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W Lisch

Publications and source records attributed to W Lisch.

At least 19 recordsLinked to original sources

[The different opacity patterns of Lisch corneal dystrophy].

BACKGROUND: Lisch corneal dystrophy is an epithelial corneal dystrophy where diffuse gray corneal opacifications are seen in direct illumination that appear in different patterns. Examination in retroillumination shows that these gray changes do consist of multiple densely crowded clear microcysts according to the opacity units. We wanted to investigate if one single case of Lisch corneal dystrophy can be differentiated easily from the other epithelial corneal dystrophies by exact analysis of the opacity units. PATIENTS AND METHODS: We examined three non-related patients with Lisch corneal dystrophy at the slit-lamp. The epithelial corneal opacities were documented photographically with direct slit-lamp illumination and retroillumination. RESULTS: All three patients showed the characteristic intraepithelial densely crowded clear microcysts in retroillumination. Diffuse gray radial opacities were noted in both corneas of a 15-year-old female patient. A club-shaped opacity was noted in one cornea of a 35-year-old female patient and a feathery gray opacity pattern was found in one cornea of a 49-year-old male patient. All three patients showed the characteristic densely crowded clear microcysts of Lisch corneal dystrophy in retroillumination. CONCLUSION: Taking the previous literature into account, five different opacity patterns can be summarised: radial, band, flame/feathery, whorled and club-shaped gray epithelial corneal opacities. A single case of Lisch corneal dystrophy can be differentiated from other epithelial corneal dystrophies by means of an exact analysis of the opacity pattern and units by slit-lamp examination.

Adolescent↗

[Best disease].

Explore the source record for details and available documents.

Bestrophins↗

[Ocular complications of adjuvant interferon therapy for malignant melanoma: a review].

BACKGROUND AND OBJECTIVE: In dermatology, interferon Alfa 2 is used in adjuvant therapy of melanoma (stage IIa/b) as well as in treatment of cutaneous lymphoma or melanoma (stage III or higher). In the past 2 years, several case reports have described interferon-induced ocular side effects, including sudden loss of vision and ischemic retinopathy. We wondered if incidence and prognosis of ocular complications were elevated in patients receiving an adjuvant treatment of melanoma with interferons. PATIENTS/METHODS: We analyzed 1334 reports of adverse effects of interferons, which had been spontaneously reported in Germany since 1990. RESULTS: 8.4% of all reported adverse effects of interferons were ocular. More than the half of these patients developed significant visual loss including retinal ischemia. CONCLUSIONS: These data underscore the importance to inform patients concerning ocular adverse effects and emphasize the need to monitor the retina during adjuvant interferon therapy.

Antineoplastic Agents↗

[Corneal ring opacity (Ascher ring)--a case report].

PATIENT: A 63-year-old man without complaints presented with a bilateral symmetric opacity of the cornea between the mid and the outer periphery which was classified as Ascher's ring. CONCLUSIONS: Ascher's ring is a very rare entity. Characteristic slit lamp features allow its differentiation from other anular corneal opacities. Corneal or systemic alterations of the lipid metabolism can be discussed. Heredity can be suspected but could not be proved as yet. The visual prognosis is excellent. Therapy is not necessary.

Cornea↗

Lisch corneal dystrophy is genetically distinct from Meesmann corneal dystrophy and maps to xp22.3.

PURPOSE: There is an ongoing discussion whether Lisch corneal dystrophy (band-shaped and whorled microcystic dystrophy of the corneal epithelium) represents a disorder that is different from Meesmann corneal dystrophy. The purpose of this study was to evaluate at the molecular level if Lisch and Meesmann corneal dystrophies are genetically distinct. METHODS: We examined at the slit lamp a total of 48 members of a family with an aggregation of Lisch corneal dystrophy. Genomic DNA was extracted from leukocytes of the peripheral blood of seven affected and six unaffected members of this family. Mutational hotspots in the cornea-specific keratin genes K3 and K12 were scanned for mutations by single-strand conformation analysis. To test for linkage to the keratin K3 or K12 loci or for X-chromosomal inheritance, six (K3) and four (K12) microsatellite markers each flanking the keratin loci as well as 22 microsatellite markers covering the X-chromosome were typed. Linkage was analyzed using the MLINK and FASTMAP procedures. RESULTS: A total of 19 trait carriers were identified in six generations of the family. No hereditary transmission from father to son was observed. Linkage was excluded for the keratin K3 and K12 genes. Furthermore, single-strand conformation analysis detected no mutations in these genes. Multipoint linkage analysis revealed linkage with a maximum likelihood of the odds (LOD) score of 2.93 at Xp22.3. Linkage was excluded for Xp22.2 to Xqter. CONCLUSIONS: Lisch corneal dystrophy is genetically different from Meesmann corneal dystrophy. Evidence was found for linkage of the gene for Lisch corneal dystrophy to Xp22.3.

