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)

This is a clinicopathologic study of 62 cases of persistent hyperplastic primary vitreous (PHPV). The cases were divided into two main groups. Group 1 consisted of 55 unilateral cases not associated with any systemic abnormalities, including 36 eyes (58%) which were considered "pure cases" (Group 1A) and 19 (31%) which disclosed other ocular abnormalities in addition to PHPV (Group 1B). Group 2 consisted of 7 (11%) bilateral cases of PHPV accompanied by other ocular and systemic malformations. The most common presenting clinical signs are leukocoria, microphthalmia and cataract. The main histopathologic features of this condition are outlined, including those responsible for the disastrous results to the eye (retinal detachment, glaucoma, phthisis bulbi). Several clinical entities, usually mistaken for or associated with PHPV, such as retinoblastoma, congenital cataract, retinal dysplasia, trisomy 13 syndrome, and falciform retinal folds are discussed briefly.
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PMID:Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature. 10 Aug 93

The clinical and pathological details of a case of reno-facial dysplasia are presented and described. Hyperplasia and lamination of Bowman's membrane, corneal endothelial dystrophy with degenerative changes in the posterior stroma, hypoplasia of the retinal ganglion cell and nerve fibre layers, and severe changes in the retinal and iris blood vessels are believed to be pathognomonic ocular features of this syndrome, in addition to cataract and signs of immaturity. These findings are compared with the few descriptions of the syndrome previously published. The corneal damage is postulated to result indirectly from oligohydramnios which causes chronic compression of the globes and thus endothelial damage due to contact with the spherical cataracts. The pathological changes in the retinal and iris vessels are either genetically determined or the result of chronic hypoxia.
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PMID:[Ocular findings in reno-facial dysplasia (Potter-syndrome) (author's transl)]. 10 56

A 1,350 gm female infant with features of the Potter syndrome (dysplasia renofacialis) had multiple ocular anomalies. Absence of keratocytes in the central corneal stroma, cataract, retinal ganglion cell and nerve fiber hypoplasia, loss of optic nerve bundles, and angiomatoid proliferation in the area of the optic disc are most striking. Some of these findings, especially in the cornea, may reflect mesoectodermal dysgenesis. Abnormal ocular angiogenesis may well comprise a part of Potter's syndrome.
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PMID:Multiple congenital ocular anomalies with bilateral agenesis of the urinary tract. 11 86

Thirty-eight newborn Beagle puppies from eight litters of a specific pathogen-free colony maintained in isolation were inoculated with canine herpesvirus. Pups were killed between one and 30 days after inoculation. Histopathologic studies were carried out on the eyes and other tissues in conjunction with fluorescent antibody and viral isolation studies. Evidence of ocular inflammation manifested by panuveitis with the presence of intranuclear inclusion bodies was usually seen by the fourth day after infection. Eyes with severe inflammation showed peripheral anterior synechiae, cataract, and keratitis. The presence of the virus was confirmed by viral isolation from ocular tissues and fluorescent antibody studies. Developmental anomalies included retinal dysplasia with fold and tube formation of the neural retina, retardation of retinal maturation, and areas of necrosis and reorganization were seen. The retinal pigment epithelium showed initially patchy depigmentation and vacuolization and, subsequently, folding hypertrophy and duplication as well as areas of widespread atrophy and patchy loss. In some animals ectopic retina was found within cystic spaces of the optic nerve. These experiments confirm the ability of canine herpes infection in neonatal pups to produce severe ocular inflammation with subsequent retinal dysplasia and associated ocular anomalies.
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PMID:Canine herpes-induced retinal dysplasia and associated ocular anomalies. 17 83

