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Biomedical subjects

B Fiedler

Publications and source records attributed to B Fiedler.

At least 19 recordsLinked to original sources
PubMed2006

[Secondary malignant transformation of an enchondroma of the hand].

The discussion in literature about chondrosarcoma of the hand arising from solitary preexistent enchondroma is very controversial. Well-documented, proved cases are rare. Treatment of choice a 77-year-old woman with chondrosarcoma (Grade 2) of proximal phalanx of the right index finger was ray resection. The 43-year-old X-ray film showed typical enchondroma in diaphysis of this finger. We concluded a long-time period for malignant transformation of a benign enchondroma on hand skeleton localisation.

Aged↗
PubMed2005

SCN1A mutation analysis in myoclonic astatic epilepsy and severe idiopathic generalized epilepsy of infancy with generalized tonic-clonic seizures.

Severe myoclonic epilepsy in infancy (SMEI), severe idiopathic generalized epilepsy of infancy (SIGEI) with generalized tonic clonic seizures (GTCS), and myoclonic astatic epilepsy (MAE) may show semiological overlaps. In GEFS+ families, all three phenotypes were found associated with mutations in the SCN1A gene. We analyzed the SCN1A gene in 20 patients with non-familial myoclonic astatic epilepsy -- including 12 probands of the original cohort used by Doose et al. in 1970 to delineate MAE. In addition, 18 patients with sporadic SIGEI -- mostly without myoclonic-astatic seizures -- were analyzed. Novel SCN1A mutations were found in 3 individuals. A frame shift resulting in an early premature stop codon in a now 35-year-old woman with a borderline phenotype of MAE and SIGEI (L433fsX449) was identified. A splice site variant (IVS18 + 5 G --> C) and a missense mutation in the conserved pore region (40736 C --> A; R946 S) were detected each in a child with SIGEI. We conclude that, independent of precise syndromic delineation, myoclonic-astatic seizures are not predictive of SCN1A mutations in sporadic myoclonic epilepsies of infancy and early childhood.

Child↗
PubMed2001

Intraductal component in invasive breast cancer: analysis of 250 resected surgical specimens.

The presence of an intraductal component together with an invasive carcinoma is known to be associated with a higher rate of local recurrence. The results of reviewing 250 resected surgical specimens from patients with breast cancer are reported. Two-hundred and fifty mastectomy specimens of invasive breast cancer were retrospectively analysed in order to determine intraductal components within the primary tumour as well as additional foci. In addition to the invasive carcinoma, a ductal carcinoma in situ (DCIS) of varying extent was identified in 127 instances. The intraductal components were marginal in 27.6% of the cases, extensive in 61.4%, and predominant in 11.0%. In addition, 21 patients had isolated DCIS only. Such in situ components were more frequently found in the age group younger than 41 years and in premenopausal patients. Seventeen percent of carcinomas associated with an intraductal component were multicentric in location as opposed to only 5% of the breast lesions without an intraductal component. The highest proportion of residual tumour was seen in poorly differentiated invasive carcinomas with DCIS. Intraductal carcinomas with intraductal component tended to have a higher incidence of a positive surgical margin. Small carcinomas with an extensive in situ component require careful surgical management in order to achieve a tumour-free margin.

Journal Article↗
PubMed2000

The cardiac Fas (APO-1/CD95) Receptor/Fas ligand system : relation to diastolic wall stress in volume-overload hypertrophy in vivo and activation of the transcription factor AP-1 in cardiac myocytes.

BACKGROUND: Fas (APO-1/CD95) is a transmembrane receptor belonging to the tumor necrosis factor receptor superfamily. Cross-linking of Fas by Fas ligand (FasL), a tumor necrosis factor-alpha-related cytokine, promotes apoptosis and/or transcription factor activation in a highly cell-type-specific manner. The biological consequences of Fas activation in cardiomyocytes and the regulation of Fas and FasL abundance in the myocardium in vivo remain largely unknown. METHODS AND RESULTS: As shown by immunohistochemistry, Fas was expressed on the sarcolemma of cardiomyocytes in left ventricular tissue sections. Moreover, FasL was constitutively expressed in the myocardium and in isolated cardiomyocytes, as revealed by reverse transcription polymerase chain reaction and Western blotting. Left ventricular abundance of Fas but not FasL was upregulated in a rat model of compensated volume-overload hypertrophy and was closely related to diastolic but not systolic wall stress as determined by MRI. Cardiomyocyte apoptosis was not enhanced in volume-overload hypertrophy despite the increased expression of Fas and the presence of FasL in the myocardium. Moreover, injection of mice with an agonistic anti-Fas antibody promoted hepatocyte but not cardiomyocyte apoptosis in vivo. Stimulation of isolated cardiomyocytes with recombinant FasL promoted an activation of the transcription factor AP-1 as shown by electrophoretic mobility shift assays but did not induce cell death. CONCLUSIONS: Fas and FasL are constitutively expressed in the myocardium and in cardiomyocytes. Myocardial expression of Fas is closely related to diastolic loading conditions in vivo. Signaling pathways emanating from Fas are coupled to an activation of the transcription factor AP-1 in cardiomyocytes.

