Cases reported "Retinitis"

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1/16. retinitis pigmentosa, Coats disease and uveitis.

    PURPOSE: To study the anamnestic immune response to retinal specific antigens of two patients suffering from a rare triad of retinitis pigmentosa, Coats disease and uveitis. patients: 17-year-old girl presented with an acute episode of panuveitis, and her 19-year-old brother suffered from chronic uveitis. On examination, both patients showed retinal vascular changes and subretinal exudations typical of Coats disease, with bone-spicule pigmentary changes as observed in retinitis pigmentosa. RESULTS: All routine examinations were unrevealing. However, the peripheral lymphocytes from these two siblings gave a specific anamnestic response to retinal antigens in vitro. A stimulation index of 4.6 was obtained when the sister's lymphocytes were stimulated with interphotoreceptor binding protein, IRBP--during the acute stage of the uveitis. The brother's lymphocytes showed a stimulation index of 2.7 towards S-Ag during the chronic phase of his uveitic condition. CONCLUSIONS: These results indicate that autoimmunity towards retinal antigens may play some role in specific types of retinitis pigmentosa. Whether these autoimmune reactions are a primary pathological mechanism or are secondary to the extensive destruction of the photoreceptor layer resulting from the retinitis pigmentosa remains debatable.
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2/16. Atypical healing of cytomegalovirus retinitis. Significance of persistent border opacification.

    PURPOSE: To analyze a phenomenon seen in patients with acquired immune deficiency syndrome (AIDS) with cytomegalovirus (CMV) retinitis undergoing systemic antiviral treatment: a persistent white border opacification on the edge of healed CMV retinitis. patients AND methods: The authors prospectively evaluated a population of 137 patients with AIDS and CMV retinitis during a 44-month period. Eleven patients (12 eyes) who were undergoing maintenance antiviral treatment were identified with an atypical healing response--the persistence of a white flat border opacification that did not advance for many weeks to months. Patient records and photographs were reviewed. Results of one autopsy were analyzed with histopathology and special stains. RESULTS: The persistent white edge maintained (without advancement or smoldering) for an average of 11.6 weeks (range, 4 to 41 weeks). This border opacification was not affected by reinduction treatment in the six patients to whom reinduction was given. Results from histopathologic examination of one patient with a persistent white border are presented: these results show that dead cytomegalic cells formed stable structures within the retina, causing white opacification that could be confused with active lesions. Immunoperoxidase stains identified CMV antigens. CONCLUSION: This persistent white border opacification, which does not advance or smolder, represents an important clinical entity that should be recognized during antiviral treatment for CMV retinitis. It can often be observed. If it is not recognized as a stable configuration, patients may undergo unnecessary reinductions with potentially toxic doses of antiviral medications.
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3/16. Concurrent uveoretinitis and pineocytoma in a child suggests a causal relationship.

    Uveoretinitis was observed in a 9-year-old girl 6 months prior to the clinical appearance of a pineal tumour. Surgical removal was not successful but biopsy revealed a parenchymal neoplasm with differentiated pinealocytes and absent mitotic activity. Some of the tumour cells contained S-antigen, rhodopsin, and serotonin. Systemic glucocorticoid therapy followed by radiation therapy caused considerable reduction in size of the tumour and a complete normalisation of all eye symptoms. This report demonstrates for the first time that a pineocytoma can occur together with uveoretinitis in humans. The latter resembles the experimentally induced autoimmune uveoretinitis described in animals. It is speculated that the retinitis might reflect an autoimmune response to S-antigen present in some tumour cells of the pineocytoma.
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4/16. Protein-losing gastroenteropathy and retinitis associated with cytomegalovirus infection in an immunocompetent infant: a case report.

    A 6-week-old immunocompetent girl developed protein-losing gastroenteropathy (PLGE) and retinitis associated with cytomegalovirus (CMV) infection. At presentation, CMV antigenaemia (6 cells/46,000 white blood cells) and its dna were detected in the patient's blood and in the mother's milk. Intravenous ganciclovir and gamma-globulin rapidly ameliorated all symptoms and CMV antigenaemia disappeared. No immunological defects were identified in this patient. To the best of our knowledge, this case involves the youngest known immunocompetent patient demonstrating CMV-induced PLGE and retinitis. CONCLUSION: breast-feeding by a cytomegalovirus-positive mother can be a primary cause of early onset cytomegalovirus infection in infants.
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5/16. Varicella-zoster virus retinitis in patients with the acquired immunodeficiency syndrome.

