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Biomedical subjects

A Weber

Publications and source records attributed to A Weber.

At least 253 records · Page 14Linked to original sources
PubMed1995

Adrenocorticotropin receptor gene mutations in familial glucocorticoid deficiency: relationships with clinical features in four families.

Familial glucocorticoid deficiency is an autosomal recessive syndrome of adrenal unresponsiveness to ACTH characterized by glucocorticoid deficiency, high plasma ACTH levels, and a normal renin-aldosterone axis. Defects of the ACTH receptor have been suggested as a possible cause, and we have previously reported a number of novel mutations of the ACTH receptor gene in some, but not all, cases, suggesting that familial glucocorticoid deficiency may have a heterogeneous molecular etiology. Here we report the clinical features and ACTH receptor gene analysis in four patients from different families. We found that two patients were compound heterozygotes for the S74I and R128C mutations (patient A) and I44M and L192fs frame shift mutations (patient B). The other two patients (C and D) were of different ethnic ancestry, but were both homozygous for a R146H mutation. Segregation studies within families revealed heterozygosity in the parents and several other family members. Human CRH tests in the parents of patients A and B showed normal cortisol and ACTH responses in the S74I, R128C, and I44M heterozygotes and exaggerated cortisol and ACTH responses in the L192fs heterozygote, suggesting that the physiological ACTH increment induced in this test did not reveal evidence of subclinical ACTH resistance, and that this test may not be of value in ascertaining heterozygosity.

Adrenocorticotropic Hormone↗
PubMed1995

Fibrous dysplasia of the temporal bone: imaging findings.

Lichtenstein [1] in 1938 coined the term fibrous dysplasia to describe a disorder characterized by the progressive replacement of normal bone elements by fibrous tissue. Histopathologically, these lesions consist of an abnormal proliferation of fibrous elements intermixed with haphazardly arranged trabeculae of woven bone. The disease can involve any bone in the body. In the head and neck, the skull and facial bones are involved in 10-25% of cases of monostotic fibrous dysplasia and in 50% of the polyostotic variety. Involvement of the temporal bone, however, is relatively rare, and only 53 cases have been reported. The three major radiographic classifications of fibrous dysplasia are pagetoid, sclerotic, and cystic. Any of these types may involve the temporal bone and related structures, including the external canal, middle ear, jugular foramen, or, rarely, the otic capsule. In this essay, we illustrate the radiographic features of the disease based on our experience with seven cases, seen at our institution since 1977, of fibrous dysplasia involving the temporal bone.

Adult↗
PubMed1995

Comparison between high dose 5-aminosalicylic acid and 6-methylprednisolone in active Crohn's ileocolitis. A multicenter randomized double-blind study. German 5-ASA Study Group.

BACKGROUND: The value of 5-aminosalicylic acid (5-ASA) in Crohn's disease (CD) is still under discussion. In a previous study 2 g 5-ASA per day were inferior to a standard glucocorticoid treatment with 6-methylprednisolone (6-MPred) (Can J Gastroenterol 1990; 4: 446-51). In the present study we tested whether in active CD response rates to 4.5 g 5-ASA/day were not different from those to 6-MPred. METHODS: Multicenter randomized double-blind double-dummy trial. 34 patients with active CD (CDAI > 150) were included. 17 patients were in the 5-ASA group (Salofalk, 4.5 g/day), 17 patients in the 6-MPred group (Urbason, initial dose 48 mg/day, weekly tapering). Duration of treatment was 8 weeks. Main outcome measure was remission of CD (CDAI < 150) and decrease of at least 60 points. RESULTS: Both groups were comparable with respect to demographic and clinical parameters. The median CDAI decrease in the 5-ASA group was 85, in the 6-MPred group 122 (p = 0.7437). The median AUC of the CDAI in the 5-ASA group was 1027, in the 6-MPred group 950 (p = 0.137). The median AUC of the CDAI per treatment day was 22.94 in the 5-ASA group, and 17.33 in the 6-MPred group (p = 0.0555). On an intention-to-treat basis remission rates after 8 weeks were 40.0% in the 5-ASA group and 56.3% in the 6-MPred group (p = 0.5867). CONCLUSIONS: Response rates to 5-ASA or 6-MPred were not significantly different although there was a trend towards a higher efficacy of 6-MPred. 5-ASA may be considered as alternative treatment in patients with activer CD who are intolerant to or refuse glucocorticoids.

