Abstract:
Purpose:Little is known regarding the therapeutic effect of carbonic anhydrase inhibitors (CAIs) in the management of cystic macular lesions in children with X-linked juvenile retinoschisis (XLRS) despite the fact that this disease often manifests during childhood. Therefore, our goal was to determine the efficacy of CAIs in the treatment of cystic macular lesions in children with XLRS. Methods:We used CAIs to treat cystic macular lesions in 18 eyes of nine children with XLRS. We evaluated the therapeutic effect of CAI treatment with the best-corrected visual acuity and foveal zone thickness (FZT) with spectral-domain optical coherence tomography. A reduction of at least 22.4% in FZT was defined as objective evidence of response. Results:Five of nine (55.6%) XLRS patients showed a significant reduction of FZT in both eyes over a median treatment interval of 6.8 months (range, 1-23). In four of five (80.0%) patients, this reduction was already apparent after 1 month of treatment. An improvement of visual acuity was observed in five eyes (27.8%) of three patients (33.3%). Six patients (66.6%) reported minor side effects. Conclusions:Treatment with CAIs decreased FZT in more than half of the children with XLRS. This effect was observed within 1 month in the majority of patients. Carbonic anhydrase inhibitor treatment restores retinal anatomy and may contribute to creating optimal circumstances for gene therapy.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Verbakel SK,van de Ven JP,Le Blanc LM,Groenewoud JM,de Jong EK,Klevering BJ,Hoyng CBdoi
10.1167/iovs.16-20078subject
Has Abstractpub_date
2016-10-01 00:00:00pages
5143-5147issue
13eissn
0146-0404issn
1552-5783pii
2565694journal_volume
57pub_type
杂志文章abstract:Purpose:To test the amblyopic suppression at mid to low spatial frequencies when compensating for signal attenuation. Methods:Eight amblyopes with (n = 5) or without (n = 3) strabismus and 10 normal controls with normal or corrected to normal visual acuity (≥20/20) and normal stereopsis (≤40 arcseconds) participated. ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-24206
更新日期:2018-08-01 00:00:00
abstract:Purpose:To assess the agreement between structural (optical coherence tomography [OCT]) and functional (visual field [VF]) glaucomatous damage with an automated method and deviation/probability maps, and to compare this method to a metric method. Methods:Wide-field spectral-domain OCT scans, including the disc and mac...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.19-27920
更新日期:2019-10-01 00:00:00
abstract::This study was undertaken to observe the effect on the differential light sensitivity in glaucoma suspects produced by a long-term reduction in intraocular pressure (IOP) with timolol maleate. The results are taken from an ongoing 6 year follow-up study of glaucoma suspects randomly selected for treatment and nontreat...
journal_title:Investigative ophthalmology & visual science
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1988-10-01 00:00:00
abstract:PURPOSE:Strabismus is a common eye disorder with a prevalence of 1% to 4%. Comitant strabismus accounts for approximately 75% of all strabismus, yet more is known about the less common incomitant disorders. Comitant strabismus is at least partly inherited, but only one recessive genetic susceptibility locus, on chromos...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1631
更新日期:2009-07-01 00:00:00
abstract:PURPOSE:To characterize the possible role of transducin Gtβγ-complex in modulating the signaling properties of photoactivated rhodopsin and its lifetime in rod disc membranes and intact rods. METHODS:Rhodopsin photolysis was studied using UV-visible spectroscopy and rapid scanning spectroscopy in the presence of hydro...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-9350
更新日期:2012-03-09 00:00:00
abstract:PURPOSE:The increase in intraocular pressure in primary open-angle glaucoma (POAG) may involve transforming growth factor (TGF)-beta2 signaling, as TGF-beta2 is found in higher amounts than normal in the aqueous humor of patients with POAG. In vitro, TGF-beta2 causes an accumulation of extracellular matrix (ECM) in the...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-0226
更新日期:2007-02-01 00:00:00
abstract:PURPOSE:To compare action spectra for visual discomfort in the fovea and the parafovea and to determine the effect of macular pigment (MP). METHODS:Visual discomfort thresholds to lights from 440 to 600 nm were obtained for six young (<35 y), visually normal subjects with a wide range of MP densities (0.10-0.71 at 30'...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-12365
更新日期:2013-09-19 00:00:00
abstract:PURPOSE:To examine the relative prevalence of ocular conditions among children who are unable to perform preschool vision screening tests and the impact on measures of screening test performance. METHODS:Trained nurse and lay screeners each administered a Lea Symbols visual acuity (VA) test (Good-Lite, Inc., Steamwood...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,多中心研究
