Abstract:
:We examined three affected members of a Chinese-American family with Bietti's crystalline retinopathy. The clinical characteristics of a 24-year-old proband are contrasted to the clinical findings of her grandmother, for whom we have 26 years of follow-up data. Lymphocytes and fibroblasts from a skin biopsy of the grandmother contained crystalline lysosomal material, which supports the diagnosis. Biochemical studies of the crystalline lysosomal material failed to identify the stored compounds but did not show them to be cholesterol or cholesterol ester. Finally, histopathologic studies performed for this condition demonstrated advanced panchorioretinal atrophy, with crystals and complex lipid inclusions seen in choroidal fibroblasts.
journal_name
Am J Ophthalmoljournal_title
American journal of ophthalmologyauthors
Kaiser-Kupfer MI,Chan CC,Markello TC,Crawford MA,Caruso RC,Csaky KG,Guo J,Gahl WAdoi
10.1016/s0002-9394(14)76572-9subject
Has Abstractpub_date
1994-11-15 00:00:00pages
569-82issue
5eissn
0002-9394issn
1879-1891pii
S0002-9394(14)76572-9journal_volume
118pub_type
杂志文章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:PURPOSE:To evaluate the efficacy of spectral-domain optical coherence tomography (SD OCT) compared to indocyanine green angiography (ICGA) in detecting idiopathic polypoidal choroidal vasculopathy (PCV) and in differentiating between PCV and occult choroidal neovascularization (CNV). DESIGN:Retrospective observational...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2014.08.025
更新日期:2014-12-01 00:00:00
abstract::Three hundred two cases of epibulbar tumors in children were retrospectively reviewed histologically and examined by diagnostic categories over a 49-year period. Of the 302 cases, 33% were choristomas; 29% nevi; 11%, epithelial inclusion cysts; 7%, papillomas; 6%, pyogenic granulomas; and 5% granulomas. Three cases of...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(75)90685-6
更新日期:1975-06-01 00:00:00
abstract:PURPOSE:To perform 11- and 2-year health care sector (ophthalmic) and societal cost perspective reference case, cost-utility analyses comparing bevacizumab, ranibizumab, and aflibercept monotherapies for neovascular age-related macular degeneration (NVAMD). DESIGN:Cost-utility analysis. METHODS:The authors performed ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2020.05.029
更新日期:2020-10-01 00:00:00
abstract:PURPOSE:To prospectively compare the safety and efficacy of deep lamellar keratoplasty (DLKP) and penetrating keratoplasty (PKP). DESIGN:Prospective, randomized clinical trial. METHODS:Consecutive 26 eyes of 24 patients who had stromal opacity without endothelial abnormalities were randomly assigned to either PKP or ...
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/s0002-9394(02)01523-4
更新日期:2002-08-01 00:00:00
abstract:PURPOSE:To investigate thermal changes produced by a chandelier fiber probe (CFP). DESIGN:Interventional single case report and laboratory investigation. METHODS:When CFP was used for 25-gauge vitrectomy, an abnormal odor was emitted, and the CFP was instantly removed. To investigate this incident, the thermal change...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2006.07.049
更新日期:2007-01-01 00:00:00
abstract:PURPOSE:To evaluate the effects of the Boston Ocular Surface Prosthesis (Boston Foundation for Sight, Needham, Massachusetts, USA) on visual acuity (VA) and visual functioning in patients with severe corneal ectasia, irregular astigmatism, or ocular surface disease. DESIGN:Prospective, interventional case series. MET...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2009.07.037
更新日期:2010-01-01 00:00:00
abstract:PURPOSE:We performed a histopathologic and immunohistologic study to determine the macromolecular and cellular components of subfoveal neovascular membranes removed at the time of submacular surgery. METHODS:Subfoveal neovascular membranes were surgically removed from ten patients (seven with age-related macular degen...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)72158-0
更新日期:1995-09-01 00:00:00
abstract:PURPOSE:To document and study the cross-sectional structures of polypoidal elements using optical coherence tomography in eyes with idiopathic polypoidal choroidal vasculopathy. METHODS:Optical coherence tomography images of two eyes with idiopathic polypoidal choroidal vasculopathy were correlated with slit-lamp biom...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(98)00411-5
