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  • 1
    Electronic Resource
    Electronic Resource
    Hot spot mutations in keratin 2e suggest a correlation between genotype and phenotype in patients with ichthyosis bullosa of Siemens (2000)
    Copenhagen : Munksgaard International Publishers
    Experimental dermatology 9 (2000), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Ichthyosis bullosa of Siemens (IBS) is a rare disorder of cornification characterized by blister formation in the upper suprabasal layers of the epidermis. Molecular analysis of IBS has identified mutations in the keratin 2e (K2e) gene, which is located in the type II keratin gene cluster on chromosome 12q. We have studied two IBS families and have identified heterozygous point mutations in codon 493 of the K2e gene in both families. Whereas a non-conservative amino acid substitution at position 117 of the 2B region of K2e (E117K) was associated with a severe phenotype in family 1, family 2 showed mild clinical features as a result of a conservative substitution (E117D). These data suggest a phenotype–genotype correlation in these families.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1034/j.1600-0625.2000.009001011.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Benchmarking competitive responses to pioneering new product introductions (2000)
    Bingley : Emerald
    Benchmarking 7 (2000), S. 324-342 
    Details
    ISSN: 1463-5771
    Source: Emerald Fulltext Archive Database 1994-2005
    Topics: Technology
    Notes: Given that the majority of firms are followers of pioneering firms into new, related product markets, managers of almost all firms can benefit from benchmarking their competitive responses to pioneering new product introductions. This paper examines the competitive responses of firms not only in terms of the time until their responding new product introduction, but also in terms of the firms' preceding stages of competitive response: awareness, interest, and evaluation. For example, how long does - and should - it take a follower firm to become aware of a pioneering new product introduction? A general conceptual framework and basic methodology is proposed for firms to evaluate and benchmark their competitive responses. Follower firm responses to pioneering new low-fat food product introductions in North America are examined and illustrate the opportunity for benchmarking firms' competitive responses.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1108/14635770010359883
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    A General Framework for Prioritizing Land Units for Ecological Protection and Restoration (2000)
    Springer
    Environmental management 25 (2000), S. 23-35 
    Details
    ISSN: 1432-1009
    Keywords: KEY WORDS: Cost–benefit; Ecological indicators; Ecological end points; Prioritization; Restoration and protection; Risk; Wetlands
    Source: Springer Online Journal Archives 1860-2000
    Topics: Energy, Environment Protection, Nuclear Power Engineering
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002679910003
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Prevention and control of infections in intensive care (2000)
    Springer
    Intensive care medicine 26 (2000), S. S022 
    Details
    ISSN: 1432-1238
    Keywords: Key words Nosocomial infection ; Antibiotics ; Environment ; Handwashing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Small intensive care units (ICUs) functioning within their capacity and caring mainly for post-operative patients have fewer problems with infection control than larger ICUs with a varied case mix, sub-optimal staffing levels, and high levels of antibiotic consumption. Under these circumstances chronic cross colonisation and infection are inevitable and outbreaks may occur. Little can be done to reduce the risks of infection which arise as a direct result of the patient's clinical condition and prior colonisation status. However, selection pressure from antibiotic usage can be modified, as can environmental hygiene, ventilation and architectural design. One of the simplest measures for reducing cross infection remains one of the most intractable: compliance by staff with protocols and standards for maintaining hand hygiene. Simplification of procedures by the ready availability of alcohol hand rub preparations with or without chlorhexidine may improve matters.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001340051114
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  • 5
    Electronic Resource
    Electronic Resource
    Analysis of single-nucleotide polymorphisms in the interleukin-4 receptor gene for association with inflammatory bowel disease (2000)
    Springer
    Immunogenetics 51 (2000), S. 1-7 
    Details
    ISSN: 1432-1211
    Keywords: Key words Inflammatory bowel ; Crohn's ; Ulcerative colitis ; Interleukin-4 receptor ; Association
