Evidence map›Paper›PMID 42484594›Full record

ArticleInvestigative ophthalmology & visual science2026

Lens Opacity in ABCA4-Associated Stargardt Disease Patients in the ProgStar Study.

Folahan Ibukun, Bai Xi Jasmine Chan, Jehanzeb Khan, Yan Wang, Ann-Margret Ervin, Laura Kühlewein, Rupert W Strauss, Nancy Aychoua, David Birch, Artur Cideciyan and 5 more

Abstract readMulticenter Study
In one paragraph

Article in Investigative ophthalmology & visual science, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors.

Folahan IbukunWilmer Eye Institute, Johns Hopkins Medicine, Baltimore, Maryland, United States.
Bai Xi Jasmine ChanBloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, United States.
Jehanzeb KhanWilmer Eye Institute, Johns Hopkins Medicine, Baltimore, Maryland, United States.
Yan WangBloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, United States.
Ann-Margret ErvinWilmer Eye Institute, Johns Hopkins Medicine, Baltimore, Maryland, United States.
Laura KühleweinUniversity of Tuebingen, Tübingen, Baden-Württemberg, Germany.
Rupert W StraussMedical University of Graz, Graz, Styria, Austria.
Nancy AychouaMoorfields Eye Hospital, London, England, United Kingdom.
David BirchRetina Foundation of the Southwest, Dallas, Texas, United States.
Artur CideciyanUniversity of Pennsylvania, Philadelphia, Pennsylvania, United States.
Mandeep SinghWilmer Eye Institute, Johns Hopkins Medicine, Baltimore, Maryland, United States.
Elias TraboulsiCole Eye Institute, Cleveland Clinic, Cleveland, Ohio, United States.
Paul BernsteinUniversity of Utah, Salt Lake City, Utah, United States.
Michel MichaelidesMoorfields Eye Hospital, London, England, United Kingdom.
Xiangrong KongWilmer Eye Institute, Johns Hopkins Medicine, Baltimore, Maryland, United States.

Funding

Structure-Function Relationships in Stargardt Disease.R21EY032955 · NEI · JOHNS HOPKINS UNIVERSITY · PI KONG, XIANGRONG · 2021 to 2022
$444k
NEI NIH HHS R21 EY032955
6 · The paper itself

Abstract

Purpose: The purpose of this study was to estimate lens opacity (LO) and associated risk factors in patients with ABCA4 associated Stargardt disease (STGD1). Methods: This is a secondary analysis of the multicenter ProgStar study. Patients with molecularly confirmed STGD1 aged ≥6 years from the United States and Europe were enrolled and followed for 2 years. LO was graded using the Age-Related Eye Disease Study (AREDS) protocol during slit lamp examinations. Baseline prevalence and 2-year incidence of LO were estimated. Logistic regression with generalized estimating equation was used to identify risk factors associated with LO. Results: Of the 259 participants, 54% were women, 86% were Caucasian, and the median age of STGD1 symptom onset was 19 years. At baseline, median age = 31 (interquartile range [IQR] = 21-44, range = 6-68) years; LO prevalence = 14.3% (69/483) of study eyes. Older age and later disease symptom onset were significantly associated with LO prevalence: for every decade older, adjusted odds ratio (adjOR) = 4.6 (95% confidence interval [CI] = 2.6-7.9); adjOR for every decade later in symptom onset = 0.7 (95% CI = 0.4-1.0). Among the 354 eyes without LO at baseline, 2-year incidence was 5.6%. Older age was associated with incidence: adjOR for every decade older = 4.1 (95% CI = 1.8-9.1). Posterior-subcapsular cataract was rare and nuclear cataract was the most prevalent or incident type. Conclusions: LO prevalence in ProgStar is higher than in age-matched general population. Age and age of symptom onset were risk factors of LO, suggesting both biological and accelerated aging due to retinal degeneration in STGD1. Trials of STGD1 should closely monitor safety in the anterior segment and consider excluding patients expecting cataract surgery soon.

Indexed as

ATP-Binding Cassette TransportersCataractMacular DegenerationAdolescentAdultAgedChildEuropeFemaleHumansIncidenceMaleMiddle AgedPrevalenceRisk FactorsStargardt DiseaseABCA4 protein, humanATP-Binding Cassette Transporters

Identifiers

PMID42484594
PMCPMC13401119

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Registered trials

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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.