Abstract：
:Gyrate atrophy of the choroid and retina is caused by deficient activity of ornithine ketoacid aminotransferase, a pyridoxal phosphate dependent enzyme. Besides the typical eye findings, abnormalities have been found on muscle biopsy, electro-encephalography, electromyography and electrocardiography, establishing this as a generalized disorder. Ornithine is markedly elevated in plasma and other body fluids. Plasma lysine, glutamate, glutamine and creatine are reduced. The possible contributions of these biochemical disturbances to the pathogenesis of gyrate atrophy are discussed. The disease is one of the few examples of an inherited chorioretinal dystrophy whose underlying biochemical defect is known. It therefore offers a unique opportunity to develop and test rational approaches to therapy. These include lowering of the abnormally high ornithine by dietary restriction of its precursor arginine, facilitation of ornithine excretion by administration of alpha-aminoisobutyric acid, replacement of deficient products such as lysine or creatine, or increasing residual enzyme activity by high levels of cofactor (vitamin B6). The results of several studies employing such approaches to therapy are presented as well as preliminary indications of possible benefit in a few patients.
journal_name
Int Ophthalmoljournal_title
International ophthalmologyauthors
Weleber RG,Kennaway NG,Buist NRdoi
10.1007/BF00139577subject
Has Abstractpub_date
1981-08-01 00:00:00pages
23-32issue
1-2eissn
0165-5701issn
1573-2630journal_volume
4pub_type
杂志文章,评审abstract：PURPOSES:This research aimed to investigate effects and risk factors on non-contact tonometer (NCT) readings in healthy myopic subjects by employing cross-sectional study design. METHODS:Totally, sixty otherwise healthy myopic volunteers (mean 28.4 years old) with 90% female were recruited in ophthalmic clinic. The ro...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-019-01203-8
更新日期：2020-02-01 00:00:00
abstract：PURPOSE:To investigate the changes in the anterior chamber volume (ACV) with swept-source optical coherence tomography (SS-OCT) after cataract surgery and the factors that influence these ACV changes. METHODS:This was a prospective cohort study. Fifty-one patients who underwent cataract surgery were enrolled. Their AC...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-018-1012-1
更新日期：2019-08-01 00:00:00
abstract：PURPOSE:To analyze the relationship between the seasonal allergic conjunctivitis (SAC), eotaxin-2, matrix metalloproteinase-9 (MMP-9), and the topographical findings in the keratoconus patients. METHODS:Thirty-four eyes of patients without SAC (Group 1), 34 eyes of patients with SAC (Group 2), and 20 eyes of control s...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-019-01149-x
更新日期：2020-01-01 00:00:00
abstract：OBJECTIVE:To evaluate the efficacy and safety of the bilateral simultaneous XEN (BISIXEN) surgery in open-angle glaucoma patients. METHODS:Retrospective analysis of a prospective data base conducted on uncontrolled glaucoma patients who underwent BISIXEN surgery. Primary endpoint measure was the incidence of sight-thr...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-020-01640-w
更新日期：2021-01-02 00:00:00
abstract：PURPOSE:To assess ophthalmic manifestations in patients with stroke and emphasize the importance of a formal screening for visual problems in stroke patients in hospital and rehabilitation settings. METHODS:This was a cross-sectional study of 50 newly diagnosed patients with stroke with Glasgow Coma Scale (GCS) > 8 ex...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-019-01131-7
更新日期：2019-12-01 00:00:00
abstract：:We report the functional outcome of a patient who had undergone Boston keratoprosthesis type I for extensive ocular surface damage caused by latex of Euphorbia. The milky sap or latex of the Euphorbia plant is highly toxic and an irritant to the skin and eye. Damage to the eye ranges from mild to severe corneal edema,...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-012-9515-7
更新日期：2012-02-01 00:00:00
abstract：PURPOSE:The purpose of this study was to evaluate the accuracy of the convolutional neural network (CNN) model in glaucoma identification with three primary colors (red, green, blue; RGB) and split color channels using fundus photographs with a small sample size. METHODS:The dataset was prepared using color fundus pho...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-020-01485-3
更新日期：2020-11-01 00:00:00
abstract：PURPOSE:Numerous studies suggest that reactive oxygen species play a crucial role in the development of glaucoma. Since glaucoma patients exhibit posterior vitreous detachment earlier than controls, it has been suggested that reactive oxygen species-increased in glaucoma-also affect the vitreous. In the present study w...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-019-01268-5
更新日期：2020-04-01 00:00:00
abstract：:To investigate the efficacy of single-dose intravitreal dexamethasone implantation in the treatment of persistent diabetic macular edema (DME) unresponsive to 3 consecutive ranibizumab injections over a period of 6 months. Forty-one patients with a previous history of treatment for DME including at least three consecu...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-015-0155-6
更新日期：2016-08-01 00:00:00
