Abstract:
PURPOSE:To investigate the clinical and electrophysiologic natural history of Stargardt disease and correlate with the genotype. DESIGN:Cohort study of 59 patients. METHODS:Clinical history, examination, and electrophysiologic assessment were undertaken in a longitudinal survey. Patients were classified into 3 groups based on electrophysiologic findings, as previously published: Group 1 had dysfunction confined to the macula; Group 2 had macular and generalized cone system dysfunction; and Group 3 had macular and both generalized cone and rod system dysfunction. At baseline, there were 27 patients in Group 1, 17 in Group 2, and 15 in Group 3. Amplitude reduction of >50% in the relevant electroretinogram (ERG) component or a peak time shift of >3 ms for the 30 Hz flicker ERG or bright flash a-wave was considered clinically significant ERG deterioration. Molecular screening of ABCA4 was undertaken. RESULTS:The mean age at baseline was 31.7 years, with the mean follow-up interval being 10.5 years. A total of 22% of patients from Group 1 showed ERG group transition during follow-up, with 11% progressing to Group 2 and 11% to Group 3. Forty-seven percent of patients in Group 2 progressed to Group 3. There was clinically significant ERG deterioration in 54% of all subjects: 22% of Group 1, 65% of Group 2, and 100% of Group 3. At least 1 disease-causing ABCA4 variant was identified in 47 patients. CONCLUSIONS:All patients with initial rod ERG involvement demonstrated clinically significant electrophysiologic deterioration; only 20% of patients with normal full-field ERGs at baseline showed clinically significant progression. Such data assist counseling by providing more accurate prognostic information and are also highly relevant in the design, patient selection, and monitoring of potential therapeutic interventions.
journal_name
Am J Ophthalmoljournal_title
American journal of ophthalmologyauthors
Fujinami K,Lois N,Davidson AE,Mackay DS,Hogg CR,Stone EM,Tsunoda K,Tsubota K,Bunce C,Robson AG,Moore AT,Webster AR,Holder GE,Michaelides Mdoi
10.1016/j.ajo.2013.01.018subject
Has Abstractpub_date
2013-06-01 00:00:00pages
1075-1088.e13issue
6eissn
0002-9394issn
1879-1891pii
S0002-9394(13)00074-3journal_volume
155pub_type
杂志文章abstract:PURPOSE:To compare retinal thickness (RT) measurements obtained by spectral-domain optical coherence tomography (SD-OCT) and time-domain OCT (TD-OCT) in eyes with and without posterior segment disease diagnoses. DESIGN:Retrospective, observational chart review. METHODS:Scans of 184 healthy and diseased eyes from 106 ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2008.11.019
更新日期:2009-05-01 00:00:00
abstract::We used 21 cadaver eyes to study transsclerally sutured, ciliary sulcus-fixated intraocular lens implantation. Results showed that transscleral sutures should exit the sclera less than 1 mm posterior to the corneoscleral limbus for true ciliary sulcus fixation. The relationship of the ciliary sulcus to the overlying p...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(89)90121-9
更新日期:1989-09-15 00:00:00
abstract::We successfully treated six patients with culture-proven acute bacterial endophthalmitis by a combination of emergency pars plana vitrectomy and the instillation of intravitreal antibiotics. All six patients regained satisfactory vision. ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)76806-0
更新日期:1978-08-01 00:00:00
abstract:PURPOSE:To compare race-related differences in estimated rate of change of Bruch's membrane opening-minimum rim width (BMO-MRW) and circumpapillary retinal nerve fiber layer thickness (RNFLT) in healthy, glaucoma suspect, and glaucoma eyes of individuals of European (ED) and African descent (AD). DESIGN:Prospective co...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2018.08.050
更新日期:2018-12-01 00:00:00
abstract:PURPOSE:To examine the association between optic disk cupping and retinal vein occlusion (RVO). DESIGN:Prospective epidemiologic study. METHODS:setting: Population-based prospective study in Beaver Dam, Wisconsin. study population: Adults aged 43 to 86 years at baseline (n = 4926). observation procedures: Optic disk ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2005.12.015
更新日期:2006-05-01 00:00:00
abstract::We analyzed 34 sets of optic disk photographs from 19 patients with glaucoma who had been followed up for ten years to evaluate changes in the position of the vasculature of the optic disks over time. Each photograph was analyzed by an image analyzer's edge-defining program for optic disk analysis and by three experie...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)74101-7
更新日期:1987-11-15 00:00:00
