Abstract：
PURPOSE:To study the topography of photoreceptor loss early in the course of Leber congenital amaurosis (LCA) caused by RPE65 mutations. METHODS:Young patients with RPE65-LCA (n = 9; ages, 6-17 years) were studied with optical coherence tomography (OCT) in a wide region of central retina. Outer nuclear layer (ONL) thickness was mapped topographically and compared with that in normal subjects and in older patients with RPE65-LCA. RESULTS:Photoreceptor layer topography was abnormal in all young patients with RPE65-LCA. Foveal and extrafoveal ONL was reduced in most patients. There were interindividual differences, with ONL thicknesses at most retinal locations ranging from near the detectability limit to a significant fraction of normal. These differences were not clearly related to age. In most patients, there was a thinner ONL inferior to the fovea compared with that in the superior retina. Summary maps obtained by aligning and averaging photoreceptor topography across all young patients showed a relative preservation of ONL in the superior-temporal and temporal pericentral retina. These retinal regions also showed the greatest magnitude of interindividual variation. CONCLUSIONS:Photoreceptor loss in the foveal and extrafoveal retina was prominent, even in the youngest patients studied. Differences in the topography of residual photoreceptors in children with RPE65-LCA suggest that it may be advisable to use individualized ONL mapping to guide the location of subretinal injections for gene therapy and thereby maximize the potential for efficacy.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Jacobson SG,Cideciyan AV,Aleman TS,Sumaroka A,Windsor EA,Schwartz SB,Heon E,Stone EMdoi
10.1167/iovs.08-2121subject
Has Abstractpub_date
2008-10-01 00:00:00pages
4573-7issue
10eissn
0146-0404issn
1552-5783pii
iovs.08-2121journal_volume
49pub_type
杂志文章abstract：PURPOSE:The majority of patients with X chromosome-linked retinitis pigmentosa (XlRP) carry mutations in the RPGR gene. The authors studied whether patients with RPGR mutations show additional splice defects that may interfere with RPGR properties. METHODS:Patient-derived cell lines with RPGR mutations were raised in ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4031
更新日期：2010-03-01 00:00:00
abstract：:We have investigated alterations of the metabolic state in vitamin A-deficient (A-) corneas using phosphorus-31 (31P) nuclear magnetic resonance (NMR) spectroscopy. Comparing to the control, A- corneas showed a prominent rise of phosphocreatine (PCr) as well as a total loss of glycerophosphorylcholine (GPC). Further, ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1989-04-01 00:00:00
abstract：:The morphology of the apical surface of rabbit retinal pigment epithelium was studied by scanning electron microscopy from the first minute to several hours after making small nonrhegmatogenous retinal detachments (blebs). From 0 to 5 min, there were only slight changes in the homogeneous, dense mat of filamentous mic...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1986-12-01 00:00:00
abstract：PURPOSE:Leber congenital amaurosis (LCA) is an inherited retinal disease that causes early-onset severe visual impairment. To evaluate the mutation spectrum in the Chinese population, we performed a mutation screen in 145 Chinese LCA families. METHODS:First, we performed direct Sanger sequencing of 7 LCA disease genes...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-15972
更新日期：2015-06-01 00:00:00
abstract：:The distribution and organization of type I and type V collagens were studied in the embryonic chicken cornea using anti-collagen, type specific, monoclonal antibodies and immunoelectron microscopy. These studies were performed on lathyritic 17-day corneas treated at 4 degrees C or 37 degrees C. At the lower temperatu...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1986-10-01 00:00:00
abstract：PURPOSE:Neuronal and glial alterations precede the overt vascular change that characterizes diabetic retinopathy. Because retinal astrocytes modulate neuronal and vascular function, this study investigated the time course of astrocyte, Müller cell, and neuronal change during diabetes to determine whether astrocytes may...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-7879
更新日期：2011-12-02 00:00:00
abstract：PURPOSE:To evaluate the performance and potential clinical role of three-dimensional (3D) 1060-nm OCT by generating choroidal thickness (ChT) maps in patients of different ages with different degrees of ametropia and axial lengths and to investigate the effect of cataract grade on OCT retinal imaging quality. METHODS:...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-5196
