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Biomedical subjects

R S Mehta

Publications and source records attributed to R S Mehta.

At least 37 records · Page 2Linked to original sources
PubMed1981

Zellweger syndrome. Lenticular opacities indicating carrier status and lens abnormalities characteristic of homozygotes.

Cataracts were found in four patients with pathologically confirmed Zellweger syndrome. By careful slitlamp examination with the pupil completely dilated, there is a denser cortex that produces a cortical-nuclear interface. These opacities have ultrastructural analogues, which are inclusion bodies restricted to the cortical lens fibers. The lens epithelium shows abnormal mitochondrial proliferation that is age dependent. The parents of these four infants with Zellweger syndrome have lenticular opacities that are seen only biomicroscopically after maximal pupillary dilation. These changes consist of curvilinear condensations in the cortical region corresponding to the locus of the cataractous changes in the homozygous state. In the clinical setting of an infant who is failing to thrive, has the Zellweger facies, and demonstrates an absent electroretinogram, these heterozygote lens opacities are useful in making the diagnosis of Zellweger syndrome before pathologic substantiation.

Cataract↗
PubMed1981

Ocular manifestations of the Smith-Lemli-Opitz syndrome.

To our knowledge, this article describes the first ocular histopathologic condition of a Smith-Lemli-Opitz proband, despite almost 60 clinical histories that exist in the literature. The sole retinal abnormality in this 1-month-old infant with congenital bilateral cataracts is the extensive dropout of peripheral ganglion axons with incipient optic nerve demyelination. Unusual amorphous cytoplasmic masses that are continuous with photoreceptor discs are prominent aspects of the peripheral subretinal space. The morphological data imply that the localized mitochondrial disintegration is restricted to the corneal endothelium and retinal pigment epithelium and is an important element in the etiology. All children who fail to thrive with vomiting, are mentally deficient, have anteverted nostrils, broad maxillary alveolar ridges, syndactyly of the second and third toes, and ambiguous genitalia should be carefully screened for incipient corneal endothelial changes, mild cataracts, and peripheral retinal changes.

Abnormalities, Multiple↗
PubMedDate not supplied

Vitamin E protects against retinopathy of prematurity through action on spindle cells.

In the premature infant, exposure of the incompletely vascularized retina to increased oxygen tension can result in the development of a blinding disease, retinopathy of prematurity (ROP). Despite the judicious curtailment of oxygen, the incidence of ROP is on the increase due to the technological advances that have improved the survival of the very young preterm infant. Six clinical trials have documented the efficacy of vitamin E supplementation in suppressing the development of severe ROP, but the mechanism of this protection has remained unknown. This report proposes that spindle cells, mesenchymal precursors of the inner retinal capillaries, are the primary inducers of the neovascularization associated with ROP. Exposure of spindle cells to elevated oxygen tension increases their gap junction area. This early morphologic event immediately halts the normal vasoformative process and eventually triggers the neovascularization that is observed clinically 8-12 weeks later. Vitamin E supplementation above the deficient plasma levels of these infants suppresses gap junction formation and clinically reduces the severity without altering the total incidence of ROP.

Humans↗
PubMedDate not supplied

Irregular astigmatism induced by annular tinted contact lenses.

Three patients developed irregular corneal astigmatism while wearing annular tinted soft contact lenses on a daily basis for 1.5 to 3 years. There was severe keratometer mire distortion, and photokeratoscopy revealed central and midperipheral corneal topographical irregularities in four of six eyes. In a masked protocol, scanning electron microscopy of four contact lenses revealed physical deformations in three lenses worn on affected eyes. We propose that latent stress vectors were created when the affected contact lenses were tinted. With patient usage, the stress vectors matured into physical deformations that induced irregular astigmatism. The astigmatism resolved upon discontinuing wear of these lenses, and the patients were able to wear other lenses with no recurrence of symptoms.

Adult↗
PubMedDate not supplied

Behcet's disease.

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Adult↗