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Biomedical subjects

G Ziegler

Publications and source records attributed to G Ziegler.

At least 19 recordsLinked to original sources
PubMed1992

Hereditary progressive dystonia with diurnal fluctuation (Segawa's syndrome)--an unusual case.

A young girl with hereditary progressive dystonia with diurnal fluctuation or Segawa's syndrome, completely handicapped and confined to a wheelchair between the age of 5 and 9, revealed an unusually slow response to levodopa. The ability to walk returned only after 12 to 14 months of treatment. Apart from peculiarities of behaviour due to longstanding immobility and ensuing parental overprotection, there were no psychological or mental abnormalities. Other organic diseases were ruled out. A series of regular follow-ups over the course of 7 years was performed. While residual and irregular day-to-day variation of functional capacities almost disappeared after conversion to a preparation with a decarboxylase inhibitor, some mild neurological abnormalities and a slight choreic hyperkinesia persisted with doses providing functional results. The patient today leads an almost normal teenage life and has performed well in school.

Body Weight↗
PubMed1992

Cysteine endopeptidases and their inhibitors in malignant progression of rat embryo fibroblasts.

Cathepsins B and L and their endogenous inhibitors were evaluated in rat embryo fibroblast lines which have been developed as a model system for the study of malignant progression and metastatic capability. Three groups of lines were analyzed: 1) immortalized/non-tumorigenic, 2) tumorigenic/metastatic lines transfected with c-Ha-ras, and 3) metastatic revertants transfected with c-Ha-ras+the E1A region of adenovirus type 2. The metastatic revertants are tumorigenic, but non-metastatic. No correlation was seen between tumorigenicity and metastatic potential and the level of expression of cathepsin B or the subcellular distribution of cathepsins B and L. However, cathepsin L activity was increased 2-fold in the 4R metastatic line. Although transfection of aneuploid 3T3 fibroblasts with ras has been shown to increase the expression of cathepsin L and cathepsin B, transfection of the diploid rat embryo fibroblasts with ras did not correlate with increased expression of cathepsin L or cathepsin B. However, ras transfection of the rat embryo fibroblasts was associated with a significant (4-15-fold) decrease in the activity of heat-stable cysteine endopeptidase inhibitors. Thus, in tumorigenic rat embryo fibroblast lines, regulation of the activities of cysteine endopeptidases by their endogenous inhibitors may be compromised, resulting in increased effective activities of the cysteine endopeptidases.

Adenoviridae↗
PubMed1992

[Lupus chorea revealing. Study in magnetic resonance imaging. Success of plasma exchanges after resistance to pulsed cortisone].

Chorea as a manifestation of SLE is infrequent (1% of all cases of SLE). A new case is reported herein. The patient, a seventeen-year-old female, was admitted with a one-week history of choreic movements of the left half of the body and arthritis of both wrists. Biologic findings confirmed the diagnosis of SLE with presence of an antiprothrombinase circulating anticoagulant. Findings upon cerebral CT scan and magnetic resonance imaging were normal. Clinical symptoms worsened despite corticosteroids in a daily dosage of 1 mg/kg with three pulses of 800 mg methylprednisolone. High-dose neuroleptic therapy was given and three plasma exchanges were performed. A dramatic improvement in clinical symptoms and biological anomalies occurred and persisted during follow-up which now exceeds one year. The lack of MRI anomalies suggests that the pathogenesis of SLE-associated chorea involves functional neurone activation by immune complexes; the dramatic effectiveness of plasma exchanges may obviate the need for using immunosuppressant agents in patients who fail to respond to corticosteroids.

Adolescent↗
PubMed1992

Multicentric reticulohistiocytosis. Immunohistological and ultrastructural study: a pathology of dendritic cell lineage.

A case of multicentric reticulohistiocytosis is presented with extensive immunohistochemical study of the infiltrate in both paraffin and cryostat sections. Factor XIIIa dermal dendrocyte marker was demonstrated in the cytoplasm of histiocytes, which has not been reported previously in this disease. In addition, the S100 protein stained positive. This immunophenotyping study suggests a dermal dendrocyte lineage with an unusual expression of S100 protein.