abstract:PURPOSE:To evaluate the specificity of current definitions used to identify progressive change of the average peripapillary retinal nerve fiber layer (RNFL) thickness measurements obtained on optical coherence tomography (OCT) imaging. DESIGN:Prospective observational cohort study. METHODS:Setting: University of Cali...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2017.06.017
更新日期:2017-09-01 00:00:00
abstract:PURPOSE:To investigate long-term responsiveness of patients with severe uveitis associated with Behçet disease to interferon alpha2a (IFN-alpha2a). DESIGN:Retrospective study. METHODS:Thirty-two patients with sight-threatening uveitis associated with Behçet disease who relapsed despite corticosteroids and immunosuppr...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2008.08.038
更新日期:2008-12-01 00:00:00
abstract:PURPOSE:To determine provider compliance with hydroxychloroquine screening following the revised recommendations published in 2011 by the American Academy of Ophthalmology. DESIGN:Evaluation of adherence to a screening protocol. METHODS:Subjects were identified with hydroxychloroquine as a medication by electronic qu...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2015.06.009
更新日期:2015-09-01 00:00:00
abstract::We performed serial slit-lamp examinations and topography analysis on a patient whose initial topographic map suggested a diagnosis of keratoconus to us but which others interpreted as normal topography. Topography analysis documented cone progression during a two-year period. The initial map showed a cone apex power ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)76210-5
更新日期:1991-07-15 00:00:00
abstract::We reviewed the charts of 29 patients with large subretinal hemorrhages involving the center of the fovea to evaluate factors that might be prognostic of visual outcome. The average final visual acuity was 20/480 with a mean follow-up of three years. Patients with thick hemorrhages (causing an obvious elevation of the...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)75575-8
更新日期:1990-01-15 00:00:00
abstract:PURPOSE:To determine whether eyestrain symptoms predict eye conditions in 6-year-old children. DESIGN:Cross-sectional population-based study. METHODS:Reports of eyestrain symptoms were sought in parental questionnaires; 1740 children (79% response) underwent eye examinations (visual acuity, cover testing, cycloplegic...
journal_title:American journal of ophthalmology
pub_type: 杂志文章,多中心研究
doi:10.1016/j.ajo.2006.03.047
更新日期:2006-09-01 00:00:00
abstract::A 32-year-old obese woman with hypertension and a three-year history of pseudotumor cerebri developed bilateral juxtapapillary subretinal neovascular membranes. To our knowledge, this is the first reported case of bilateral subretinal neovascular membranes complicating the course of this disease. The subretinal neovas...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(81)90282-8
更新日期:1981-03-01 00:00:00
abstract:PURPOSE:To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). DESIGN:Retrospective cohort study. METHODS:Patients diagnosed with PEVAC were identi...
journal_title:American journal of ophthalmology
pub_type: 杂志文章,多中心研究
doi:10.1016/j.ajo.2017.10.009
更新日期:2017-12-01 00:00:00
abstract::We measured the magnitude and time course of the increase in palpebral fissure width in ten normal volunteers in response to direct-acting (2.5% phenylephrine, 1% apraclonidine) and indirect-acting (10% cocaine, 1% hydroxyamphetamine) topical adrenergic drugs given in one eye. The increase in the palpebral fissure wid...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)76775-3
更新日期:1991-06-15 00:00:00
abstract::Recurrent erosion of the cornea has been well documented in patients with nontraumatic anterior membrane dystrophies of various types. We examined five patients who, in addition to an erosion, developed stromal keratitis. Three of these patients were subjected to a complete microbiologic workup, but the lesions were a...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)75006-8
更新日期:1985-07-15 00:00:00
abstract::Amyloid fibrils occurring in primary and myeloma-associated (AL), secondary (AA), and certain neuropathic hereditary forms of systemic amyloidosis can be distinguished biochemically or immunohistologically as being composed of immunoglobulin light chain, protein AA, or prealbumin respectively. All types of systemic an...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(87)90357-6
更新日期:1984-08-15 00:00:00
abstract::Corneal epithelial iron lines commonly occur, and their shape is characteristically influenced by the underlying corneal surface topography. We studied a pattern of iron deposition, observed after implantation of the Intrastromal Corneal Ring (KeraVision, Inc., Santa Clara, California). In five of ten patients undergo...
