{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T06:25:08Z","timestamp":1793082308330,"version":"4.5.0"},"reference-count":25,"publisher":"BMJ","issue":"4","license":[{"start":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T00:00:00Z","timestamp":1727827200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100017559","name":"Recordati Rare Diseases","doi-asserted-by":"publisher","award":["unrestricted grant from RECORDATI"],"award-info":[{"award-number":["unrestricted grant from RECORDATI"]}],"id":[{"id":"10.13039\/100017559","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Br J Ophthalmol"],"accepted":{"date-parts":[[2024,9,16]]},"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>To analyse the correlation between the physician categories defined by the 3C classification (crystal-complication-compliance) and the ocular manifestations of nephropathic cystinosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>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\u2019s correlation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Photophobia (0.647, p&lt;0.001), visual acuity (\u22120.695, p&lt;0.001), Gahl\u2019s score (0.603, p&lt;0.001), optical coherence tomography (OCT)% (0.713, p&lt;0.001) and in vivo confocal microscopy (IVCM)% (0.845, p&lt;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&lt;0.001), 0.634 (p&lt;0.001), 0.726 (p&lt;0.001) and 0.677 (p&lt;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&lt;0.001) and 0.631 (p&lt;0.001), respectively. With all cystinosis markers, the 3C classification showed a stronger correlation than age and crystal scores by Gahl\u2019s and OCT. Category and age were strongly correlated (0.656, p&lt;0.001). There was a moderate negative correlation with therapeutic compliance with cysteamine eye-drops and categorisation (\u22120.422, p&lt;0.001). The compliance pattern observed may help to explain why the disease progresses in some patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>3C classification is a reliable tool to categorise ocular cystinosis and can support clinical management decisions allowing more reliable comparison of datasets.<\/jats:p><\/jats:sec>","DOI":"10.1136\/bjo-2023-325093","type":"journal-article","created":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T17:20:15Z","timestamp":1727889615000},"page":"442-449","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Evaluation of a new classification system for measuring the progression of ocular cystinosis: an analysis of 64 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