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Biomedical subjects

W Storch

Publications and source records attributed to W Storch.

At least 55 records · Page 3Linked to original sources
PubMed1980

[Immunologic studies with the lymphocyte transformation test on the side effects of D-penicillamine therapy in Wilson's disease].

18 patients with Wilson's disease with and without proteinuria and 19 test persons without Wilson's disease were examined for a disturbance of the transformation on account of phythemagglutinin, streptolysin-O and several D-penicillamine preparations by means of the lymphocyte transformation test. Also during the therapy with D-penicillamine was no cause for a disturbed transformation of lymphocytes on the mitogen phythemagglutinin. A cell-conditioned immune reaction to D-penicillamine which could be established by means of the lymphocyte transformation test under the conditions chosen seems to be present in patients with Wilson's disease neither in lacking or insignificant nor in pronounced proteinuria. The increased transformation rates to streptolysin-O in Wilson's disease need confirmation in a larger number of patients. Like the increased spontaneous transformation rates they might refer to an immune-stimulating effect of the D-penicillamine.

Drug Hypersensitivity↗
PubMed1980

[Wilson's disease in the German Democratic Republic. III. Diagnosis and therapy].

Taking into consideration the manifold symptomatology of Wilson's disease on the one hand and the necessity of an early--possibly already at the asymptomatic stage before the 6th year of life--diagnostic ascertainment on the other hand, the diagnostic approach performed in the GDR is described. Furthermore, the directives of treatment and the successes of treatment are discussed as well as the various side-effects of the D-penicillamine therapy described, in which case the severe nephrotic syndromes are particularly considered.

Adult↗
PubMed1980

[Wilson's disease in the German Democratic Republic. II. Pathogenesis and clinical aspects].

From the point of view of the Leipzig Centre for Wilson's disease in the GDR the authors adopt a definite attitude to the questions of pathogenesis and clinic. Since for the performance of the life-saving long-term therapy the disease must be diagnosed early, in detail are described the preclinical (asymptomatic) and clinical stage--including its various forms of manifestation--and their differential diagnosis and it is dealt with the changed radio-copper-kinetics.

Adolescent↗
PubMed1980

[Standardization of indirect immunofluorescence for detection of autoantibodies (author's transl)].

Seven laboratories made a chessboard titration with a serum containing antimitochondrial antibodies and two conjugates labeled with fluorescein isothiocyanate. The variation of the plateau titer and the titer of the serum as well as the plateau-end point was slight with the exception of one case. The standardization of indirect immunofluorescence needs a close contact between the laboratories, the availability of reference sera and conjugates, and similar microscopic equipment.

Autoantibodies↗
PubMed1980

[Immunohistochemical detection of antibodies against smooth muscle (author's transl)].

Smooth muscles antibodies are heterogeneous. Their fluorescent staining patterns and their antigens are not exactly known. The author's own experiences are based on 170 smooth muscle antibodies, which were tested by means of indirect immunofluorescence for their binding to rat stomach, liver, kidney, heart, and bovine skeletal muscle, further to human lymphocytes (only 10 cases were tested). 123 different fluorescent patterns and 21 combinations of various organs were found. Few unusual patterns were demonstrated. There was evidence that antibodies with individuum specificity exist, e.g. antibodies reacted only with the muscularis mucosae of the stomach. Possibilities of errors were discussed shortly. Smooth muscle antibodies reacting with stomach, liver, kidney (especially glomerular mesangium), and heart were more frequent in liver diseases than in non liver diseases (ratio about 4 : 1). Antibodies reacted with smooth, heart, and skeletal muscle were most anti-skeletal antibodies cross-reacted with smooth muscle.

Animals↗
PubMed1980

[The significance of electron microscopy for the assessment of membranous glomerulonephritis (author's transl)].

In 35 biopsy specimens taken from 21 patients with membranous glomerulonephritis (mGN) the ultrastructure of the glomeruli was investigated with special regards to the degradation of the immune complexes. Electron microscopy allows the most comprehensive assessment of the manifold basement membrane changes. This is of high value for the practical diagnosis.

Antigen-Antibody Complex↗
PubMed1980

[immunohistologic findings of kidney biopsies in Wilson's disease].

12 patients with Wilson's disease were examined by means of renal biopsy. In 9 of these patients a slight (4 patients) or a strong proteinuria (5 patients) appeared during the therapy with D-penicillamine. A biopsy was performed in 3 patients before the beginning of the therapy with D-penicillamine. The patients with severe proteinuria showed the largest immunohistological changes (partly distinct immune complex nephritis with epimembranous IgG- and complement-(C3-) depositions -- electronoptically membranous glomerulonephritis. In rebiopsy after a stoppage of the medicament of 1 year only a slight tendency to improvement was to be recognized.

Biopsy↗
PubMed1979

[Autoantibodies in arteriosclerosi and endangiitis obliterans].

10 of 14 patients with obliterating endangiitis and 2 of 17 patients with obliterating arteriosclerosis had in the serum immunoflorescence-optically provable low titre antibodies against different constituents of smooth musculature (once cross-reacting with transverse and cross striation of the myocardium). Further antibodiies against brush border of proximal renal tubuli, parietal cells, cardiac sarcolemma, cytoplasm of the liver and distal renal tubuli were found. Antibodies against nuclear material, skeletal musculature, further typical antibodies against mitochondria, ribosomes and membranes of the endoplasmatic reticulum as well as so-called pemphigus and pemphigoid antibodies could not be proved. The proof of low titre antibodies against constituents of smooth musculature is in this case regarded as an unspecific marker of an (often) viral inflammation.