doi:10.1167/iovs.06-0384
更新日期:2007-01-01 00:00:00
abstract:PURPOSE:To determine which components present in oxidized LDL are responsible for the cytotoxicity associated with its internalization by culture ARPE19 cells. METHODS:ARPE19 cells were grown in 24-well and 96-well plates. Cell viability was measured by MTT and/or adenosine triphosphate (ATP) content. LDL was oxidized...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-0075
更新日期:2004-08-01 00:00:00
abstract:PURPOSE:The purpose of this study was to determine whether a better IOP reduction can be observed in conscious, normotensive monkeys treated with ONO-9054, a novel dual EP3 and FP receptor agonist, compared with prostaglandin F2α analogs. METHODS:The binding affinities and agonistic activities of ONO-AG-367, a carboxy...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-16181
更新日期:2015-04-01 00:00:00
abstract:PURPOSE:This study describes the effects of the cyclic adenosine monophosphate (cAMP) pathway on the tight junctional barrier of the corneal endothelium, which plays a critical role in maintaining the corneal stroma in an underhydrated, transparent state. METHODS:Subcultured bovine corneal endothelial cells grown on f...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1995-11-01 00:00:00
abstract:PURPOSE:Acute optic neuritis (ON) is often followed by recovery of visual function. Although this recovery is mainly attributable to resolution of the acute inflammation, the redistribution of ion channels along the demyelinated membrane, and subsequent remyelination, part of it may be the result of neural plasticity. ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-4577
更新日期:2010-05-01 00:00:00
abstract:PURPOSE:To test the association between TCF4, a gene recently found to confer susceptibility to Fuchs' corneal dystrophy (FCD) in Caucasian populations, and Chinese patients with FCD. METHODS:Fifty-seven Chinese subjects with clinically diagnosed FCD and 121 normal control subjects were recruited. Genomic DNA was extr...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7568
更新日期:2011-07-29 00:00:00
abstract::Interactions between direction-selective mechanisms for motion were examined in strabismic and anisometropic amblyopia. Amblyopes were found to have a marked reduction in sensitivity to flicker or movement when the amblyopic eye was presented with a combination of two gratings drifting in opposite directions at the sa...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1980-09-01 00:00:00
abstract:PURPOSE:To investigate whether tissue plasminogen activator (tPA) can prevent and/or reverse steroid-induced IOP elevation in an ovine model. METHODS:Three animal groups were subjected to bilateral steroid-induced IOP elevation using thrice daily topical ocular prednisolone administration. In the first group (N = 8), ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-12801
更新日期:2013-12-03 00:00:00
abstract:PURPOSE:We investigated the phenotype of macrophages infiltrating rejected corneal allografts. METHODS:We performed allogeneic or syngeneic corneal transplantation in mice, and humanely killed animals at day 28 during allograft rejection when 60% of corneal allografts were rejected. We divided allografts into two grou...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-12650
更新日期:2013-11-21 00:00:00
abstract:Purpose:To explore patterns of disease progression in nonneovascular age-related macular degeneration (AMD) associated with hyperreflective crystalline deposits (HCDs) in the sub-retinal pigment epithelium-basal laminar space. Methods:Retrospective review of medical records, multimodal imaging, and longitudinal eye-tr...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.19-26589
更新日期:2019-07-01 00:00:00
abstract:PURPOSE:The purpose of this study was to investigate the mutational incidence and spectrum of mitochondrial ND1 gene in subjects with Leber's hereditary optic neuropathy (LHON). METHODS:A cohort of 1281 Han Chinese probands and 478 control subjects underwent sequence analysis of mitochondrial (mt)DNA. Resultant varian...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,多中心研究
doi:10.1167/iovs.16-19243
更新日期:2016-05-01 00:00:00
abstract:PURPOSE:To determine whether there is rod system dysfunction in the central retina of patients with age-related macular degeneration (AMD). METHODS:Dark-adapted sensitivity (500-nm stimulus) and light-adapted sensitivity (600 nm) were measured psychophysically at 52 loci in the central 38 degrees (diameter) of retina ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2000-01-01 00:00:00
abstract:PURPOSE:To test whether the incidence of long-term drift to timolol can be reduced by a "pulsatile" treatment (6 months timolol-2 months dipivefrin). METHODS:In a randomized clinical trial, 100 consecutive subjects with ocular hypertension or high-tension primary open-angle glaucoma in at least one eye were randomly a...