Animals↗
PubMed1998

Centrotemporal spikes in families with rolandic epilepsy: linkage to chromosome 15q14.

OBJECTIVE: To localize a gene predisposing to benign epilepsy of childhood with centrotemporal spikes (BECTS). BACKGROUND: BECTS, or rolandic epilepsy, is the most prevalent idiopathic epilepsy syndrome in childhood. Functional relevant defects in the alpha 4 subunit of the neuronal nicotinic acetylcholine receptor (AChR) have been demonstrated in autosomal dominant nocturnal frontal lobe epilepsy, which, like BECTS, is an idiopathic partial epilepsy. METHODS: A DNA linkage study was conducted screening all chromosomal regions known to harbor neuronal nicotinic AChR subunit genes. Twenty-two nuclear families with BECTS were analyzed. RESULTS: In an "affected-only" study, best p values and lod scores were reached between D15S165 and D15S1010 on chromosome 15q14. In multipoint nonparametric linkage analysis a nominal p value of 0.000494 was calculated by GENEHUNTER. Best parametric results were obtained under an autosomal recessive model with heterogeneity (multipoint lod score 3.56 with 70% of families linked to the locus). These markers are localized in direct vicinity to the alpha 7 subunit gene of the AChR. CONCLUSIONS: We found evidence for linkage of BECTS to a region on chromosome 15q14. Either the alpha 7 AChR subunit gene or a closely linked gene are implicated in pedigrees with BECTS. The disorder is genetically heterogeneous. Surprisingly, the same chromosomal area has been reported to be linked to the phenotype in families with an auditory neurophysiologic deficit as well as in families with juvenile myoclonic epilepsy, another idiopathic but generalized epilepsy syndrome.

Adolescent↗
PubMed1996

Transmembrane topology of alpha- and beta-subunits of Na+,K+-ATPase derived from beta-galactosidase fusion proteins expressed in yeast.

Various models of the transmembrane topology of the Na+,K+-ATPase predict either 8 or 10 membrane spans for the alpha-subunit and one to three membrane spans for the beta-subunit. Structure/function analysis, however, requires precise knowledge about the folding of enzymes. Therefore, the intention of this work was to establish a transmembrane topology model for the subunits of Na+,K+-ATPase. The bacterial enzyme beta-galactosidase was fused to the C termini of truncated alpha- and beta-subunits of Na+,K+-ATPase. Fusions were generated at Arg60 (LTTAR60), Glu116 (AATEE116), Ala247 (VEGTA247), Leu311 (YTWEL311), Ala444 (VAGDA444), Ala789 (IFIIA789), Met809 (LGTDM809), Asp884 (RVTWD884), Ile946 (MKNKI946), and Arg972 (GVALR972) of the sheep alpha1-subunit and at Pro236 (LGGYP236) of the dog beta-subunit. The fusion constructs were expressed in yeast cells for studies on the localization of the fused reporter enzyme. Activity measurements of the reporter enzyme revealed that only intracellular fusion sites lead to active beta-galactosidase. Indirect immunofluorescence microscopy with cells expressing alpha1/beta-galactosidase and beta/beta-galactosidase hybrid proteins demonstrated that inactive beta-galactosidase is associated with the yeast plasma membrane and can be detected from the extracellular side. The data obtained suggest that Pro236 of the beta-subunit is located on the extracellular surface, corresponding to a model with one transmembrane segment, and that the alpha-subunit of the Na+,K+-ATPase consists of 10 membrane-associated spans. They also suggest that a stretch of the alpha1-subunit between membrane spans M7 and M8 might be hidden within the membrane, surrounded by the other hydrophobic spans, in analogy to the P-loop of Na+ or K+ channels and to the "hourglass" structure of water channels.

Amino Acid Sequence↗
PubMed1996

[Struma ovarii--a case report].

A 43-year old women was admitted to our hospital for investigation and treatment of a right ovarian tumour. Presenting symptoms and signs included recurrent pelvic pain since half a year, nervousness and intercurrent insomnia. At operation a solid cystic tumour was found arising from the right ovary. Histologically a combination tumour was found to consist of a multilocular cystadenoma and a true struma ovarii as a rare neoplasia of teratomatous nature.

