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Biomedical subjects

M Beck

Publications and source records attributed to M Beck.

At least 199 records · Page 11Linked to original sources
PubMed1995

Inter- and intrafamilial variability in mucolipidosis II (I-cell disease).

In this paper nine patients with mucolipidosis II (I-cell disease) are described. They had clinical features commonly found in mucolipidosis II, including disproportionate dwarfism, coarse facial features and mental retardation. However, there was remarkable variability in age of onset, organ manifestation and radiological findings. Some had unusual clinical symptoms including pericardial effusion and profound brain atrophy. Striking differences in phenotypic expression were also seen in two affected siblings. Clinical heterogeneity is observed not only in mucolipidosis II but also in many other lysosomal storage disorders. The factors that may contribute to this clinical diversity are discussed.

Age of Onset↗
PubMed1995

[In-vitro stability of rotator cuff repair techniques].

Rotator cuff repair techniques were examined in vitro. First, the mechanical properties of nine different tendon-grasping techniques were compared using 159 normal infraspinatus sheep tendons. The clinically most frequently used simple stitch and mattress suture failed at low-to-moderate loads (two stitches, 184 N and 269 N) with the sutures pulling out of the tendons. A modification of the Mason-Allen grasping technique improved the ultimate tensile strength to 359 N (two stitches) without allowing relevant gap formation. Augmentation with synthetic materials did not improve the mechanical properties of the tendon-grasping techniques tested. The mechanical properties of different anchoring techniques to bone were assessed using osteoporotic bone specimens with mechanical properties comparable to those of proximal humeri with long-standing cuff defects. Single as well as double transosseous suture fixation (139 N and 146 N) and suture anchor fixation (142 N) were weak. Tying the knots over a plate-like cortical bone augmentation device improved the failure strength to 329 N. Some of these data were presented in the English literature in 1994. Considering the excellent preliminary experience in the clinical application of the modified Mason-Allen grasping technique and cortical augmentation, it seemed appropriate to present them in German as well.

Animals↗
PubMed1995

Family violence.

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Adult↗
PubMed1995

[Violent patients. A problem for the therapist in psychiatric clinics?].

At four psychiatric hospitals, 128 doctors, 50 psychologists and 55 social workers were asked to complete a questionnaire about their experience with aggressive patients. Fifty-five percent reported having been assaulted seriously during their career; physical assaults during the last year had occurred for 29%. Forty-nine percent had experienced a situation judged as fairly or very dangerous. Women were concerned less than men, social workers less than psychologists and doctors.

Aggression↗
PubMed1995

Role of larval cadavers in recycling processes of Bacillus sphaericus.

The influence of larval cadavers of Culex pipiens on recycling processes of Bacillus sphaericus was investigated by bioassays and spore counts in the laboratory. Studies conducted with 3 different B. sphaericus concentrations (0.005, 0.01, 0.05 mg B. sphaericus/liter) indicated that the presence of cadavers in the water contributed to the maintenance of toxic levels of B. sphaericus. Larval cadavers seem to contain all the nutrients necessary both for vegetative multiplication and for toxin synthesis associated with the sporulation process. Bioassays of B. sphaericus revealed that the mortality of Culex pipiens remained on a high level over a period of 26 days when larval cadavers were added every second day to the test vessels. This result was supported by a sharp increase in spore density when cadavers were added at the same interval. The test series showed B. sphaericus recycles in intact cadavers of Culex pipiens, whereas this phenomenon could not be observed when crushed cadavers were used in the trials. Therefore, our results demonstrated that for successful recycling processes it seems of crucial importance that infected cadavers remain intact at least for a certain time and also that the dosage of the applied B. sphaericus plays a major role in recycling processes whereas larval density is only of minor importance to these processes.

Animals↗
PubMed1994

Proteoglycan synthesis by cultured human chondrocytes.

Iliac crest biopsies are important in the detection of human skeletal dysplasias. Therefore, culture of these cells may serve as a valuable method for studying proteoglycan metabolism in chondrocytes of individuals with skeletal abnormalities. Morphological and biochemical studies were performed on human iliac crest chondrocytes grown in monolayer and in agarose gels. Two proteoglycan populations of different hydrodynamic size and glycosaminoglycan composition were synthesized by cells grown in monolayer. Chondrocytes cultured in an agarose gel for 2 weeks synthesized proteoglycans identical to those of the native tissue with respect to hydrodynamic size and glycosaminoglycan chain length. However, the ratio of chondroitin-6-sulfate to chondroitin-4-sulfate was higher than in the native tissue. This ratio was not influenced by different sulfate concentrations in the medium. Moreover, treatment with ascorbic acid did not influence proteoglycan synthesis; however, there was a pericellular accumulation of proteoglycans.