Dendritic Cells↗
PubMed1991

Knee arthritis revealing acute leukemia in a patient with rheumatoid arthritis.

We report one case of leukemic synovitis in a patient with a 40-year history of rheumatoid arthritis. The synovial fluid sample and synovial biopsy specimen showed myeloblastic cells. So, leukemic synovitis can be suspected in a patient with inflammatory rheumatism and adequate diagnostic procedures should be carried out.

Aged↗
PubMed1991

The malignant phenotype and cysteine proteinases.

Expression, membrane association and secretion of both cathepsin B and cathepsin L have been associated with the malignant phenotype of murine B16 melanomas. Only native forms of the two enzymes were found in the lysosomal fractions, whereas both native and latent forms were found in the membrane fractions. Brief exposure to acid pH induced secretion of only native forms of both cathepsin B and cathepsin L and a concomitant reduction in membrane-associated activities. Thus, the pericellular acidification associated with malignant tumors may provide optimal conditions for proteolysis by the cysteine proteinases in terms of their enhanced stability and their induced release in native forms not requiring proteolytic activation.

Animals↗
PubMed1990

Possible interaction of fluoroquinolones with the benzodiazepine-GABAA-receptor complex.

1. The possible involvement of the benzodiazepine (BZD)-GABAA-receptor complex in mediating CNS stimulatory effects of fluoroquinolones was tested in vitro, in a binding inhibition assay and in vivo, in a clinical drug interaction study using electro-encephalogram (EEG) monitoring. 2. The specific binding of [3H]-flunitrazepam to rat synaptic brain membranes was inhibited by various fluoroquinolones in a concentration-dependent manner. 3. Ofloxacin had CNS-stimulating effects as revealed by the EEG which were slightly augmented by flumazenil but reversed by coadministration of midazolam. 4. In conclusion, our findings suggest that clinically observed CNS adverse effects of fluoroquinolones could be mediated at least in part through interaction with the BZD-GABAA-receptor complex and may be controlled by BZD agonist administration.

Adult↗
PubMed1989

The common occurrence of furan fatty acids in plants.

The observation that F-acids (1) occur in rat chow initiated a search for F-acids in human diet. We observed that the amount of F-acids with a pentyl side chain in alpha-position taken up with a one-day diet correlates well with the amount of excreted degradation products, the pentyl urofuran acids (2), (3) and (4). Therefore it can be concluded that F-acids with a pentyl side chain are not produced in the human body but are introduced through the diet. The origin of F-acids carrying an alpha-propyl side chain is less clear. The amount of propyl-urofuran acids (2) and (3) excreted in urine was found in one case out of three to be five times higher than the amount of F-acids carrying a propyl group in alpha-position taken up by the diet. Therefore, it can presently not be excluded that a portion of the propyl F-acids is produced by the body. F-acids found in human food are mainly introduced into the body by vegetables and fruits. F-acids were found also in birch leaves in considerable amounts, as well as in grasses, dandelion and clover leaves. Thus, we can conclude that F-acids are common constituents of plants.

Chromatography, Gas↗
PubMed1989

[Systematic study of various tumoral markers in prevalent bone metastasis].

In the presence of prevalent bone metastases, the precise histo-pathological diagnosis of the primary tumor is often difficult. The authors study the diagnostic value of systematic serum assay of a series of tumoral tracers (ACE, AFP, PAP and PSA, SCC, CA 19:9, CA 15:3, CA 125) which until now were used in evolutive and therapeutic monitoring. 34 patients were selected for this preliminary retrospective study (including 20 with a demonstrated histopathological diagnosis). 70 p. cent of prevalent bone metastases express a target tracer corresponding to the initial location. In some cases, an elevated tracer, because of its specificity, may bring about a diagnostic or therapeutic decision (always according to the context). No conclusion may currently be drawn in case of discordance between the anatomo-clinical context and the "profile" of the markers (1 case in our series).

Biomarkers, Tumor↗