Adult↗
PubMed1996

Palytoxin-induced Na+ influx into yeast cells expressing the mammalian sodium pump is due to the formation of a channel within the enzyme.

Palytoxin forms ionic channels in animal cell membranes but does not have similar effects on bacteria or yeast cells. These channels appear to be associated with the sodium pump. Using a heterologous expression system for the mammalian sodium pump in the yeast Saccharomyces cerevisiae, we recently demonstrated palytoxin-induced K+ efflux from yeast cells. Using the same system, we now show that the palytoxin-induced Na+ influx measured by others in animal cells is also directly associated with the sodium pump. Under the influence of palytoxin, yeast cells that express the mammalian sodium pump exchange extracellular Na+ ions for intracellular K+ ions with a stoichiometry of approximately 1:1. Both fluxes can be inhibited by ouabain. K+ efflux can also be observed when extracellular Na+ is replaced by Li+, Cs+, or NH4+. These data suggest that all palytoxin-induced ion fluxes measured so far in various cell systems are directly associated with the sodium pump. Palytoxin-induced Na+ influx or K+ efflux does not occur with yeast cells that express a truncated form of the sodium pump that is missing 44 of the carboxyl-terminal amino acids of the alpha 1 subunit. Scatchard analysis reveals only a slightly lower affinity of the truncated form for [3H]ouabain compared with the affinity of the native enzyme. Yeast cells expressing the truncated enzyme can bind [3H]ouabain, which can be displaced by palytoxin. Therefore, the inability of the truncated form to conduct ions under the influence of palytoxin is not due to the removal of the palytoxin binding site but rather to the removal of a part of the enzyme that participates in a direct or indirect way in the formation of the palytoxin-induced channel. Based on these findings, we conclude that palytoxin opens a channel within and not merely in the vicinity of the sodium pump. This might be the same channel that under normal conditions actively transports Na+ and K+ ions.

Acrylamides↗
PubMed1995

Comparative hygienic surveillance of contamination with pseudomonads in a cystic fibrosis ward over a 4-year period.

In order to study the long-term distribution and population dynamics of Pseudomonas aeruginosa strains in a highly contaminated hospital environment, two 4-week epidemiological studies, with an interval of 4 years, were carried out in the cystic fibrosis (CF) ward of the Paediatric Clinic of the Medical School of Hannover. Out of the 1948 specimens taken, P. aeruginosa was mainly identified in those from moist, inanimate sources (200 isolates) and hospitalized CF patients (168 isolates). A correlation was established between the frequency with which P. aeruginosa-positive patients came into contact with hospital facilities and the rate of contamination of these facilities. Rooms reserved for colonized patients were more frequently contaminated with P. aeruginosa in contrast to function rooms in the same ward and the outpatient clinic. However, no direct exchange between patients' strains and the inanimate hospital environment was detected. Out of the 11 genotypes of P. aeruginosa found in 1989 and the 13 genotypes found in 1993, four genotypes were present on both occasions. The most predominant clone was found in tap-water, sinks, wash-basins and creams with an incidence of 34 and 68% in the environmental isolates. The strains seemed to have spread into the adjacent control ward during the 4-year interval. Thus, the separation of colonized and non-colonized patients was undermined through the transfer of strains from a highly contaminated environment without additional hygiene precautions.

Bacterial Typing Techniques↗
PubMed1994

Epidemiology of chronic Pseudomonas aeruginosa infections in cystic fibrosis.

The epidemiology of chronic colonization of airways with Pseudomonas aeruginosa was monitored in 44 patients with cystic fibrosis (CF) by DraI/SpeI macrorestriction analyses of 489 isolates. Sequential P. aeruginosa isolates (144) that had been collected from 32 CF patients over < or = 2.5 years were investigated, and 12 patients were followed for 8 years after onset of colonization. Forty-eight different genotypes were uncovered from 481 typeable isolates. Ten genotypes were found in > 1 unrelated CF patient. The 6 most frequent clones were identified in 58% of isolates. Ten of the 12 patients monitored for 8 years were harboring their initially acquired P. aeruginosa clone at all times, with subtle shifts of fragment patterns indicating subclonal variation. During colonization, the bacteria gradually lost pyocin and phage typing responses, supporting the view that genotypically discordant P. aeruginosa strains develop a common phenotype.

Adolescent↗
PubMed1993

[Pathomorpholigical findings in ketothiolase deficiency].

