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Biomedical subjects

W Müller

Publications and source records attributed to W Müller.

At least 703 records · Page 39Linked to original sources
PubMed1983

alpha 1-Antitrypsin (Pi) types and subtypes in the Tyrolean population.

Since nine patients with infantile liver cirrhosis or hepatopathy associated with the Pi ZZ phenotype had been observed in recent years in the Children's Hospital of the University of Innsbruck, Tyrol, the distribution of the Pi types and the PiM subtypes was determined in the Tyrolean population. Apparently healthy blood donors (868) from different regions of Tyrol were examined. Isoelectricfocusing was used for classification of Pi types. The frequency of the allele PiZ was 0.0138, which corresponded to the range observed in other Middle European populations. The frequencies for the suballeles of PiM were PiM1 = 0.7062, PiM2 = 0.1480, and PiM3 = 0.1037. PiS had a frequency of 0.0225, the other rare alleles occurred with a combined frequency of 0.0058.

Alleles↗
PubMed1983

Supratentorial lobar ependymomas: reports on the grading and survival periods in 80 cases, including 46 recurrences.

We report 80 supratentorial lobar ependymomas, including 46 recurrences. All underwent operation. In the total collection 34.5% survived for more than 5 years. The behaviour of children and adults was nearly identical. Based on a histological grading into three steps of malignancy (E1, E2, E3), the ependymomas in low grade 1 showed a 5 years' survival time of 41.5%. Compared to a 28.5% survival in group E2 at the same time. Results in group E3, which histologically resembled malignant glioma or glioblastoma, are not relevant because of the small number of cases. More than 50% of the E1 recurrences showed transformation to E2. No transformation into a lower grade of malignancy has been observed.

Adolescent↗
PubMed1983

Characterization of blood mononuclear cells of rheumatoid arthritis patients. II. Depressed PPD presentation by monocytes to T lymphocytes.

Purified blood monocytes from patients with rheumatoid arthritis (RA) were significantly less capable of presenting purified protein derivative of tuberculin (PPD) to autologous lymphocytes than monocytes from patients with osteoarthritis, degenerative spine diseases, or healthy controls. Since lymphocytes from RA patients exhibited a normal response to soluble PPD or concanavalin A, the lowered T-cell reactivity had to be attributed to a diminished antigen-presenting capacity of monocytes. Several reasons may be responsible for this altered monocyte function in rheumatoid arthritis: a shift of monocytes to subpopulations expressing less Ia-like antigens, an inherent monocyte disorder, or a "preactivation" of monocytes associated with a reduced antigen-presenting capacity.

Adult↗
PubMed1983

Oral immunization to milk protein in human infants in the presence of passive antibody.

The possible influence of maternal antibody on the immune response to bovine serum albumin (BSA), a normal cow's milk protein, was investigated in fullterm human neonates. Antibody production to BSA of 12 infants with passively acquired anti-BSA (Group I) was compared to the immune response of nine infants without passive anti-BSA at birth (Group II) during the first 6 months of life. All infants were raised on commercial cow's milk formulas containing BSA in concentrations from 0.4-4.0 mg/dl. From 4 wk of age concentrations of circulating anti-BSA as measured by radioimmunoassay and enzyme-linked-immunosorbent-assay (ELISA) were higher in Group II, but differences were not statistically significant. There was no differences in the immune response between infants ingesting formulas with high BSA content compared to infants ingesting low concentrations of antigenic BSA. The main isotype associated with anti-BSA formation was IgG. IgA in measurable amounts appeared later and accounted for approximately 10% of circulating antibody in both groups at 6 months of age. Ony small amounts of IgM- and IgE-anti-BSA were detected.

Animals↗
PubMed1983

NK cell function in severe combined immunodeficiency (SCID): evidence of a common T and NK cell defect in some but not all SCID patients.