journal_title:Investigative ophthalmology & visual science
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1996-03-01 00:00:00
abstract::The sequential electrophysiologic and pathologic changes in the retina in ceroid-lipofuscinosis were recorded in a time-course study in the ovine model. Over a relatively short period in the course of the disease, a severe reduction in both rod and cone b-wave amplitudes developed with rod b-wave changes preceeding th...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1984-03-01 00:00:00
abstract:PURPOSE:To evaluate the potential role that FoxO transcription factors play in modulating resveratrol's protective effects against oxidative stress in lens epithelial cells. METHODS:Primary human or porcine lens epithelial cells (LECs) were treated with resveratrol (RES) 25 μM and incubated under either physiologic (5...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-6652
更新日期:2011-06-21 00:00:00
abstract:PURPOSE:Viral conjunctivitis is a highly contagious infection often causing major epidemics. A safe broad-spectrum antiviral agent is needed to treat this unmet medical need. The purpose of this study is to demonstrate that in vitro NVC-422 is a safe, broad-spectrum topical virucidal agent with activity against ophthal...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.12-10700
更新日期:2013-02-19 00:00:00
abstract:PURPOSE:CD4(+)CD25(+)FoxP3(+) naturally occurring regulatory T cells (Tregs) maintain self-tolerance and function to suppress overly exuberant immune responses. However, it is unclear whether innate immune cells modulate Treg function. Here the authors examined the role of innate immunity in lymphomyeloid homeostasis. ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-6536
更新日期:2011-04-08 00:00:00
abstract:Purpose:Patients with diabetes have a propensity to develop dry eye symptoms (DES), with reduced tear secretion and corneal sensitivity. The underlying pathologic basis of DES was explored in patients with Type 1 diabetes. Methods:Forty-two patients with Type 1 diabetes mellitus (T1DM) (age: 49.21 ± 2.53 years, durati...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-25321
更新日期:2018-11-01 00:00:00
abstract:PURPOSE:To correlate retinal histopathology with functional changes caused by the rhodopsin Q64ter mutation. METHODS:A 50-year-old female heterozygote was evaluated clinically and with psychophysical and electroretinographic measurements of rod and cone function. The retinas obtained after death were examined microsco...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1996-04-01 00:00:00
abstract:PURPOSE:To investigate the effects of reducing connexin (Cx) diversity in the lens when the amount of connexin protein is nearly constant. METHODS:Lenses in which the Cx50 coding region was replaced by that of Cx46 (knockin [KI]), were compared with wild type (WT) and Cx50-knockout (KO) lenses. Gap junctional conducta...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-0445
更新日期:2004-10-01 00:00:00
abstract:PURPOSE:Changes in blood vessel barrier properties contribute to retinal edema in diabetic retinopathy (DR). However, limited data are available to describe the routes of transport for fluids and solutes across the inner blood-retinal barrier (iBRB). In this study, a three-pore model was developed to characterize such ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-5971
更新日期:2011-02-28 00:00:00
abstract:PURPOSE:Organized conjunctiva-associated lymphoid tissue (O-CALT) is constantly exposed to environmental antigens and plays a central role in the common mucosal immune system. This study was undertaken to investigate whether O-CALT in rabbit lymphoid tissue, as in other lymphoid tissue in other mammals, changes with ag...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0856
更新日期:2008-02-01 00:00:00
abstract:PURPOSE:To compare the mydriatic effect and the short-term corneal endothelial safety of intracamerally injected N-methyl-3,4-dihydroxyphenylamine (epinine) to phenylephrine in a porcine eye model. METHODS:One hundred twelve eyes from newly slaughtered pigs were used in this study. After pretreatment with 20 mg intrac...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-3651
更新日期:2009-11-01 00:00:00