Abstract：
Purpose:The purpose of this study was to evaluate the efficacy of new viscous cysteamine hydrochloride (CH) eye drops (vCH 0.55%) compared with standard CH 0.10% drops treatment. Methods:This was an open-label, phase III, randomized, two-arm multicenter trial conducted at two centers in France. Cystinosis patients ≥2 years old were randomized 1:1 to receive eye drops, four times per day for 90 days in both eyes. We compared the superiority in reducing corneal cystine crystal density as assessed by in vivo confocal microscopy (IVCM). We also evaluated photophobia, corneal cystine crystal scores (CCCSs), and cystine crystal depth measured by optical coherence tomography. Safety objectives were to assess adverse events (AEs), local adverse drug reactions, and ocular safety parameters. Results:We included 15 patients with vCH 0.55% and 16 patients with CH 0.10% drops for 90 days. The mean absolute change in IVCM total score at day 90 in the vCH 0.55% drops group (-4.6 ± 3.1) was significantly greater than and superior to the mean absolute change in the CH 0.10% drops group (-0.46 ± 3.38; P < 0.0001). Photophobia, CCCS, and corneal cystine crystal depth were significantly more improved in the vCH 0.55% drops group than in the CH 0.10% group. The most frequent local adverse drug reactions in both groups were stinging, burning, redness, and blurred vision. Conclusions:vCH 0.55% was effective in reducing corneal cystine crystal density and superior to treatment with CH 0.10% drops, which offer advantages over hospital pharmacy formulations and is a more preferable and convenient treatment option.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Liang H,Labbé A,Le Mouhaër J,Plisson C,Baudouin Cdoi
10.1167/iovs.16-21080subject
Has Abstractpub_date
2017-04-01 00:00:00pages
2275-2283issue
4eissn
0146-0404issn
1552-5783pii
2621983journal_volume
58pub_type
杂志文章,多中心研究,随机对照试验abstract：Purpose:Conventional full-field flash electroretinography (ERG) yields a single response waveform that can be useful in the early detection and diagnosis of many diseases affecting the retina. It is an objective measurement that probes the entire retina. However, localized areas of dysfunction have relatively small inf...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-20726
更新日期：2017-06-01 00:00:00
abstract：PURPOSE:Patients with typical clinical features of retinitis pigmentosa were found to have the atypical electroretinographic finding of a negative waveform to a bright flash in the dark-adapted state. The full-field electroretinogram (ERG) was studied in seven such patients to understand better the pathophysiology. ME...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1993-11-01 00:00:00
abstract：PURPOSE:Oxidative stress seems to be an important factor in the development of age-related macular degeneration (AMD). The role of DNA repair mechanisms has also received attention recently in AMD pathogenesis. This case-control study was conducted to determine the frequency of polymorphisms in two DNA repair enzyme ge...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4842
更新日期：2010-09-01 00:00:00
abstract：:This study demonstrated that growth and differentiation of rabbit conjunctival epithelial cells could be promoted by such substrata as collagen gel, matrigel or various mixtures of collagen and matrigel in a defined culture system. On conventional plastic or glass culture, the conjunctival epithelial cells adopted a m...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1988-10-01 00:00:00
abstract：PURPOSE:To investigate the impact of diabetic macular ischemia (DMI) on visual acuity (VA), through the analysis of novel fluorescein angiography (FA) parameters. METHODS:Data were retrospectively collected over a 6-month period. DMI severity was graded using Early Treatment Diabetic Retinopathy Study (ETDRS) protocol...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-11103
更新日期：2013-03-28 00:00:00
abstract：Purpose:To investigate the role of mitogen-activated protein kinase-activated protein kinase-2 (MK2) in ocular surface damage of dry eye. Methods:MK2 inhibition was performed in mice subjected to desiccating stress (DS) by topical application of MK2 inhibitor (MK2i) or vehicle eye drops. The total and phosphorylated M...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.17-22240
更新日期：2017-09-01 00:00:00