Adult↗
PubMed1995

[Providing practice-oriented social medicine in medical education--a lecture concept].

This article outlines the concept on which lectures in Social Medicine offered at the Institute for Occupational, Social and Environmental Medicine of the University of Erlangen-Nuremberg for the past three years are based. In addition to the fundamentals of Social Medicine, in our lectures we impart practical and career-oriented knowledge of the social security system and socio-medical diagnostics and how to compile medical assessments. We thus try to make it easier for the prospective physicians to later give help and advice to their patients about gaining social welfare benefits. Additionally we instruct the students in the basics of preventive medicine and the economics of medical activity. Consequently they recognise the importance of these topics for their later work in a clinic or as general practitioners. Our concept is in accordance with the intended modification of the German legislation governing the permission of doctors to practise medicine. Improvements are planned which will gear medical training better with the requirements of a medical practitioner as well as offering more intensive instruction in preventive medicine and the economy aspects of health care.

Curriculum↗
PubMed1995

The association of occupational asbestos dust exposure and laryngeal carcinoma.

Controversy exists as to whether occupational asbestos dust exposure is responsible, or at least partially responsible, for the development of laryngeal carcinoma. The aim of this study is to present the current scientific knowledge on this subject based on a comprehensive literature research and to critically review the epidemiological investigations published. The results of 31 cohort studies and 24 case-control studies, and the conclusions in 11 more recent reviews are contradictory. In most studies there was no statistically significant indication of a casual relationship. It is noteworthy that an increased risk of laryngeal carcinoma among persons exposed to asbestos dust was observed mostly in older studies in which smoking habits and/or alcohol consumption as the most important nonoccupational risk factors were usually not taken into account. In addition, since most of the calculated positive associations are very weak, no reliable conclusion can be reached. While there may be a causal relationship between occupational exposure to asbestos dust and the occurrence of laryngeal carcinoma, this cannot be regarded as certain on the basis of the evidence reviewed.

Asbestosis↗
PubMed1995

Treatment of active Crohn's ileocolitis with oral pH-modified budesonide. Germany Budesonide Study Group.

Budesonide is a topical steroid which after intestinal absorption is rapidly degraded into inactive metabolites in the liver. Its systemic bioavailability is only 10-15%. In the present open trial the efficiency and safety of oral pH-modified release budesonide were assessed in patients with active Crohn's ileocolitis. This report describes the results of the first 30 of 78 patients. After 6 weeks of treatment with 3 x 3 mg budesonide/day 67% of the patients were in clinical remission. Typical steroid-related side effects were observed in only one patient. Budesonide therefore seems to be suited as an alternative for classical steroids in patients with Crohn's ileocolitis. Its clinical efficacy is comparable with classical steroids but it's rate of steroid-related side effects is lower.

Administration, Oral↗
PubMed1995

Polycystic disease of the parotid glands.

A 31-year-old woman had bilateral swelling of the parotid glands at 4 months of pregnancy. MR imaging showed marked enlargement of the parotid glands with increased signal on images with long repetition times. A diagnosis of polycystic disease of the parotid gland was made after biopsy and histologic examination. The radiographic and histologic features of this rare disease are discussed.

Adult↗
PubMed1995

[Individual medical diagnosis in clinical environmental medicine--references for single-case risk analysis].

