CFF Long: Long-Term Visual Acuity Trajectories in Japanese LHON Patients

Yasuyuki Takai's dataset contains longitudinal visual acuity data for a cohort of Japanese patients with Leber hereditary optic neuropathy (LHON). The data includes best-corrected visual acuity (BCVA) measurements from onset to 120 months for 27 patients (53 eyes) who were followed continuously for at least 10 years. The dataset was last updated on April 28, 2026.

Use Cases

• Modeling long-term visual acuity trajectories based on the described three-phase (Acute, Chronic, Late Chronic) progression.
• Estimating time-to-event outcomes, such as achieving specific BCVA thresholds (≤1.6 or ≤1.3 logMAR), based on the Kaplan–Meier analyses mentioned.
• Analyzing potential ethnic differences in LHON progression by comparing this Japanese cohort data with other populations.
• Benchmarking clinical trial outcomes against the phase-specific, long-term descriptive trends provided in this cohort.

Strengths

• Provides rare long-term follow-up data (≥10 years) for a genetically confirmed rare disease cohort.
• Includes detailed temporal analysis with piecewise linear mixed-effects models over three distinct clinical phases.
• Data is shared under a permissive CC-BY-4.0 license, facilitating reuse.

Limitations

• Row count is unknown, which may limit suitability assessment.
• Column-level documentation is absent; field semantics must be inferred after download.
• The authors note a modest sample size (27 patients) and selection bias towards long-term attendees, limiting generalizability.

Provenance

Source

Single-center retrospective cohort study at a tertiary eye hospital in Japan, authored by Yasuyuki Takai.

Collection Method

Retrospective cohort study of genetically confirmed LHON patients with continuous follow-up.

Time Range

Follow-up data from disease onset to 120 months (10 years).

Freshness

Last updated 2026-04-28 17:27:01; freshness should be verified.

Geography

Japan (specifically, a tertiary eye hospital cohort).