Biomedical subjects
W Lisch
Publications and source records attributed to W Lisch.
[Clinical types of immunologic transplant reactions following perforating keratoplasty].
The incidence of allograft rejection was determined for 740 penetrating keratoplasties performed between 1980 and 1987. All 740 cases were followed up for at least 1 year. The reaction forms of allograft rejection were grouped according to biomicroscopic appearance. The incidence and progression of symptoms are described. Rejection types are subdivided into patients with favorable versus poor prognosis. Of the patients 37.9% demonstrated an immune response (including discrete forms). Clear reaction patterns within the two groups of patients became apparent when reaction variations were carefully differentiated. Epithelial immune reaction was found in 5.2%/10.5% and subepithelial infiltration in 1.7%/4.8% of the patients with favorable/poor prognosis. The largest disparity in frequency occurred in progressive endothelial reaction; 3.8% in patients with favorable versus 36.7% in patients with poor prognosis. Focal endothelial reactions occurred in both groups with comparable frequencies (14.1%/13.3%). The large percentage of immunological reactions, including late manifestations (approximately 12% after 1 year) and some with irreversible progression, warrants continuing efforts to treat and prevent this complication.
Linkage analysis in granular corneal dystrophy (Groenouw I), Schnyder's crystalline corneal dystrophy, and Reis-Bücklers' corneal dystrophy.
Tight linkage was excluded for 8 markers in 37 blood relatives from 3 families, 29 of whom had granular corneal dystrophy (Groenouw I). Inconclusive results were obtained for linkage with four marker loci. The highest positive LOD score was 0.57 for linkage between glutamic pyruvic transaminase and granular corneal dystrophy. Tight linkage was excluded for glyoxalase-1 in eight individuals from one family with Schnyder's crystalline corneal dystrophy. Results were inconclusive for another six markers. Positive LOD scores were obtained for linkage with adenylate-kinase 1 and the ABO blood group, with values of 1.16 and 0.67, respectively. Among six blood relatives with Reis-Bücklers' corneal dystrophy, the highest positive LOD score was 1.17 for linkage with mitochondrial malic enzyme. For another six markers informative for linkage analysis, the results were inconsistent.
[Multiple vitelliform retinal cysts. Case report and review of the literature].
Description of a patient with multiple vitelliform retinal cysts, including the fovea, with normal EOG and no indications of hereditary origin. The changes were apparent as hypofluorescent zones in the angiogram. After three years an increase in size and partial confluence of the cysts was observed, but two years later cicatrization occurred in the previously cystic areas. So far 30 persons with multiple vitelliform cysts have been described in the literature; the principal findings in these cases are summarized in a table together with the present authors' findings. As regards heredity, two groups may be distinguished: (1) multiple cysts associated with Best's vitelliform dystrophy and (2) so-called isolated cases with no indication of heredity. All Group 1 cases seen so far have had a pathologic EOG, while in Group 2 cases the EOG may be both normal and pathologic. The average age of Group 1 patients is 31, that of Group 2 patients 39 years. The findings in Group 2 cannot be intepreted without reservation as adult or degenerative changes. Multiple vitelliform cysts must be clearly distinguished from multiple pigment epithelium detachments.
[Pathogenesis of congenital vitreous cysts].
Unilateral congenital vitreous cysts (VC) were observed in two young patients. The vitreous was highly liquefied in both. Biomicroscopially, the ball-shaped VC in the first patient was seen to be detached from the surrounding tissues and hence highly mobile. The potato-shaped VC in the second patient was attached to the posterior lens surface by a short, thread-like strand and was therefore only slightly mobile. Partially vascularized prepapillary strands were observed in the patient's fellow eye. Two different pathogenic mechanisms may be postulated, namely (1) that the VC was squeezed or jarred loose from the ciliary body pigment epithelium (first patient), or (2) that the VC resulted from impaired retrogression of the primary vitreous or the hyaloid artery (second patient).
Recognizing familial lecithin cholesterol acyltransferase deficiency at the slit lamp.
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[Corneal opacity as the leading symptom of hereditary lecithin-cholesterol acyltransferase (LCAT) deficiency. Case report and a review of the literature].
