Ocular Cystinosis Experience in Southwestern Ontario
Why this work is in the frame
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Bibliographic record
Abstract
Purpose To evaluate ocular treatment adherence and its impact on clinical outcomes in patients with cystinosis in southwestern Ontario, where the disease incidence is higher due to a founder effect in the Old Order Amish population. Methods This was a retrospective case series of patients with ocular cystinosis seen at Victoria Hospital and the Ivey Eye Institute at St Joseph's Health Care in London, Ontario, Canada, from 2008 to 2023. The authors investigated the demographics, ocular manifestations, and visual outcomes and characteristics in pediatric patients with ocular cystinosis. They also conducted qualitative analysis to characterize medication compliance and identify treatment barriers to compliance. Results This study included 14 patients with ocular cystinosis. In southwestern Ontario, the incidence of cystinosis is approximately 1 in 4,700 live births. Most patients were Old Order Amish, with a family history of cystinosis and consanguineous parents. During treatment, patients were typically asymptomatic, but photophobia was the most reported symptom. Slit-lamp examination revealed cystine crystals in the cornea of all patients; however, best corrected visual acuity and intraocular pressure were unremarkable in every case. A Fisher's exact test revealed a trend toward lower compliance in Amish patients compared to non-Amish patients (odds ratio: 0.067, P = .103). Significant treatment barriers for patients with ocular cystinosis were identified, including compliance issues with frequent eye drop regimens, educational and financial burdens, and geographical and cultural challenges, all impacting patient care and follow-up. Conclusions This study highlights the need for improved strategies to improve treatment compliance and overcome barriers to care for patients with ocular cystinosis, particularly within the Old Order Amish population in southwestern Ontario, to ensure better clinical outcomes and quality of life. [ J Pediatr Ophthalmol Strabismus . 2025;62(2):105–115.]
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Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it