更新日期:1999-03-01 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 compare visual and topographic outcomes 1 year after conventional (C-CXL) vs accelerated corneal cross-linking (A-CXL) in pediatric keratoconus (KC). DESIGN:Comparative, retrospective, consecutive case series. METHODS:Patients with topography-confirmed, progressive KC and a corneal thickness of ≥400 μm at ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2017.08.015
更新日期:2017-11-01 00:00:00
abstract:PURPOSE:To investigate the impact on patient management of posterior segment echographic evaluation at an academic institution. DESIGN:Retrospective, noncomparative, consecutive case series. METHODS:Records were reviewed of all patients evaluated for the first time with ocular echography for posterior segment evaluat...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(03)00910-3
更新日期:2004-01-01 00:00:00
abstract:PURPOSE:To evaluate the outcome of sodium morrhuate 5% injections in patients with low flow vascular lesions, which consist of orbital lymphangiomas, and in one patient with intraosseous cavernous hemangioma. DESIGN:Prospective, interventional consecutive case series. METHODS:Intralesional sodium morrhuate 5% was inj...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2005.09.026
更新日期:2006-02-01 00:00:00
abstract:PURPOSE:To describe a newly identified clinical entity tentatively named white dot fovea. METHODS:We examined by scanning laser ophthalmoscopy 58 eyes of 30 patients (mean age, 64 years) who had white dots in the fovea (anatomically defined as the foveola) simulating macular hole. In addition, the retinal surfaces of ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)70995-x
更新日期:1997-01-01 00:00:00
abstract:PURPOSE:To compare the prevalence of dengue maculopathy among patients hospitalized for dengue fever during a 2007 epidemic with its prevalence during the 2005 epidemic. DESIGN:Cross-sectional observational study of consecutive patients hospitalized in two general hospitals with dengue fever during the dengue epidemic...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2009.06.030
更新日期:2009-12-01 00:00:00
abstract:PURPOSE:To evaluate the indications for and outcomes of intraocular lens (IOL) exchange at the same clinical setting over the past decade, as well as compare the efficacy and safety of anterior chamber lens (AC-IOL) and posterior chamber lens (PC-IOL) implantation for IOL exchange. DESIGN:Retrospective, nonrandomized ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2005.05.015
更新日期:2005-10-01 00:00:00
abstract:PURPOSE:To review the contributions to ophthalmic genetics through the American Journal of Ophthalmology (AJO). DESIGN:Perspective. METHODS:A literature search to retrieve original articles, letters, editorials, and published lectures from 1966 to 2017, providing a 50-year review. Titles were excluded that gave no re...
journal_title:American journal of ophthalmology
pub_type: 杂志文章,评审
doi:10.1016/j.ajo.2018.03.004
更新日期:2018-06-01 00:00:00
abstract::A 26-year-old woman experienced bilateral hearing loss, progressive nasal obstruction, and rhinorrhea. Examination disclosed a retropharyngeal mass. A needle biopsy specimen of the mass showed that it was a chordoma. The patient underwent surgery to remove the mass and received a postoperative course of radioactive co...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)75899-4
更新日期:1981-07-01 00:00:00
abstract:PURPOSE:To report two cases of diffuse unilateral subacute neuroretinitis (DUSN) with unusual findings. DESIGN:Observational and interventional case series. METHODS:Two patients with DUSN were found to have the unusual findings of a large live worm in one eye and macular cyst in the other. Both patients underwent fun...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2006.06.005
更新日期:2006-11-01 00:00:00
abstract::Of 45 patients with proliferative sickle retinopathy in stages III, IV, and V, nine patients (eight with hemoglobin SC disease, one with sickle cell thalassemia) showed spontaneous regression (autoinfarction) of retinal sea fans. One mechanism involved in autoinfarction of neovascular tissue is progressive, centripeta...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(75)90285-8
