Abstract:
:The role of heterozygous galactokinase deficiency in the development of presenile cataracts is presently undetermined. Erythrocyte galactokinase activity was measured from 95 normal Caucasian subjects and from 39 Caucasian patients who had developed idiopathic bilateral cataracts between ages 20 and 55. The diagnosis of heterozygous galactokinase deficiency was based on the following criteria: galactokinase activity more than 2.0 SD below the control population mean; when available, familial evidence for heterozygous galactokinase activity was used as additional evidence. Three of 39 patients (1/13) with cataracts were found to be carriers of the galactokinase deficiency allele (P less than 0.001). Two heterozygotes had high dietary galactose intake suggesting a possible relationship between a high galactose diet and cataract formation. Dietary information was unavailable for the third heterozygote. We conclude that there is a high prevalence of heterozygous galactokinase deficiency existing in patients less than 55 yr of age with cataracts, and recommend that adults at risk restrict their consumption of dairy products.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Stambolian D,Scarpino-Myers V,Eagle RC Jr,Hodes B,Harris Hsubject
Has Abstractpub_date
1986-03-01 00:00:00pages
429-33issue
3eissn
0146-0404issn
1552-5783journal_volume
27pub_type
杂志文章abstract::The outer horizontal cell (OHC) of the frog Rana pipiens was studied by light and electron microscopy of Golgi-stained and horseradish peroxidase-injected cells. Responses of OHCs were recorded with intracellular electrodes. The OHCs are probably axonless cells. Their dendritic terminals are lateral processes at synap...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1985-05-01 00:00:00
abstract::We tested the state of retinal correspondence at different positions in the visual field of ten observers with strabismic and/or anisometropic amblyopia, four strabismic subjects with alternating fixation and three normal controls. Correspondence was evaluated by the subjective displacement of dichoptic stimuli; to es...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1989-09-01 00:00:00
abstract:PURPOSE:Excessive and continuously produced free radicals in the outer retina are implicated in retinal aging and the pathogenesis of sight-threatening retinopathies, yet measuring outer retinal oxidative stress in vivo remains a challenge. Here, we test the hypothesis that continuously produced paramagnetic free radic...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-18420
更新日期:2015-12-01 00:00:00
abstract:PURPOSE:To describe and analyze developmental change from birth through maturity of human electroretinogram (ERG) response, especially in terms of rod phototransduction as represented in the ERG a-wave. METHODS:Electroretinograms were recorded from 16 human infants from 5 to 270 days of age, two children 1.9 and 3.4 y...
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:The values of Heidelberg Retinal Tomograph (HRT; Heidelberg Engineering, Heidelberg, Germany) stereometric parameters depend on the reference plane (RP), the instability of which results in parameter variability. Identification of change depends on RP stability. This study was undertaken to evaluate the influen...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-1764
更新日期:2008-09-01 00:00:00
abstract:PURPOSE:Leukocytes play a critical role in ocular diseases such as uveitis, diabetic retinopathy, and choroidal neovascularization. Intercellular adhesion molecule (ICAM)-1 is essential for the migration of leukocytes. Control of ICAM-1 expression may lead to therapies for these diseases. Small-interfering ribonucleic ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-3457
更新日期:2010-01-01 00:00:00
abstract:Purpose:Lens epithelial cell (LEC) conversion to myofibroblast is responsible for fibrotic cataract surgery complications including posterior capsular opacification. While transforming growth factor beta (TGFβ) signaling is important, the mechanisms by which the TGFβ pathway is activated post cataract surgery (PCS) are...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-25067
更新日期:2018-10-01 00:00:00
abstract:PURPOSE:To determine variations in eye volume, surface area, and shape with refractive error in young children using a three-dimensional magnetic resonance imaging (MRI) model. METHODS:A subset of Singaporean Chinese boys enrolled in the population-based Strabismus, Amblyopia, and Refractive Error in Singapore (STARS)...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7269
更新日期:2011-11-17 00:00:00
abstract:Purposes:Trabeculectomy is a surgical procedure for lowering intraocular pressure in glaucoma patients, in which excessive scarring leading to failure of the filtering bleb adversely affects the surgical outcome. Heightened Tenon's capsule fibroblast (TCF) proliferation and extracellular matrix (ECM) deposition are imp...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-19480
更新日期:2016-10-01 00:00:00
abstract:Purpose:Prospective treatments for age-related macular degeneration and inherited retinal degenerations are commonly evaluated in the Royal College of Surgeons (RCS) rat before translation into clinical application. Historically, retinal thickness obtained through postmortem anatomic assessments has been a key outcome ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-20363
更新日期:2017-03-01 00:00:00
abstract:PURPOSE:To compare confocal scanning laser tomography (CSLT) to optic disc photography for detecting progressive glaucomatous disc changes. METHODS:Four expert observers first examined a training set of 40 pairs of monoscopic disc photographs to help set a criterion for change. They then classified 44 pairs of monosco...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-2457
更新日期:2009-04-01 00:00:00
abstract:PURPOSE:Diabetic retinopathy is shown to share many similarities with chronic inflammatory disease, and in diabetes, accelerated apoptosis of retinal capillary cells is evident before histopathology can be seen. The purpose of this study was to examine the effect of interleukin (IL)-1beta on capillary cell apoptosis in...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-0633
更新日期:2004-11-01 00:00:00
abstract::Neodymium: yttrium aluminum garnet (Nd:YAG) laser transscleral cyclophotocoagulation has been shown to be an effective method of lowering intraocular pressure (IOP). Transmission and absorption features of diode laser radiation (810 nm) make these new laser sources suitable for production of transscleral thermal lesio...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1991-04-01 00:00:00
abstract:PURPOSE:To evaluate the contribution that fixation errors make to the overall variability of perimetric responses in patients with glaucoma. METHODS:Frequency of seeing curves were established, with and without fixation error correction, at two locations in each of 14 patients with glaucoma and good visual acuity. One...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1996-02-01 00:00:00
abstract:PURPOSE:To determine the size variation of the X-chromosomal human red and green visual pigment gene array in the general population using pulsed field gel electrophoresis and Southern blotting. METHODS:Peripheral blood lymphocytes were prepared from 67 anonymous males. The cells were embedded in agarose and the genom...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1997-04-01 00:00:00
abstract:PURPOSE:The authors examined the muscarinic acetylcholine receptor (mAChR) subtypes in rabbit corneal epithelial and endothelial cells and in subcellular fractions of these cell types. A Chinese hamster ovary (CHO) cell line (nontransfected CHO K1), expected to be a negative control, also was investigated. METHODS:Who...
