Persistent hyperplastic primary vitreous associated with protein C deficiency.
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Biomedical subjects
Publications and source records attributed to V M Hermsen.
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The proliferation and fibrous metaplasia of retinal glial and pigment epithelial cells cause proliferative vitreoretinopathy. The immunotoxin 454A12 MAB-rRA is composed of a murine monoclonal antibody, specific for the human transferrin receptor, and is chemically linked to recombinant ricin A chain, a cellular toxin. The rapidly proliferating cells take up the immunotoxin, but non-proliferating cells do not. Using a collagen-gel medium to simulate the vitreous, we have studied the effect of the immunotoxin on fibroblast proliferation in vitro. Exposure of the fibroblasts to 1000 ng of immunotoxin per milliliter of the collagen gel medium for 10 minutes kills 96% or more of the cells for 20 days. These in vitro data indicate that the immunotoxin is effective in an environment similar to the vitreous; however, in vivo studies will be necessary to prove if it is a suitable agent for the long-term prevention of cell proliferation in the human eye.
Photoradiation therapy (PRT) is a new technique that is currently under investigation for the treatment of a variety of solid malignant tumors. The technique involves systemic administration of hematoporphyrin derivative (HpD), a photosensitizing compound that is preferentially retained by malignant cells and photoactivation of the neoplasm with red light (630 nm) to achieve selective destruction of cancer cells. We used HpD PRT for seven patients with malignant melanoma of the uvea and conclude that initial results of HpD PRT for uveal malignant melanoma are encouraging and justify further investigation.
7 affected and 4 normal individuals from three consecutive generations of a family with central areolar pigment epithelial dystrophy were examined. The pedigree was indicative of an autosomal dominant disorder with variable expression. The macular changes varied from numerous small drusen-like defects to large colobomatous appearing lesions while the visual acuities ranged from 6/4 to 3/120. Some of those with excellent visual acuity had small central scotomata. Urinary metabolic screening, plasma amino acid levels and 24-hour urinary amino acid levels were normal.
Iris fluorescein angiography was used to perform the first prospective, controlled, masked evaluation of the effect of panretinal photocoagulation on diabetic rubeosis. Eyes with rubeosis had iris fluorescein angiograms five to seven weeks apart. Eyes either had laser treatment immediately after the first angiogram or had no laser treatment between angiograms. The rubeosis was said to have improved if all three masked readers independently selected the angiogram performed last as having the less severe rubeosis. In eyes with severe rubeosis, only two of 11 (18%) spontaneously improved whereas 11 of 15 (73%) improved following laser therapy. In eyes with mild rubeosis, one of ten improved spontaneously and one of ten improved following laser therapy. Iris fluorescein angiography documented regression of severe but not mild rubeosis following panretinal photocoagulation.
A brief review of the anatomy of the eye, the eyelid, and the lacrimal system provides reference material for the articles that follow in this issue. Pathologic changes in the normal anatomy that can help the physician diagnose an ophthalmic or systemic problem are highlighted.