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Biomedical subjects

V Ziegler

Publications and source records attributed to V Ziegler.

At least 19 recordsLinked to original sources

[Chemically-induced scleroderma].

Scleroderma-like diseases can be induced by a number of chemical compounds, such as plastics, solvents and drugs. Contaminated rapeseed oil was the cause of the toxic oil syndrome and L-tryptophan induces the so-called eosinophilia-myalgia-syndrome. On the other hand, paraffin and silicon can trigger so-called adjuvant disease, while long-term exposure to silica can lead to idiopathic scleroderma (associated with silicosis in some cases). In addition to the clinical features, some pathogenetic data in the literature, such as genetic factors (HLA, chromosomal anomalies, enzyme deficiencies) and the metabolism of chlorinated ethylenes via reactive epoxide intermediate products, and our own findings are reported. Silica-induced scleroderma cannot be distinguished from the idiopathic form by epidemiological, clinical or immunological studies or by parameters referring to the blood vessels or collagen metabolism. In cell culture studies it has been shown that macrophages/monocytes release IL1, IL6 and TNF after ingestion of silica, which affects fibroblasts, T-helper cells and endothelial cells. Comparative results from the silicosis literature are reported. Finally, the possibly stimulating role of ionizing irradiation (uranium mining) in favouring the development of scleroderma is discussed.

Ethylenes

[The mentally ill artist in the work of E. T. A. Hoffmann in relation to psychological theories at that time].

The work of one of the greatest writers of German Romanticism, E.T.A. Hoffmann, incorporates a great deal of current medical knowledge, which Hoffmann used in a skillful and detailed manner in the portrayal of his characters and their motives. Immersed as he was in contemporary medical practice, an interest fuelled by his own deep-seated hypochondria, he was particularly taken with the works of Philippe Pinel, Johann Christoph Reil, Carl A.F. Kluge, and Gotthelf Heinrich Schubert, who were all interested in the working of the mind. This article demonstrates how attention to Hoffmans's medical reading list offers insights useful for critical understanding of his work, using as an example the analysis of the mad goldsmith Cardillac in one of Hoffmann's most famous stories, 'Das Fräulein von Scuderi'.

Creativity

[Contact eczema and "standard test"--do "International Standard Test Series" coincide with Austrian local characteristics?].

Testing with a "standard" group of substances has proved of great value when allergic contact dermatitis is suspected and the offending agent cannot be pinpointed by careful history-taking. But internationally standardized series do not take into account locally important and individual factors, changing consumer habits or economic aspects. We have, thus, compared the suitability of two standard series, the ICDRG-European and the DKG-German standard, for Austrian purposes. 1,478 patients with a clinical diagnosis of contact dermatitis were patch tested between 11/88 and 4/90 with the ICDRG allergens; the second collective of 505 patients was tested between 5/90 and 1/91 with the DKG series. Only 12 out of the 27 substances of the ICDRG series exceeded the critical incidence of 1%; 6 were below this limit but positive in more than 0.5% and 9 hardly ever aroused positive reactions. In the DKG series, 17 out of 23 substances surpassed the limit, 2 were positive in more than 0.5%, and 4 caused virtually no reactions. However, 4 allergens with frequent sensitization rates were included neither in the ICDRG nor in the DKG series and 2 others were missing in the over-dimensioned ICDRG standard. Our results demonstrate that internationally established standard series have to adapted for the local situation in Austria, and that a constant review is essential with replacement of allergens as necessary according to changing production techniques and consumer habits. Suggestions for an "Austrian standard patch test series" are proposed on the basis of our results but in spite of being helpful in general terms interpretation of such a test in the individual patient with eczema remains problematic.

Allergens

[Quartz crystals in the skin as a cause of progressive systemic scleroderma].

There is a close correlation between the systemic sclerosis and the exposure to crystalline silica in the GDR. The silica content was investigated in the skin of the patients. Silica particles were found using phase-contrast and polarizing microscopy. The size of the crystals varied from 1 to 90 micrometer. Below a silica content of 10% in the dust it is unlikely that systemic sclerosis is developing.

Biopsy

Brofaromine (CGP 11 305 A): estimation of plasma concentrations by a biologic technique as compared to liquid chromatography.

In an open clinical trial 13 depressives significantly improved under the reversible and selective type-A monoamine oxidase (MAO) inhibitor brofaromine. The inhibitory potency of deproteinated plasma on a crude MAO preparation from human placenta was measured as a parameter for plasma brofaromine. There were no significant differences in plasma MAO inhibitory potency between responders (improvement greater than 50%; n = 5) and non-responders. MAO inhibitory potency significantly (p less than 0.05) increased parallel to the increase of the dosage from 50 mg b.i.d. to t.i.d. confirming the validity of this technique. The biologic assay, however, overestimated brofaromine by a factor of two in acute kinetic experiments with healthy volunteers as compared to a chromatographic technique, although both methods significantly correlated (r = 0.928).

Adult

Silica-induced scleroderma.

