Publications by authors named "Amir H Hariri"

Purpose: To evaluate the effect of subretinally transplanted human central nervous system stem cells (HuCNS-SC) on the progression of geographic atrophy (GA) in patients with nonneovascular age-related macular degeneration (AMD).

Design: Multicenter, prospective, phase 1 open-label clinical trial.

Participants: Fifteen patients with bilateral GA solely the result of AMD.

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Importance: Sensitive outcome measures for disease progression are needed for treatment trials of Stargardt disease.

Objective: To estimate the progression rate of atrophic lesions in the prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) study over a 12-month period.

Design, Setting, And Participants: This multicenter prospective cohort study was conducted in an international selection of tertiary referral centers from October 21, 2013, to February 15, 2017.

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Purpose: To report a genotype-phenotype correlation study of patients with retinitis pigmentosa (RP) based on ultra-widefield (UWF) fundus autofluorescence (FAF) imaging.

Design: Case series.

Participants: Thirty-four patients with RP.

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Background/aims: To better understand the pattern of degeneration progression in cases with choroideremia.

Methods: A cohort of genotypically confirmed choroideremia cases who underwent optical coherence tomography (OCT) and fundus autofluorescence (FAF) imaging was studied. Using HEYEX review software, the foveal centre was marked on FAF images under guidance of corresponding OCT images, followed by application of an ETDRS grid.

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Background And Objective: To compare macular thickness measurement algorithms of two different spectral-domain optical coherence tomography (SD-OCT) devices in eyes affected by dry age-related macular degeneration (AMD).

Patients And Methods: Patients with dry AMD and healthy volunteers from the retina clinic of the Doheny Eye Center - UCLA were imaged using two different SD-OCT devices: the RS-3000 Advance (Nidek, Padova, Italy) and the Cirrus HD-OCT (Carl Zeiss Meditec, Dublin, CA). All patients had been previously diagnosed with drusen or geographic atrophy due to AMD.

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Background/aims: To systematically compare the intermodality and inter-reader agreement for two blue-light confocal fundus autofluorescence (FAF) systems.

Methods: Thirty eyes (21 patients) with a diagnosis of geographic atrophy (GA) were enrolled. Eyes were imaged using two confocal blue-light FAF devices: (1) Spectralis device with a 488 nm excitation wavelength (488-FAF); (2) EIDON device with 450 nm excitation wavelength and the capability for 'colour' FAF imaging including both the individual red and green components of the emission spectrum.

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Purpose: To quantify the extent of topographic correspondence between baseline (BSL) choroidal neovascularization (CNV) and macular atrophy (MA) at follow-up in eyes with neovascular age-related macular degeneration (NVAMD).

Design: Post hoc analysis of randomized controlled clinical trial data.

Methods: Sixty treatment-naïve NVAMD patients from the TREX-AMD trial were followed for 18 months.

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Importance: Sensitive outcome measures for disease progression are needed for treatment trials of Stargardt disease.

Objective: To describe the yearly progression rate of atrophic lesions in the retrospective Progression of Stargardt Disease study.

Design, Setting, And Participants: A multicenter retrospective cohort study was conducted at tertiary referral centers in the United States and Europe.

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Purpose: The junctional zone at the border of areas of geographic atrophy (GA) in eyes with nonneovascular age-related macular degeneration is an important target region for future therapeutic strategies. The goal of this study was to perform a detailed classification and quantitative characterization of the junctional zone using spectral domain optical coherence tomography.

Methods: Spectral domain optical coherence tomography volume cube scans (Spectralis OCT, 1024 × 37, Automatic Real Time > 9) were obtained from 15 eyes of 11 patients with GA because of nonneovascular age-related macular degeneration.

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Importance: Outcome measures that are sensitive to disease progression are needed as clinical end points for future treatment trials in Stargardt disease.

Objective: To examine the incidence of atrophic lesions of the retinal pigment epithelium in patients with Stargardt disease as determined by fundus autofluorescence imaging.

Design, Setting, And Participants: In this retrospective multicenter cohort study, 217 patients 6 years and older at baseline at tertiary referral centers in Europe, the United States, and the United Kingdom who were harboring disease-causing variants in the adenosine triphosphate (ATP)-binding cassette subfamily A member 4 (ABCA4) gene and who met the following criteria were enrolled: (1) at least 1 well-demarcated area of atrophy with a minimum diameter of 300 µm, with the total area of all atrophic lesions being less than or equal to 12 mm2 in at least 1 eye at the most recent visit, and (2) fundus autofluorescence images for at least 2 visits with a minimum of 6 months between at least 2 visits.