Arteriosclerosis Obliterans↗
PubMed1979

[Atypical antibody against liver cell cytoplasm in virus induced autoimmune hepatitis (author's transl)].

A high titer (1:500) antibody against liver cell cytoplasm was found in one case of atypical viral hepatitis. The central areas of the rat liver lobule react more intensive than the periphery. Inconstantly it was found a reaction also with the parietal cells of the stomach, with the 3rd segment of proximal rat kidney tubules, and with smooth muscle. Possibly, this antibody is a marker of a virus induced autoimmune hepatitis.

Animals↗
PubMed1979

[Serious side effects with D-penicillamine therapy for Wilson's disease].

Immunocomplex nephritis as one of the serious side-effects is dealt with on the basis of 41 patients with Wilson's Disease who have been treated for many years and were stabilised on D-penicillinamine. In one quarter of the patients, proteinuria was found 1 to 5 years after the beginning of the therapy. Until now, an immunocomplex nephritis with diffuse granular, mainly epimembranous IgG and C3 deposits on the glomerular basement membran was found in four patients. No circulating antibodies against cell nuclei were found. The finding of immunocomplex nephritis calls for the discontinuation of the therapy for an at present unknown period of time.

Basement Membrane↗
PubMed1979

[Some problems of Wilson's disease].

The Leipzig Center for Wilson's Disease in the GDR is charged with the registration and diagnosis of all homozygous Wilson gene carriers, the clarification of all suspected cases, including the heterozygote test, and the co-ordination of long-term treatment. At present, there are 78 recorded Wilson gene carriers living. On the basis of our own comprehensive observations and investigations over prolonged periods of time, questions concerning pathogenesis, genetics, diagnosis and therapeutic measures, including their side-effects, are dealt with.

Adolescent↗
PubMed1978

[About the differentiation of antibodies against the connective tissue (author's transl)].

Unfixed cryostat sections of an organ block comprising the liver, stomach, and kidney of rats as well as the kidney of mice were used as antigenic substrate for detecting antibodies against connective tissues by indirect immunofluorescence. There were found more than 14 different fluorescent patterns. Two types could be distinguished easily: The "type 1" shows a clear fluorescence of fine perivascular fibres and a faint fluorescence of vascular endothelium in the liver and in the kidney. The sinusoids are negative. The "type 2" is characterized by a bright and broad fluorescence of the sinusoids and of the portal tract without perivascular staining of the liver. On the sections of the kidney a fluorescence of vascular endothelium, of the tubular basement membrane or of the peritubular connective tissue fibrils is typical.

Adult↗
PubMed1977

[Standardized indirect immunofluorescence. Differentiation of mitochondrial, microsomal and ribosomal antibodies].

By an extensive standardisation of the indirect immunofluorescence for the demonstration espeially of mitochondrial antibodies we succeeded in recognizing atypical fluorescence patterns and in describing their exact localisation. On the basis of absorption studies with mitochondrias, microsomas and ribosomas by comparative observation of sections of liver, stomach and kidneys of rats the preferred sort of reaction and the intensity of fluorescence of antibodies against mitochondria, microsomas and ribosomas were empirically established. Antimitochondrial antibodies react above all with the parietal cells of the stomach and the distal epithelia of the tubulus of the kidney. Antibodies against microsomas of liver and kidney are characterized by a brilliant diffuse cytoplasmatic fluorescence of the hepatocytes and by a comparatively weaker fluorescence of exclusively proximal tubuli of the kidneys of rats. Antibodies against ribosomas lead to a fluorescence especially of the main cells of the stomach. The differentiation of several cytoplasmatic antibodies is among others of interest for the diagnosis of certain autoimmune diseases. Although there are numerous still unclear findings and "overlap" phenomena the existence of high titre antibodies against mitochondrias speaks for a primarily biliary cirrhosis or a pseudo-LE-syndrome, the existence of antibodies against microsomas of kidney and liver of rats for a special form of a chronically active hepatitis and the existence of the very rare antibodies against ribosomas for an active lupus erythematodes disseminatus.

Animals↗
PubMed1977

[Immunopathogenetic aspects of chronic hepatitis].

In a short survey aspects to the immunopathogenesis of chronic hepatitides are explained. Hereby, partly critically, humoral and cellular immunophenomena are discussed, furthermore, the author deals with genetic factors, viruses and drugs as a cause of autoimmune hepatitides. Finally a simple working hypothesis to the pathogenesis is formulated and it is tried to classify the chronic aggressive hepatitis into six sub-groups on the basis of immunological, clinical and biochemical criteria: 1. much HBAg-associated, 2. lupoid, 3. drug-induced, 4. autoimmune cholostatic mixed form, 5. much associated with Ak to endoplasmatic reticulum and 6. undefined.

Antibodies, Antinuclear↗
PubMed1977

The immunoelectron-microscopical demonstration of antibodies against endoplasmic reticulum (microsomes) in chronic aggressive hepatitis and liver cirrhosis.

Electron microscopic findings made on microsomal fractions of rat liver after treatment with microsomal antibodies obtained from the blood of patients diagnosed as having chronic aggressive hepatitis and liver cirrhosis and after incubation with peroxidase-labelled antihuman IgG are described and presented in this paper. Interpretation of these findings as the substrate of an antibody reaction directed against membranes of the endoplasmic reticulum of hepatocytes is discussed.

Antibodies↗