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract  Genetic linkage analysis in families with multiple cases of inflammatory bowel disease (IBD) has mapped a gene which confers susceptibility to IBD to the pericentromeric region of chromosome 16 (IBD1). The linked region includes the interleukin(IL)-4 receptor gene (IL4R). Since IL-4 regulation and expression are abnormal in IBD, the IL4R gene is thus both a positional and functional candidate for IBD1. We screened the gene for single-nucleotide polymorphisms (SNPs) by fluorescent chemical cleavage analysis, and tested a subset of known and novel SNPs for allelic association with IBD in 355 families, which included 435 cases of Crohn's disease and 329 cases of ulcerative colitis. No association was observed between a haplotype of four SNPs (val50ile, gln576arg, A3044G, G3289A) and either the Crohn's disease or ulcerative colitis phenotypes using the transmission disequilibrium test. There was also no evidence for association when the four markers were analyzed individually. The results indicate that these variants are not significant genetic determinants of IBD, and that the IL4R gene is unlikely to be IBD1. Linkage disequilibrium analyses showed that the val50ile and gln576arg variants are in complete equilibrium with each other, although they are separated by only about 21 kilobases of genomic DNA. This suggests that a very dense SNP map may be required to exclude or detect disease associations with some candidate genes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002510050001
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  • 6
    Electronic Resource
    Electronic Resource
    Characterization of Radioactive Hazardous Waste (2000)
    Springer
    Journal of radioanalytical and nuclear chemistry 243 (2000), S. 551-553 
    Details
    ISSN: 1588-2780
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Energy, Environment Protection, Nuclear Power Engineering
    Notes: Abstract The characterization of radioactive hazardous waste, also known as transuranic "mixed waste" has to be completed before it can be classified for proper treatment (incinerator, mechanical compaction or thermal treatment), packing, and transport. The characterization of the TRU mixed waste is not only complex process but rather an expensive undertaking. The process knowledge is the basic foundation of characterization. It is the documented knowledge of processes and materials that generated the waste. The transuranic waste Quality Assurance Program Plan (QAPP) defines the Data Quality Objectives (DQO's) and provides the scope of analytical parameters and methods required to accurately characterize the radioactive mixed waste. Based on the historical data and process knowledge a sampling and analysis plan can be developed to characterize the radioactive hazardous waste. Based on the characterization, an assessment of the regulatory status can be made before the waste could be accepted for disposal at the WIPP facility. The Waste Acceptance Criteria (WAC) developed by WIPP defines the parameters for receiving and final disposal of the TRU waste. The sets of criteria, such as: heat generated, fissile gram equivalent (FGE), plutonium-equivalent (PE) curies, and specifications of a dose rate have to be met before the waste is accepted for deep geological disposal. The characterization of radioactive waste becomes even more complex due to the presence of iron base metals/alloys, aluminum base metals/alloys, organic, chelating agents that are mixed with plastic, rubber, cellulose, soils and cement. Some of the modern characterization technologies that are under development and currently used for TRU mixed wastes are: nondestructive examination, nondestructive assay, headspace gas analysis, and drum coring for Resources Conservation Recovery Act (RCRA) sampling.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1016027625604
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  • 7
    Electronic Resource
    Electronic Resource
    Improving permissive infection of human cytomegalovirus in cell culture (2000)
    Springer
    Archives of virology 145 (2000), S. 2431-2438 
    Details
    ISSN: 1432-8798
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary.  Changes in human cytomegalovirus (HCMV) titre occurring under different conditions were studied using plaque assay. No significant change in titre was found using primary embryonic fibroblasts or primary foreskin fibroblasts, or with the addition of dexamethasone to the medium. Significant increases in titre were found when standard cultures were pre-incubated in medium containing DEAE-dextran and/or calcium chloride. However, DEAE-dextran and/or calcium chloride had no significant effect on HCMV detection using the shell vial assay, possibly because enhancement affects permissive infection, but not surface expression of viral antigens. DEAE-dextran and calcium chloride can be included in the medium of standard cultures as a means of obtaining higher titres of HCMV, and are particularly useful for isolates that are difficult to culture.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s007050070032
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