更新日期：2010-10-01 00:00:00
abstract：PURPOSE:To determine the effect of triamcinolone acetonide (TA) on outflow facility in mice. METHODS:Animals received 20 μL of TA (40 mg/mL) suspension subconjunctivally either bilaterally or unilaterally and were euthanized after either 1 week or 3 weeks. Before mice were killed, IOP was measured with a rebound tonom...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-11223
更新日期：2013-02-01 00:00:00
abstract：PURPOSE:BM88 is a cell-cycle exit and neuronal differentiation protein that has been used as a marker of surviving retinal ganglion cells (RGCs) after optic nerve injury. Thy1.1 has also been used as a marker for RGC loss, but after optic nerve crush (ONC) a decrease in Thy1.1 expression precedes the loss of RGCs. The ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.13-12986
更新日期：2014-03-28 00:00:00
abstract：:A group of 32 patients with low vision who were considered clinically appropriate candidates for visual rehabilitation with telescopic spectacles were prospectively studied before the first attempted use of these visual aids. Laboratory measurements were made of: (1) rotational head stability in pitch and yaw during q...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1991-09-01 00:00:00
abstract：Purpose:To describe the pathology of AMD in eyes with geographic atrophy (GA) using confocal scanning laser ophthalmoscopy (SLO) blue light autofluorescence (BAF), and near-infrared (IR) AF and to correlate it with the histology and immunohistochemistry analysis at the margins of the GA lesion. Methods:Enucleated, fix...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.61.8.15
更新日期：2020-07-01 00:00:00
abstract：PURPOSE:To explore factors affecting the glaucoma diagnostic capability of macular thickness and circumpapillary retinal nerve fiber layer (cRNFL) thickness as determined by spectral domain-optical coherence tomography. METHODS:Area under the curve (AUC) of the receiver operating characteristics (ROC) discriminating h...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-7833
更新日期：2011-10-11 00:00:00
abstract：:As part of an ophthalmic and anthropometric survey of two free-breeding rhesus monkey (Macaca mulatta) populations, intraocular pressure (IOP) of 114 animals was measured under ketamine catalepsia with the use of a floating-tip pneumatonometer. The mean IOP of the 102 animals included in the main analyses was 14.9 +/-...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1979-08-01 00:00:00
abstract：PURPOSE:To determine whether basic fibroblast growth factor (bFGF) and transforming growth factor-beta 2 (TGF-beta 2) influence the contractile activity and the expression of alpha-smooth muscle actin (alpha-SMA) in bovine lens epithelial cells (LECs). To examine whether modulation of contractile activity by these grow...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1995-07-01 00:00:00
abstract：PURPOSE:To investigate the relationship between percentage of body fat and macular pigment (MP) optical density. METHODS:One hundred healthy subjects of ages between 22 and 60 years volunteered to participate in this study. MP optical density was measured psychophysically, serum lutein and zeaxanthin were quantified b...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-0273
更新日期：2004-11-01 00:00:00
abstract：PURPOSE:Concentrations of aqueous humor endothelin (ET)-1 are increased in patients with primary open-angle glaucoma (POAG) as well as in animal models of glaucoma. Glucocorticoids have also been associated with glaucoma, in that topical administration of glucocorticoids can increase intraocular pressure by increasing ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-0463
更新日期：2003-12-01 00:00:00
abstract：PURPOSE:The main purpose of this study was to develop a cell culture model of immortalized epithelium from the human cornea for drug permeability testing. METHODS:Immortalized human corneal epithelial (HCE) cells were grown on filters, with various filter materials and coating procedures. In the optimal case, HCE cell...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2001-11-01 00:00:00
abstract：:Previous studies have used Badal laser optometry to demonstrate significant variance in the distribution of tonic (or "dark-focus") resting positions of accommodation (TA) for groups of observers. This study investigates whether individual differences in TA are due to individual differences in autonomic tone of the ci...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1985-07-01 00:00:00
abstract：PURPOSE:Levels of some cytokines are significantly higher in the vitreous fluid of patients with acute uveitis than in normal vitreous fluid. The authors sought to determine which proinflammatory cytokines were upregulated in the vitreous fluid of patients with ocular sarcoidosis. METHODS:Samples of vitreous fluid wer...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-9244
