1/13. Acute unilateral corneal immunoprotein deposition in IgM monoclonal gammopathy.A healthy 43-year-old officer of a merchant ship at sea developed pain, redness, and photophobia in his right eye. During the next 2 weeks, he noted the presence of a band of opacity spreading from his temporal limbus toward his central cornea. His episcleral vessels were engorged in a distribution contiguous with the peripheral, sectorial, fleck-like corneal opacities. The opacity had progressed during topical and systemic antibiotic therapy, but halted with use of topical corticosteroids. Systemic evaluation showed mild IgM monoclonal gammopathy. Transmission electron microscopy of a corneal biopsy specimen revealed electron-dense fibrils identified as immunoprotein. To our knowledge, this is the first report of a case of acute unilateral deposition of corneal immunoprotein in a patient with monoclonal gammopathy. Clinicians should begin with a broad differential diagnosis when evaluating patients with corneal opacity.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/13. Oligosymptomatic cornea verticillata in a heterozygote for fabry disease: a novel mutation in the alpha-galactosidase gene.BACKGROUND: fabry disease is an X-linked genetic disorder involving sphingolipid catabolism, which is caused by lysosomal alpha-galactosidase A deficiency. Ophthalmological findings such as corneal and lens opacities and conjunctival and retinal vessel abnormalities can be the only and/or the first recognizable symptoms, especially in heterozygous females. methods: We report on a 34-year-old German woman with cornea verticillata. The alpha-galactosidase A activity was determined in leukocytes using a fluorescence substrate, and the sequence analysis of the alpha galactosidase A gene was performed with genomic dna. RESULTS: The alpha-galactosidase A activity in leukocytes was significantly low (0.24 nmol/min/mg protein; normal range, 0.4-1.2), which is compatible with a heterozygote for fabry disease. The following sequence analysis revealed a heterozygous transition in position IVS5 2 T > C. Transition of thymine (T) to cytosine (C) affects the donor splice motive of exon 5 and most probably leads to an aberrant splicing procedure of the alpha-galactosidase A gene. CONCLUSION: Our case emphasizes the importance of ophthalmological findings in fabry disease. The subsequent biochemical and molecular analysis provides a secure diagnosis of female carriers of fabry disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/13. Q-switched Nd:YAG laser treatment for corneal neovascularization.Q-switched Nd:YAG laser treatment to occlude newly formed corneal vessels was performed in patients with herpetic keratitis to reduce corneal opacity and the risk of graft rejection. Nine neovascularized corneas of 9 patients were treated. In 8 of the 9 patients, corneal neovascularization was markedly reduced with a resulting decrease in corneal opacity. Penetrating keratoplasty was performed in 3 patients after the treatment. Two patients in whom YAG laser treatment was effective had uneventful postoperative courses for 13 and 17 months, respectively. In one patient with ineffective YAG laser treatment, rejection occurred 3 months after keratoplasty, and systemic steroid and ciclosporin therapy was needed. Ultrastructural examination of the corneal button removed during keratoplasty from a patient with successful laser treatment showed destruction of vascular endothelial cells and occlusion of the vascular lumen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/13. A novel two nucleotide deletion in the apolipoprotein a-i gene, apoA-I Shinbashi, associated with high density lipoprotein deficiency, corneal opacities, planar xanthomas, and premature coronary artery disease.Familial HDL deficiency (FHD) is a rare autosomal dominant lipoprotein disorder. We describe a novel genetic variant of the apolipoprotein a-i (apoA-I) gene resulting in FHD. The proband is a 51-year-old woman who was hospitalized due to severe heart failure. Her plasma HDL-cholesterol (C) and apoA-I concentrations were 0.08mmol/l and 1mg/dl, respectively. She exhibited corneal opacities and planar xanthomas on eyelids and elbows. coronary angiography demonstrated extensive obstructions in two major vessels. Genomic dna sequencing of the patient's apoA-I gene revealed a homozygosity for a GC deletion between 5 GC repeats in exon 4, creating a frameshift and a stop codon at residue 178. We designated this mutation as apoA-I Shinbashi. The proband's father, son, and daughter were found to be heterozygous for this mutation and their HDL-C and apoA-I levels were about half of normal levels, demonstrating a gene dosage effect. The father underwent coronary bypass surgery at age of 70 years. Lecithin-cholesterol acyltransferase (LCAT) activity was decreased by 63% in the homozygote and 31% in heterozygotes, respectively. This new case of apoA-I deficiency, apoA-I Shinbashi, is the first case involving a single gene defect of the apoA-I gene to develop all the characteristics for apoA-I deficiency, including premature coronary heart disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/13. Free autologous buccal mucosal graft transplantation to treat ocular complications after toxic epidermal necrolysis: case report.BACKGROUND: Toxic epidermal necrolysis (TEN) can lead to ocular surface scarring associated with pain, dry eye symptoms, and decreased visual acuity that often are difficult to treat. history AND SIGNS: A 34-year-old woman was referred to our department two-years after TEN induced by lamotrigin (lamictal(R)). She was complaining of severe