Aims To analyse the correlation between the physician categories defined by the 3C classification (crystal-complication-compliance) and the ocular manifestations of nephropathic cystinosis. Methods The last visit data of 64 patients aged between 2 and 64 attending the centre for management of cystinosis were reviewed. Each patient had been placed into one of four categories by the clinician based on disease severity. The correlation between these categories and markers of the disease was assessed using Pearson’s correlation. Results Photophobia (0.647, p<0.001), visual acuity (−0.695, p<0.001), Gahl’s score (0.603, p<0.001), optical coherence tomography (OCT)% (0.713, p<0.001) and in vivo confocal microscopy (IVCM)% (0.845, p<0.001), showed a strong, highly significant correlation between key signs and symptoms and the 3C classification. Corneal complications were strongly correlated with the 3C classification with scores of 0.802 (p<0.001), 0.634 (p<0.001), 0.726 (p<0.001) and 0.677 (p<0.001) for band keratopathy, keratitis, neovascularisation and corneal ulceration, respectively. 75% of those classified as most severe had all four complications. The use of artificial tears and ciclosporin strongly correlated with the categorisation, 0.574 (p<0.001) and 0.631 (p<0.001), respectively. With all cystinosis markers, the 3C classification showed a stronger correlation than age and crystal scores by Gahl’s and OCT. Category and age were strongly correlated (0.656, p<0.001). There was a moderate negative correlation with therapeutic compliance with cysteamine eye-drops and categorisation (−0.422, p<0.001). The compliance pattern observed may help to explain why the disease progresses in some patients. Conclusion 3C classification is a reliable tool to categorise ocular cystinosis and can support clinical management decisions allowing more reliable comparison of datasets. All data relevant to the study are included in the article or uploaded as online supplemental information.

中文翻译：

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测量眼胱氨酸病进展的新分类系统的评估：64 例患者的分析


目的 分析 3C 分类 （晶体并发症-依从性） 定义的医生类别与肾病性胱氨酸病的眼部表现之间的相关性。方法 回顾了 64 例在胱氨酸病管理中心就诊的 2 至 64 例患者的末次就诊数据。临床医生根据疾病严重程度将每位患者分为四类之一。使用 Pearson 相关性评估这些类别与疾病标志物之间的相关性。结果 畏光 （0.647， p<0.001）、视力 （-0.695， p<0.001）、Gahl 评分 （0.603， p<0.001）、光学相干断层扫描 （OCT） % （0.713， p<0.001） 和体内共聚焦显微镜 （IVCM） % （0.845， p<0.001） 显示关键体征和症状与 3C 分类之间存在很强、高度显着的相关性。角膜并发症与 3C 分类密切相关，带状角膜病变、角膜炎、新生血管形成和角膜溃疡的评分分别为 0.802 （p<0.001） 、0.634 （p<0.001）、0.726 （p<0.001） 和 0.677 （p<0.001）。75% 被归类为最严重的患者患有所有四种并发症。人工泪液和环孢素的使用与分类密切相关，分别为 0.574 （p<0.001） 和 0.631 （p<0.001）。对于所有胱氨酸病标志物，3C 分类显示出比年龄更强的相关性，Gahl 和 OCT 的晶体评分。类别和年龄密切相关 （0.656，p<0.001）。与半胱胺滴眼液的治疗依从性和分类呈中度负相关 （-0.422，p<0.001）。观察到的依从性模式可能有助于解释为什么某些患者会进展疾病。 结论 3C 分类是眼胱氨酸病分类的可靠工具，可以支持临床管理决策，从而实现更可靠的数据集比较。与研究相关的所有数据都包含在文章中或作为在线补充信息上传。