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Biomedical subjects

W Gebhart

Publications and source records attributed to W Gebhart.

At least 19 recordsLinked to original sources

"Lumpy-bumpy" elastic fibers in the skin and lungs of a patient with a penicillamine-induced elastosis perforans serpiginosa.

Penicillamine-induced cutaneous elastosis perforans serpiginosa associated with a large air-cyst in the right lung is described in a 29-year-old female patient with Wilson disease. Identical light and electron-microscopic changes were present in both dermal and pulmonary elastic tissue, suggesting a disseminated drug-induced cutaneo-visceral elastosis. Lung cysts have not been previously reported in association with long term penicillamine treatment. The electron-microscopic morphology of the elastic fibers was found to be "specific" enough to allow separation of penicillamine-induced elastosis perforans serpiginosa from other forms of this disease.

Adult

Effect of different preparation procedures on the ultrastructure of gonococci.

Culture samples of Neisseria gonorrhoeae and Escherichia coli were studied by transmission and electron microscopy to evaluate the effect of different preparation procedures on the pilation of these organisms. Unfixed as well as fixed bacteria showed only few, long, filamentous appendages when investigated in ultrathin sections, negatively stained specimens, or critical-point dried preparations. Snap-frozen specimens of E. coli showed many short and thin pili after being shadowed with carbon and platinum whereas those of N. gonorrhoeae showed only some type-C-like pili. Thus, the number and morphological appearance of pili appear to be greatly influenced by the preparation techniques used for study by electronmicroscopy. Conclusions as to the type and the infectivity of a bacterial strain can, therefore, not be based on purely morphological criteria.

Bacteriological Techniques

[Mucopolysaccharidosis V (Ullrich-Scheie syndrome) (author's transl)].

Mucopolysaccharidosis V (Scheie's syndrome, MPS-IS) is a very rare, autosomal recessively inherited metabolic disease. The degradation of dermatan sulphate and heparan sulphate is disturbed due to alpha-L-iduronidase deficiency, leading to intracellular storage and excessive urinary secretion of these substances. The characteristic clinical features are contractures (claw-like flexion of the fingers), umbilical and inguinal herniae, corneal opacity, hepatomegaly, myocardiopathy and minor skeletal malformations. A patient with Scheie's syndrome is now reported for the first time in Austria; the results of the clinical, biochemical, chromosomal, dermatoglyphic and electron optical investigations are described and discussed.

Adolescent

The relation of nervous elements to intradermal nevi. As electron microscopic study.

Intratumoral nerves of dermal nevi were investigated by electron microscopy in order to elucidate the role of neural elements in the development of these tumors. The following results were obtained: 1. Intratumoral nerves exhibit an increased cellularity, but the number of myelinated fibers is reduced. 2. Nevus cells type B and C are frequently present within nerve fascicles. 3. Unmyelinated axons were found in close relationship to nevus cells. 4. Schwann cells may show proliferations similar to early stages of neurofibroma and can be differentiated from unpigmented tumor cells by their relation to axons only. These findings indicate that identical cell populations are present within the intratumoral nerves and the tumor itself. Therefore, the theory of a neural component in the histogenesis of intradermal nevi is supported.

Adult

[Cytoid bodies in the human skin].

Cytoid bodies represent roundish or polygonal, approximately cell-sized structures in histological sections. Such bodies have been reported to be of pathologic significance in several cutaneous disorders. Histological, immunological and electron microscopic investigations were performed in order to (1) characterize the different types of cytoid bodies in human skin, (2) elucidate their origin and nature and (3) determine their significance in cutaneous histopathology. A distinct identification of elastic globes, Russell bodies, Civatte bodies, amyloid bodies and cytoid fibrin bodies could be made. In addition, other substances such as nuclear or cytoplasmic debris, cytolytic fragments or basement membrane proliferations were found in form of cytoid aggregates in several dermatoses. However, none of the different groups of cytoid bodies was evaluated to be of pathognomonic significance for a specific disease. All of them are rather symptomatic for a number of anatomical or pathological principles and events in the dermo-epidermal junction area. Thus, the presence of cytoid bodies is not a major diagnostic aid in histopathology but may give valuable hints for a better interpretation of the morphogenesis of etiologically unrelated skin disorders.

Amyloid

Demonstration of specific storage material within cutaneous nerves in metachromatic leukodystrophy.

Biopsies of clinically unaltered skin from a 5-year-old girl with metachromatic leukodystrophy were studied by light, fluorescence and analytical electron microscopy. Investigation of hematoxylin-eosin-stained sections revealed no pathologic changes, but a brown metachromatic material was found within cutaneous nerves after acetic acid cresyl violet staining of frozen sections. In semithin Epon sections small dermal nerve fascicles contained endoneural deposits, which proved to correspond with the typical prismatic, dense or Tuffstein bodies described in other organs of patients suffering from MLD. The inclusions were located mainly within Schwann cells and exhibited a bright orange fluorescence after trypaflavine-phosphotungstic acid-treatment as well as a high electron density in serial ultrathin sections. Energy dispersive X-ray microanalysis revealed a high sulfur content in the membrane bound granules. These findings demonstrate the presence of the specific storage material also within cutaneous nerves in MLD and thus suggest skin biopsies as an addiitonal and simple diagnostic acid in this disease.

Child, Preschool

Different types of benign nerve sheath tumors. Light microscopy, electron microscopy and autoradiography.

In a light-, electronmicroscopic and autoradiographic study different types of nerve sheath tumors were classified. Their cellular population was quantitatively evaluated in the electron microscope. In the neurinoma the predominant cell was found to be the Schwann cell, but in the different types of neurofibromata a variable content of connective tissue cells was noted. T-HE DIFFUSE NEUROFIBROMATA SHOWED A QUANTITATIVE CELLULAR COMPOSITION SIMILAR TO NORMAL PERIPHERAL NERVES. In the plexiform neurofibroma a large number of fibroblasts were present and in the argyrophilic neurofibroma high content of perineurial cells was found. In autoradiographic sections the tumors showed in general a low proliferation rate (L.i. 1-3.6%). In the argyrophilic neurofibrom a higher labelling index (9.5%) was found.

Adolescent

The epidermal cell proliferation in lichen planus.

The proliferative activity of epidermal structures was determined in 6 patients suffering from lichen planus (i.p.). By using the in vitro 3H-thymidine labelling technique, semithin Epon sections of lesional and perilesional uninvolved skin were compared autoradiographically with normal controls. The following results were obtained. The labelling index of epidermal cells was significantly increased within the i.p. papules. The mean values ranged from 41% in acanthotic i.p. to 23% in atrophic i.p. whereas in adjacent unaffected skin (15% and 11% respectively) as well as in normal controls (6%) a lower 3H-index was observed. As regards damage to basal cells within i.p. lesions, a factor--the relation between the number of basal cells in involved and uninvolved skin in reference to the epidermal length--was calculated in order to correct the autoradiographic results in involved skin. The differences between i.p. papules, adjacent skin and controls remained statistically significant even after correction. The intradermal eccrine ducts revealed a significantly increased labelling index in i.p. lesions as compared with perilesional and normal skin. In this respect no difference could be observed between the acanthotic and the atrophic variant of i.p. These findings indicate that replacement of damaged basal cells in i.p. is achieved by an increase in actively dividing keratinocytes in both epidermis and skin appendages within the lesion.

Cell Count