Adolescent↗

A new, band-shaped and whorled microcystic dystrophy of the corneal epithelium.

Five family members and three unrelated patients (four women, four men, 23 to 71 years old) had a dystrophy of the corneal epithelium. Direct slit-lamp examination showed bilateral or unilateral, gray, band-shaped, and feathery opacities that sometimes appeared in whorled patterns. Retroillumination showed intraepithelial, densely crowded, clear microcysts. Light and electron microscopy disclosed diffuse vacuolization of the cytoplasm of epithelial cells in the affected area. Visual acuity was so reduced in three patients that abrasion of the corneal epithelium was performed. The corneal abnormalities recurred within months, with the same reduction in visual acuity as before. The corneal opacities were progressive in two patients but diminished noticeably in another after he began using a hard contact lens. We found no other ophthalmic irregularities or associated systemic abnormalities and no indication of drug-induced keratopathy.

Adult↗

[Clinical aspects, follow-up and results of cataract extraction in uveitis].

Over a 5-year period, a consecutive series of 52 eyes in 46 patients with uveitis underwent extracapsular cataract extraction. Twenty-eight of these cases received a posterior chamber intraocular lens (IOL). During an average follow-up of 25 months (range 7 to 58 months) 71% of eyes receiving an IOL achieved postoperatively a visual acuity of 0.5 or better; 54% of aphakic eyes reached this level. Persistent cystoid macular edema limited the visual improvement to 20/200 in 6 patients; none of the patients developed cystoid macular edema postoperatively on clinical observation. Intraocular hypertension occurred postoperatively in 12 eyes, but was limited to a 4-week postoperative period in 9 cases. YAG laser capsulotomy was performed in 2 eyes with opacification of the posterior lens capsule without any further complications. The results suggest that uveitis patients benefit from cataract extraction and in selected cases can tolerate IOL implantation without major complications.

Adolescent↗

Paraproteinemic corneal deposits in plasma cell myeloma.

We treated two patients who had IgG-kappa-light chain monoclonal gammopathy with partially crystalline and partially amorphic corneal deposits. Impairment of vision made keratoplasty necessary for each patient. Histologic examination of the corneal specimens showed deposits that stained positively for Masson's trichrome in all corneal cells. Immunohistochemical tests identified these deposits as IgG-kappa-light chain immunoglobulin fragments. Electron microscopy showed intracellular, rhomboid-shaped deposits enveloped by a membrane. The same deposits appeared in the conjunctival epithelium, within subconjunctival fibrocytes, and in the plasma cells of the bone marrow. Immunoelectrophoresis showed IgG-kappa-light chain fragments in the blood serum, the lacrimal film, and the aqueous humor. This suggests that the intracellular immunoglobulin fragments may have entered the corneal and conjunctival epithelium by way of the lacrimal film, the keratocytes by way of the corneo-scleral limbus vasculature, and the endothelial cells from the aqueous humor.

Aged↗

Elevated prolactin levels in human aqueous humor of patients with anterior uveitis.

Evidence is accumulating that prolactin (PRL) may play a physiological role in the regulation of humoral and cell-mediated immune responses. On the basis of these observations, we measured levels in the serum and aqueous humor of 28 patients with cataract or anterior uveitis with concomitant cataract. Intraocular concentrations were measured in a range from 0.1 to 3.4 ng/ml. Whereas serum PRL levels failed to show significant differences between the two groups (P = 0.39), intraocular concentrations were significantly higher in uveitis patients (P less than 0.001). The level in aqueous humor did not reflect the serum concentration in either group. To the best of our knowledge, this is the first study measuring PRL concentrations in human aqueous humor.

Adolescent↗

[Cornea guttata and Fuchs' endothelial-epithelial dystrophy. Clinico-histologic study of 73 patients].

In a retrospective, clinico-histological study, 84 corneas from 73 patients with diagnosed Fuchs' dystrophy (FD) were examined after penetrating keratoplasty. Seven types of posterior corneal degeneration were found: six with diversely arranged and pronounced guttae and a thickened Descemet's membrane, and a seventh with no guttae in either eye. This last variant appeared histologically in only two patients. Whether it represents a different syndrome can only be speculated. Our study showed, however, that the frequency of cornea guttata (CG) in FD is statistically significant in all the histological variants.

Aged↗

[Current status of linkage studies for gene localization in corneal dystrophies].

Corneal dystrophies with primarily autosomal dominant inheritance have been clearly identified both histologically and histochemically. No information is available to date on the causal enzymatic protein defect of the individual forms of dystrophy. By using linkage analysis to study families with various corneal dystrophies, an attempt is being made with polymorphous markers to find an indirect indication for localization in a chromosome. Numerous factors which exclude linkage can be named for granular, lattice and Schnyder's corneal dystrophy. Our linkage analysis studies of a second family with Schnyder's dystrophy support our indications of linkage with the sites for adenylate kinase (AK1) and the AB0 blood groups on chromosome 9.

Chromosome Aberrations↗