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Purpose: To identify valid and reproducible methods for quantifying anatomic outcome measures for eyes with choroideremia (CHM) in clinical trials.

Design: Reliability analysis study.

Methods: In this multicenter study, patients with confirmed genetic diagnosis of CHM were enrolled.

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Background And Objective: To determine the correlation between mesopic retinal sensitivity and optical coherence tomographic metrics of the outer retina in patients with intermediate age-related macular degeneration (AMD).

Patients And Methods: Participants with nonatrophic dry AMD underwent mesopic MP-3 microperimetry (Nidek, Padova, Italy) and both Nidek and Cirrus (Carl Zeiss Meditec, Dublin, CA) spectral-domain optical coherence tomography (SD-OCT). The volume of the outer retinal layers was measured on the Nidek SD-OCT scans using the automatic segmentation algorithm of Navis-EX software.

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Article Synopsis
  • The study aimed to evaluate the effectiveness of aflibercept therapy in treating Type 1 and Type 3 neovascularization associated with age-related macular degeneration.
  • Results showed that Type 3 lesions had a significantly greater improvement in vision and required fewer injections than Type 1 lesions over a 12-month period.
  • The findings suggest that understanding the neovascularization subtype at diagnosis could lead to more personalized and effective treatment strategies.
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Background And Objective: To evaluate the spectral-domain optical coherence tomography (SD-OCT) characteristics of the junctional zone corresponding to areas of increased autofluorescence (IAF) at the margin of geographic atrophy (GA) in patients with age-related macular degeneration (AMD).

Patients And Methods: SD-OCT and fundus autofluorescence (FAF) images from untreated eyes with GA available from archived studies at Doheny Image Reading Center were evaluated. Areas of definite decreased autofluorescence (DDAF) corresponding to GA, and areas of IAF at the margins of the GA were manually segmented.

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Purpose: To compare the retinal sensitivity at the junctional zone and uninvolved retina of eyes with geographic atrophy (GA) due to age-related macular degeneration (AMD).

Design: Cross-sectional, observational study.

Methods: Patients with dry AMD were evaluated by microperimetry and Cirrus optical coherence tomography (OCT).

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Purpose: The aims of this study were to determine the prevalence of vitreomacular adhesion (VMA) in a random sample of clinical patients at three US retina clinics and to assess comorbid retinal conditions, ocular diseases, prior treatment history, and other medical histories.

Patients And Methods: This observational, retrospective cohort study was based on patients from the Doheny Eye Centers, Duke Eye Center, and Tufts Medical Center who received a bilateral spectral domain optical coherence tomography (SD-OCT) scan (one scan/eye) for clinical evaluation with available medical records. The study had three phases: 1) collection of retrospective patient data; 2) review of OCT scans at a reading center to assess VMA and associated conditions; and 3) analyses and reporting of data on the prevalence of VMA, patient demographics, and comorbid conditions.

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Importance: New methods are needed to quantify the change in the outer retinal structures in retinitis pigmentosa (RP).

Objective: To implement an alternate method for tracking ellipsoid zone (EZ) changes in RP by quantifying the EZ area on en face spectral domain-optical coherence tomographic (SD-OCT) images.

Design, Setting, And Participants: Data for this observational case study were collected at the Department of Ophthalmology, University of California, Los Angeles, from May 1 to July 30, 2015, and included SD-OCT images of a subset of patients from the Trial of Oral Valproic Acid for Retinitis Pigmentosa.

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Purpose: To evaluate manual and semiautomated grading techniques for assessing decreased fundus autofluorescence (DAF) in patients with Stargardt disease phenotype.

Methods: Certified reading center graders performed manual and semiautomated (region finder-based) grading of confocal scanning laser ophthalmoscopy (cSLO) fundus autofluorescence (FAF) images for 41 eyes of 22 patients. Lesion types were defined based on the black level and sharpness of the border: definite decreased autofluorescence (DDAF), well, and poorly demarcated questionably decreased autofluorescence (WDQDAF, PDQDAF).

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