Ascorbic Acid↗
PubMed1994

Managing the mind.

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Health Care Reform↗
PubMed1994

Prenatal diagnosis and carrier detection in mucopolysaccharidosis type II by mutation analysis. A 47,XXY male heterozygous for a missense point mutation.

Identification of iduronate-2-sulphatase (IDS) gene mutations in patients with mucopolysaccharidosis type II (MPS II, Hunter syndrome) allows fast and reliable carrier detection and prenatal diagnosis. We describe here three cases of prenatal diagnosis by direct detection of the gene mutation. In addition to two affected male fetuses from two different families, a 47,XXY fetus carrying both the normal and the mutant allele was diagnosed in a third family. The latter pregnancy was carried to term and the child is obviously not affected by MPS II.

Alleles↗
PubMed1994

Riedel's thyroiditis associated with high titers of antimicrosomal and antithyroglobulin antibodies and hypothyroidism.

Riedel's thyroiditis is a rare, chronic inflammatory disease of the thyroid gland. The aggressive fibrosis with extension beyond the thyroid into adjacent tissues contrasts with the diffuse, but intracapsular fibrosis of Hashimoto's thyroiditis. Most current studies refute the possibility of progression from a highly fibrosing form of Hashimoto's thyroiditis to a Riedel's thyroiditis based on the distinct clinical and laboratory data, although an unknown immunological basis is suggested for both diseases. The authors describe a patient with Riedel's thyroiditis, probably associated with Hashimoto's thyroiditis, sent to surgery because her cytological examination suggested thyroid malignancy. This patient had clinical and laboratory features of hypothyroidism and very high titers of antimicrosomal and antithyroglobulin antibodies, which decreased after surgery. Pathology studies disclosed Riedel's thyroiditis with intense lymphocytic infiltration suggestive of Hashimoto's thyroiditis. Quantitative immunohistochemical studies were not able to distinguish between both diseases.

Adult↗
PubMed1994

Mucopolysaccharidosis type I: identification of 8 novel mutations and determination of the frequency of the two common alpha-L-iduronidase mutations (W402X and Q70X) among European patients.

A group of 46 European patients with mucopolysaccharidosis type I (MPS I) was screened for mutations of the alpha-L-iduronidase gene. The 2 common nonsense mutations, W402X and Q70X, were identified in, respectively, 37% and 35% of mutant alleles. Considerable differences were seen in the frequency of these 2 mutations in patients from North Europe (Norway and Finland) and other European countries (mainly The Netherlands and Germany). In Scandinavia, W402X and Q70X account for 17% and 62% of the MPS I alleles, respectively, while in other European countries W402X is about 2.5 times more frequent (48%) than Q70X (19%). Eight novel mutations are described including 4 missense mutations, 1 nonsense mutation, 1 insertion of 2 base pairs, and 2 deletions of 1 and 12 base pairs.

Alleles↗
PubMed1994

RTX toxin genotypes and phenotypes in Actinobacillus pleuropneumoniae field strains.

Actinobacillus pleuropneumoniae serotype reference strains and 204 A. pleuropneumoniae field strains representing all 12 serotypes and both biovars 1 and 2, obtained from laboratories from various countries worldwide, were analyzed for the presence of the toxin genes apxIC, apxIA, apxIB, apxID, apxIIC, apxIIA, apxIIIC, apxIIIA, apxIIIB, and apxIIID by DNA-DNA hybridization with specific gene probes. Expression of the toxins ApxI, ApxII, and ApxIII was assessed by immunoblot analysis with monoclonal antibodies. The results show that the patterns of apx genes and those of the expressed Apx toxins in biovar 1 field strains are the same as those of the genes and toxins of corresponding serotype reference strain. We found only three strains which had certain apx genes missing compared with the genes in their serotype reference strains. Analysis of the expression of the three toxins showed that nearly all strains expressed their apx genes and produced the same Apx toxins as their serotype reference strain. We found only one strain that did not produce ApxI, although it contained the apxICABD genes, and one strain which did not express ApxII but which contained apxIICA. Several field strains which initially showed that their serotype did not correspond to the apx gene profile of the reference strain and which had an unexpected virulence for the given serotype revealed that their initial serotyping was erroneous. We show that the apx gene profiles are inherent to a given serotype. The method cannot differentiate between all 12 serotypes. However, it allowed us to distinguish five groups of toxin gene patterns which showed pathological, toxicological, and epidemiological significance. None of the biovar 2 strains contained apxIII genes. The apxI and apxII genes in the biovar 2 strains, however, were the same as those found in the serotype reference strains of biovar 1.

Actinobacillus pleuropneumoniae↗