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遗传性视网膜疾病患儿的视网膜血管血氧测定法。

Retinal vessel oximetry in children with inherited retinal diseases.

机构信息

Department of Ophthalmology, University of Basel, Basel, Switzerland.

Institute of Molecular and Clinical Ophthalmology Basel (IOB), Basel, Switzerland.

出版信息

Acta Ophthalmol. 2021 Feb;99(1):52-60. doi: 10.1111/aos.14466. Epub 2020 Jun 22.

Abstract

BACKGROUND

Retinal vessel oximetry (RO) has been used to show altered metabolic function in patients with inherited retinal diseases (IRDs). The aim of this study was to investigate RO parameters of children with IRDs and presumed IRD carriers (pIRDc) and to compare them to controls.

METHODS

In this cross-sectional cohort study, 142 eyes from 71 Caucasian subjects were included: 40 eyes with IRDs, 26 eyes with pIRDc and 76 control eyes. The oxygen saturation was measured with the Retinal Vessel Analyser (IMEDOS Systems UG, Jena, Germany). Mean oxygen saturations in the peripapillary retinal arterioles (A-SO ; %) and venules (V-SO ; %) were estimated, and their difference (A-V SO ; %) was calculated. In addition, we evaluated the mean diameter in all major retinal arterioles (D-A; μm) and venules (D-V; μm). anova-based linear mixed-effects models were calculated with SPSS .

RESULTS

In general, children suffering from IRDs differed from controls when the A-SO and A-V SO were taken into account: both the A-SO and the A-V SO were significantly increased (p = 0.012). In subgroup analyses, children suffering from rod-cone dystrophy (RCD) presented an A-SO increase (99.12 ± 8.24%) when compared to controls (91.33 ± 10.34%, p = 0.014) and pIRDc (92.37 ± 6.57%, p = 0.065). For V-SO significant changes in RCD (67.42 ± 9.19%) were found in comparison with controls (58.24 ± 11.74%, p < 0.041), pIRDc (56.67 ± 7.16%, p = 0.007), cone-rod dystrophies (CRD, 52.17 ± 5.32%, p < 0.001) and inherited macular dystrophies (IMD, 55.74 ± 6.96%, p = 0.004), In addition, A-V SO was decreased in RCD (31.69 ± 3.92%) when measured against CRD (41.9 ± 8.87%, p = 0.017) or IMD (39.52 ± 8.95%, p = 0.059).

CONCLUSION

In general, we found that children with IRDs presented early metabolic changes. Within IRDs, children with RCD showed more affected metabolic changes. Thus, RO may support early screening to rule out IRDs in children, and more precisely may help to differentiate those suffering from RCD.

摘要

背景

视网膜血管血氧计(RO)已被用于显示遗传性视网膜疾病(IRDs)患者代谢功能的改变。本研究的目的是研究 IRD 患儿和疑似 IRD 携带者(pIRDc)的 RO 参数,并将其与对照组进行比较。

方法

在这项横断面队列研究中,纳入了 71 名高加索受试者的 142 只眼睛:40 只眼睛患有 IRD,26 只眼睛患有 pIRDc,76 只眼睛为对照组。使用视网膜血管分析仪(IMEDOS Systems UG,耶拿,德国)测量氧饱和度。估计视盘周围视网膜小动脉(A-SO;%)和小静脉(V-SO;%)的平均氧饱和度,并计算它们之间的差异(A-V SO;%)。此外,我们评估了所有主要视网膜小动脉(D-A;μm)和小静脉(D-V;μm)的平均直径。使用 SPSS 计算基于anova 的线性混合效应模型。

结果

一般来说,患有 IRD 的儿童与对照组相比,当考虑到 A-SO 和 A-V SO 时,两者的 A-SO 和 A-V SO 均显著增加(p=0.012)。在亚组分析中,患有杆状-锥状营养不良(RCD)的儿童的 A-SO 增加(99.12±8.24%),与对照组(91.33±10.34%,p=0.014)和 pIRDc(92.37±6.57%,p=0.065)相比。对于 V-SO,与对照组(58.24±11.74%,p<0.041)、pIRDc(56.67±7.16%,p=0.007)、锥杆营养不良(CRD,52.17±5.32%,p<0.001)和遗传性黄斑营养不良(IMD,55.74±6.96%,p=0.004)相比,RCD 患者的 V-SO 变化明显。此外,与 CRD(41.9±8.87%,p=0.017)或 IMD(39.52±8.95%,p=0.059)相比,RCD 患者的 A-V SO 降低(31.69±3.92%)。

结论

一般来说,我们发现患有 IRD 的儿童存在早期代谢变化。在 IRD 中,患有 RCD 的儿童表现出更多受影响的代谢变化。因此,RO 可能有助于对儿童进行早期筛查以排除 IRD,并且更准确地可能有助于区分患有 RCD 的儿童。

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