供应链创新在改善供应链运作流程、提高供应链效率方面发挥着关键作用,厘清与供应链创新有关文献的发展脉络,有助于进一步深入分析该研究领域。基于此,以2010—2021年(截至2021年4月23日)Web of Science核心合集和中国知网(CNKI)中以"...
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供应链创新在改善供应链运作流程、提高供应链效率方面发挥着关键作用,厘清与供应链创新有关文献的发展脉络,有助于进一步深入分析该研究领域。基于此,以2010—2021年(截至2021年4月23日)Web of Science核心合集和中国知网(CNKI)中以"供应链"和"创新"为标题的论文为样本,运用CiteSpace可视化软件,梳理了国内外供应链创新的现状、研究热点和前沿趋势。分析发现:国内外期刊文献发文量呈上升趋势,虽然国内对于供应链创新的关注度大于国外,但是存在作者合作频率低,研究机构较为分散的情况;国内外供应链创新在研究热点、研究趋势和应用场景上存在显著差异。总结了过去11年供应链创新的发展,并根据对已有文献的整理,归纳了供应链创新的研究框架,以期展现现有研究成果与未来研究方向。
We report three unrelated patients affected at birth with an unusually severe form of epidermolysis bullosa simplex Dowling-Meara type (EBS-DM) because of mutations in KRT14 encoding keratin 14. Two patients were hete...
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We report three unrelated patients affected at birth with an unusually severe form of epidermolysis bullosa simplex Dowling-Meara type (EBS-DM) because of mutations in KRT14 encoding keratin 14. Two patients were heterozygous for the previously described p.M119T mutation. The third patient was heterozygous for a novel c.1246delC mutation predicting the replacement of the helix termination peptide and the tail domain by a 25 amino-acid aberrant carboxy terminal sequence. At age 2 years, patients carrying the p.M119T mutation still suffered from severe EBS-DM, whereas the patient harboring the c.1246delC mutation has improved over time. These cases illustrate genotype-phenotype correlations and have implications for genetic counselling of EBS.
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