更新日期:1975-11-01 00:00:00
abstract:PURPOSE:To estimate the cumulative probability of Nd:YAG laser posterior capsulotomy after cataract extraction in a geographically defined population. METHODS:Rochester Epidemiology Project databases were used to identify retrospectively all Nd:YAG laser posterior capsulotomies performed on Olmsted County, Minnesota, ...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(00)00795-9
更新日期:2001-02-01 00:00:00
abstract:PURPOSE:To analyze the features of retinal detachment (RD) occurring after cataract surgery performed by Kelman phacoemulsification (KPE) and to identify any potential correlations between KPE intraoperative complications and the features of RD (incidence apart) as well as the final visual outcome. DESIGN:Intervention...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(02)01347-8
更新日期:2002-05-01 00:00:00
abstract:PURPOSE:To document the variations in normal anatomy that occur at the insertion of the inferior oblique muscle and in the vicinity of its surgical capture site (10 to 12 mm from the insertion). METHODS:One hundred intact cadaver orbits with no history of eye muscle or orbital disorders during life were carefully diss...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(99)00225-1
更新日期:1999-10-01 00:00:00
abstract:PURPOSE:To develop patient-derived Pediatric Eye Questionnaires (PedEyeQ) to separately assess eye-related quality of life (ER-QOL) and functional vision in children with eye conditions. DESIGN:Questionnaire development study. METHODS:A total of 444 children (0 to <18 years old), across 10 diagnostic categories, were...
journal_title:American journal of ophthalmology
pub_type: 杂志文章,多中心研究
doi:10.1016/j.ajo.2019.01.001
更新日期:2019-04-01 00:00:00
abstract::To investigate whether Fuchs' heterochromic iridocyclitis may be an immune complex vasculitis, we used an immunofluorescence technique to detect immunoglobulins and complement in iris biopsy specimens from nine patients with Fuchs' heterochromic iridocyclitis, 12 patients with other types of uveitis, and nine patients...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(14)75757-5
更新日期:1992-01-15 00:00:00
abstract::A 7-year-old boy with acute lymphocytic leukemia developed iris infiltration, blood-streaked hypopyon, and high intraocular pressure. A diagnostic anterior chamber paracentesis was performed, which provided a cytologic diagnosis. The aspirate was studied by both the millipore filter technique with Papanicolaou stain a...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/0002-9394(80)90112-9
更新日期:1980-02-01 00:00:00
abstract:PURPOSE:Macular imaging with optical coherence tomography (OCT) measures the most critical retinal ganglion cells (RGCs) in the human eye. The goal of this perspective is to review the challenges to detection of glaucoma progression with macular OCT imaging and propose ways to enhance its performance. DESIGN:Perspecti...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2020.09.026
更新日期:2020-09-18 00:00:00
abstract:PURPOSE:To determine the optical coherence tomographic images that are commonly observed in eyes with choroidal osteoma (choroidal ossification). METHODS:Three patients with choroidal osteoma were examined by optical coherence tomography. RESULTS:We found two optical coherence tomographic patterns in the eyes with ch...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(00)00503-1
更新日期:2000-07-01 00:00:00
abstract:PURPOSE:To report a new method to perform intraoperative video fluorescein angiography (VFA) using an optical fiber-free intravitreal surgery system (OFFISS) and retinal blood flow analysis during surgery. DESIGN:Observational case series. METHODS:Four eyes of four patients with macular edema as a result of branch re...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/j.ajo.2004.06.079
更新日期:2004-12-01 00:00:00
abstract:PURPOSE:To report a patient with persistent subepithelial corneal opacities 18 months after adenoviral keratoconjunctivitis who underwent photorefractive keratectomy for the correction of myopia. METHODS:Case report, review of medical literature, and slit-lamp photography. RESULTS:The patient underwent photorefractiv...
journal_title:American journal of ophthalmology
pub_type: 杂志文章
doi:10.1016/s0002-9394(98)00224-4
更新日期:1998-12-01 00:00:00