The post-mortem findings in two brothers who had suffered from clinically and biochemically confirmed ketothiolase deficiency are reported. They had died as a consequence of metabolic-acidotic crisis at the age of 6 years and 9 months and 4 years and 1 month, respectively. Autopsy revealed cardiac hypertrophy and brain pathology in both children. The latter consisted of loss of neurons, spongiosis and slight reactive astrocytosis affecting parasagittal areas of the parietal and occipital cortex, visual cortex, putamen, caput nuclei caudati and claustrum. Furthermore demyelination of the visual pathways, including chiasma opticum, was seen. Changes in both hemispheres were almost symmetric. In the younger child, changes were less severe than in the older one in whom the course of the disease had been longer. To the best of our knowledge this is the first report of autopsy findings in siblings with ketothiolase deficiency.

Acetyl-CoA C-Acyltransferase↗
PubMed1992

Uvulopalatopharyngoplasty for obstructive sleep apnea: a community's experience.

Uvulopalatopharyngoplasty (UPPP) has become an accepted method for treating obstructive sleep apnea (OSA), with a reported success rate as high as 77%, depending upon inclusionary and outcome criteria. The authors reviewed the records of 90 patients with moderately severe OSA (apnea plus hypopnea index [AHI] greater than 20) who underwent UPPP at either a private community or an academic hospital. Forty percent of patients experienced more than a 50% reduction in their AHI with UPPP. Only 22 (24%) of the patients had a postoperative AHI less than 50% of the preoperative AHI and less than 20, i.e., met the authors' criteria for surgical success. The success rate for community otolaryngologists was no different than that achieved in the academic institution. When data from previously published reports were analyzed using these criteria for success, similar results were observed. This study suggests that the effectiveness of UPPP performed by the general otolaryngologic community is equivalent to that reported in the literature. However, more rigorous criteria must be applied to UPPP when evaluating its results and in counseling potential candidates for this procedure.

Adult↗
PubMed1991

[Morphologic evaluation of glandular inclusions in the peritoneum in relation to the prognostic value of second look laparotomy in ovarian cancer].

Today second-look-laparotomy is recommendable for corroboration of complete remission in ovarian carcinomas. In these cases fluids of peritoneal lavage and biopsies will be got from different regions of abdomen. Physicians, oncologists and pathologists should think at the possibility of existence of benign glands by valuation of biopsies or fluids of laverage. This should be included in the differential diagnostic concept.

Biopsy↗
PubMed1989

[Determination of the protein binding of drugs by continuous ultrafiltration. 9. Comparison of the binding of nonsteroid antirheumatics to human serum albumin and their interaction with phenprocoumon].

Binding to HSA has been determined for diflunisal (alpha = 0.03%), diclofenac (0.09), ibuprofen (0.10), bumadizone (0.11), ketoprofen (0.14), oxyphenbutazone (0.28), indomethacin (0.39), mofebutazone (0.57), tenoxicam (0.59), piroxicam (0.91), salicylic acid (1.00), o-carbamoylphenoxyacetic acid (11.58) and salicylamide (24.91). The free concentration of phenprocoumon was raised by diflunisal up to 35% in a dose dependent manner. Ibuprofen and piroxicam did not show significant effects. It is concluded that diflunisal is bound only slightly to the phenoprocoumon binding site of HSA while ibuprofen has no affinity to this part of the albumin molecule.

4-Hydroxycoumarins↗
PubMed1985

On the stability of polymorphic host-pathogen populations.

The stability of populations of hosts and micro-parasites is investigated where each consists of n varieties that are equal in every respect except that each strain of parasites can infect only one specific strain of hosts and none of the others. Collectively the host strains are limited by a carrying capacity and through this limitation the host populations interact with each other. Hosts are assumed to reproduce asexually or such that different strains do not mate or are not fertile if they do. When the excess death rate caused by the pathogenic parasites is sufficiently large, then the host population is regulated to an equilibrium below the carrying capacity of the environment. This polymorphic equilibrium is shown to be locally asymptotically stable. When one of the parasite strains is absent, then all the other strains die out asymptotically. However, if host resistance to all infectious strains of parasites is achieved at the cost of a lower birthrate of the resistant host strain, then, if a certain condition for the various parameters is satisfied, stable coexistence between infected and resistant hosts is possible. There are many examples where susceptibility and resistance of hosts depends upon the conformation of specific proteins that are involved in host-parasite interactions and hence upon alleles at genetic loci that code for these proteins. We propose that polymorphism in wildtype populations which has been the subject of much theorizing in mathematical genetics may be due to host-pathogen interactions. Our model suggests how a polymorphic population, once established, can remain polymorphic indefinitely.

Animals↗