Temporal bone involvement in polyostotic fibrous dysplasia.
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Biomedical subjects
Publications and source records attributed to D Berson.
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There is considerable evidence which supports a link between abnormalities in copper metabolism and some pigmentary retinopathies. In a previous study, we found such abnormalities in high myopic Caucasian patients. Since the incidence of myopia, and the connection of the latter with retinal detachment, in the black are different from those found in the Caucasian, we modeled the present study on the black after the above-mentioned one carried out on the Caucasian. In the present study no abnormalities in copper metabolism were present, in the black high myopic patient.
Mandibulofacial dysostosis is a syndrome caused by delay in the development of the first visceral arch. This report describes a case which had fusion of the gums and temporomandibular joint, together with coloboma of both lids. This variant has not previously been reported.
Marfanoid hypermobility syndrome together with Duane's retraction syndrome in two sisters is presented. To our knowledge, this is the first report of such a combination. A possible mode of inheritance is suggested.
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A case of total eversion of both upper eyelids of late onset is reported. There were no associated ocular or general anomalies. No apparent cause was found. The treatment was directed toward diminishing the swelling of the lids and preventing corneal involvement, secondary infections, and epidermalization of the conjunctiva. Within the first week, the eyelids returned to their normal position. In a three-month follow-up period, no complication or other anomalies were found.
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A 20-year-old Coloured woman presented on three occasions with recurrent right-sided spontaneous pneumothorax which proved to be associated with pulmonary histiocytosis X. After corticosteroid therapy there was no recurrence of pneumothorax or radiographic evidence of progressive disease during a 6-month follow-up period.
Of 70 eyes with therapeutic peripheral iridectomy (PI), 51% suffered a loss of two or more lines on the Snellen chart; 57% developed posterior synechiae (PS) and 57% showed lens opacities. Thirty-three per cent of eyes that underwent PI prophylactically had a vision drop of two lines or more, 39% developed PS and 42% showed varying degrees of lens opacities. Although vision drop, lens opacities and PS were less marked in the prophylactic group, it appears that PI is a surgical procedure not without its hazards. We therefore suggest that peripheral iridectomy should not be performed routinely on the second eye not suffering an acute attack. This procedure should be undertaken only in cases with positive provocative tests and/or clinical signs of closed angle glaucoma.
A brief description is given of the main clinical, histological and epidemiological circumstances of retinal vein occlusion. In an assessment of the pathogeneis of the condition, primacy is given to the role of arteriolar flow insufficiency and stress is laid on the long-standing capillaropathy which precedes the heamorrhagic phase of the disease. Basic therapeutic considerations are mentioned but stress is laid on the diagnosis of the pre-occlusive stage and on measures of a preventive nature that may be taken. Retinal vein occlusion and haemorrhagic cerebral infarct are compared with regard to their pathogenesis and the advantages are discussed of a common ophthalmoscopic study of retinal vein occlusion with neurologists interested in cerebral vascular disease leading to an interchange of therapeutic experience in both conditions. Finally, there are detailed the studies in retinal vein occlusion being conducted by the Jerusalem Institute for the Prevention of Blindness. These are studies of incidence, natural history and of pathogenesis as observed in the experimental disease produced in rhesus monkey.
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