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Papers by David Birch

Research paper thumbnail of Progression of atrophic lesions prospectively determined by fundus autofluorescence: the natural history of the progression of atrophy secondary to Stargardt disease (ProgStar) study

Investigative Ophthalmology & Visual Science, 2016

Research paper thumbnail of Progressive Loss of Rod Sensitivity in Patients with Autosomal Dominant Retinitis Pigmentosa (adRP) due to RHO Pro23His Mutation

Investigative Ophthalmology & Visual Science, 2017

Research paper thumbnail of Longitudinal Changes in Scotopic and Mesopic Macular Function as Assessed with Microperimetry in Patients With Stargardt Disease: SMART Study Report No. 2

American Journal of Ophthalmology, 2021

PURPOSE To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losse... more PURPOSE To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losses measured by microperimetry, and to report and compare the longitudinal rates of scotopic and mesopic macular sensitivity losses in ABCA4 gene associated Stargardt Disease (STGD1). DESIGN Multicenter prospective cohort study. METHODS Participants: 127 molecular confirmed STGD1 patients enrolled from 6 centers in the USA and Europe and followed every 6 months for up to 2 years. OBSERVATION PROCEDURES The Nidek MP-1S device was used to measure macular sensitivities of the central 20° under mesopic and scotopic conditions. The mean deviations (MD) from normal for mesopic macular sensitivity for the fovea (within 2° eccentricity) and extrafovea (4°-10° eccentricity), and the MD for scotopic sensitivity for the extrafovea were calculated. Linear mixed effects models were used to estimate mesopic and scotopic changes. MAIN OUTCOME MEASURES Baseline mesopic mean deviation (mMD) and scotopic MD (sMD) and rates of longitudinal changes in the mMDs and sMD. RESULTS At baseline, all eyes had larger sMD, and the difference between extrafoveal sMD and mMD was 10.7 dB (p<.001). Longitudinally, all eyes showed a statistically significant worsening trend: the rates of foveal mMD and extrafoveal mMD and sMD changes were 0.72 (95%CI: 0.37 to 1.07), 0.86 (95%CI: 0.58 to 1.14) and 1.12 (95%CI: 0.66 to 1.57) dB/year, respectively. CONCLUSIONS In STGD1, in extrafovea, loss of scotopic macular function preceded and was faster than the loss of mesopic macular function. Scotopic and mesopic macular sensitivities using microperimetry provide alternative visual function outcomes for STGD1 treatment trials.

Research paper thumbnail of Behavioral measurements of rat spectral sensitivity

Vision Research, Jun 1, 1975

Abstract Behavioral measurements of visual sensitivity were obtained from hooded rats on a threec... more Abstract Behavioral measurements of visual sensitivity were obtained from hooded rats on a threechoice discrimination task. Increment threshold measurements reveal the occurrence of a distinct Purkinje shift. Photopic spectral sensitivity functions for this animal have peaks at 500 and 580 nm. The results of chromatic adaptation suggest that this photopic sensitivity function results from interactions between two mechanisms with peak sensitivities near 520 and 540 nm. Along with previous behavioral and electrophysiological results, these findings suggest that the rat possesses at least three different spectral mechanisms—a scotopic mechanism with a peak sensitivity at 500 nm and two photopic mechanisms whose peak sensitivities may be about 520 and 540 nm.

Research paper thumbnail of Abnormal Cone Structure in Foveal Schisis Cavities in X-Linked Retinoschisis from Mutations in Exon 6 of the<i>RS1</i>Gene

Investigative Ophthalmology & Visual Science, Dec 20, 2011

Research paper thumbnail of Factors Modifying Severity and Penetrance in Males and Females with Retinitis Pigmentosa Caused by Mutations in the X-Linked RPGR Gene

Research paper thumbnail of Visual function improvements with BIIB112 (NSR-RPGR) in X-linked retinitis pigmentosa: XIRIUS (dose-expansion) and XOLARIS (natural disease progression) studies

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

Investigative Ophthalmology & Visual Science, Jun 10, 2020

Research paper thumbnail of The Transcription Factor PRDM8 Is Required for Rod and Cone Bipolar Cell Maintenance

Research paper thumbnail of Natural History of Progression of Choroideremia (NIGHT) Study: Cross-Sectional Analysis of Baseline Characteristics

