Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0086543 (cataract)
 29,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied 62 eyes submitted to the Armed Forces Institute of Pathology (AFIP) from 1958 through 1980 that satisfied our criteria for the histologic diagnosis of Coats' disease. Our histopathologic definition of Coats' disease was the presence of a primary vascular lesion consisting of retinal telangiectasia with leakage of plasma to form intraretinal and subretinal exudates. In the cases we reviewed, Coats' disease occurred more frequently in boys, it usually affected only one eye, and was generally detected in the first decade of life. In 52 cases (79%) the clinical manifestations, strabismus and leukokoria, were thought to be caused by retinoblastoma. Angle closure glaucoma was present in 36 cases (58%). In all but one of the cases studied, the lesion was located peripheral to the equator. We further identified diffuse involvement of capillaries in the peripheral retina using trypsin-digest preparations. Associated histologic findings included: rubeosis iridis, cataract, vitreous neovascularization, and nodules resulting from fibrous metaplasia of the retinal pigment epithelium. These fibrous nodules typically occurred in the macular area and occasionally contained calcium or bone.
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PMID:Coats' disease: a study of 62 histologically confirmed cases. 650 5

This thesis presents the results of a study of 384 eyes of 192 patients with a mean age of 39.1 years who presented with typical retinitis pigmentosa. The major findings are outlined below, together with suggested hypotheses: Cataract was found in 46.4% of the eyes. Among these, 93.6% showed posterior subcapsular opacification. The incidence of cataract increased with age. The vitreous degeneration that is characteristic of the RP syndrome and begins in childhood was described as showing dust-like, particulate matter throughout the gel; posterior vitreous separation; formation of a posterior matrix of coarse, white, interconnected strands and opacities; and final collapse of the residual gel. Ultrastructural studies of vitreous material from eight eyes revealed that the particles were isolated pigment granules and the coarse strands were composed of condensed collagen fibers. Notwithstanding the vitreous degeneration and prevalence of myopia in RP, neurosensory retinal breaks and/or rhegmatogenous detachment were found in only 7 (1.8%) of the 384 eyes studied. Premature separation of the vitreous from the retina, absence of lattice retinal degeneration, and perhaps a stronger than normal RPE-neurosensory retinal bond are thought to be possible protective factors. Rather than searching for a "toxin," elaborated by diseased retina, that causes vitreous degeneration and cataract formation, it is suggested that the ocular media be studied for an absence of moieties that are normally produced by healthy retina for vitreous and lens maintenance. The classic criteria for diagnosis of RP were met by 96.3% of eyes that showed retinal vascular attenuation and by 52.0% that showed pallor of the optic disc. Less frequent manifestations included solitary retinal hemorrhage, peripheral microaneurysms, telangiectasia, and fluorescein leakage at the macula and disc. Seven additional cases with a Coats'-like retinal detachment were added to the 14 already presented in the literature. Two of the seven had autosomal dominant RP, the first such cases reported. The vascular malformations and detachments were most often inferior. Unlike typical Coats' syndrome, the condition was usually bilateral, showed no sex preference, and appeared to affect older individuals.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Retinitis pigmentosa: clinical observations and correlations. 667 82

The goal of radiation therapy in pediatric cancer is to destroy cancer cells and preserve functional surrounding normal cells. Although all radiation for pediatric cancers does not result in complications of the eye, acute and long-term radiation effects can occur after treatment. Acute radiation effects to the eye include erythema, epilation, conjunctivitis, dermatitis, keratitis, corneal ulceration, iritis, and retinal edema. Long-term radiation effects include tissue necrosis, decreased tear production, telangiectasia, scleral melting, cataract, corneal neovascularization, radiation retinopathy, retarded bone growth (of bones within the irradiated field), and radiation-induced cancers. Nursing interventions and implications will be presented in conjunction with medical management for each of these acute and long-term effects.
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PMID:Acute and long-term effects of radiation therapy to the eye in children. 826 86

