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Perspective April 2021

Tackling the Challenges of Product Development Through a Collaborative Rare Disease Network: The Foundation Fighting Blindness Consortium

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Todd A. Durham; Jacque L. Duncan; Allison R. Ayala; David G. Birch; Janet K. Cheetham; Frederick L. Ferris, III; Carel B. Hoyng; Mark E. Pennesi; José-Alain Sahel; for the Foundation Fighting Blindness Consortium Investigator Group
TAGS: blindness, rare diseases
Trans. Vis. Sci. Tech.. 2021; 10(4):23. doi: https://doi.org/10.1167/tvst.10.4.23

Articles October 2020

The RUSH2A Study: Best-Corrected Visual Acuity, Full-Field Electroretinography Amplitudes, and Full-Field Stimulus Thresholds at Baseline

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David G. Birch; Peiyao Cheng; Jacque L. Duncan; Allison R. Ayala; Maureen G. Maguire; Isabelle Audo; Janet K. Cheetham; Todd A. Durham; Abigail T. Fahim; Frederick L. Ferris, III; Elise Heon; Rachel M. Huckfeldt; Alessandro Iannaccone; Naheed W. Khan; Eleonora M. Lad; Michel Michaelides; Mark E. Pennesi; Katarina Stingl; Ajoy Vincent; Christina Y. Weng; for the Foundation Fighting Blindness Consortium Investigator Group
TAGS: electroretinography, visual acuity
Trans. Vis. Sci. Tech.. 2020; 9(11):9. doi: https://doi.org/10.1167/tvst.9.11.9

Includes: Supplemental Content

Articles June 2020

Quantification of RPE Changes in Choroideremia Using a Photoshop-Based Method

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Yi Zhai; Manlong Xu; Ioannis S. Dimopoulos; David G. Birch; Paul S. Bernstein; Jenny Holt; David Kirn; Peter Francis; Ian M. MacDonald
TAGS: choroideremia, bland-altman plot
Trans. Vis. Sci. Tech.. 2020; 9(7):21. doi: https://doi.org/10.1167/tvst.9.7.21

Includes: Supplemental Content

ARVO Annual Meeting Abstract June 2020

The natural history of choroideremia; progressive loss of visual function and retinal structure

David G Birch; Paul S Bernstein; Ian M MacDonald; Timothy Stout; David S Liao; Kirsten G Locke; Yi Zhai; Audra K Miller; Jenny Holt; Adia Jackson Leung; Somayeh Honarmand; Paul Jordan; Ulrich F O Luhmann; David H Kirn; Peter J Francis

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):1908. doi:

ARVO Annual Meeting Abstract June 2020

The Association between Rod Function and Low Luminance Acuity

Abigail christie; Renee A Denlar; Amy Thompson; Timothy Nguyen; Martin Klein; David G Birch; Karl G Csaky

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):3990. doi:

ARVO Annual Meeting Abstract June 2020

Structure and function from en-face optical coherence tomography, fundus autofluorescence, and rod and cone perimetry in Stargardt disease

Brandon Winward; Vivienne C Greenstein; Donald C Hood; David G Birch

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):1048. doi:

ARVO Annual Meeting Abstract June 2020

Measuring progression at the edge of dense macular scotomas in Stargardt disease

Etienne Schönbach; Rupert Strauss; Mohamed I. Ahmed; Jessica Janes; David G Birch; Artur Cideciyan; Janet Sunness; Xiangrong Kong; Beatriz E Munoz; Michael S Ip; Srinivas Sadda; Hendrik P Scholl

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):3189. doi:

ARVO Annual Meeting Abstract June 2020

Test-retest variability of static perimetry and microperimetry results from the RUSH2A study

Allison Ayala; Wendi Liang; David G Birch; Janet K Cheetham; Elvira N Chegarnov; Jacque L Duncan; Todd Durham; Frederick L Ferris; Eleonora M Lad; Maureen Maguire; Mark E Pennesi

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):3342. doi:

