Spectral-domain optical coherence tomography measures of outer segment layer progression in patients with X-linked retinitis pigmentosa.

Spectral-domain optical coherence tomography measures of outer segment layer progression in patients with X-linked retinitis pigmentosa.
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X连锁性视网膜炎的患者外部段层进展的光谱域光学相干断层扫描测量。

DOI:
10.1001/jamaophthalmol.2013.4160
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发表时间:
2013-09
期刊:
影响因子:
8.1
通讯作者:
Hood, Donald C.
Hood, Donald C.
中科院分区:
医学1区
文献类型:
--
作者:
Birch, David G.;Locke, Kirsten G.;Wen, Yuquan;Locke, Kelly I.;Hoffman, Dennis R.;Hood, Donald C.

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在短期变异性的背景下,确定内段椭球区(EZ,即内/外段边界)宽度的年变化率,应使我们能够更好地理解这一措施在x -联性视网膜色素变性(XLRP)的未来治疗试验中的价值。从RP的光谱域光学相干断层扫描(SD-OCT)测量中确定显示EZ的中心区域的宽度,并确定EZ的短期重复变异性和年变化率。隐性或单纯性RP患者(年龄范围8-65岁,平均年龄40.5岁)在同一天进行两次扫描以评估重测变异性。参加正在进行的双盲治疗试验(二十二碳六烯酸vs安慰剂;clinicaltrials.gov NCT00100230)的XLRP患者(年龄范围8-27岁,平均年龄15.2岁)每隔3年进行一次横贯水平经络的光谱域光学相干断层扫描。医学视网膜研究中心。48例RP患者,其中隐性或单纯性RP 20例,XLRP 28例,健康对照23例。计算及比较3次年度访港期间的经济隔离区宽度。重测差异呈正态分布,差异的大小与平均EZ宽度无关。EZ宽度的重测差异的平均值(SD)为0.08°(0.22°)(范围为- 0.30°至0.60°)。因此,95%的重测差异在±0.43°(124µm)范围内。在28例XLRP患者中,27例患者在2年后显着减少EZ宽度。XLRP患者的EZ宽度平均每年减少0.86°(248µm,或7%)。EZ宽度平均下降率(7%)转化为等效功能视网膜面积的平均变化率为13%。这一变化率与视野和全视场视网膜电图的结果一致。然而,与视野和视网膜电图不同,重复变异性小于年变化率。这些结果支持EZ宽度作为RP前瞻性临床试验结果测量的有效性。
Determining the annual rate of change in the width of the inner segment ellipsoid zone (EZ; ie, inner/outer segment border) in the context of short-term variability should allow us to better understand the value of this measure for future treatment trials in X-linked retinitis pigmentosa (XLRP). To identify the width of the central region showing an EZ and to determine the short-term repeat variability and the annual rate of change in the width of the EZ from spectral-domain optical coherence tomography (SD-OCT) measures in RP. Patients with recessive or simplex RP (age range, 8–65 years; mean age, 40.5 years) underwent scanning twice on the same day to evaluate test-retest variability. Patients with XLRP (age range, 8–27 years; mean age, 15.2 years) from a larger group participating in an ongoing double-blind treatment trial (docosahexaenoic acid vs placebo; clinicaltrials.gov NCT00100230) underwent spectral-domain optical coherence tomography line scanning across the horizontal meridian at 3 yearly intervals. Research center specializing in medical retina. Forty-eight patients with RP, including 20 with recessive or simplex RP and 28 with XLRP, and 23 healthy control subjects. Widths of the EZ calculated and compared among the 3 annual visits. Test-retest differences were normally distributed, and the magnitude of the difference was independent of mean EZ width. The mean (SD) for test-retest differences in EZ width was 0.08° (0.22°) (range, −0.30° to 0.60°). Thus, 95%of all test-retest differences fall within ±0.43° (124 µm). Of the 28 patients with XLRP, 27 showed a significant decrease in EZ width after 2 years. Patients with XLRP showed a mean annual decrease in EZ width of 0.86° (248 µm, or 7%). The mean rate of decline in EZ width (7%) translates into a mean rate of change of 13%for the equivalent area of functioning retina. This rate of change is consistent with that reported for visual fields and full-field electroretinograms. Unlike visual fields and electroretinograms, however, the repeat variability is less than the annual rate of change. These results support the validity of EZ width as an outcome measure in prospective clinical trials in RP.
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