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Biomedical subjects

W Gebhart

Publications and source records attributed to W Gebhart.

At least 73 records · Page 4Linked to original sources
PubMed1982

A monoclonal antibody against a surface antigen shared by human large granular lymphocytes and granulocytes.

A monoclonal IgM antibody VEP13 was raised against a cell membrane antigen by immunizing BALB/c mice with a cell preparation enriched for human large granular lymphocytes (LGL). These cells are known to include the effector cells responsible for natural killer (NK) and killer (K) cell activity. By indirect immunofluorescence, VEP13 antibody reacted with 21.4 +/- 7.9% of peripheral blood lymphocytes (PBL) and more than 95% granulocytes but not with B lymphocytes, monocytes, erythrocytes, thrombocytes, thymocytes, and immature bone marrow cells. Cell suspensions enriched for T cells by means of E-rosetting using neuraminidase-treated sheep red blood cells contained 14.7 +/- 5.5% of VEP13+ cells. When PBL were treated with VEP13 antibody and complement, NK activity against a variety of target cells was greatly reduced and K cell activity was also strongly affected. Enrichment experiments of VEP13+ cells by means of the fluorescence-activated cell sorter revealed that all the NK activity resides in the VEP13+ fraction. This cell fraction was a homogeneous population of LGL. Studying the coexpression of various other antigens as defined by monoclonal antibodies, 84.6 +/- 4.6% of VEP13+ cells also expressed M1, 81.6 +/- 5.5% T10, 47.9 +/- 14.4% Leu 2a, 51.6 +/- 17.0% human Lyt-3, and 6.0 +/- 4.0% HLA-DR antigens. Leu 1, Leu 3a, and T6 antigens were found to be absent from VEP13+ cells. We conclude that VEP13 antibody should be helpful in determining LGL in peripheral blood as well as in tissue sections of tumor patients.

Antibodies, Monoclonal↗
PubMed1982

[Genodermatosis in a pair of brothers: Dowling-Degos, Grover, Darier, Hailey-Hailey or Galli-Galli disease?].

A peculiar polymorphous eruption in two brothers is reported. Histological examination of hyperpigmented macular lesions on the face and neck revealed all diagnostic features of the Dowling-Degos disease. With the electron microscope, singly dispersed melanosomes could be demonstrated in the basal keratinocytes. In biopsies from the erythematosquamous pruriginous lesions in the flexural areas, on the trunk and extremities, acantholysis, formation of lacunae, and some corps ronds were the predominant histological findings. Reduction of desmosomes, separation of desmosomes and tonofilaments, as well as thickened collagen-like tonofilaments could be shown. Not infrequently within a single lesion, all the features compatible with Dowling-Degos, Hailey-Hailey, Darier, and Grover disease were present. Pending further investigations and observation of patients with similar findings we propose with the consent of the patients, the term "Galli-Galli" disease as preliminary designation of this genodermatosis.

Acantholysis↗
PubMed1981

[Oculodermal melanocytosis (nevus of ota) and blue nevus (author's transl)].

Report is made of a patient having concurrently an Ota nevus and a blue nevus. The dermal melanocytes showed a marked formation of large intracytoplasmatic vacuoles. Reference to the important role of an "extracellular sheath" is made. The nearly identical ultrastructural characteristics of dermal melanocytes in various dermal melanocyte disorders seems to imply a common pathological origin in their development.

Adult↗
PubMed1981

[Penicillamine-induced elastosis perforans serpiginosa and pulmonary cyst in Wilson's disease (author's transl)].

A large air cyst was removed from the right lung of a 29-year-old female patient with Wilson's disease and penicillamine-induced perforating elastosis, the cyst first appearing after 9 years of treatment with penicillamine. Since, on the one hand, the microscopic and ultrastructural changes in the elastic tissue of the lungs were identical to those observed in the skin, both in areas of clinically-demonstrable elastosis perforans serpiginosa and clinically unaffected skin and, on the other hand, no other pulmonary disease was demonstrable to account for the development of the cystic lesion, it is concluded that penicillamine is the causative factor by means of extensive alteration of the elastic tissue. The morphological changes in the elastic fibres are so characteristic that it is easy to distinguish penicillamine-induced elastosis perforans serpiginosa from the idiopathic variant.

Adult↗
PubMed1981

[Linear epidermal nevus with multiple malformations. (author's transl)].

We report about a 12 year old boy who was born with a linear epidermal nevus. This nevus extended from the umbilicus strictly along the midline to the symphysis and continued on the right leg caudally to the medial brim of the foot and the first and second toe. Changes of bone structure are seen at-x-ray examination of the right hip and leg. Vascular dysplasia with arteriovenous shunts and spinal angiomas were found by angiography in the vascular bed of the left iliac artery. The length of the legs differed due to hyperplasia of the left lower extremity and both feet resembled the "Friedreich-foot".

Abnormalities, Multiple↗
PubMed1981

The "lumpy-bumpy" elastic fiber. A marker for long-term administration of penicillamine.

A 29-year-old woman with Wilson's disease developed dermolytic skin lesions 2 years after initiation of treatment with penicillamine. Eight years later, still on penicillamine therapy, striae appeared over both of her breasts. Biopsy of involved skin during the 10th year of treatment with penicillamine revealed characteristic lumpy-bumpy alterations of dermal elastic fibers which were not present in the first skin biopsy 8 years previously. Biopsy of a stria showed changes similar to those in the dermolytic skin lesions. Lumpy-bumpy elastic fibers are pathognomonic for penicillamine-induced elastosis. They are easily recognizable with examination by conventional microscopy. Their appearance may serve as a warning of potentially serious, widespread elastic tissue involvement. These abnormal elastic fibers are not only found in the skin, but also in the lungs.

Adult↗
PubMed1981

[Penicillamine-induced dermolytic dermatosis in a patient with Wilson's disease (author's transl)].

A 29-year-old female patient developed a dermolytic dermatosis after 2 years' treatment with penicillamine for Wilson's disease. The skin lesions were localized on skin areas exposed to trauma. Light and electron microscopic investigations showed alterations of all compartments of connective tissue. Patients under long-term high-dose treatment with penicillamine should be closely monitored for cutaneous and systemic connective tissue disease.

Adult↗
PubMed1979

"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↗
PubMed1979

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↗
PubMed1978

[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↗
PubMed1978

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↗