Eyes from cardiomyopathic hamsters (UM-X7.1 strain) were examined histologically for evidence of ocular defects. Changes observed included microphthalmia, scleral ectasia, scleral rupture, keratoconus, retinal detachment, retinal dysplasia, retinal fragmentation, retinal thinning, fibrosis of iris and ciliary body, ectopia lentis, and cataract formation. Lesions characteristic of cardiomyopathic hamsters were observed in the myocardium and skeletal muscle. This strain may be a suitable animal model to study the pathogenesis of ocular changes seen in certain congenital connective tissue disorders in man.
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PMID:Ocular abnormalities in the myopathic hamster (UM-X7.1 strain). 41 Jul 51

We studied the abnormal ocular and systemic findings in one case of true triploidy and two cases of triploid mosaicism. A liveborn triploid child 69,XXY, had abnormalities including cebocephaly, a single midline nostril, incomplete cleft palate, transverse palmar creases, partial syndactyly, and ambiguous genitalia. Ocular abnormalities included hypotelorism, blepharophimosis, microcornia, iris coloboma, cataract, persistent hyaloid vasculature, retinal dysplasia, and optic atrophy. A 16-year-old girl with triploid mosaicism had congenital left facial and body hemiatrophy, both growth and mental retardation, left-sided grand mal seizures, incontinentia pigmenti of both legs, partial syndactyly, and generalized weakness. Results of her ocular examination were within normal limits. A 13-year-old boy with triploid mosaicism exhibited both growth and mental retardation, truncal obesity, and required a brace to support his back. Ocular findings included synophrys, bilateral blepharoptosis, and abnormal results of Schirmer tear test. Studies indicate a wide spectrum of ocular and systemic abnormalities occur that are presumably associated with the chromosome error.
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PMID:Ocular findings in triploidy. 41 37

Severe ocular malformations, including microphthalmos, intraocular cartilage, cataract, persistent hyperplastic primary vitreous, and retinal dysplasia, occurred in a premature baby girl. The mother had ingested LSD during the first trimester of pregnancy. To our knowledge, this is the third case reported of ocular teratogenesis associated with maternal LSD ingestion. Further cases must be documented to establish an actual cause and effect relationship between the drug and the induced malformations.
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PMID:Multiple ocular anomalies associated with maternal LSD ingestion. 41 6

The clinical picture of the Kniest's syndrome is described. The syndrome is a rare hereditary condition with generalized bone dysplasia, disproportional dwarfism, conduction deafness and severe myopia, retinal detachment, cataract and amaurosis.
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PMID:[Kniest's syndrome (author's transl)]. 60 Feb 74

The clinical, radiographic, and pathologic features of skeletal and ocular dysplasia in 6 Labrador Retrievers were studied. Abnormalities in the appendicular skeleton included retarded growth in the radius, ulna, and tibia; ununited and hypoplastic anconeal and coronoid processes; hip dysplasia, and delayed development of epiphyses. Ocular changes were characterized by retinal dysplasia, with retinal detachment and cataract formation. Dogs from 4 litters were affected.
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PMID:Retinal dysplasia associated with skeletal abnormalities in Labrador Retrievers. 83 Jun 31

Several lines of transgenic mice carrying a transgene construct consisting of the regulatory enhancer element of the Moloney murine sarcoma virus and the Simian virus 40 genome coding for the SV 40 promoter and the large T antigen were established. We describe several abnormalities found in the eyes of transgenic animals of which heritable cataract formation, probably due to disturbances in primary lens fibre differentiation, showed a close correlation to large T antigen expression. Additionally, lenticonus anterior, retinal dysplasia and one case of malignant transformation of lens epithelium were found. The introduction of the deleted MSV-enhancer linked to the large T coding region led to less severe postnatally occurring cataracts. Thus, the partial deletion of the MSV enhancer resulted in differences in the degree of severity of lens disturbances. However, tissue specificity remained constant. Our results indicate that large T antigen seems to play an important role in cataract formation but not in the pathogenesis of retinal dysplasia.
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PMID:Eye pathology in transgenic mice carrying a MSV-SV 40 large T-construct. 165 Dec 51


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