abstract：:The cone dystrophies can be subdivided into 3 functional stages: central cone disease, peripheral cone disease and diffuse cone disease, respectively. In the patient material of our clinic the sex distribution of patients presenting with te diffuse cone disease stage was abnormal: 22 males and 3 females. The authors s...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/BF00155631
更新日期：1987-08-01 00:00:00
abstract：:Evaluation of brain cluster activation using the functional magnetic resonance imaging (fMRI) and diffusion tensor imaging (DTI) was sought in strabismic amblyopes. In this hospital-based case-control cross-sectional study, fMRI and DTI were conducted in strabismic amblyopes before initiation of any therapy and after ...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-015-0159-2
更新日期：2016-08-01 00:00:00
abstract：PURPOSE:To report a case of bilateral Terson Syndrome with sub inner limiting membrane hemorrhage associated with a rare finding: perimacular fold. METHODS:The patient, a 34 years old female with aneurysmal subarachnoid hemorrhage and bilateral Terson syndrome was admitted to the Ophthalmology department, complaining ...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-018-0822-5
更新日期：2019-02-01 00:00:00
abstract：:To evaluate the efficacy of 25-ga pars plana vitrectomy (PPV) and 25-ga PPV combined with phacoemulsification surgery for managing ciliary block (malignant) glaucoma. The medical records of 11 eyes, in nine consecutive patients with malignant glaucoma were retrospectively reviewed to determine the efficacy of 25-ga PP...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-014-9974-0
更新日期：2015-08-01 00:00:00
abstract：PURPOSE:To provide normal corneal elevation data for a large Caucasian population and to determine the impacts on these data of age, sex, axial length (AXL) and horizontal white-to-white (WW). SETTING:Centro Internacional de Oftalmología Avanzada, Madrid, Spain. DESIGN:Retrospective, cross-sectional, observational. ...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-018-1028-6
更新日期：2019-09-01 00:00:00
abstract：:To evaluate the effect of intravitreal bevacizumab (IVB) before Ahmed valve implantation for treatment of neovascular glaucoma (NVG). This study is a retrospective, comparative, consecutive case series. The study group consisted of 27 eyes of 26 patients with NVG who underwent an Ahmed valve implantation. Thirteen eye...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-013-9875-7
更新日期：2014-08-01 00:00:00
abstract：:The purpose of this study was to report various conditions masquerading as congenital nasolacrimal duct obstruction (CNLDO). Retrospective review was designed in a tertiary hospital setting. 92 eyes of 65 consecutive patients were included in this study. All patients presenting with CNLDO symptomatology but where the ...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-015-0050-1
更新日期：2015-12-01 00:00:00
abstract：:The aim of this study is to report the clinical outcome of a diabetic patient with optic nerve head neovascularization treated with an intravitreal injection of triamcinolone acetonide. A 52-year-old patient presented with clinically significant diffuse macular edema and optic nerve head neovascularization due to prol...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1023/b:inte.0000031740.85124.78
更新日期：2004-03-01 00:00:00
abstract：:Trichiasis/Entropion are the severe consequences of chronic trachoma during early life. Blindness and vision loss is preventable with timely lid surgery to correct trichiasis. In a trachoma hyperendemic region of Central Tanzania, a two year follow-up survey was conducted among 205 women with trichiasis to determine t...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/BF00919249
更新日期：1994-01-01 00:00:00
abstract：PURPOSE:To assess the level of awareness among UK ophthalmic surgeons of the potential risks from starch powdered surgical gloves during ophthalmic surgery and to show by electron microscopy that starch granule contamination can occur during ophthalmic surgery. SETTING:A sample (N = 46) of UK ophthalmologists from the...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1023/a:1006392915801
更新日期：1998-01-01 00:00:00
abstract：:Out of 49 patients diagnosed as having Stargardt's disease that have been clinically reviewed, 22 of these cases have been followed for a period up to 13 years. The cases have been studied in three subgroups, according to their fundus appearance. Patients from group I were those whose lesions were confined to the macu...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/BF00137651
更新日期：1985-11-01 00:00:00
abstract：:To report a unusual case of an isolated traumatic stellated tarso-conjunctival laceration located in the upper left eyelid without eyelid margin involvement and with normal ocular examination. A 19 year-old male wounded by a bokken (wooden katana) while practicing a sham duel had an isolated eyelid tarso-conjunctival ...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-012-9573-x
更新日期：2012-08-01 00:00:00
abstract：:To document the rate of self-reported compliance and glaucoma-related knowledge in Swiss patients and to identify risk factors for their poor compliance. This was an observational study, including a total of 200 consecutive patients already under glaucoma medication in two Swiss tertiary glaucoma clinics (Geneva and B...
