Cases reported "Eye Diseases"

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1/53. A case of juvenile myelomonocytic leukemia with ocular infiltration.

    We present a case of juvenile myelomonocytic leukemia (JMML) with ocular infiltration. A 1-month-old boy presented with myeloid precursors in peripheral blood and a white blood cell count >10x10(9)/l. His peripheral blood monocyte count was >1x10(9)/l, bone marrow blasts were <20%, and no Ph chromosome was identified. The boy also presented with hepatosplenomegaly, pallor, fever, and skin rash. We diagnosed this case as JMML, although hemoglobin F was within the normal range and no spontaneous colony growth was observed from peripheral blood mononuclear cells. Neither Epstein-Barr (EB) virus nor cytomegalovirus was detected by PCR in bone marrow aspirate or peripheral blood. The patient had several lesions into which JMML cells might have infiltrated, including skin, liver, spleen, oral cavity, right lung, sigmoid colon, and both eyes. To our knowledge, this is the first reported case of JMML with ocular involvement. Since infiltration of JMML cells into both eyes causes blindness, further consideration of the timing of bone marrow transplantation (BMT) in JMML is necessary.
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keywords = bone
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2/53. amiodarone pulmonary, neuromuscular and ophthalmological toxicity.

    amiodarone is an iodinated benzofuran derivative class III antiarrhythmic that is highly effective in suppressing ventricular and supraventricular arrhythmias. It is also associated with an imposing side effect profile, which often limits its use. Numerous adverse effects have been documented including skin discolouration, photosensitivity, hepatitis, thyroid dysfunction, corneal deposits, pulmonary fibrosis, bone marrow suppression and drug interactions. These side effects are thought to be correlated with the total cumulative dose of amiodarone, but idiopathic reactions have been reported. The majority of adverse reactions resolve with discontinuation of the drug; however, rapid progression may occur, which may be fatal. The present report documents a patient who had a combination of serious amiodarone toxicities that, once recognized, were treated and eventually resulted in a good outcome.
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keywords = bone
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3/53. Ocular manifestations of graft versus host disease following bone marrow transplantation.

    The ocular manifestations of Graft Versus Host Disease (GVHD) include keratoconjunctivitis sicca, cicatricial lagophthalmos, sterile conjunctivitis, corneal epithelial defects, corneal ulceration and melting. These manifestations are more frequent in patients with chronic GVHD than in patients with acute GVHD. The more severe ocular complications are associated with severe systemic chronic GVHD and poorer survival. Recent improvements in the systemic management of these patients have led to the more frequent recognition of the ocular problems. The high prevalence of ocular involvement and potentially severe ocular problems in GVHD patients necessitate close ophthalmic monitoring.
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keywords = bone
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4/53. Hereditary arthro-ophthalmopathy (Stickler syndrome).

    We report a 34-year-old woman with hereditary arthro-ophthalmopathy (HAO; Stickler syndrome). This disorder is a rare inherited generalized connective tissue disorder involving the eyes, facial structures and the skeleton, resulting from defective type II collagen formation. There is premature degenerative arthritis and osteopenia with increased incidence of fractures. The differential diagnosis and treatment for bone loss are discussed.
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keywords = bone
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5/53. Perfluorodecalin-induced intravitreal inflammation.

    OBJECTIVE: To report an unusual case of intravitreal inflammation in a human eye caused by the presence of residual perfluorodecalin in a case of giant retinal tear and retinal detachment. methods: The posterior capsule of the lens, which was infiltrated with deposits, was collected during surgery. The specimen was stained with hematoxylin and eosin, with periodic acid-Schiff, and for melanin. Part of it was examined with electron microscopy. Immunohistochemical staining was performed to demonstrate CD68 antigens, cytokeratin, and glial fibrillary acid protein. RESULTS: Vacuolated macrophages and retinal pigment epithelial cells infiltrated the posterior capsule. Electron microscopy showed the presence of membrane-lined vacuoles within the macrophages. A monolayer of epithelial cells covered the cellular infiltration. CONCLUSION: Residual perfluorodecalin can induce an intraocular chronic macrophage response.
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ranking = 196.96233462184
keywords = macrophage
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6/53. Further clinical and sensorial delineation of Schinzel-Giedion syndrome: report of two cases.