The immunologic work-up of eight infants with the clinical diagnosis of severe combined immunodeficiency (SCID) was performed with special emphasis on natural killer (NK) cell function and ontogeny. Contrary to previous reports, our study shows that not all SCID patients lack NK activity; some may even express very high NK- and antibody-dependent cellular cytotoxicity (ADCC). The present group of eight SCID infants was homogeneous with respect to normal levels of the purine metabolism enzymes adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP). They all had low serum Ig levels and were defective for specific antibody formation against BSA and diphtheria toxin (DiT). None of the infants' peripheral blood mononuclear cells (PBMC) proliferated significantly upon in vitro stimulation with PHA, concanavalin A (Con A), pokeweed mitogen (PWM), and irradiated allogeneic lymphocytes. Seven of eight patients, however, responded significantly to mitogenic factors present in a lectin-free interleukin 2 (IL 2) preparation, and two exhibited a positive costimulation as well with simultaneous exposure to IL 2 + Con A. The lymphocyte marker analysis revealed high percentages of OKT10+ cells in seven of eight infants, whereas peripheral T cells (OKT3+) with suppressor/killer (OKT8+) or helper/inducer (OKT4+) phenotypes were abnormally low in all infants with one exception. The PBMC of two patients formed low to normal percentages of E rosettes but expressed no B cell markers (B-/SCID). The six other infants had high percentages of B cells (B+/SCID) but lacked E rosette-forming cells. High NK and ADCC activity was found in the two B-/SCID patients. The B+/SCID infants either totally lacked NK and ADCC function (four of six) or expressed low to normal NK activity together with some T cell markers as revealed by monoclonal antibody staining but not by E rosette formation (two of six). From the data presented, an ontogenic model is proposed that assumes the status of an independent cell lineage in between T cells and monocytes for human NK cells, or that places these cells in close proximity to early differentiation steps of the T cell lineage. In any case, NK cell function clearly constitutes an additional parameter of heterogeneity in the immunologic analysis of SCID.

Adenosine Deaminase↗
PubMed1982

[Cartagener syndrome. Pathogenesis and therapy].

Kartagener's syndrome (situs inversus totalis, bronchiectasis and sinusitis) was demonstrated in two patients. Electron microscopic investigations of the bronchial epithelium showed an accidental arrangement of ciliary axes, but not the expected lack of dynein arms. In both cases severe disturbance of maturation of spermiogenesis, not noted in this syndrome so far, was observed. Whereas one patient is largely free of symptoms after two operations, the other died after the second operation as a consequence of bronchopleural fistulation and bilateral pneumonic complications.

Adult↗
PubMed1982

[Seronegative rheumatic spondyloarthropathies (spondarthritis)].

The group of seronegative rheumatic spondylarthropathies (spondarthritis) includes different diseases with common features. The common characteristics of these diseases are sacroiliitis and/or spondylitis, usually oligoarticular arthritis, distinct extraarticular manifestations of the skin, mucous membranes, eyes and internal organs, and a common genetic disposition. To differentiate the diseases within this group, of which spondylitis ankylosans counts as the prototype, allowance must be made for radiomorphological changes of the spine, the various joints involved and the type of extraarticular symptoms. The therapy depends upon the type of disease, although the therapeutic principles remain the same as far as the spine is concerned; pain treatment and conservation of spine mobility are the main factors.

Arthritis, Juvenile↗
PubMed1982

[Synoviorthese].

Among the various possibilities for local treatment of rheumatoid synovitis, intra-articular injection of corticosteroids has only a very temporary effect. The same can be said of local treatment with cytostatics, though the effect is somewhat longer-lived. After intra-articular treatment with 1% osmic acid, joint effusions in particular remain under control over a lengthy period of time. The most effective conservative local treatment appears to be radionuclide therapy, and in older patients it may be a true alternative to surgical synovectomy. Radionuclides with varying physical properties are administered according to the size of the joint. The dosage of these substances also depends on the size of the joint. If precautions are taken, side effects are rare.

Adrenal Cortex Hormones↗