Cases reported "Cataract"

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1/8. Comparison of Scheimpflug images of posterior capsule opacification and histological findings in rabbits and humans.

    PURPOSE: To compare the posterior capsule opacification in Scheimpflug photographic images produced by an electronic anterior eye segment analysis system with the histopathological findings in rabbits and humans. SETTING: Department of ophthalmology, Wakayama Medical College, japan. methods: Opacified posterior capsules were photographed using the EAS-1000 system (Nidek) and were then extracted during vitreous surgery for proliferative diabetic retinopathy or proliferative vitreoretinopathy in 2 patients. In rabbits, phacoemulsification and aspiration (PEA) with intraocular lens (IOL) implantation was performed. The IOL was implanted in the bag or in the sulcus. After intervals of healing, the posterior capsule was photographed with the EAS-1000 and the animals were then killed. In both clinical and experimental specimens, the posterior capsule was processed for light microscopic histology and immunohistochemistry. RESULTS: Opacified human capsules were well imaged by the EAS-1000. histology showed that lens epithelial cells proliferated with and without an accumulation of extracellular matrix. Details such as rolling of the capsulotomy edge were seen well. Regenerated lens fibers of Soemmering's ring were seen as a mass within the capsule. In the rabbit model, Scheimpflug images accurately represented the capsules as they appeared histologically. CONCLUSION: The EAS-1000 system provided faithful, relatively high-resolution images that corresponded to the histologic findings in the posterior capsules after PEA-IOL surgery in humans and rabbits.
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2/8. Bilateral retinal phototoxic injury during cataract surgery in a child.

    The intense illumination of the operating microscope has been implicated in photic retinopathy in patients and in animal studies. We report a case of bilateral macular phototoxicity occurring in an eleven-year-old child who underwent bilateral cataract surgery for radiation cataracts. We are unaware of other reports of retinal toxicity occurring during pediatric cataract surgery. We hypothesize that this child may have been predisposed to macular injury because of previous chemotherapy and radiotherapy exposure. Ophthalmic surgeons should be aware that light toxicity from the operating microscope might also occur in the pediatric population.
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3/8. Identification of a novel, putative cataract-causing allele in CRYAA (G98R) in an Indian family.

    PURPOSE: The aim of the present study was to investigate the molecular basis underlying a nonsyndromic presenile autosomal dominant cataract in a three-generation pedigree. The phenotype was progressive from a peripheral ring-like opacity to a total cataract with advancing age from teenage to adulthood. The visual impairment started as problem in distant vision at the age of 16 years, to diminishing vision by the age of 24. methods: Clinical interventions included complete ophthalmological examination, a collection of case history, and pedigree details. Blood samples were collected from available family members irrespective of their clinical status. A functional candidate gene approach was employed for PCR screening and sequencing of the exons and their flanking regions of CRYGC, CRYGD, and CRYAA genes. For structural consequences of the mutated alphaA-crystallin we used the bioinformatics tool of the ExPASy server. RESULTS: sequence analysis of CRYGC and CRYGD genes excluded possible causative mutations but identified known polymorphisms. Sequencing of the exons of the CRYAA gene identified a sequence variation in exon 2 (292 G->A) with a substitution of Gly to Arg at position 98. All three affected members revealed this change but it was not observed in the unaffected father or sister. The putative mutation obliterated a restriction site for the enzyme BstDSI. The same was checked in controls representing the general population of the same ethnicity (n=30) and of randomly selected dna samples from ophthalmologically normal individuals from the population-based KORA S4 study (n=96). Moreover, the Gly at position 98 is highly conserved throughout the animal kingdom. For the mutant protein, the isoelectric point was raised from pH 5.77 to 5.96. Moreover, an extended alpha-helical structure is predicted in this region. CONCLUSIONS: The G98R mutation segregates only in affected family members and is not seen in representative controls. It represents very likely the fourth dominant cataract-causing allele in CRYAA. In all reported alleles the basic amino acid Arg is involved, suggesting the major importance of the net charge of the alphaA-crystallin for functional integrity in the lens.
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4/8. The calcium and magnesium content of the human lens and aqueous humour. A study in patients with hypocalcemic and senile cataract.

    Previous observations of raised calcium content in mature senile cataract are confirmed in the present study. Also human hypocalcemic cataract showed increased amounts of calcium in accordance with previous observations in animals. On the other hand, the magnesium content of the lens was not influenced by cataract development. The calcium aqueous/serum ratio was similar in all examined groups (normal, hypocalcemic-, incipient-, and mature senile cataract), whereas the corresponding magnesium ratio was elevated in the cataract patients. This observation may indicate a possible connection between magnesium metabolism and the development of cataract.
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5/8. Persistent behavioural blindness after early visual deprivation and active visual rehabilitation: a case report.

    Early long lasting binocular deprivation results in behavioural blindness in both man and experimental animals. However, few reported cases show that visual rehabilitation may improve visual behaviour. A 34-year-old man who had experienced 30 years of binocular deprivation due to bilateral cataracts received visual rehabilitation for one year. The rehabilitation included training in eye-hand co-ordination, recognition of objects, evaluation of distance and size, and mobility training. Despite signs of recovery of visual functions the patient never started to use vision in his normal life. The negative outcome of the rehabilitation is partly attributed to the patient's motivational problems and to the relatively short rehabilitation time. Visual rehabilitation may be successful when started immediately after the corrective operation on the eyes when the level of motivation is also high.
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6/8. Cochleosaccular degeneration of the inner ear and progressive cataracts inherited as an autosomal dominant trait.

    A new syndrome of cataracts and progressive sensorineural hearing loss, inherited as an autosomal dominant trait, is described. The histopathology of the inner ears in the proband demonstrated severe degeneration limited to the cochlea and saccule. This case represents the second reported example of genetically determined cochleosaccular degeneration in man. The cochleosaccular pattern of histopathology is found in both man and animals and may be the end result of prenatal dysplasia or postnatal degeneration. It also appears that cochleosaccular dysplasia and degeneration may be the result of genetically determined or acquired defects. Previously reported examples of cochleosaccular dysplasia and degeneration are reviewed including human cases in which the histopathology is similar to, but not characteristic of, the cochleosaccular pattern.
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7/8. Metabolic cataracts in newly diagnosed diabetes.

    The morphologically distinct diabetic or 'metabolic' cataract is rare in newly diagnosed insulin dependent diabetes. The cases described are of five adolescents (three girls, two boys) with newly diagnosed insulin dependent diabetes who developed metabolic cataracts close to the time of diagnosis (0-16 months). They all had a prolonged duration of symptoms before diagnosis (4-24 months) and high glycated haemoglobin levels at diagnosis (15-21%). The pathogenesis of diabetic cataract is not well understood in humans. An attempt is made to link clinical observations with evidence from experimental animal models to understand the mechanism of cataract formation, with particular reference to the aldose reductase pathway. It is recommended that the lens and retina are examined at the onset of diabetes in all children, especially those who have a prolonged duration of symptoms before diagnosis and who report persistent blurred vision.
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8/8. The diabetic cataract: an unusual presentation in a young subject: case report.

    This case report concerns a 14-year-old female patient, whose insulin-dependent diabetes mellitus was displayed by one infrequent complication, the cataract. This is an unusual manifestation in a 14-year-old patient; indeed, there are many findings in experimental animals demonstrating the development of this complication by maintaining blood glucose levels above 12 mM. After surgical therapy, complete vision was recovered, but we think that an earlier diagnosis and therapy of metabolic derangement of diabetes may have avoided this complication.
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