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:Intramuscular innervation of horizontal rectus extraocular muscle (EOMs) is segregated into superior and inferior (transverse) compartments, whereas all EOMs are also divided into global (GL) and orbital (OL) layers with scleral and pulley insertions, respectively. Mechanical independence between both types of ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15968
更新日期:2014-12-11 00:00:00
abstract::Contrast sensitivity functions for sinusoidal gratings of different spatial frequencies and stimulus durations have been determined for both eyes of amblyopic subjects. For both long and short stimulus durations, the entire contrast sensitivity function of the amblyopic eyes is lower than that of the nonamblyopic eyes...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract:PURPOSE:Retinal pigment epithelial (RPE) cells play important roles in ophthalmologic diseases such as proliferative vitreoretinopathy, AMD, and diabetic retinopathy. MicroRNA-34a (miR-34a) has been reported to be important in the regulation of cell proliferation, migration, differentiation, and apoptosis. In this stud...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-11873
更新日期:2013-10-03 00:00:00
abstract:Purpose:To quantify organelles impacting imaging in the cell body and intact apical processes of human retinal pigment epithelium (RPE), including melanosomes, lipofuscin-melanolipofuscin (LM), mitochondria, and nuclei. Methods:A normal perifovea of a 21-year-old white male was preserved after rapid organ recovery. An...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.61.8.13
更新日期:2020-07-01 00:00:00
abstract:PURPOSE:To determine the efficacy and safety of naked plasmid gene therapy to the corneal stroma and epithelium. METHODS:Naked plasmid DNA was injected under pressure into the cornea of mice. The expression of genes coding for beta galactosidase (beta-gal), enhanced green fluorescent protein (EGFP), vascular endotheli...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2001-08-01 00:00:00
abstract:Purpose:To define the nature and extent of temporal frequency abnormalities in diabetics who have mild or no nonproliferative diabetic retinopathy (NPDR) by using the flicker electroretinogram (ERG). Methods:Light-adapted flicker ERGs were recorded from 20 diabetics who have no clinically apparent retinopathy, 20 diab...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-25199
更新日期:2018-10-01 00:00:00
abstract:PURPOSE:In severe ocular surface diseases, pathologic keratinization of the ordinarily nonkeratinized corneal and conjunctival mucosal epithelia results in severe visual loss. The expression in conjunctivalized corneas of various proteins known to play important roles in the physiological keratinization process in huma...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2001-03-01 00:00:00
abstract::The level of oxygen at the anterior corneal surface beneath the closed eyelid is shown to be equivalent to an atmosphere of 7.7% oxygen. This finding is in good agreement with assumptions which have been based on the oxygen level at the palpebral conjunctiva. However, in some instances a significant amount of oxygen i...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract:PURPOSE:Point and null mutations in interphotoreceptor retinoid-binding protein (IRBP) cause retinal dystrophy in affected patients and IRBP-deficient mice with unknown mechanism. This study investigated whether IRBP protects cells from damages induced by all-trans-retinal (atRAL), which was increased in the Irbp(-/-) ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-18551
更新日期:2016-04-01 00:00:00
abstract:PURPOSE:This study observed biofilm formation by Pseudomonas aeruginosa and Staphylococcus aureus in contact lens cases and investigated the inhibitory effects of 2,2'-dipyridyl (2DP) and 1,2,3,4,6-penta-O-galloyl-b-D-glucopyranose (PGG). METHODS:Biofilm formation of P. aeruginosa ATCC 9027 and S. aureus ATCC 25923 an...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-17723
更新日期:2015-11-01 00:00:00
abstract:PURPOSE:To identify if whole-body hydration plays an important role in dry eye (DE). We hypothesized that individuals classified as DE have higher plasma osmolality (Posm), indicating suboptimal hydration, compared with those classified as non-DE. METHODS:Using a hospital-based observational cross-sectional design, as...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.12-10175
更新日期:2012-09-25 00:00:00
abstract:PURPOSE:To establish an experimental mouse model of retinal detachment (RD) created by corneal puncture (CP). METHODS:Mouse corneas were punctured with a 30.5-gauge beveled needle, and the anterior chamber was penetrated. Histologic and functional changes of the retina were examined by light microscopy and electroreti...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-8241
更新日期:2012-03-26 00:00:00
abstract:Purpose:The prevalence of age-related macular degeneration (AMD) increases dramatically with age. This large collaborative study investigates the effects of 51 late-AMD-associated genetic variants in different ages, focusing on individuals above the age of 90 years. Methods:The study included 27,996 individuals of the...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.61.14.17
更新日期:2020-12-01 00:00:00
abstract:PURPOSE:To determine whether fellow eyes with normal visual acuity of patients with unilateral neovascular age-related macular degeneration (AMD) have retinal malfunction. METHODS:Foveal cone electroretinograms (ERGs) were recorded from fellow eyes with visual acuities of 20/25 or better of 73 patients with unilateral...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1993-11-01 00:00:00