Corneal superficial unwettable defect. Clinical and histopathological study.
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Biomedical subjects
Publications and source records attributed to F Kloucek.
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We examined the peripheral blood chromosomes of eight patients with retinoblastoma. In two of them an interstitial deletion of 13q was found. The breakpoints were determined as follows: case 1, 13q12 leads to 21; case 2 13q12 leads to 31. In both cases, band 13q14 was deleted. In case 2 the lymphocytes of the mother showed the identical interstitial 13q deletion in 3 of 100 mitoses, thus raising the possibility of maternal origin of the 13q deletion in a child. In one patient, retinoblastoma was unilateral; in the other, bilateral. Both patients were mentally retarded.
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Presumptive primary band-shaped keratopathy was described in a 35-year-old man and his 62-year-old paternal uncle. Lamellar keratoplasty was performed in one eye in each of these patients. The light and electron microscopic studies were carried out on both corneal specimens. Histologic stains for calcium were negative. Noncalcific band-shaped keratopathy was confirmed by electron microscopic findings too. Electron-dense globular deposits were found in the region of Bowman's membrane and superficial stroma. These may be characterized as a secondary form of spheroidal degeneration.
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Corneal biopsy specimens of 6 patients with this particular affection were studies with the electron microscope. In addition to the routine electron microscopic methods, staining with ruthenium red was performed on one excised specimen. Marked changes were found in the region of Bowman's membrane, which was altered either superficially or in its total width. Its collagen fibrils became rough and intensively electron-dense, forming a granular or structureless trabecular meshwork. Intertrabecular spaces were filled with special electron-lucent material, which also formed a continuous layer on the surface of the altered Bowman's membrane. Filamentous structures resembling fingerprints were observed in certain areas of these spaces; and after staining with ruthenium red, numerob granules were also visible. Both these structures are considered by the author to be acid mucopolysaccharides. There is further discussed on the correlation of these findings with previous histochemical findings; the author gives special attention to the question whether waxy substances-eye ointment bases-could have been deposited in the pathologically altered Bowman's membrane.
In 11 patients, the clinical, histological and histochemical picture of an unusual corneal affection termed "corneal superficial unwettable defect", is reported. It develops during the healing of various corneal lesions. Clinically, it is described as a defect of the epithelium with a denuded and changed Bowman's membrane, the surface of which repells tears and cannot be stained with fluorescein. In the histological picture, the changed Bowman's membrane has fibrillar or foamy appearence. It stains strongly with colloidal iron and alcian blue, but PAS and toluidine blue reactions are weak. Histochemical reactions for neutral fats are strongly positive, but those for phospholipids are negative. The results of extractions speak for the presence of some lipid component, which is neither neutral fat nor phospholipid. Hence it is supposed that there may be some waxes-ointment bases, reacting with the pathologically changed Bowman's membrane. The possible mechanisms injuring or influencing the Bowman's membrane are also discussed.
The essence of the process in which the corneal tissue disappears in atrophy of the eye has not yet been studied. The authors of this investigation studied the changes in cornea as they developed after a gross perforating injury to the eye in rabbit. They found that the diameter of the cornea had grown smaller by 2 mm and its radius by 0.5 mm. The tensile strength of the cornea had decreased and hydration increased. Histologically there were but little changes and histochemically a decreased amount of acid mucopolysaccharides was ascertained in the corneal stroma. Incorporation of radioactive sulphate (Na2-35SO4) in vivo disclosed decreased biosynthesis of glycosaminoglycans. Clinically, even though the eye ball atrophied, complete atrophy with flattening and complete diminution, as in the human eye, never developed. The question arises whether the six-months period was sufficient for rabbit cornea to develop clinically similar atrophy as in human cornea or whether the clinical features are principally different in the two species. Foci of ossification, as found in atrophic eye balls, which are known to develop after a long time, would be evidence of the second alternative.
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