72 research outputs found

    Relevance and justification of the regional development agency approach for the Brcko district of BiH

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    Still facing the consequences of a post-war and a post-communist transition, BiH is trying to consolidate the strategies for a sustainable peace, based on institutionalisation of a pluralistic democracy - less obsessed by the ethnic questions - and a socio-economic development - that does not necessarily have to rely on the international assistance. Bosnia and Herzegovina is far from being an unitarian institutional, political and social subject and the differences that have marked the years of the war have got consolidated in a constitutional structure that is for sure one of the most complicated that are currently existing in the world. The lack of the presence of a State in the policy making process, is often filled up in Bosnia by the emerging role of local authorities. The case of Brcko District of Bosnia and Herzegovina is rather unique and is a sort of paradox in the paradox of the BiH situation. After getting an autonomous status which was a result of a long and tiring political mediation, Brcko District is now facing the challenge of building a regional development strategy, which has not only to be "politically correct" (like almost all the decisions that have been recently regarding this little but strategic territorial area, so crucial for the BiH peace stability) but has as well to match the difficulties of achieving a future sustainability. The following paper aims to give an answer to the following crucial questions: Does it make sense to promote a regional development strategy and the institutionalisation of a Development Agency for a limited territorial area like Brcko, created as a consequence of a war scenario? What is the specificity of Brcko and what -on the contrary- can be used to build up a model replicable elsewhere in BiH?

    Unaware Actors: Policies and Random Combinations of Aid Interventions in Bosnia and Herzegovina (1992-1995)

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    In the last ten years, international organizations have defined more clearly the role and the tasks of humanitarian aid in contemporary international relations. The case of Bosnia and Herzegovina is a good illustration of the advantages but also of the disadvantages of today’s humanitarian operations. The peacekeeping operation in Bosnia and Herzegovina is the largest operation of this kind that has been set in motion by the international community. In its humanitarian aspect it is complex and multi-layered. Humanitarian campaigns today are more philanthropically- than organizationally-driven, but it is the latter aspect that makes them fail or succeed. It is not enough to provide humanitarian aid, but its distribution to the recipients who need it must be organized. That process involves several essential factors: international and inter-state organizations, governmental organizations as well as non-governmental (international and local) organizations and associations. The example of Bosnia and Herzegovina shows to what extent the mentioned factors contribute to the success of a humanitarian operation, but also the problems which stem from their undefined role and tasks

    Foreign Aid and Cultural Diplomacy. Challenges and Opportunities in the New World (Dis) Order

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    Phenotype and genotype of 87 patients with Mowat-Wilson syndrome and recommendations for care

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    Phenotype and genotype of 87 patients with Mowat-Wilson syndrome and recommendations for care

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    Phenotype and genotype of 87 patients with Mowat-Wilson syndrome and recommendations for care

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    Mowat-Wilson syndrome (MWS) is a rare intellectual disability/multiple congenital anomalies syndrome caused by heterozygous mutation of the ZEB2 gene. It is generally underestimated because its rarity and phenotypic variability sometimes make it difficult to recognize. Here, we aimed to better delineate the phenotype, natural history, and genotype-phenotype correlations of MWS.MethodsIn a collaborative study, we analyzed clinical data for 87 patients with molecularly confirmed diagnosis. We described the prevalence of all clinical aspects, including attainment of neurodevelopmental milestones, and compared the data with the various types of underlying ZEB2 pathogenic variations.ResultsAll anthropometric, somatic, and behavioral features reported here outline a variable but highly consistent phenotype. By presenting the most comprehensive evaluation of MWS to date, we define its clinical evolution occurring with age and derive suggestions for patient management. Furthermore, we observe that its severity correlates with the kind of ZEB2 variation involved, ranging from ZEB2 locus deletions, associated with severe phenotypes, to rare nonmissense intragenic mutations predicted to preserve some ZEB2 protein functionality, accompanying milder clinical presentations.ConclusionKnowledge of the phenotypic spectrum of MWS and its correlation with the genotype will improve its detection rate and the prediction of its features, thus improving patient care.GENETICS in MEDICINE advance online publication, 4 January 2018; doi:10.1038/gim.2017.221

    Phenotype and genotype of 87 patients with Mowat–Wilson syndrome and recommendations for care

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    Purpose: Mowat–Wilson syndrome (MWS) is a rare intellectual disability/multiple congenital anomalies syndrome caused by heterozygous mutation of the ZEB2 gene. It is generally underestimated because its rarity and phenotypic variability sometimes make it difficult to recognize. Here, we aimed to better delineate the phenotype, natural history, and genotype–phenotype correlations of MWS. Methods: In a collaborative study, we analyzed clinical data for 87 patients with molecularly confirmed diagnosis. We described the prevalence of all clinical aspects, including attainment of neurodevelopmental milestones, and compared the data with the various types of underlying ZEB2 pathogenic variations. Results: All anthropometric, somatic, and behavioral features reported here outline a variable but highly consistent phenotype. By presenting the most comprehensive evaluati
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