abstract：PURPOSE:Accommodative effort during nearwork is thought to be a causative factor in the development of myopia. It has been proposed that an anomaly in autonomic control may be a precursor to the development of myopia. In the present study the closed-loop accommodation response after variations in fixation period was in...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1999-08-01 00:00:00
abstract：:Posterior eye cups of the frog Rana ridibunda were prefixed in the presence of tannic acid, osmicated and stained en bloc with uranyl acetate. Electron microscopy revealed the following features of membrane traffic in cells of the retinal pigment epithelium (RPE): (1) intracellular membranes with increased electron de...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1987-12-01 00:00:00
abstract：:The visual field of normal infants was assessed using a perimeter with LED stimuli and a forced-choice observation procedure. Central fixation was elicited by four central, pulsing LEDs and maintained with the aid of auditory stimuli. Field extent was derived from the four-alternative, forced-choice judgments of an ad...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1988-03-01 00:00:00
abstract：PURPOSE:The retina of the blind mole rat Spalax ehrenbergi was compared with other vertebrate photosensitive organs in an attempt to correlate its histologic organization with a presumptive nonvisual photoreceptor role. METHODS:The eyes of eight adult animals were analyzed by light and electron microscopy, using conve...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2002-07-01 00:00:00
abstract：:Vasoactive intestinal peptide (VIP) and d-ala2-methionine enkephalinamide (DALA, a long-lasting enkephalin analogue) were used to investigate the peptidergic control of lacrimal gland function. To characterize the mechanism by which VIP stimulates and DALA inhibits lacrimal peroxidase secretion, the effect of these pe...
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:Primary open angle glaucoma-associated mutations in myocilin (MYOC) cause protein "nonsecretion," rendering secreted MYOC difficult to detect using conventional techniques. This study focused on developing and using an assay that can quickly and easily detect mutant MYOC secretion. METHODS:We fused Gaussia luc...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-18789
更新日期：2016-04-01 00:00:00
abstract：PURPOSE:To identify risk factors for the development of high-risk proliferative diabetic retinopathy (PDR) and for the development of severe visual loss or vitrectomy (SVLV) in eyes assigned to deferral of photocoagulation in the Early Treatment Diabetic Retinopathy Study (ETDRS). METHODS:Multivariable Cox models were...
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：1998-02-01 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 iron carrier transferrin is expressed at remarkably high levels in normal retinas and is upregulated during retinal degeneration. The authors characterized the consequences of genetically reduced retinal transferrin production on retinal structure and function. METHODS:Hypotransferrinemic (HPX⁻/⁻) mice tre...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-7436
更新日期：2012-02-02 00:00:00
abstract：PURPOSE:To determine whether the atmospheric pressure (ATM) change experienced during diving can induce changes in the intraocular pressure (IOP) of eyes in a normal population. METHODS:The IOP of 27 healthy volunteers (ages, 23.8 +/- 4.9 years; range, 18-44) was measured with a Perkins applanation tonometer by two in...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.07-1578
更新日期：2008-12-01 00:00:00
abstract：:To compare the effects on the postnatal development of the eye of both total and partial form deprivation in diurnally reared chicks and of dark-rearing, chicks were reared with occluders covering one eye from hatching for up to 6 weeks. In diurnally reared birds, both total and partial form deprivation resulted in se...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1987-08-01 00:00:00
abstract：PURPOSE:To evaluate genotypic and macular morphologic correlations in patients with RPE65-, CEP290-, GUCY2D-, or AIPL1-related Leber congenital amaurosis (LCA) using spectral-domain optical coherence tomography (SD-OCT). METHODS:SD-OCT macular scans were performed in 21 patients, including 10 with RPE65, 7 with CEP290...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-3734
更新日期：2010-05-01 00:00:00
