肾病性胱氨酸病中的畏光与角膜晶体密度：一项共聚焦显微镜活体检查及眼前节光学相干断层扫描研究

Photophobia and corneal crystal density in nephropathic cystinosis: an in vivo confocal microscopy and anterior-segment optical coherence tomography study.

作者信息

Liang Hong, Baudouin Christophe, Tahiri Joutei Hassani Rachid, Brignole-Baudouin Françoise, Labbe Antoine

机构信息

Department of Ophthalmology III Quinze-Vingts National Ophthalmology Hospital, Paris, France 2Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, DHU View Maintain, Institut national de la santé et de la recherche médicale (INSERM)-DHOS CIC 142.

INSERM, U968, Paris, France 4Sorbonne Universités, UPMC University Paris 06, UMR_S 968, Institut de la Vision, Paris, France 5Centre national de la recherche scientifique (CNRS), UMR_7210, Paris, France.

出版信息

Invest Ophthalmol Vis Sci. 2015 May;56(5):3218-25. doi: 10.1167/iovs.15-16499.

DOI:10.1167/iovs.15-16499

PMID:26024106

Abstract

PURPOSE

To analyze the correlation between photophobia and corneal crystal density in nephropathic cystinosis using in vivo confocal microscopy (IVCM) and anterior-segment optical coherence tomography (AS-OCT).

METHODS

Forty eyes of 20 patients with nephropathic cystinosis aged 7 to 37 years were included in this study. Ophthalmologic investigations included clinician-assessed and self-assessed evaluations of photophobia, slit-lamp biomicroscopy analysis, the depth of crystal deposition (DCD) and the central corneal thickness (CCT) in the central cornea measured with AS-OCT, and IVCM analysis of the crystal density score (IVCM-CysS), inflammatory cell density (IVCM-inf), and nerve damage (IVCM-N). Age, sex, intraleukocyte cystine concentrations (ICC), and the need for renal transplantation were also recorded.

RESULTS

The average subjective and objective photophobia scores were 2.10 ± 1.28 and 1.70 ± 1.41, respectively. Using AS-OCT, the average percentage of crystal infiltration (OCT-CysP) and was 49.56 ± 27.31% (range, 11.45%-95.81%). The mean IVCM-CysS was 8.84 ± 4.34, the mean density of inflammatory cells (IVCM-inf) was 178.28 ± 173.00 cells/mm2, and the mean IVCM-N score was 3.11 ± 2.11. Clinician- and self-assessed estimations of photophobia were correlated (R2 = 0.61). No significant correlation was observed between clinician- and self-assessed photophobia scores and ICC or sex. There were significant correlations between clinician- and self-assessed photophobia scores and age, OCT-CysP, IVCM-CysS, IVCM-inf, and IVCM-N. The IVCM-CysS was also correlated with OCT-CysP (R2 = 0.27), IVCM-inf (R2 = 0.37), and IVCM-N (R2 = 0.56).

CONCLUSIONS

In vivo confocal microscopy and AS-OCT are reliable tools to quantify cystinosis corneal crystals. In patients with nephropathic cystinosis, the intensity of photophobia is associated with the density of crystals, infiltration of inflammatory cells, and nerve damage within the cornea.

摘要

目的

使用共聚焦显微镜（IVCM）和眼前节光学相干断层扫描（AS - OCT）分析肾病性胱氨酸病中畏光与角膜晶体密度之间的相关性。

方法

本研究纳入了20例年龄在7至37岁之间的肾病性胱氨酸病患者的40只眼睛。眼科检查包括临床医生评估和患者自我评估的畏光情况、裂隙灯生物显微镜分析、用AS - OCT测量中央角膜的晶体沉积深度（DCD）和中央角膜厚度（CCT），以及对晶体密度评分（IVCM - CysS）、炎症细胞密度（IVCM - inf）和神经损伤（IVCM - N）进行IVCM分析。还记录了年龄、性别、白细胞内胱氨酸浓度（ICC）以及肾移植需求。

结果

主观和客观畏光评分的平均值分别为2.10±1.28和1.70±1.41。使用AS - OCT，晶体浸润的平均百分比（OCT - CysP）为49.56±27.31%（范围为11.45% - 95.81%）。IVCM - CysS的平均值为8.84±4.34，炎症细胞的平均密度（IVCM - inf）为178.28±173.00个细胞/mm²，IVCM - N评分的平均值为3.11±2.11。临床医生评估和患者自我评估的畏光情况具有相关性（R² = 0.61）。临床医生和患者自我评估的畏光评分与ICC或性别之间未观察到显著相关性。临床医生和患者自我评估的畏光评分与年龄、OCT - CysP、IVCM - CysS、IVCM - inf和IVCM - N之间存在显著相关性。IVCM - CysS也与OCT - CysP（R² = 0.27）、IVCM - inf（R² = 0.37）和IVCM - N（R² = 0.56）相关。