    Schinzel-Giedion syndrome is a rare multiple congenital malformation syndrome defined by an evocative midfacial retraction, kidney and urinary malformations and multiple skeletal abnormalities associated to a recently described neurodegenerative process. Two children with SGS are reported with identical clinical findings: megacalycosis, progressive neurodegeneration with infantile spasms and hypsarrhymtic activity. Ocular investigations revealed alacrimia and corneal hypoesthesia. Computed tomography of the temporal bone showed a tuning-fork malformation of the stapes for both children. These features may contribute to further delineation of SGS as additional clinical criteria.
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keywords = bone
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7/53. interleukin-8 expressed in the granulocytes of the eye in a patient with Behcet's disease complicated by lens-induced endophthalmitis.

    BACKGROUND: interleukin-8 (IL-8) is believed to be involved in the progression of intraocular inflammation. We sought the source of IL-8 in the enucleated eye of the present patient. CASE: A 40-year-old Japanese man was diagnosed as having Behcet's disease. His vision deteriorated due to persistent uveitis and secondary glaucoma. His left eye had lens-induced endophthalmitis. OBSERVATIONS: The left eye had to be enucleated, and it was investigated by an immunohistochemical analysis using antibodies for CD 1a (dendritic cells), CD 3 (T cells), CD 68 (monocytes/macrophages), interferon-gamma, or IL-8. Fibrovascular tissue had formed on and beneath the lens where inflammatory cells had infiltrated. Most of the mononuclear inflammatory cells were T cells. A large number of macrophages were observed especially around the lens. interferon-gamma-positive cells were scattered, while IL-8 was observed only in the accumulated granulocytes, but not in either mononuclear cells or macrophages. CONCLUSION: IL-8 is thus considered to play a role in the progression of intraocular inflammation, and granulocytes are thought to be a possible source of IL-8 in endophthalmitis.
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ranking = 196.96233462184
keywords = macrophage
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8/53. Cellular infiltration of the vitreous in a patient with X-linked immunodeficiency with increased IgM.

    We studied visual impairment caused by benign lymphoid infiltration of the vitreous bilaterally, as a complication of a primary immunodeficiency, X-linked immunodeficiency with increased IgM in an 8-year-old boy. immunophenotyping of a vitreous aspirate showed a mixed cell population, including lymphocytes (T helper, suppressor-cytotoxic T cells, and B cells) and macrophages. Cultures of the vitreous were negative for bacterial or fungal pathogens. The vitreous infiltrates have been resistant to treatment with corticosteroids and cytotoxic agents.
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ranking = 65.654111540614
keywords = macrophage
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9/53. Pulmonary and ophthalmic involvement with erdheim-chester disease: a case report and review of the literature.

    erdheim-chester disease is a rare nonfamilial histiocytic disorder of unknown etiology with characteristic long bone findings. The 3-year survival rate for patients with erdheim-chester disease is 50%. Approximately 50% of patients have disease involvement in other tissues, including skin, retro-orbital and periorbital tissues, pituitary-hypothalamic axis, heart, kidney, retroperitoneum, breast, skeletal muscle, and sinonasal mucosa; about 20% of patients have lung involvement. prognosis generally depends on the extent of the extraosseous disease. For patients with lung involvement, gender distribution is equal, but men typically present at an older age than do women. Approximately 80% of patients present with dyspnea, and most patients have diffuse interstitial infiltrates and pleural and/or interlobar septal thickening on chest radiology. Characteristic lung histopathology includes the accumulation of histiocytes with variable amounts of fibrosis and a variable lymphoplasmacytic infiltrate in a lymphangitic distribution. Immunostains are diagnostically useful, showing immunopositivity for CD68 and factor xiiia and immunonegativity for CD1a. Birbeck granules are uniformly absent ultrastructurally.
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ranking = 0.33333333333333
keywords = bone
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10/53. Frontoethmoidal encephalocele with ocular leakage of cerebrospinal fluid. Case report.

    The authors present the case of a 2-year-old boy who was born with a small bulging mass in the middle frontonasal area and hypertelorism. In the neonatal period he suffered from a continuous lacrimal secretion mistaken for recurrent conjunctivitis. Cranial computerized tomography scanning and magnetic resonance imaging revealed a frontoethmoidal encephalocele associated with an ocular leakage of cerebrospinal fluid. One-stage repair of the encephalocele, along with correction of hypertelorism and bone grafting of the forehead, was performed with good result. During a 2-year follow-up period no neurological deficits appeared.
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keywords = bone
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