Evidence map›Paper›PMID 39357989›Full record

ArticleThe British journal of ophthalmology2025

Evaluation of a new classification system for measuring the progression of ocular cystinosis: an analysis of 64 patients.

Hong Liang, Christophe Baudouin, Vincenzo Giordano

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Article in The British journal of ophthalmology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

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0cells of the map it votes in
3citing papers in PubMed
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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

Who cites it

3 citing papers in PubMed.

  1. CystadropsOphthalmology and therapy · 2025
    Article
  2. Article
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4 · The record

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5 · Who and what money

Authors and funding

3 authors.

Hong LiangNational Hospital Centre for Ophthalmology Quinze-Vingts, Paris, France lianghongfr@yahoo.fr.ORCID http://orcid.org/0000-0002-0579-8651
Christophe BaudouinNational Hospital for Ophthalmology Quinze-Vingts, Paris, France.
Vincenzo GiordanoRecordati Rare Diseases SARL, Puteaux, France.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

aimsTo analyse the correlation between the physician categories defined by the 3C classification (crystal-complication-compliance) and the ocular manifestations of nephropathic cystinosis.

methodsThe 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.

resultsPhotophobia (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.

conclusion3C classification is a reliable tool to categorise ocular cystinosis and can support clinical management decisions allowing more reliable comparison of datasets.

Indexed as

Corneal DiseasesCystinosisAdolescentAdultChildChild, PreschoolCysteamineDisease ProgressionFemaleHumansMaleMicroscopy, ConfocalMiddle AgedRetrospective StudiesTomography, Optical CoherenceVisual AcuityCysteamineCorneaInflammationNeovascularisationVisual perception

Identifiers

PMID39357989
PMCPMC12013570

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