journal_title：International ophthalmology
pub_type： 杂志文章,多中心研究
doi：10.1007/s10792-011-9468-2
更新日期：2011-10-01 00:00:00
abstract：:In serial experiments on cats, intravenous digital subtraction angiography (DSA) was utilized to observe orbital and ocular circulation. Images of the orbital arterial system as well as normal ocular vasculature were obtained, utilizing dosage of contrast material acceptable for human study. Images obtained after intr...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/BF00136492
更新日期：1985-09-01 00:00:00
abstract：:We describe a case illustrating a new technique of using intraoperative optical coherence tomography (IOCT) for foreign body removal from the limbus and cornea. In this case, fragments of a fish hook in the temporal limbus and cornea were identified on the OCT. The residual pieces were then removed, assisted by high-r...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-016-0304-6
更新日期：2017-06-01 00:00:00
abstract：PURPOSE:To describe and analyze short-term posterior vitreous abnormalities following intravitreal ocriplasmin in eyes with symptomatic vitreomacular traction syndrome (VMT). METHODS:In this institutional, prospective and interventional study enrolled patients with symptomatic focal VMT syndrome treated with intravitr...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-019-01177-7
更新日期：2020-01-01 00:00:00
abstract：:Ocular wall dimensions (axial eye length, anterior chamber depth, lens thickness, vitreous length, thickness and volume of the ocular wall) were determined by echobiometry on 10 eyes of 10 patients with glaucoma one day before and four days after trabeculectomy. The results showed significant differences: decreased ax...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/BF00917990
更新日期：1992-09-01 00:00:00
abstract：PURPOSE:To report the first European case of endogenous endophthalmitis secondary to a liver abscess due to Klebsiella pneumoniae expressing MagA gene. METHODS:A 33-year-old diabetic patient was admitted for fever and right upper quadrant abdominal pain. Abdominal computed tomography and laboratory studies were perfor...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-007-9111-4
更新日期：2008-04-01 00:00:00
abstract：PURPOSE:The authors investigated the use of dapiprazole, an alpha-adrenergic blocking agent, in preventing pigment release and IOP increase after exercise in patients affected with PDS. METHODS:Three young myopic males (31, 34 and 35 years old) affected with pigmentary dispersion syndrome performed 30 minutes of joggi...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/BF00130855
更新日期：1995-01-01 00:00:00
abstract：PURPOSE:To compare the actual depth of the tunnel created with femtosecond laser for intracorneal ring segment (ICRS) implantation with the target depth in keratoconus patients. METHODS:In this mix design study, eligible keratoconus patients were identified through chart review. The inclusion criterion was a history o...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-017-0595-2
更新日期：2018-08-01 00:00:00
abstract：:The purpose of this study is to evaluate whole lysyl oxidase like 1 (LOXL1) gene by sequence analysis in Turkish patients with exfoliation glaucoma (XFG). A total of 48 (35 male, 13 female) patients with XFG were enrolled. Besides routine ophthalmological examination, peripapillary retinal nerve fibre layer (RNFL) ana...
journal_title：International ophthalmology
pub_type： 杂志文章
doi：10.1007/s10792-016-0174-y
更新日期：2016-10-01 00:00:00
