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一大群非综合征性视网膜色素变性患者中的高反射性神经节细胞层带:发生率及临床相关性

Hyperreflective ganglion cell layer band in a large cohort of non-syndromic retinitis pigmentosa: Frequency and clinical correlations.

作者信息

Dias Margarida Q, Gouveia Nuno, Franca Maria, Murta Joaquim, Silva Rufino, Marques João Pedro

机构信息

Ophthalmology Unit, Unidade Local de Saúde Coimbra, (ULS Coimbra), Coimbra, Portugal.

Clinical Academic Center of Coimbra (CACC), Coimbra, Portugal.

出版信息

Acta Ophthalmol. 2025 May;103(3):e148-e155. doi: 10.1111/aos.16772. Epub 2024 Oct 9.

DOI:10.1111/aos.16772
PMID:39382145
Abstract

PURPOSE

Recently, an 'hyperreflective ganglion cell layer band' (HGB) has been described on spectral-domain optical coherence tomography (SD-OCT) in a subset of patients with retinitis pigmentosa (RP). This study aims to validate and describe the frequency of HGB in a large cohort of Portuguese patients with RP.

METHODS

Single-centre, cross-sectional cohort study included consecutive patients with a genetic diagnosis of RP. SD-OCT images were reviewed to identify the presence of the HGB and other retinal comorbidities. The HGB was defined as a continuous hyperreflective band within the thickness of the ganglion cell layer (GCL). We built mixed-effects regression models, accounting for inter-eye correlations, to investigate features predictive of visual acuity. Subsequently, a reduced model was fitted.

RESULTS

A total of 398 eyes from 201 patients were included. HGB was identified in 69 (17.3%) eyes from 39 (19.4%) patients. Patients presenting with the HGB were significantly younger at diagnosis and at symptom onset. Median BCVA [ETDRS (IQR)] was 65 (29) letters in eyes with the HGB and 70 (21) letters in eyes without HGB (p < 0.001). In both the full and reduced mixed-effects models, the presence of HGB and macular hole (MH) was significantly associated with worse BCVA.

CONCLUSIONS

This study validates the recent description of HGB within the GCL in a subset of patients with RP. Eyes with HGB demonstrated significantly worse BCVA compared to those without HGB, suggesting that the presence of HGB may serve as an SD-OCT biomarker of worse visual prognosis in these patients.

摘要

目的

最近,在一部分视网膜色素变性(RP)患者的光谱域光学相干断层扫描(SD-OCT)中发现了一种“高反射性神经节细胞层带”(HGB)。本研究旨在验证并描述一大批葡萄牙RP患者中HGB的出现频率。

方法

单中心横断面队列研究纳入了连续的经基因诊断为RP的患者。对SD-OCT图像进行评估,以确定HGB和其他视网膜合并症的存在情况。HGB被定义为神经节细胞层(GCL)厚度内的连续高反射带。我们建立了考虑眼间相关性的混合效应回归模型,以研究预测视力的特征。随后,拟合了一个简化模型。

结果

共纳入201例患者的398只眼。在39例(19.4%)患者的69只眼(17.3%)中发现了HGB。出现HGB的患者在诊断时和症状出现时明显更年轻。有HGB的眼的最佳矫正视力(BCVA)中位数[ETDRS(四分位间距)]为65(29)个字母,无HGB的眼为70(21)个字母(p<0.001)。在完整和简化的混合效应模型中,HGB和黄斑裂孔(MH)的存在均与较差的BCVA显著相关。

结论

本研究验证了最近在一部分RP患者的GCL中对HGB的描述。与无HGB的眼相比,有HGB的眼的BCVA明显更差,这表明HGB的存在可能是这些患者视力预后较差的SD-OCT生物标志物。

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