Correction: Long-term outcome after allogeneic hematopoietic stem cell transplantation for Shwachman–Diamond syndrome: a retrospective analysis and a review of the literature by the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation (SAAWP-EBMT)

An amendment to this paper has been published and can be accessed via a link at the top of the paper

Transient Facial Nerve Paralysis (Bell's Palsy) following Intranasal Delivery of a Genetically Detoxified Mutant of Escherichia coli Heat Labile Toxin

BACKGROUND: An association was previously established between facial nerve paralysis (Bell's palsy) and intranasal administration of an inactivated influenza virosome vaccine containing an enzymatically active Escherichia coli Heat Labile Toxin (LT) adjuvant. The individual component(s) responsible for paralysis were not identified, and the vaccine was withdrawn. METHODOLOGY/PRINCIPAL FINDINGS: Subjects participating in two contemporaneous non-randomized Phase 1 clinical trials of nasal subunit vaccines against Human Immunodeficiency Virus and tuberculosis, both of which employed an enzymatically inactive non-toxic mutant LT adjuvant (LTK63), underwent active follow-up for adverse events using diary-cards and clinical examination. Two healthy subjects experienced transient peripheral facial nerve palsies 44 and 60 days after passive nasal instillation of LTK63, possibly a result of retrograde axonal transport after neuronal ganglioside binding or an inflammatory immune response, but without exaggerated immune responses to LTK63. CONCLUSIONS/SIGNIFICANCE: While the unique anatomical predisposition of the facial nerve to compression suggests nasal delivery of neuronal-binding LT-derived adjuvants is inadvisable, their continued investigation as topical or mucosal adjuvants and antigens appears warranted on the basis of longstanding safety via oral, percutaneous, and other mucosal routes

CIBERER : Spanish national network for research on rare diseases: A highly productive collaborative initiative

Altres ajuts: Instituto de Salud Carlos III (ISCIII); Ministerio de Ciencia e Innovación.CIBER (Center for Biomedical Network Research; Centro de Investigación Biomédica En Red) is a public national consortium created in 2006 under the umbrella of the Spanish National Institute of Health Carlos III (ISCIII). This innovative research structure comprises 11 different specific areas dedicated to the main public health priorities in the National Health System. CIBERER, the thematic area of CIBER focused on rare diseases (RDs) currently consists of 75 research groups belonging to universities, research centers, and hospitals of the entire country. CIBERER's mission is to be a center prioritizing and favoring collaboration and cooperation between biomedical and clinical research groups, with special emphasis on the aspects of genetic, molecular, biochemical, and cellular research of RDs. This research is the basis for providing new tools for the diagnosis and therapy of low-prevalence diseases, in line with the International Rare Diseases Research Consortium (IRDiRC) objectives, thus favoring translational research between the scientific environment of the laboratory and the clinical setting of health centers. In this article, we intend to review CIBERER's 15-year journey and summarize the main results obtained in terms of internationalization, scientific production, contributions toward the discovery of new therapies and novel genes associated to diseases, cooperation with patients' associations and many other topics related to RD research

Museums Brand Equity and Social Media: Looking into Current Research Insights and Future Research Propositions

Abstract. Extensive research has repeatedly acknowledged the link between traditional and digital marketing communication tools and branding performance. Particularly, both within For Profit Organizations (henceforth, FPOs) and Non-Profit Organizations (henceforth NPOs), social media as the milestone of the digital era has rebutted the foundations of corporate and personal communication through the emergence of new participatory communication terms, such as ''prod-user'' and “co-creation”. Consequently, a growing research trend has emerged towards e-e marketing tools and social media impact on destination branding, as well. Simultaneously, thanks to its multidimensional benefits both at the communicational, educational, and promotional levels, social media are emerging as an essential feature in the branding of the new museum era. To date, within the NPOs sector, few studies have investigated the effect of social media on brand equity. Moreover, far too little attention has been paid to the link between social media and museums' brand equity. Based on the systematic qualitative critical review methodology, this paper attempts to identify the basic trends and research status by 2018. Drawing on a review of 78 papers that are the result of systematic desk research, this study categorizes and presents, for the first time, the effects of social media use on museums’ brand components. The study offers new and valuable insights into the multidisciplinary research interests of the research and industry community relating to communication and marketing, NPOs, tourism, and museums context. Keywords: Social Media, Museums, Brand Equity, NPOs, Cultural Tourism

Instituto Sorolla, Valencia, España

This original building meets accurately the conditions laid down in the specifications, namely; optimum illumination and ventilation for the lecture halls and other rooms, maximum free space for sporting actives, and specially good acoustical properties in the lecture halls, chapel, bar and gymnasium. The main external features of this school are the untreated concrete surfaces, and the strong and novel expressiveness of the reinforced concrete structure.Este edificio singular cumple perfectamente las premisas fijadas en el proyecto, economía; aulas y demás dependencias con iluminación y ventilación óptimas; máximo de espacio libre para zonas deportivas; acústica determinada en salón de actos, capilla, bar y gimnasio, etc. El hormigón visto y la recia y original expresividad de la estructura de hormigón armado, son las notas más características y distintivas de este conjunto escolar

Plasmidic versus Insertional Cloning of Heterologous Genes in Mycobacterium bovis BCG: Impact on In Vivo Antigen Persistence and Immune Responses

Bivalent recombinant strains of Mycobacterium bovis BCG (rBCG) expressing the early regulatory nef and the structural gag(p26) genes from the simian immunodeficiency virus (SIV) SIVmac251 were engineered so that both genes were cotranscribed from a synthetic operon. The expression cassette was cloned into a multicopy-replicating vector, and the expression levels of both nef and gag in the bivalent rBCG(nef-gag) strain were found to be comparable to those of monovalent rBCG(nef) or rBCG(gag) strains. However, extrachromosomal cloning of the nef-gag operon into a replicative plasmid resulted in strains of low genetic stability that rapidly lost the plasmid in vivo. Thus, the nef-gag operon was inserted site specifically into the BCG chromosome by means of mycobacteriophage Ms6-derived vectors. The resulting integrative rBCG(nef-gag) strains showed very high genetic stability both in vitro and in vivo. The in vivo expression of the heterologous genes was much longer lived when the expression cassette was inserted into the BCG chromosome. In one of the strains obtained, integrative cloning did not reduce the expression levels of the genes even though a single copy was present. Accordingly, this strain induced cellular immune responses of the same magnitude as that of the replicative rBCG strain containing several copies of the genes