In the general population there is an increasing tendency to attribute health damage to exposure to hazardous substances in the environment. With the increasing demand for consultation and care there is at present a growing need for doctors specialised in environmental medicine. In the Federal Republic of Germany environmental medicine is represented in particular by environmental hygiene and clinical environmental medicine. Clinical environmental medicine can be characterised as being halfway between care and clinical diagnosis with emphasis on secondary prevention (early recognition of any disturbance of health). The discipline is geared to the individual and this means that the diagnostic strategy and risk evaluation must be considered individually for every case. In addition to scientific facts of environmental toxicology and epidemiology, fundamental medical and pathophysiological knowledge must also come into play. A scientifically founded medical appraisal clarifying possible negative effects on health due to hazardous substances in the environment is presented, based on long-standing experience gathered during consultation work on environmental medical problems at the Institute for Occupational, Social and Environmental Medicine of the University of Erlangen-Nuremberg. Focus is mainly on the effects of chemicals. To evaluate the individual risk, an analytical procedure of many steps is required which includes in particular the collection of anamnestic data specific to the problem, objectifying and quantifying of exposure to hazardous substances and/or specific biological effect reactions, limitation of competing influences and an overall medical assessment of environmental toxicity.

Environmental Exposure↗
PubMed1995

[The occurrence of Listeria monocytogenes in fecal samples of pigeons].

Listeria spp. were isolated from faeces samples from 12 (3.4%) of 350 pigeons examined. Three isolates were identified as L. monocytogenes (1x serovar 1/2a, 1/2b, and 4ab), eight isolates were L. innocua (2x serovar 6a, 6x serovar 6b), and two isolates L. seeligeri (serovar 6b). One fecal sample yielded a mixed culture of L. monocytogenes and L. innocua. The results indicate that pigeons are not important in the epidemiology of listeriosis in man and animals.

Animals↗
PubMed1995

[Treatment of chronic diarrhea in dogs and cats under field conditions using oral E. coli vaccines].

An autogenous oral E. coli vaccine was tested for efficacy in the treatment of chronic diarrhoea in dogs (n = 82) and cats (n = 50) under field conditions. The data were collected through evaluation of questionnaires completed and returned by veterinarians. After oral application of the E. coli vaccine the symptoms of diarrhoea were stopped in 71% of the treated animals within two to five days. In further 15% of the cases the enteric symptoms continued but were not so severe as in the beginning of the treatment. There was no therapeutic success with the oral E. coli vaccine in further 14% of the animals in cause of food allergy, ascariasis, volvulus or pancreatic insufficiency.

Animals↗
PubMed1995

[Occurrence of Salmonella in fecal samples of dogs and cats in northern Bavaria from 1975 to 1994].

103 (3.45%) of 2985 fecal samples from dogs respectively 39 (1.92%) of 2024 fecal samples from cats bacteriologically examined during the years 1975 to 1994 yielded Salmonellae. The 103 Salmonella strains of dogs belonged to 32 different serovars and in the cats the 39 strains found represented a total of 17 different serovars. The most frequently detected serovar was S. typhimurium including var. copenhagen, with 33% in the dogs and 49% in the cats.

Animals↗
PubMed1995

Activation of Moloney murine leukemia virus LTR enhances the titer of recombinant retrovirus in psi CRIP packaging cells.

The critical physiological function of the long terminal repeat in Moloney murine leukemia virus (MoMLV) gene expression has been thoroughly explored and shown to include binding sites for ubiquitous and tissue-specific transcription factors, such as the glucocorticoid responsive element (GRE) and the LVb sequence recognized by phorbol 12-myristate 13-acetate (TPA)-induced factors. The present study was undertaken to determine the effect of different activators, known to enhance expression of MoMLV, on their ability to modulate retroviral transcripts in psi CRIP producing cell lines. Improvement of recombinant retrovirus production by two psi CRIP producer cells was tested by using dexamethasone, TPA and sodium butyrate (Na-But) alone or in combination. We demonstrate that 5 mM Na-But, or 5 mM Na-But plus 1 microM dexamethasone significantly enhanced viral production. These compounds could induce a 10-fold increase in viral production. There was also a good correlation between the increased viral production and the titer measured after transduction of NIH 3T3. This improvement is of general interest, since a major goal for gene therapy is the production of high retroviral titer for increased transduction efficiency.

3T3 Cells↗
PubMed1995

[Studies on the occurrence of Listeria monocytogenes in fecal samples of domestic and companion animals].