Research paper thumbnail of The Natural History of the Progression of Choroideremia (NIGHT) Study: Longitudinal Changes in Visual Acuity over 12 Months

Research paper thumbnail of Argus II Retinal Prosthesis System Post-Approval US Cohort 1 Year Outcomes

Research paper thumbnail of SAG (S-antigen visual arrestin-1) mutations cause autosomal dominant retinitis pigmentosa (adRP) without the Oguchi disease phenotype

Investigative Ophthalmology & Visual Science, Jun 23, 2017

Research paper thumbnail of Natural progression of scotopic and photopic macular function loss in patients with Stargardt disease: The SMART Study

Research paper thumbnail of EZ Width Reflects Disease Severity in adRP Patients with PRPF31 Gene Mutations

Investigative Ophthalmology & Visual Science, Jun 23, 2017

Research paper thumbnail of Detectable rod function in patients with retinitis pigmentosa (RP) with or without a measureable rod electroretinogram (ERG) response

Investigative Ophthalmology & Visual Science, Sep 26, 2016

Research paper thumbnail of Local and global visual function deficits in patients with ABCR gene mutations

We examined the ability of patients with Stargardt disease to perform a global hyperacuity task. ... more We examined the ability of patients with Stargardt disease to perform a global hyperacuity task. Stimuli were circular D4 contours and their deformations. A spatial 2 AFC paradigm was employed. In each trial, the patients indicated which of two stimuli was deformed. The patients showed a substantial elevation in the threshold for detecting circular D4 deformation while still retaining relatively good visual acuity. The results support the hypothesis that optimal performance for detecting circular D4 deformation involves some kind of global mechanism. The results also suggest the global hyperacuity is an effective test for quantifying early visual loss from macular degeneration.

Research paper thumbnail of Identification of a Large Insertion/Deletion in the PRPF31 Gene in a Family with adRP

Investigative Ophthalmology & Visual Science, May 1, 2006

Research paper thumbnail of A Novel GCAP1 Mutation Linked to Dominant Cone Dystrophy

Investigative Ophthalmology & Visual Science, May 10, 2007

Research paper thumbnail of Heparin Enhances AAV2–mediated Transduction Efficiency In The Mouse Retina

Investigative Ophthalmology & Visual Science, May 1, 2004

Research paper thumbnail of Progression of atrophic lesions prospectively determined by fundus autofluorescence: the natural history of the progression of atrophy secondary to Stargardt disease (ProgStar) study

Investigative Ophthalmology & Visual Science, 2016

Research paper thumbnail of Progressive Loss of Rod Sensitivity in Patients with Autosomal Dominant Retinitis Pigmentosa (adRP) due to RHO Pro23His Mutation

Investigative Ophthalmology & Visual Science, 2017

Research paper thumbnail of Longitudinal Changes in Scotopic and Mesopic Macular Function as Assessed with Microperimetry in Patients With Stargardt Disease: SMART Study Report No. 2

American Journal of Ophthalmology, 2021

PURPOSE To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losse... more PURPOSE To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losses measured by microperimetry, and to report and compare the longitudinal rates of scotopic and mesopic macular sensitivity losses in ABCA4 gene associated Stargardt Disease (STGD1). DESIGN Multicenter prospective cohort study. METHODS Participants: 127 molecular confirmed STGD1 patients enrolled from 6 centers in the USA and Europe and followed every 6 months for up to 2 years. OBSERVATION PROCEDURES The Nidek MP-1S device was used to measure macular sensitivities of the central 20° under mesopic and scotopic conditions. The mean deviations (MD) from normal for mesopic macular sensitivity for the fovea (within 2° eccentricity) and extrafovea (4°-10° eccentricity), and the MD for scotopic sensitivity for the extrafovea were calculated. Linear mixed effects models were used to estimate mesopic and scotopic changes. MAIN OUTCOME MEASURES Baseline mesopic mean deviation (mMD) and scotopic MD (sMD) and rates of longitudinal changes in the mMDs and sMD. RESULTS At baseline, all eyes had larger sMD, and the difference between extrafoveal sMD and mMD was 10.7 dB (p<.001). Longitudinally, all eyes showed a statistically significant worsening trend: the rates of foveal mMD and extrafoveal mMD and sMD changes were 0.72 (95%CI: 0.37 to 1.07), 0.86 (95%CI: 0.58 to 1.14) and 1.12 (95%CI: 0.66 to 1.57) dB/year, respectively. CONCLUSIONS In STGD1, in extrafovea, loss of scotopic macular function preceded and was faster than the loss of mesopic macular function. Scotopic and mesopic macular sensitivities using microperimetry provide alternative visual function outcomes for STGD1 treatment trials.