ARVO Annual Meeting Abstract June 2020

Randomized clinical trial evaluating the pharmacodynamics of emixustat in subjects with macular atrophy secondary to Stargardt disease

Jeff Gregory; David G Birch; Ryo Kubota

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):3190. doi:

ARVO Annual Meeting Abstract June 2020

Accelerating research in rare inherited retinal disorders (IRDs) through an international consortium

Janet K Cheetham; Allison Ayala; David G Birch; Jacque L Duncan; Todd Durham; Frederick L Ferris; Maureen Maguire; Mark E Pennesi

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):3356. doi:

ARVO Annual Meeting Abstract June 2020

Baseline visual fields in the RUSH2A study: Associated factors and correlation with measures of disease severity

Jacque L Duncan; Wendi Liang; Isabelle S Audo; David G Birch; Joseph Carroll; Janet K. Cheetham; Laura R Erker; Frederick L Ferris; Elise Heon; Robert Hufnagel; Alessandro Iannaccone; Glenn J Jaffe; Christine N Kay; Maureen Maguire; Michel Michaelides; Mark E Pennesi

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):3364. doi:

ARVO Annual Meeting Abstract June 2020

Deep learning-based automatic segmentation of intact ellipsoid zone area on optical coherence tomography images of USH2A-related retinal degeneration

Sina Farsiu; Jessica Loo; Jacque L Duncan; David G Birch; Glenn J Jaffe

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):485. doi:

ARVO Annual Meeting Abstract June 2020

Rod visual fields in comparison to the recommended functional and structural screening tests for patients treated with hydroxychloroquine

Yan Nuzbrokh; LUZ AMARO-QUIREZA; Donald C Hood; Stephen Tsang; Janet R Sparrow; David G Birch; Vivienne C. Greenstein

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):1058. doi:

ARVO Annual Meeting Abstract June 2020

Evaluation of Two Convolutional Neural Network (CNN) Models for Automatic Segmentation of Retinal Layers from OCT images in Retinitis Pigmentosa (RP)

Yi-Zhong Wang; Wenxuan Wu; David G Birch

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):1632. doi:

ARVO Annual Meeting Abstract June 2020

Meta-learning approach to automatically register multivendor retinal images

Ali Hasan; Zengtian Deng; Jessica Loo; Dibyendu Mukherjee; Jacque L Duncan; David G Birch; Glenn J Jaffe; Sina Farsiu

Invest. Ophthalmol. Vis. Sci.. 2020; 61(7):1634. doi:

Review June 2020

Advancing Clinical Trials for Inherited Retinal Diseases: Recommendations from the Second Monaciano Symposium

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Debra A. Thompson; Alessandro Iannaccone; Robin R. Ali; Vadim Y. Arshavsky; Isabelle Audo; James W. B. Bainbridge; Cagri G. Besirli; David G. Birch; Kari E. Branham; Artur V. Cideciyan; Steven P. Daiger; Deniz Dalkara; Jacque L. Duncan; Abigail T. Fahim; John G. Flannery; Roberto Gattegna; John R. Heckenlively; Elise Heon; K. Thiran Jayasundera; Naheed W. Khan; Henry Klassen; Bart P. Leroy; Robert S. Molday; David C. Musch; Mark E. Pennesi; Simon M. Petersen-Jones; Eric A. Pierce; Rajesh C. Rao; Thomas A. Reh; Jose A. Sahel; Dror Sharon; Paul A. Sieving; Enrica Strettoi; Paul Yang; David N. Zacks; The Monaciano Consortium
TAGS: outcome measures, retinal diseases, surrogate endpoints, genetics, vision, natural history, ethics, gene therapy
Trans. Vis. Sci. Tech.. 2020; 9(7):2. doi: https://doi.org/10.1167/tvst.9.7.2

Articles April 2020

Disease Progression in Patients with Autosomal Dominant Retinitis Pigmentosa due to a Mutation in Inosine Monophosphate Dehydrogenase 1 (IMPDH1)