Familial lecithin: cholesterol acyltransferase (LCAT) deficiency is an inborn error of lipid metabolism clinically characterized by anemia, proteinuria, and corneal opacification. The authors review the literature dealing with 34 biochemically proven and 2 probable cases of LCAT deficiency, and describe the first case from a German-speaking country. Ocular findings were bilateral diffuse nebulous corneal opacification composed of innumerable minute grayish dots throughout the stroma. At the periphery of the cornea these dots increased, forming a ringlike band with indistinct margins. A small lipid deposit was also seen in the retina of the right eye. Vision was 20/20, but glare disability was significantly increased. Corneal opacification was also noticed in all cases of the literature. Anemia was detected at the time of diagnosis in 92% and proteinuria in 76% of the reviewed cases. Corneal opacification is the one absolutely obligatory clinical feature; moreover it is uniform and pathognomonic: a true indicating sign of LCAT deficiency.
[Corneal complications in Goldenhar-Gorlin syndrome].
The authors describe 3-year-old Italian girl with oculoauriculovertebral dysplasia (Goldenhar-Gorlin syndrome) accompanied by corneal anesthesia and reduced tear production complicated by progressive neuroparalytic corneal ulcer.
[Molecular biology of hereditary corneal diseases].
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[Neuroparalytic corneal ulcer in Goldenhar-Gorlin syndrome].
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Schnyder's dystrophy. Progression and metabolism.
In the first long-term cohort study of Schnyder's corneal dystrophy the authors examined affected and unaffected members of two unrelated families in 1975 and 1976 respectively, and again in 1984. They identified carriers, catalogued changes in the diffuse and crystalline corneal opacities which characterize this dystrophy and analysed the patient's lipid metabolism. Corneal opacities never regressed. Progression was more frequent in diffuse than in crystalline opacities. Both crystalline and diffuse opacities reappeared and progressed following penetrating keratoplasty. Mean cholesterol levels in the carrier group were above normal and six had a moderate type IIa dyslipoproteinemia; conversely, two carriers had low apo B. The degree of corneal opacification showed no relationship to dyslipoproteinemia. Schnyder's corneal dystrophy appears to involve the corneal lipid metabolism only and not to be a systemic disease.
Management of the opacified posterior lens capsule: an excision technique for membranous changes.
In 16 patients ranging from six months to 85 years a membranous-changed posterior lens capsule was partially excised during an originally extracapsular-planned lens surgery. After removing the lens contents the anterior chamber was filled with sodium hyaluronate. The opacified posterior capsule was punctiformly opened and the retrolenticular space widened with sodium hyaluronate, too. The isolated posterior capsule could be excised with microscissors without damaging the corneal endothelium or the anterior vitreous face. Integrity of the vitreous was preserved in 94% of the operated eyes. The described technique can be regarded as the method of choice for management of solid capsular opacities in infants and may be also helpful in some cases of membranous capsular changes in adults.
[Is luxation of the lens an indication for surgical removal?].
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[Excision of the posterior lens capsule with preservation of the anterior vitreous face].
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[Posterior crystalline corneal dystrophy].
A mother and daughter are described who have symmetrically arranged crystalline opacities in the area anterior to Descemet's membrane. These appear in the slit-lamp as symmetrically arranged, multicolored scintillating dots in the central part of the cornea. The daughter has, in addition, crystalline vitreal opacities. Microscopic examination of the endothelium revealed irregularly situated white spots in a circumscribed area anterior to the endothelium. None of the eyes showed any signs of inflammation. Metabolic tests yielded no signs of systematic metabolic disease.
[Various forms of opacities of granular corneal dystrophy].
Opacities in granular corneal dystrophy are examined as units as overall opacity patients. Unit opacities are individual spots and can be qualitatively divided into three basic groups: drop-shaped, crumb-shaped and ring-shaped. The typical overall opacity pattern is ray or disk-shaped. The different opacities enable the disease to be classified into early, middle and late stages. Recurrences within grafts show the same characteristics.
[Epithelial corneal basement membrane dystrophy].
Dystrophy of the basement membrane of the corneal epithelium is an autosomal-dominant disease. Three distinct forms of opacity occur, either in isolation or in combination with one another. These are (1) dotlike opacities, (2) maplike opacities and (3) fingerprint lines. The present paper describes a family in which 6 members show only the characteristic signs of superficial maplike corneal opacities. There have been no recurrent corneal erosions. In addition, 7 "isolated cases" are described in which both the pure and combined forms of opacity have been observed. The dotlike opacities are demonstrable only when maplike alterations are also present. The patholomechanism leading to these dotlike changes cannot be explained by aberrance of the basement membrane alone. Cases of the three forms of opacity which may occur as secondary phenomena have to be distinguished from the dystrophic form.
Hereditary vitreoretinal degenerations.
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