SynthesisOphthalmology. Retina2020
Long-term Natural History of Atrophy in Eyes with Choroideremia-A Systematic Review and Meta-analysis of Individual-Level Data.
Synthesis in Ophthalmology. Retina, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
19 citing papers in PubMed, 2 syntheses or guidelines pooled it, 23 citations in OpenAlex.
- Long-term natural history of visual acuity in eyes with choroideremia: a systematic review and meta-analysis of data from 1004 individual eyes.The British journal of ophthalmology · 2021Pooled it
- Progression of Unifocal versus Multifocal Geographic Atrophy in Age-Related Macular Degeneration: A Systematic Review and Meta-analysis.Ophthalmology. Retina · 2020Pooled it
- Progression of Macular Tissue Loss in Choroideremia and Its Association with Baseline Fundus Autofluorescence Morphology.Ophthalmology science · 2026Article
- Validation of Ultra-Widefield Autofluorescence Imaging as an Endpoint for Disease Progression in Choroideremia: A Comparative Study.Ophthalmology science · 2026Article
- A 48-Month, Prospective, Multicenter, Natural History Study of Choroideremia.Ophthalmology science · 2026Article
- Somatic mosaicism and skewed X-chromosome inactivation associated with male-pattern choroideremia phenotype in a female patient.Documenta ophthalmologica. Advances in ophthalmology · 2026Article
- Longitudinal and cross-sectional study of retinal phenotypes and visual function in choroideremia carriers: a new grading system.Eye and vision (London, England) · 2026Article
- Longitudinal Assessment of Cone Structure, Choriocapillaris, and Retinal Sensitivity in Choroideremia.Investigative ophthalmology & visual science · 2025Article
- Gene therapy for choroideremia: progress, potential and pitfalls.Expert opinion on biological therapy · 2025Review
- Long-term natural history of ellipsoid zone width inThe British journal of ophthalmology · 2025Article
- Longitudinal assessment of female carriers of choroideremia using multimodal retinal imaging.The British journal of ophthalmology · 2025Article
- Early diagnosis and treatment by newborn screening (NBS) or family history is associated with improved visual outcomes for long-chain 3-hydroxyacylCoA dehydrogenase deficiency (LCHADD) chorioretinopathy.Journal of inherited metabolic disease · 2024Article
- Using Goldmann Visual Field Volume to Track Disease Progression in Choroideremia.Ophthalmology science · 2023Article
- Local progression kinetics of macular atrophy in recessive Stargardt disease.Ophthalmic genetics · 2023Article
- Choroideremia: The Endpoint Endgame.International journal of molecular sciences · 2023Review
- Natural history of central sparing in geographic atrophy secondary to non-exudative age-related macular degeneration.The British journal of ophthalmology · 2022Article
- A hierarchical Bayesian entry time realignment method to study the long-term natural history of diseases.Scientific reports · 2022Article
- An In Silica Model for RPE Loss Patterns in Choroideremia.Investigative ophthalmology & visual science · 2021Article
- Longitudinal Study to Assess the Quantitative Use of Fundus Autofluorescence for Monitoring Disease Progression in Choroideremia.Journal of clinical medicine · 2021Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
purposeTo conduct a systematic review and meta-analysis of the natural history of atrophy secondary to choroideremia (CHM). CLINICAL RELEVANCE: A sensitive and reliable anatomic measure to monitor disease progression is needed in treatment trials for CHM. However, the long-term natural history of the residual retinal pigment epithelium (RPE) is unclear, with reported RPE area decline rates varying widely among patients.
methodsWe searched in 7 literature databases up through July 17, 2019, to identify studies that assessed the residual RPE area in untreated eyes with CHM using fundus autofluorescence (FAF). We sought individual-eye data and investigated the RPE decline pattern using 3 models: the area linear model (ALM), radius linear model (RLM), and area exponential model (AEM), in which the area, radius, and log-transformed area of RPE change linearly with time, respectively. To account for different eyes' entry times into the studies, we added a horizontal translation factor to each dataset. The RPE decline rate was estimated using a 2-stage random-effects meta-analysis. We assessed the risk of bias using the Quality In Prognosis Studies tool.
resultsOf 807 articles screened, we included 9 articles containing cross-sectional data (257 eyes) from 6 studies and longitudinal data (229 visits from 68 eyes) from 5 studies. The residual RPE area followed a trend of exponential decay as a function of patient age. After the introduction of horizontal translation factors to longitudinal datasets of individual eyes, the datasets fit along a straight line in the AEM over nearly 60 years (r
conclusionsThe loss of residual RPE area in untreated eyes with CHM follows the AEM over approximately 60 years. Log-transformed residual RPE area measured by FAF can serve as an anatomic endpoint to monitor CHM.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.