In a survey done in East Germany between 1981 and 1988, we found that 93 of 120 male scleroderma patients had long-term exposure to silica dust. We describe our findings in 12 patients with scleroderma and silicosis. The exposure time to silica dust was between 3 and 34 years; the interval between the beginning of exposure and the onset of scleroderma averaged 27.3 years (range 9 to 40 years). Antinuclear antibodies in titers between 80 and 10,240 with nucleolar and/or speckled patterns were found in 10 patients, antibodies against double-stranded DNA in three, Scl-70 (topoisomerase I) in three, and anticentromere antibodies in five. The following markers of collagen metabolism were increased in serum: beta-galactosidase in 12 patients, laminin peptide-P1 in 10 patients, N-terminal procollagen type III peptide in 10, and urinary sialic acid excretion in 7. We propose that crystalline particles of silica less than 5 microns may be phagocytosed by macrophages and release lymphokines and monokines, which activate fibroblasts and enhance their collagen and glycosaminoglycan synthesis. In addition, silica may act as an adjuvant to increase immune reactivity.

Biopsy

Type III collagen aminopropeptide and laminin P1 levels in serum of patients with silicosis-associated and idiopathic systemic scleroderma.

A group of 191 patients with systemic scleroderma and 12 patients with silicosis-associated scleroderma were investigated for connective tissue turnover. The serum levels of type III collagen aminopropeptide (P-III-P), the laminin PI (Lam PI) fragment and the acid lysosomal beta-galactosidase (beta-Gal) were determined by specific radioimmunoassays and spectrofluorometry, respectively. Increased levels of type III collagen aminopropeptide strongly correlated with enhanced activity of beta-galactosidase. Both parameters correlated with the clinical course in idiopathic systemic scleroderma and in silicosis-associated scleroderma. Serum levels of Lam PI were also found to be elevated in both groups, although there was no correlation with the severity of the disease. Autoantibodies directed against the DNA topoisomerase Scl-70 and against centromeric proteins were found in a similar range in patients with idiopathic systemic and silicosis-associated scleroderma. These results suggest that P-III-P, Lam PI and beta-Gal are useful serological markers of fibrotic activity and demonstrate similarities between idiopathic systemic scleroderma and scleroderma associated with silica-dust exposure.

Aged

Silica-associated systemic sclerosis is clinically, serologically and immunologically indistinguishable from idiopathic systemic sclerosis.

To determine whether the clinical, immunological and serological features of patients with silica-associated systemic sclerosis are different from patients with the 'idiopathic' form of systemic sclerosis (SS) we studied 22 underground coal miners who were exposed to silica dust (SD), 30 mine workers who later developed silicosis (S) and 17 mine workers exposed to silica dust who subsequently developed a systemic sclerosis-like disease (SA-SS). The patients with SA-SS had features clinically indistinguishable from individual patients with SS. They all had Raynaud's phenomenon, 14 had cutaneous sclerosis identical to that seen in acrosclerosis and three had a generalized cutaneous sclerosis. Sixteen patients had bibasilar pulmonary fibrosis, 10 had necrosis of the fingertip pulps, nine had oesophageal involvement and only one patient had renal involvement. Antinuclear antibodies and circulating immune complexes were detected in three and eight patients with SD, 14 and five patients with S and in 16 and nine patients with SA-SS, respectively. Anti-Scl-70 antibody was detected in eight of the 17 patients with SA-SS. Evidence for in vivo endothelial cell damage, as determined by elevated levels of von Willebrand factor, was found in nine patients with SD, 14 patients with S and in 10 patients with SA-SS. Following incubation of the patient's serum with confluent cultures of human umbilical vein endothelial cells there was only a significant reduction in calcium ionophore-induced release of prostacyclin with the serum from SA-SS patients compared to that with control serum (NC). The mean +/- SEM release of 6-keto-PGF1 alpha (the stable metabolite of prostacyclin expressed as ng/10(4) cells) decreased from 2.90 +/- 0.27 to 2.01 +/- 0.33 (SD), 3.34 +/- 0.42 to 1.76 +/- 0.31 (S), 1.98 +/- 0.12 to 0.64 +/- 0.07 (SA-SS) and 2.28 +/- 0.33 to 1.36 +/- 0.21 (NC) with 1 and 20% serum, respectively. This study demonstrates that immune complex and antinuclear antibody formation and in vivo endothelial cell damage occurs following occupational exposure to silica. The patients who subsequently develop a systemic sclerosis-like disease have clinical, immunological and serological features which are indistinguishable from the idiopathic form of the disease although as a group the SA-SS patients have a higher prevalence of pulmonary involvement and the anti-Scl-70 antibody.

6-Ketoprostaglandin F1 alpha

[Early detection of scleroderma in quartz dust exposed workers and workers with silicosis by determining serum beta-galactosidase activity].

Progressive systemic sclerosis (PSS) is a rare disease belonging to the collagen diseases. PSS is frequently observed in workers with an intensive exposure to crystalline silica and with silicosis in the GDR. The recognition as an occupational disease is regulated by law. The elevated beta-galactosidase activity in the serum of patients with silicosis and beginning PSS can be used for detecting of early stages of PSS. References are given to medical care of patients with silicosis and exposure to quartz.

Adolescent

[Dermatological incompatibility reaction to X5Cr-Ni-Mo 18.10 steel implants in animal experiments].

Metal allergy is surveyed in detail. Especially the development of eczema by means of metallic implantates containing chromium or nickel. The results from two series of experiments on guinea-pigs are presented. The first series proves that after preceding sensibilization by potassium bichromate or nickel-II-sulfate eczemas may be induced in the guinea-pig by X 5 Cr Ni Mo 18.10 steel implantates. Positive skin reactions in the patch test prove the possibility of sensibilization by X 5 Cr Ni Mo 18.10 steel in a second series of animals preoperatively negative.

Animals