[Heredity in frosted degeneration. Preliminary results].
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Biomedical subjects
Publications and source records attributed to P Bec.
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Three cases of ocular candidosis involving heroin abusers have been observed in 1983 in Toulouse department of ophthalmology. These three patients had used iranian brown heroin. Twenty similar cases have been published in these last years. This new pathology can be explained on two reasons. The first is that the drug abusers have some immunity pertubation; however, immunity exploration in these patients does not reveal any immunodeficiency. The second reason, certainly more important, is the method of using heroin. The diagnosis of Candida endophthalmitis of course based on clinical context must be proved by biological tests. Candida albicans is never identified in aqueous humor. For this reason, it seems very interesting to detect anti-candida antibodies in aqueous humor. It has been used as methods of dosage laser Nephelemetry for IgG and immunofluorescence for candidosis antibodies. The criterion used is similar to the toxoplasmosis coefficient established by Desmonts (3). In two cases, this test was the only way that permits us to have certitude of candidosis ocular diagnosis. Otherwise the observations show that anterior chamber punction is more significant when there is an anterior uveitis.
The ultrastructural study of the vitreoretinal juncture was carried out by comparing two groups of patients: 6 young subjects under 25 years and 6 older subjects over 70. Retinal samples were taken from the posterior pole, the equator and the extreme periphery. Our work shows that the morphological modifications of the vitreoretinal juncture during the aging process vary following location. The most original alteration, never observed before, appears in the equatorial region: it consists of a widening of the intercell space filled with fibrils adhering to Müller's cells. It builds up an adhesion zone between the vitreous and the retina which is not visible during clinical investigations; it can create a tear during the posterior vitreous detachment.
The ultrastructural study of a case of snail track degeneration shows the presence of lipid inclusions in both the glial and the macrophage cells in every layer of the retina, and the existence of intraretinal fibers different from collagen fibers appearing to be glial filaments similar to those found in astrocytic gliomes and to the Rosenthal fibers observed in senile nervous cells. Other features were thinning of the retina and absence of blood vessels in the retina. There are no abnormalities of the vitreo-retinal juncture. All the lesions are in agreement with those observed by Daicker [Ophthalmologica, Basel 165: 360-365, 1972; Klin. Mbl. Augenheilk. 172: 581-583, 1978] with some differences, however. They are different from those found in lattice degeneration. They show that snail track degeneration is a specific form of peripheral retinal degeneration which is quite different from lattice degeneration and must not be considered similar.
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The Octopus automatic perimeter was used to determine changes in visual field of 90 patients with chronic open angle glaucoma. Mean sensitivity value calculated from 62 of the 73 points of the apparatus program 33 - after elimination of those 11 points corresponding to Mariottes's zone and the central 10 degrees - provides an effective paracentral visual field monitoring numerical parameter. The authors named the parameter "global value". Statistical analysis of "global value" variations between two successive examinations showed: significant improvement between the first and the second tests; disappearance of the phenomenon between two further successive tests. The reasons for this pseudo-improvement remain unclear: perhaps the patients experience gained from the precious tests plays a part in the phenomenon. This should in any case be taken into account when assessing possible aggravation between two tests. Analysis of results suggests that, as a standard evaluation method, any "global value" degradation of the order of 1 db between the first two tests and 3 db between two successive tests performed after the second one is significant of a visual field degradation with a 1% range of error.
Argon laser trabeculoplasty (or trabeculoretraction) was used following WISE's technique in 100 cases of open angle glaucoma. The patients, all of different ages, presented with various forms of open angle glaucoma. In some cases, the intraocular pressure had not been normalized by the medical treatment, and in some others, the patients showed drug intolerance. We eliminated the patients whose angle was too narrow to allow photocoagulation. They were examined at regular intervals of 1 week, 2 weeks, 1 month, 3 months, 6 months and 12 months after therapy. Pressure figures only were considered in the therapeutic follow-up. After 12 months, the mean pressure drop was 7.3 mmHg after 180 degrees trabeculoplasty and 11.43 mmHg after 360 degrees trabeculoplasty, the latter consequently entailing a more considerable drop. Older subjects however showed a greater pressure decrease. We obtained less satisfactory results in cases of exfoliative syndrome glaucoma and pigmentary glaucoma. The important point is that pressure normalization was obtained--in most cases--only if the prior medical treatment was maintained either without any change or lightly reduced. The trabeculoplasty alone reduced ocular pressure in only 3.57% and in 9.09% respectively after 180 degrees and 360 degrees trabeculoplasty. We can therefore conclude from these observations that this technique is only an auxiliary method in open angle glaucomas that do not sufficiently respond to medical treatment.
Optic nerve head drusen are rare, inherited concretions, which are almost always calcified; their appearance must be known because they represent one of the major causes of pseudopapilledema. The computed tomographic (CT) scan can show small and buried drusen, which are sometimes difficult to diagnose by the ophthalmoscopic examination. Four cases of drusen (two bilateral and two unilateral) were confirmed or diagnosed by high-resolution CT. The CT appearance of drusen is characteristic because the calcifications are well defined, punctate, and strictly located in the optic disc. The use of high-resolution CT scanners is very helpful.
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A case of retinitis pigmentosa associated with astrocytic hamartomas of the optic disc is described. The rarity of tuberous sclerosis in this association is discussed, as well as differences between optic nerve head drusen and astrocytic hamartomas ('giant drusen') which can both be associated with retinitis pigmentosa.
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Eight patients with cystine stones (7 adults, 1 children) are treated by D-penicillamine since at least 5 years and at the most 9 years. The goal of the treatment is to obtain free cystinuria concentration less than 200 mg/l by the conjunction of a high urine flow and D-penicillamine. Cystinuria is measured by automatic analyser chromatography. In 2 patients, lithiasis got worse and cystinuria remained highly pathologic because bad compliance. In the 6 other cases, no lithiasis recurrence was noted; 5 patients had renal unilateral or bilateral lithiasis before treatment. A partial stone dissolution was obtained in 3 patients and total stone dissolution in 1 patient. Despite important posology often used, D-penicillamine has been remarkably well tolered since the only side effect observed was erythrodermia in 1 case at the beginning of the treatment.
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A vitrectomy was performed in a diabetic patient because of unilateral vitreous hemorrhage, which persisted for six months. There was only background retinopathy in the other eye. In the operated eye, no proliferative retinopathy was found. During surgery a partial posterior vitreous detachment was noted with persistent vitreoretinal attachment to the superotemporal vascular arcade. Postoperative fundus examination and fluorescein angiography showed background retinopathy and the presence of a venous loop involving the superotemporal vein. Histologic description of venous loops with vitreoretinal adhesion was recently reported and it was suggested that this could lead to vitreous hemorrhage even in absence of severe proliferative retinopathy. To our knowledge, there is no previous report of diabetic vitreous hemorrhage probably due to a venous loop with vitreoretinal attachment.