更新日期：2012-06-20 00:00:00
abstract：:Failure of corneal grafts is thought to involve the development and activation of specifically sensitized T-cells. One method which might be used to circumvent the development of such cells is the phenomenon of anterior chamber-associated immune deviation (ACAID). Injection of antigen into the anterior chamber of the ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1990-10-01 00:00:00
abstract：PURPOSE:To measure the effects of cerebrospinal fluid pressure (CSFp) on retrolaminar tissue pressure (RLTp) and the translaminar pressure gradient (TLPG), particularly at low CSFp, which is the normal situation in erect posture. METHODS:Micropipettes coupled to a servonull pressure system were passed into eyes of ane...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1998-07-01 00:00:00
abstract：Purpose:To quantify and evaluate macular superficial capillaries and large vessels separately using an optical coherence tomographic angiography (OCTA)-based automatic segmentation algorithm. Methods:In this cross-sectional study, all eyes were scanned using an OCTA device with 3 × 3 mm cube centered on the fovea. Ret...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.17-23676
更新日期：2018-04-01 00:00:00
abstract：Purpose:To discover novel therapies that lower IOP by increasing aqueous humor outflow facility, ex vivo ocular perfusion systems provide a valuable tool. However, currently available designs are limited by their throughput. Here we report the development of a compact, scalable perfusion system with improved throughput...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-20974
更新日期：2017-06-01 00:00:00
abstract：PURPOSE:To assess changes in rod and cone visual functions in a mouse model of Fundus albipunctatus with disrupted 11-cis-retinol dehydrogenase (RDH) genes after pharmacologic treatment with an artificial retinal chromophore. METHODS:Retinoid levels and photoreceptor functions of Rdh5-/-Rdh11-/- mice at a variety of l...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.06-0215
更新日期：2006-10-01 00:00:00
abstract：:PURPOSE. To evaluate the shape of the posterior corneal surface in eyes with normal myopia or myopic astigmatism and the changes after LASIK and epi-LASIK. METHODS. A total of 152 eyes were included in the study. The posterior corneal elevation was measured with Scheimpflug imaging (Pentacam; Oculus, Wetzlar, Germany)...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4811
更新日期：2010-07-01 00:00:00
abstract：:Stiles-Crawford functions were obtained from the maculas of 22 patients with different genetic types of retinitis pigmentosa and visual acuity of 20/40 or better. Reduced cone directional sensitivity was seen in the fovea with both focal cone electroretinographic testing and psychophysical testing. Functions from the ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1982-02-01 00:00:00
abstract：:Flicker contrast sensitivity was tested in the "good" eyes of 13 patients with monocular exudative age-related maculopathy (ARM). The stimulus was a foveal, long-wavelength, low spatial frequency 2.8 degrees circle in an equiluminant (photopic) surround. Two of these ARM-risk eyes have since developed exudative ARM. C...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1992-10-01 00:00:00
abstract：PURPOSE:Glial cells are structurally and functionally linked to neuronal tissues. Pathologically, the cells may be activated and characterized by increased size and number and altered cellular properties. In glaucoma, pathologic mechanisms within the anterior optic nerve may include glial activation. This study examine...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2002-04-01 00:00:00
abstract：PURPOSE:To study reading acuity in children and assess the reliability of its measurement using the Italian version of the MNREAD reading chart. METHODS:One hundred sixteen children from grades 3 to 8 with normal near vision were tested at a pediatric eye care institution in northeastern Italy. Visual acuity was evalu...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-1304
更新日期：2004-09-01 00:00:00
abstract：Purpose:Candida remains the leading cause of fungal endophthalmitis. However, the pathobiology and innate immune responses in this disease are not well characterized. Here, we developed two murine models of candida endophthalmitis and evaluated their disease susceptibility and differential immune response. Methods:End...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.61.11.31
更新日期：2020-09-01 00:00:00
