
Scholl, Hendrik P. N.
影响因子：
1.2
作者：
Park SP;Chang S;Allikmets R;Smith RT;Burke TR;Gregory-Roberts E;Tsang SH
通讯作者：
Tsang SH
DOI：
10.1073/pnas.1913179117
发表时间：
2020-02-04
影响因子：
11.1
作者：
Hanany, Mor;Rivolta, Carlo;Sharon, Dror
通讯作者：
Sharon, Dror
影响因子：
3.5
作者：
Cideciyan, Artur V.;Swider, Malgorzata;Palczewski, Krzysztof
通讯作者：
Palczewski, Krzysztof
影响因子：
2.4
作者：
Nishida, Y;Murata, T;Kani, K
通讯作者：
Kani, K