    We examined five patients infected with the human immunodeficiency virus who developed a rapidly progressive necrotizing retinitis characterized by early patchy choroidal and deep retinal lesions and late diffuse thickening of the retina. In all but one case, the retinitis began in the posterior pole with little or no clinical evidence of vasculitis. All five patients had relentless progression of disease and were left with atrophic and necrotic retinae, pale optic-nerve heads, and narrowed vasculature. None of the patients developed aqueous or vitreal inflammation or retinal detachment. Clinical and laboratory evidence suggested that varicella-zoster virus was the causal agent in all five cases. First, the onset of retinitis in four cases either succeeded or was coincident with an eruption of dermatomal zoster. Second, varicella-zoster virus was cultured from the two chorioretinal specimens and varicella-zoster virus antigen was detected in the vitreal aspirate from one case. Third, by means of immunocytochemistry, varicella-zoster virus antigen was found in the outer retinae of both enucleation specimens. Fourth, viral capsids with the size and shape of herpesviridae were found in the outer retinae of both enucleation specimens. The clinical features observed in this study are distinct from those described for the acute retinal necrosis syndrome and appear to constitute a new and highly characteristic pattern of varicella-zoster virus-induced disease.
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6/16. Neuroretinitis in cat scratch disease.

    We report a patient with neuroretinitis, whose associated lymphadenopathy, exposure to cats, and strongly positive cat scratch antigen skin tests suggested the diagnosis of cat scratch disease. Cat scratch disease should be added to the list of infectious agents believed to produce the clinical picture of neuroretinitis.
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7/16. Dual infection of retina with human immunodeficiency virus type 1 and cytomegalovirus.

    We examined retinal tissue from eight human immunodeficiency virus type 1 (hiv-1) seropositive patients with acquired immunodeficiency syndrome (AIDS) or aids-related complex for evidence of dual infection with hiv-1 and cytomegalovirus. culture demonstrated simultaneous infection with hiv-1 and cytomegalovirus in two of 13 retinal specimens. This was confirmed by both immunofluorescence and immunohistochemical staining. Moreover, coinfection of individual cells with cytomegalovirus and hiv-1 was observed by immunohistochemical staining. Infection of retina with cytomegalovirus or hiv-1 alone occurred in one and six of the 13 retinal specimens, respectively. hiv-1 antigens were present on scattered cells in all layers of the retina and on retinal vascular endothelium. hiv-1 was isolated from retinal tissue derived from eyes both with and without gross ocular lesions. Cytomegalovirus antigens were found in all layers of the retina, but not on vascular endothelial cells. The atypically rapid clinical progression of retinitis in one of the patients with dual hiv-1 and cytomegalovirus infection suggests the possibility that interactions between these two viruses may influence retinal disease in patients with AIDS.
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8/16. Toxoplasmic retinochoroiditis: electron-microscopic and immunofluorescence studies of formalin-fixed tissue.

    An 82-year-old white man had bilateral, chronic recurrent uveitis caused by toxoplasma gondii. In spite of extensive therapy for toxoplasmosis, the left eye became painful and blind and was enucleated. Histopathologically, the retina disclosed unusually large numbers of necrotic cysts and a few presumably viable cysts containing crescent-shaped organisms and scanty free forms of T gondii. Electron-microscopic studies confirmed the presence of a true wall of the cysts as well as additional ultrastructural features highly characteristic of the parasite. Immunofluorescent studies of the formalin-fixed tissue demonstrated quite vividly the cysts, which stained variably according to the stage of viability of the organisms. In cases in which toxoplasmic retinochoroiditis has been suspected clinically but routine histologic preparations fail to reveal the organisms, the remaining formalin-fixed tissue should be examined by specific immunofluorescent methods to demonstrate antigenic material of the causative parasite.
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9/16. Immunological studies of uveitis. 3. Cell-mediated immunity to interphotoreceptor retinoid-binding protein.

    Three patients were seen with the same clinical characteristics which were not described previously: diffuse uveitis with bilateral involvement, onset in the second decade of life, no extraocular signs or symptoms, diffuse retinal capillary leakage on fluorescein angiography but without apparent fundus changes, many vitreous cells but no snowbank-like vitreous opacity, responsiveness to steroid, and good prognosis. The peripheral blood lymphocytes of all three patients responded to bovine and human interphotoreceptor retinoid-binding protein (IRBP), but not to bovine retinal S antigen. It was speculated that these patients were human counterparts of IRBP-induced uveoretinitis that was described in experimental animals.
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10/16. candida retinitis in bare lymphocyte syndrome.

    Bare lymphocyte syndrome (BLS) is a rare, severe combined immunodeficiency characterized by lack of expression of HLA A, B and C antigens and the absence of B2 microglobulins. patients with BLS exhibit functional deficiency of both T and B cells resulting in bacterial as well as viral and fungal infection. Ophthalmic findings in this group of disorders have not been reported. We present a case of candida retinitis in a terminally ill 5-year-old girl with BLS.
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