The authors report surgical outcomes of full-thickness macular hole repair in two patients with idiopathic macular telangiectasia in a non-comparative case series. Both patients underwent pars plana vitrectomy with indocyanine green-assisted internal limiting membrane peeling and injection of 16% C(3)F(8) gas. Patients were imaged with optical coherence tomography (OCT) before and after surgery. The first patient demonstrated macular hole closure on examination and OCT with visual improvement from 20/50 preoperatively to 20/30 after macular hole surgery and subsequent cataract surgery. The second patient's hole closed per OCT immediately after surgery but reopened 4 months later, and visual acuity remained 20/70. Macular hole surgery may be an effective treatment in patients with idiopathic macular telangiectasia and full-thickness macular holes and should be further investigated.
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PMID:Surgery for full-thickness macular hole in patients with idiopathic macular telangiectasia type 2. 2115 75

Lutein is concentrated in the primate retina, where together with zeaxanthin it forms the macular pigment. Traditionally lutein is characterized by its blue light filtering and anti-oxidant properties. Eliminating lutein from the diet of experimental animals results in early degenerative signs in the retina while patients with an acquired condition of macular pigment loss (Macular Telangiectasia) show serious visual handicap indicating the importance of macular pigment. Whether lutein intake reduces the risk of age related macular degeneration (AMD) or cataract formation is currently a strong matter of debate and abundant research is carried out to unravel the biological properties of the lutein molecule. SR-B1 has recently been identified as a lutein binding protein in the retina and this same receptor plays a role in the selective uptake in the gut. In the blood lutein is transported via high-density lipoproteins (HDL). Genes controlling SR-B1 and HDL levels predispose to AMD which supports the involvement of cholesterol/lutein transport pathways. Apart from beneficial effects of lutein intake on various visual function tests, recent findings show that lutein can affect immune responses and inflammation. Lutein diminishes the expression of various ocular inflammation models including endotoxin induced uveitis, laser induced choroidal neovascularization, streptozotocin induced diabetes and experimental retinal ischemia and reperfusion. In vitro studies show that lutein suppresses NF kappa-B activation as well as the expression of iNOS and COX-2. Since AMD has features of a chronic low-grade systemic inflammatory response, attention to the exact role of lutein in this disease has shifted from a local effect in the eye towards a possible systemic anti-inflammatory function.
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PMID:Lutein: more than just a filter for blue light. 2246 91

In our institute, we have recently found a child Japanese monkey who is characterized by deep wrinkles of the skin and cataract of bilateral eyes. Numbers of analyses were performed to identify symptoms representing different aspects of aging. In this monkey, the cell cycle of fibroblasts at early passage was significantly extended as compared to a normal control. Moreover, both the appearance of senescent cells and the deficiency in DNA repair were observed. Also, pathological examination showed that this monkey has poikiloderma with superficial telangiectasia, and biochemical assay confirmed that levels of HbA1c and urinary hyaluronan were higher than those of other (child, adult, and aged) monkey groups. Of particular interest was that our MRI analysis revealed expansion of the cerebral sulci and lateral ventricles probably due to shrinkage of the cerebral cortex and the hippocampus. In addition, the conduction velocity of a peripheral sensory but not motor nerve was lower than in adult and child monkeys, and as low as in aged monkeys. However, we could not detect any individual-unique mutations of known genes responsible for major progeroid syndromes. The present results indicate that the monkey suffers from a kind of progeria that is not necessarily typical to human progeroid syndromes.
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PMID:Sporadic premature aging in a Japanese monkey: a primate model for progeria. 2536 57

This is a case of bilateral and symmetric postoperative pseudophakic cystoid macular edema (CME) in a patient with macular telangiectasia type 2 after uneventful subsequent cataract surgeries. An 80-year-old woman with a history of macular telangiectasia type 2 had uneventful phacoemulsification procedures and developed pseudophakic CME during the early postoperative periods, remarkably symmetric in both eyes. The CME responded well to treatment with topical antiinflammatory drops (combination of steroids and nonsteroidal antiinflammatory drugs [NSAIDs] prescribed for the right eye and NSAIDs only for the left eye), and the edema resolved after some months. To our knowledge, this is the first case that describes bilateral pseudophakic CME in macular telangiectasia type 2 and highlights the specific retinal condition as a possible additional risk factor for developing postoperative CME after phacoemulsification.
J Cataract Refract Surg 2019 08
PMID:Bilateral and symmetric postoperative cystoid macular edema in a patient with macular telangiectasia type 2 after uneventful subsequent phacoemulsification procedures. 3113 18