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Lea D. Bennett; Martin Klein; Finny T. John; Bojana Radojevic; Kaylie Jones; David G. Birch
TAGS: disease progression, inosine monophosphate, mutation, oxidoreductase, perimetry, retinitis pigmentosa, spectral domain-optical coherence tomography, visual fields, electroretinography, retina
Trans. Vis. Sci. Tech.. 2020; 9(5):14. doi: https://doi.org/10.1167/tvst.9.5.14

Special Issue March 2020

Application of a Deep Machine Learning Model for Automatic Measurement of EZ Width in SD-OCT Images of RP

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Yi-Zhong Wang; Daniel Galles; Martin Klein; Kirsten G. Locke; David G. Birch
TAGS: datasets, machine learning, pixel, spectral domain-optical coherence tomography, training, learning
Trans. Vis. Sci. Tech.. 2020; 9(2):15. doi: https://doi.org/10.1167/tvst.9.2.15

ARVO Annual Meeting Abstract July 2019

A Deep Convolutional Neural Network (CNN) Model for Automatic Measurement of Ellipsoid Zone (EZ) Width in Retinitis Pigmentosa (RP)

Yi-Zhong Wang; Daniel Galles; Martin Klein; Kirsten G Locke; David G Birch

Invest. Ophthalmol. Vis. Sci.. 2019; 60(9):1527. doi:

ARVO Annual Meeting Abstract July 2019

X-Chromosome Inactivation is a Biomarker of Clinical Severity in Female Carriers of X-linked Retinitis Pigmentosa

Abigail T Fahim; Lori S Sullivan; Sara J Bowne; Kaylie Webb-Jones; Dianna K.H. Wheaton; Kari E Branham; Mohammad Othman; Athanasios J Karoukis; Chris Andrews; John R Heckenlively; David G Birch; Stephen P Daiger

Invest. Ophthalmol. Vis. Sci.. 2019; 60(9):2961. doi:

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Tackling the Challenges of Product Development Through a Collaborative Rare Disease Network: The Foundation Fighting Blindness Consortium

The RUSH2A Study: Best-Corrected Visual Acuity, Full-Field Electroretinography Amplitudes, and Full-Field Stimulus Thresholds at Baseline

Quantification of RPE Changes in Choroideremia Using a Photoshop-Based Method

The natural history of choroideremia; progressive loss of visual function and retinal structure

The Association between Rod Function and Low Luminance Acuity

Structure and function from en-face optical coherence tomography, fundus autofluorescence, and rod and cone perimetry in Stargardt disease

Measuring progression at the edge of dense macular scotomas in Stargardt disease

Test-retest variability of static perimetry and microperimetry results from the RUSH2A study

Randomized clinical trial evaluating the pharmacodynamics of emixustat in subjects with macular atrophy secondary to Stargardt disease

Accelerating research in rare inherited retinal disorders (IRDs) through an international consortium

Baseline visual fields in the RUSH2A study: Associated factors and correlation with measures of disease severity

Deep learning-based automatic segmentation of intact ellipsoid zone area on optical coherence tomography images of USH2A-related retinal degeneration

Rod visual fields in comparison to the recommended functional and structural screening tests for patients treated with hydroxychloroquine

Evaluation of Two Convolutional Neural Network (CNN) Models for Automatic Segmentation of Retinal Layers from OCT images in Retinitis Pigmentosa (RP)

Meta-learning approach to automatically register multivendor retinal images

Advancing Clinical Trials for Inherited Retinal Diseases: Recommendations from the Second Monaciano Symposium

Disease Progression in Patients with Autosomal Dominant Retinitis Pigmentosa due to a Mutation in Inosine Monophosphate Dehydrogenase 1 (IMPDH1)

Application of a Deep Machine Learning Model for Automatic Measurement of EZ Width in SD-OCT Images of RP

A Deep Convolutional Neural Network (CNN) Model for Automatic Measurement of Ellipsoid Zone (EZ) Width in Retinitis Pigmentosa (RP)

X-Chromosome Inactivation is a Biomarker of Clinical Severity in Female Carriers of X-linked Retinitis Pigmentosa

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