Atrophy Expansion Rates in Stargardt Disease Using Ultra-Widefield Fundus Autofluorescence.
Atrophy Expansion Rates in Stargardt Disease Using Ultra-Widefield Fundus Autofluorescence.
复制标题
应用超宽视野眼底自发荧光检测Stargardt病的萎缩扩张率。
DOI：
10.1016/j.xops.2021.100005
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发表时间：
2021-03
影响因子：
--
通讯作者：
Chen, Fred K.
中科院分区：
文献类型：
--
作者：
Jeffery, Rachael C. Heath;Thompson, Jennifer A.;Lo, Johnny;Lamey, Tina M.;McLaren, Terri L.;McAllister, Ian L.;Mackey, David A.;Constable, Ian J.;De Roach, John N.;Chen, Fred K.
关键词：
目的：应用超宽视野（UWF）眼底自发荧光（FAF）技术研究Stargardt病（STGD 1）的萎缩扩大率（ER）。回顾性、纵向研究。使用UWF FAF和Heidelberg 30° × 30°和55° × 55° FAF成像评价的双等位基因ABCA 4突变患者。将继发于STGD 1的萎缩患者分为基因型组：A组，双等位基因重度或空样变异伴早发性疾病; B组，1个中间变异反式伴重度或空样变异; C组，1个轻度变异反式伴重度或空样变异或迟发性疾病。在基线和随访时，手动勾勒出明确降低的自体荧光（DIFF-A）的边界，并记录面积（单位：平方毫米）。进行Bland-Altman分析以检查观察者与器械之间的一致性。采用线性混合模型对ER的预测因子进行了评价，包括面积和平方根面积（SRA）。患者和眼部的Doppler面积ER和Doppler SRA ER的预测因素包括发病年龄、症状持续时间、基因型组、基线视力和基线萎缩大小。共招募了来自69名携带61种独特ABCA 4变体的患者（33名男性[47%];平均年龄±标准差，41 ± 20岁;范围，10-83岁）的138只眼睛。超宽视场FAF测量相当于海德堡30° × 30°成像。基线糖尿病面积是糖尿病面积ER的唯一显著预测因子（P < 0.001）。基线时的年龄和基因型组是DADSRA ER的预测因素。明确减少的自体荧光面积ER范围为4.65 mm 2/年（A组）至0.62 mm 2/年（C组）。超宽视野FAF是评估STGD 1中萎缩ER的可行且可靠的方法。ABCA 4突变严重程度在预测ER萎缩中的价值值得进一步研究。
To investigate atrophy expansion rate (ER) using ultra-widefield (UWF) fundus autofluorescence (FAF) in Stargardt disease (STGD1). Retrospective, longitudinal study. Patients with biallelic ABCA4 mutations who were evaluated with UWF FAF and Heidelberg 30° × 30° and 55° × 55° FAF imaging. Patients with atrophy secondary to STGD1 were classified into genotype groups: group A, biallelic severe or null-like variants with early-onset disease; group B, 1 intermediate variant in trans with severe or null-like variant; and group C, 1 mild variant in trans with severe or null-like variant or late-onset disease. The boundaries of definitely decreased autofluorescence (DDAF) were outlined manually and areas (in square millimeters) were recorded at baseline and follow-up. Bland-Altman analysis was conducted to examine agreement between observers and devices. Linear mixed modeling was used to evaluate predictors of ER in DDAF area and square root area (SRA). Patient and ocular predictors of DDAF area ER and DDAF SRA ER included age at onset, duration of symptoms, genotype group, baseline visual acuity, and baseline atrophy size. A total of 138 eyes from 69 patients (33 men [47%]; mean age ± standard deviation, 41 ± 20 years; range, 10–83 years) carrying 61 unique ABCA4 variants were recruited. Ultra-widefield FAF measurements were equivalent to Heidelberg 30° × 30° imaging. Baseline DDAF area was the only significant predictor of DDAF area ER (P < 0.001). Age at baseline and genotype group were predictors for DDAF SRA ER. Definitely decreased autofluorescence area ER ranged from 4.65 mm2/year (group A) to 0.62 mm2/year (group C). Ultra-widefield FAF is a feasible and reliable method for assessing atrophy ER in STGD1. The value of ABCA4 mutation severity in predicting atrophy ER warrants further investigation.
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