Listeria (L.) monocytogenes was isolated from 33.3% fecal samples of 138 cattle (29 strains serovar 1/2b, 10 strains serovar 1/2a, 7 strains serovar 4ab), from 8% fecal samples of 100 hens (5 strains serovar 1/2b, 1 strain serovar 1/2a, 4ab and 4b each), from 8% fecal samples of 100 sheep (6 strains serovar 1/2a, 1 strain serovar 1/2b and 4ab each), from 5.9% fecal samples of 34 pigs (2 strains serovar 4ab), from 4.8% fecal samples of 400 horses (7 strains serovar 1/2a, 6 strains serovar 1/2b and 4ab each), from 1.3% fecal samples of 300 dogs (3 strains serovar 1/2b, 1 strain serovar 4ab), from 0.9% fecal samples of 350 pigeons (1 strains serovar 1/2a, 1/2b and 4ab each), from 0.4% fecal samples of 275 cats (1 strain serovar 4b) and from 5 respectively 2 fecal samples of 35 tortoises (3 strains serovar 4ab, 2 strains serovar 4b) respectively 5 amphibia (1 strain serovar 4ab and 4e each). Further 36.9% fecal samples of cattle, 15% fecal samples of hens, 6% fecal samples of horses, 5.9% fecal samples of pigs, 5% fecal samples of sheep, 4% fecal samples of dogs, 2.3% fecal samples of pigeons, 3 fecal samples of 35 tortoises and 1 fecal sample of 260 rabbits contained L. innocua. In further 2% fecal samples of hens, in further 1.5% fecal samples of horses and in further 0.6% fecal samples of pigeons was detected L. seeligeri. 3 fecal samples of horses and 1 fecal sample of dogs, lizards and amphibia each contained L. welshimeri, while in 1 fecal sample of tortoises and in 3 fecal samples of 33 lizards L. ivanovii was detected.

Amphibians↗
PubMed1994

Porins from plants. Molecular cloning and functional characterization of two new members of the porin family.

Porins are voltage-gated diffusion pores found in all eukaryotic kingdoms. Here we describe, for the first time, the identification and characterization of two cDNAs encoding porins from plants. Peptide sequences obtained from a 30-kDa protein of envelope membranes from pea root plastids allowed the isolation of two cDNA clones from pea and maize. On the protein level, both proteins are homologous by 58%. Sequence comparison against the Swiss-Prot sequence data base revealed a homology of about 25% to mitochondrial porins from fungi and human. Computer-aided predictions of the secondary structure of the plant porins revealed the presence of 16 antiparallel beta-strands that are also found in mitochondrial porins. Porins from non-green plastids and from the outer mitochondrial membrane were reconstituted into planar lipid bilayers. The proteins showed high pore-forming activities and similar single-channel conductances. In vitro translated porin was preferentially imported only into non-green plastids but not into chloroplasts. To our knowledge, this is the first example of selective import of a plastid protein into different types of plastids. This finding is in line with the observation that an immunoreactive 30-kDa band was only found in non-green plastids and mitochondria but not in chloroplasts. We conclude that mitochondria and non-green plastids possess homologous porin proteins, whereas chloroplasts are characterized by a different type of porin.

Amino Acid Sequence↗
PubMed1994

Glucose uptake and metabolism in grr1/cat80 mutants of Saccharomyces cerevisiae.

Glucose repression in the yeast Saccharomyces cerevisiae designates a global regulatory system controlling the expression of various sets of genes required for the utilization of alternate carbon sources. In a screen, designed for the selection of mutants with reduced glycolytic flux we obtained isolates which were shown by complementation of the cloned wild-type gene to be allelic to the glucose repression mutants grr1/cat80/cot2 previously described. We demonstrate that the grr1 lesion lead to a concentration-dependent decrease in glycolytic flux on glucose. It is very likely that this is caused by a significant decrease in the expression of various genes encoding hexose transporters (HXT1,3) leading to a reduced glucose-uptake rate. In contrast, expression of the maltose permease gene (MAL11) and maltose utilization is normal. There is indirect evidence that grr1 affects the uptake of amino acids, and others have shown that the sugar-induced transport of divalent cations is impaired. These effects are not glucose-specific. We suggest that Grr1, a putative cytoplasmic protein, has a central function in the sensing of nutritional conditions for a variety of unrelated substances, and that relief from glucose repression may be a corollary of this defect in sensing.

Biological Transport↗