Research paper thumbnail of Behavioral measurements of rat spectral sensitivity

Vision Research, Jun 1, 1975

Abstract Behavioral measurements of visual sensitivity were obtained from hooded rats on a threec... more Abstract Behavioral measurements of visual sensitivity were obtained from hooded rats on a threechoice discrimination task. Increment threshold measurements reveal the occurrence of a distinct Purkinje shift. Photopic spectral sensitivity functions for this animal have peaks at 500 and 580 nm. The results of chromatic adaptation suggest that this photopic sensitivity function results from interactions between two mechanisms with peak sensitivities near 520 and 540 nm. Along with previous behavioral and electrophysiological results, these findings suggest that the rat possesses at least three different spectral mechanisms—a scotopic mechanism with a peak sensitivity at 500 nm and two photopic mechanisms whose peak sensitivities may be about 520 and 540 nm.

Research paper thumbnail of Abnormal Cone Structure in Foveal Schisis Cavities in X-Linked Retinoschisis from Mutations in Exon 6 of the<i>RS1</i>Gene

Investigative Ophthalmology & Visual Science, Dec 20, 2011

Research paper thumbnail of Factors Modifying Severity and Penetrance in Males and Females with Retinitis Pigmentosa Caused by Mutations in the X-Linked RPGR Gene

Research paper thumbnail of Visual function improvements with BIIB112 (NSR-RPGR) in X-linked retinitis pigmentosa: XIRIUS (dose-expansion) and XOLARIS (natural disease progression) studies

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

Investigative Ophthalmology & Visual Science, Jun 10, 2020

Research paper thumbnail of The Transcription Factor PRDM8 Is Required for Rod and Cone Bipolar Cell Maintenance

Research paper thumbnail of Natural History of Progression of Choroideremia (NIGHT) Study: Cross-Sectional Analysis of Baseline Characteristics

Research paper thumbnail of The Natural History of the Progression of Choroideremia (NIGHT) Study: Longitudinal Changes in Visual Acuity over 12 Months

Research paper thumbnail of Argus II Retinal Prosthesis System Post-Approval US Cohort 1 Year Outcomes

Research paper thumbnail of SAG (S-antigen visual arrestin-1) mutations cause autosomal dominant retinitis pigmentosa (adRP) without the Oguchi disease phenotype

Investigative Ophthalmology & Visual Science, Jun 23, 2017

Research paper thumbnail of Natural progression of scotopic and photopic macular function loss in patients with Stargardt disease: The SMART Study

Research paper thumbnail of EZ Width Reflects Disease Severity in adRP Patients with PRPF31 Gene Mutations

Investigative Ophthalmology & Visual Science, Jun 23, 2017

Research paper thumbnail of Detectable rod function in patients with retinitis pigmentosa (RP) with or without a measureable rod electroretinogram (ERG) response

Investigative Ophthalmology & Visual Science, Sep 26, 2016

Research paper thumbnail of Local and global visual function deficits in patients with ABCR gene mutations

We examined the ability of patients with Stargardt disease to perform a global hyperacuity task. ... more We examined the ability of patients with Stargardt disease to perform a global hyperacuity task. Stimuli were circular D4 contours and their deformations. A spatial 2 AFC paradigm was employed. In each trial, the patients indicated which of two stimuli was deformed. The patients showed a substantial elevation in the threshold for detecting circular D4 deformation while still retaining relatively good visual acuity. The results support the hypothesis that optimal performance for detecting circular D4 deformation involves some kind of global mechanism. The results also suggest the global hyperacuity is an effective test for quantifying early visual loss from macular degeneration.

Research paper thumbnail of Identification of a Large Insertion/Deletion in the PRPF31 Gene in a Family with adRP

Investigative Ophthalmology & Visual Science, May 1, 2006

Research paper thumbnail of A Novel GCAP1 Mutation Linked to Dominant Cone Dystrophy

Investigative Ophthalmology & Visual Science, May 10, 2007

Research paper thumbnail of Heparin Enhances AAV2–mediated Transduction Efficiency In The Mouse Retina

Investigative Ophthalmology & Visual Science, May 1, 2004

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