visual acuity loss, pain, and dry eye symptoms. visual acuity was reduced to light perception in the right eye (RE) and to 0.2 in left eye (LE). Basal Schirmer test was 2 mm in RE and 3 mm in LE. With or without therapeutic contact lenses, the patient was experiencing severe discomfort requiring tear supplementation up to 50 - 80 times/day in both eyes. THERAPY AND OUTCOME: A free autologous mucosal graft (3.5 x 2.0 cm) was transplanted from the lower lip into the upper RE fornix. Six months after surgery, with therapeutic contact lenses, the need for tear supplementation was markedly reduced to 3 - 4 times/day in RE while it remained unchanged in LE. In RE, slit-lamp examination revealed decreases in the corneal stromal edema and in the diameter of neo-vessels associated with an improvement of visual acuity (counting fingers at 30 cm). Basal Schirmer test values were unchanged. It has to be mentioned that the improvement observed after surgery was markedly dependent on wearing a therapeutic contact lens. CONCLUSIONS: Free autologous buccal graft transplantation (with its presumably accessory salivary glands) in association with the use of a therapeutic contact lens can be an efficient approach to treat ocular complication following drug-induced TEN (Lyell's syndrome).- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/13. A new mucolipidosis with psychomotor retardation, corneal clouding, and retinal degeneration.A 23-year-old man had slow psychomotor development at 6 months of age and developed intermittent corneal clouding at about 18 months. He developed a truncal ataxia and hypotonia of the limbs combined with spasticity and active deep reflexes that did not progress. His skeleton and facies were normal. Between 1 and 13 years of age, he developed severe optic atrophy, absence of retinal blood vessels, and an extinguished electroretinogram. Biochemical analysis of cultured fibroblasts indicated no lysosomal hydrolase deficiency; cellular metachromasia was absent and there was no mucopolysaccharidoses. Ultrastructural studies indicated single-membrane-limited vacuoles containing lamellated membranes and a polymorphous substance in tissue-cultured cells and conjunctiva.- - - - - - - - - - ranking = 31.409567745468keywords = blood vessel, vessel (Clic here for more details about this article) |
7/13. A new mucolipidosis with psychomotor retardation, corneal clouding, and retinal degeneration.A man now 22 years of age had slow psychomotor development about 6 months after birth and developed intermittent corneal clouding at about 18 months. He developed truncal ataxia, hypotonia of the limbs combined with spasticity, and active deep reflexes. These have not progressed. His skeleton and facies are normal. Between his first and thriteenth year he developed sev ere optic atrophy, absence of retinal blood vessels, and an extinguished electroretinogram. Biochemical analysis of cultured fibroblasts indicated no lysosomal hydrolase deficiency; cellular metachromasia was absent and there was no mucopolysaccharidoses. Ultrastructural studies indicated single membrane vacuoles containing lamellated membranes and a polymorphous substance in tissue cultured cells and conjunctiva.- - - - - - - - - - ranking = 31.409567745468keywords = blood vessel, vessel (Clic here for more details about this article) |
8/13. Ocular manifestations of familial high-density lipoprotein deficiency (tangier disease).Corneal clouding is one of the manifestations of tangier disease, an inherited disorder in which cholesterol-rich lipids are deposited in various tissues of the body. The cause of the corneal clouding is unknown. This study documents the clinical course and conjunctival biopsy findings of a 60-year-old man who was one of the earliest patients to be recognized with tangier disease and in whom progressive corneal clouding developed in adult life. Noteworthy in the biopsy specimens were birefringent lipid particles that were predominantly present in degenerating pericytes of the conjunctival vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/13. Circinate-pattern interstitial keratopathy in daily wear soft contact lens wearers.Three male and two female patients (aged 16 to 22 years) developed bilateral deep corneal stromal vascularization and circinate-pattern lipid exudation resembling interstitial keratitis after wearing adequately fitting hydrogel daily wear soft contact lenses comfortably for periods of ten to 24 months. The circinate pattern consisted of lipid deposition in circular distribution either parallel to the limbus or around a deep stromal blood vessel. All had negative serologic test results for syphilis. The cause of these changes is uncertain.- - - - - - - - - - ranking = 31.409567745468keywords = blood vessel, vessel (Clic here for more details about this article) |
10/13. Late corneal opacities in the syndrome of interstitial keratitis and vestibulo-auditory symptoms.Progressive clouding of the cornea may be a delayed complication of the interstitial keratitis-vestibuloauditory syndrome. Two illustrative cases are presented. The first case illustrates progressive opacification about aberrant vessels in the deep stroma presenting a characteristic dendritiform pattern. The second case shows that the opacification is due, in this case at least, to formation of connective tissue with inclusion of lipid crystals and fat along with the blood vessels. Descemet's membrane is also thickened several fold.- - - - - - - - - - ranking = 32.409567745468keywords = blood vessel, vessel (Clic here for more details about this article) |
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