abstract：:Purpose: To compare efficacy and safety between subconjunctival and intrascleral methods of mitomycin C (MMC) application during trabeculectomy Methods: This retrospective study included 165 eyes treated by trabeculectomy and compared clinical outcomes between eyes that received subconjunctival MMC (Group 1, 80 eyes, ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-14159
更新日期：2014-07-21 00:00:00
abstract：Purpose:Diabetic retinopathy (DR) is a leading cause of vision impairment and blindness worldwide in the working-age population, and the incidence is rising. Until now it has been difficult to define initiating events and disease progression at the molecular level, as available diabetic rodent models do not present the...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.61.2.43
更新日期：2020-02-07 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 describe hereditary macular degeneration observed in the cynomolgus monkey (Macaca fascicularis), which shares phenotypic features with age-related macular degeneration in humans, and to test the involvement of candidate gene loci by mutation screening and linkage analysis. METHODS:Ophthalmic examinations w...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-1031
更新日期：2005-02-01 00:00:00
abstract：PURPOSE:To determine whether a new cyclic GMP analog, 2'-O-(4-benzoyl)benzoylguanosine 3',5' cyclic monophosphate (BB-cGMP), significantly reduces intraocular pressure in either normotensive or hypertensive eyes of rabbits. METHODS:Intraocular pressure of normal adult rabbits and/or rabbits with alpha-chymotrypsin-ind...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1994-05-01 00:00:00
abstract：PURPOSE:To assess photoreceptor structure in macular microholes by using adaptive optics scanning laser ophthalmoscopy (AO-SLO) and spectral-domain optical coherence tomography (SD-OCT) and compare with visual acuity. METHODS:Fourteen eyes from 12 patients with macular microholes underwent a full ophthalmologic examin...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.13-13792
更新日期：2014-08-21 00:00:00
abstract：PURPOSE:To examine the visual evoked potentials (VEP) and electroretinograms (ERG) generated during electrical stimulation of the human optic nerve using the optic nerve visual prosthesis. METHODS:Two volunteers blind from retinitis pigmentosa (RP) and with no light perception each received a chronically implanted opt...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4346
更新日期：2010-10-01 00:00:00
abstract：PURPOSE:The incidence of blindness is increasing, in part, because of abnormal ocular neovascularization. Anti-VEGF therapies have yielded impressive results; however, they are not a cure for blindness. Recently, metallothioneins (MTs) 1 and 2 have been implicated in the process of angiogenesis. Therefore, we investiga...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-14545
更新日期：2014-09-23 00:00:00
abstract：Purpose:The purpose of this paper was to discuss manifestations of primary mitochondrial dysfunctions and whether the retinal pigment epithelium or the photoreceptors are preferentially affected. Methods:A retrospective analysis was performed of patients with clinically and laboratory confirmed diagnoses of maternally...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.61.12.12
更新日期：2020-10-01 00:00:00
abstract：:Purpose. To understand the barriers facing primary care providers (PCPs), including nurse practitioners (NPs) and physician assistants (PAs), in the current referral-to-eye-care process and to solicit suggestions from PCPs on how to improve the current referral system. Methods. Four focus groups were conducted with a ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4512
更新日期：2010-04-01 00:00:00
abstract：PURPOSE:To examine the rate of retinal ganglion cell (RGC) layer and retinal nerve fiber layer (RNFL) changes in nonoptic neuritis (NON) eyes of relapsing remitting multiple sclerosis (RRMS) patients, and to find a specific imaging parameter useful for identifying disease progression. METHODS:Forty-five consecutive RR...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-19047
更新日期：2016-04-01 00:00:00
abstract：PURPOSE:Melanocyte is the major cell component in the uvea. Interleukin (IL)-6 is a proinflammatory cytokine. The authors studied the expression of IL-6 in cultured human uveal melanocytes (UMs) and their modulation by IL-1β and explored the relevant signal pathways. METHODS:Conditioned media and cells were collected ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-6908
更新日期：2011-06-01 00:00:00