journal_title:American journal of ophthalmology
pub_type: 临床试验,杂志文章
doi:10.1016/s0002-9394(14)71353-4
更新日期:1993-09-15 00:00:00
abstract:PURPOSE:To report an HIV-negative lymphoma patient who developed progressive outer retinal necrosis syndrome and who had a good visual outcome after treatment with two-drug antiviral therapy and intravenous immunoglobulin. METHODS:Case report. RESULTS:A 43-year-old man with small lymphocytic lymphoma was diagnosed wi...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(00)00951-x
更新日期:2001-07-01 00:00:00
abstract:PURPOSE:To classify and describe clinically meaningful classes of color vision defects using pretreatment Farnsworth-Munsell 100-hue results from the Early Treatment Diabetic Retinopathy Study (ETDRS) patients using standard statistical techniques. DESIGN:The ETDRS was a randomized trial investigating retinal photocoa...
journal_title:American journal of ophthalmology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1016/j.ajo.2004.02.009
更新日期:2004-07-01 00:00:00
abstract::A 46-year old woman and a 59-year-old man had corneal crystals, multiple myeloma, and IgG kappa hypergammaglobulinemia. In one case, crystalline deposits were also present in the lens. In both patients there was a marked decrease in the amount of crystals during chemotherapy. The crystals within the cornea of one case...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(78)90230-1
更新日期:1978-09-01 00:00:00
abstract:PURPOSE:To identify the frequency of recently acquired vs chronic systemic Toxoplasma gondii infections in patients with ocular toxoplasmosis. METHODS:Serum samples from 22 patients with primary ocular toxoplasmosis (not from scars) and 42 patients with recurrent ocular toxoplasmosis were tested for the presence of an...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(99)00266-4
更新日期:1999-10-01 00:00:00
abstract:PURPOSE/METHODS:A 49-year-old woman with a left orbital mass was referred to an orbital clinic. Clinical investigation suggested a lacrimal gland lesion and the patient underwent lateral orbitotomy and excisional biopsy for a suspected pleomorphic adenoma in the lacrimal gland. RESULTS/CONCLUSION:Histopathologic evalu...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)73823-1
更新日期:1995-01-01 00:00:00
abstract::A 17-year old girl received prednisone and azathioprine for the treatment of systemic lupus erythematosus. She developed a fever and hallucinations 18 months later; cryptococcal meningitis was diagnosed. An internal ophthalmoplegia with loss of accommodation and dilation of the pupils developed together with bilateral...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(79)90304-0
更新日期:1979-05-01 00:00:00
abstract:PURPOSE:To report a novel mutation of the CYP1B1 gene in a Japanese patient with primary infantile glaucoma. METHODS:DNA was extracted from leukocytes of six unrelated patients with primary infantile glaucoma. The coding regions of the CYP1B1 gene were amplified by polymerase chain reaction, examined by agarose gel se...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(99)00143-9
更新日期:1999-09-01 00:00:00
abstract:PURPOSE:To demonstrate whether indocyanine green stains the inner limiting membrane of the retina or residual vitreous cortex. METHODS:We report on the intraoperative staining patterns of the vitreomacular interface in 10 eyes of 10 consecutive patients who underwent vitrectomy with indocyanine green staining for macu...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(00)00924-7
更新日期:2001-03-01 00:00:00
abstract:PURPOSE:Photorefractive keratectomy has the potential to cause transient corneal haze. The purpose of this study was to evaluate the relationship between transient corneal haze as measured by an objective means and high and low contrast visual performance. METHODS:In a prospective study, 44 eyes of 28 patients were ex...
journal_title:American journal of ophthalmology
pub_type: 临床试验,杂志文章
doi:10.1016/s0002-9394(01)01003-0
更新日期:2001-08-01 00:00:00
abstract:PURPOSE:To present a large series of choroidal hemangiomas treated with proton beam irradiation and to describe the treatment outcomes. METHODS:We treated 54 eyes of 53 patients with choroidal hemangioma. The lesions consisted of 48 circumscribed hemangiomas and six diffuse hemangiomas in patients with Sturge-Weber sy...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(98)00150-0
更新日期:1998-08-01 00:00:00
abstract::Between September 1978 and December 1985, 450 case reports of serious respiratory and cardiovascular events and 32 case reports of death attributed to ophthalmic timolol were received by the United States Food and Drug Administration and the National Registry of Drug-Induced Ocular Side Effects. Two hundred sixty-seve...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(86)90532-5
更新日期:1986-11-15 00:00:00
abstract::We perfused enucleated human eyes via the anterior chamber by the constant pressure technique. Infusion of human serum into the anterior chamber of enucleated human eyes for 30 minutes at 23 mm Hg pressure induced a 42% decrease in facility of outflow, which was not relieved by irrigation of the anterior chamber with ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(78)90023-5
更新日期:1978-07-01 00:00:00