319 research outputs found

    L'acquisizione delle immobilizzazioni materiali e immateriali

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    Il capitolo tratta delle operazioni di gestione esterna determinanti costi di acquisizione dei fattori produttivi ad uso corrente e pluriennali, materiali e immateriali, da un lato, ricavi di vendita dei prodotti, dall’altro. Alla fine di ciascun paragrafo sono presentati gli schemi di conto economico e di stato patrimoniale, a sezioni divise, che si ottengono per semplice trasposizione dei saldi contabili nelle corrispondenti sezioni dare/avere dei prospetti di bilancio

    A c.3037G > A mutation in FBN1 gene causing Marfan syndrome with an atypically severe phenotype.

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    Marfan syndrome is a pleiotropic connective tissue disease inherited as an autosomal dominant trait, mostly caused by mutations in the FBN1 gene, which is located on chromosome 15q21.1 and encoding fibrillin 1. We report a case of Marfan syndrome presenting with severe ocular and systemic manifestations, such as cardiac congenital anomalies. The patient underwent a multidisciplinary approach and his clinical diagnosis was associated with a c.3037G > A mutation in the FBN1 gene. Identification of this genetic alteration should instigate a prompt multidisciplinary assessment and monitoring, in order to prevent devastating consequences such as cardiac and ocular phenotype. Molecular modeling of the mutation highlighted the importance of the preservation of the calcium-dependent structure of an epidermal -growth-factor-like domain of fibrillin-1 and consequently the microfibrillar formation process. This report aims to highlight the importance of an early clinical and molecular diagnosis and once more, the importance of the multidisciplinary approach of this genetic entity

    Estudio clínico y molecular en una familia con displasia ectodérmica hipohidrótica autosómica dominante

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    Hypohidrotic ectodermal dysplasia (HED) is a rare disease characterized by deficiency in development of structure derived from the ectoderm and is caused by mutations in the genes EDA, EDAR, or EDARADD. Phenotypes caused by mutations in these three may exhibit similar clinical features, explained by a common signaling pathway. Mutations in EDA gene cause X linked HED, which is the most common form. Mutations in EDAR and EDARADD genes cause autosomal dominant and recessive form of HED. The most striking clinical findings in HED are hypodontia, hypotrichosis and hypohidrosis that can lead to episodes of hyperthermia. We report on clinical findings in a child with HED with autosomal dominant inheritance pattern with a heterozygous mutation c.1072C>T (p.Arg358X) in the EDAR gene. A review of the literature with regard to other cases presenting the same mutation has been carried out and discussed

    Does Endogenous Technical Change Make a Difference in Climate Policy Analysis? A Robustness Exercise with the FEEM-RICE Model

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    The Italian Draft Law on the \u2018Provisions Concerning the Safeguarding of the Intangible Cultural Heritage\u2019

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    Intangible cultural heritage in Italy is still in need of a unified approach, capable of providing reliable criteria for identifying its assets and for indicating timescales and means by which they should be safeguarded. In the continued absence of up-to-date, ad hoc state legislation (since the content of those laws which do implement international Conventions is too generic in nature to be sufficiently effective), the Regions have proceeded to act in a somewhat scattered manner, giving rise to an extremely fragmented and very disorderly regulatory framework. The draft law N. 4486, "Provisions Concerning the Safeguarding of the Intangible Cultural Heritage", presented on 12th May 2017 at the Chamber of Deputies of the Italian Republic - as the result of the work of an interdisciplinary and inter-university research team coordinated by Marco Giampieretti, who has drafted the final text with the collaboration of Simona Pinton - seeks to fill the serious void that exists in Italian legal system by aligning it to the principles of international and European law, by redirecting the relevant State and Regional legislation, and by satisfying the fundamental requirements of the national community

    Land, Environmental Externalities and Tourism Development

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    A Probe into the Reform of Public Calligraphy Course in Chinese Colleges and Universities

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    中国书法是中国传统文化中的瑰宝,它既是一门艺术,又有着深厚的文化内涵。我国普通高校开设公共书法教学是与目前倡导的素质教育,培养创新型人才,提倡人的全面和谐发展的高等教育理念相一致的。加强高校公共书法教育,是全面推进素质教育的有效途径之一,也是提高当代大学生人文素质的重要手段。它对促进大学生人格完善、创造力培养以及民族文化的传承等有着不可低估的作用。本文主要针对我国普通高等院校公共书法教学现状进行分析,并由此比较借鉴日本高校中实施书法教学的成功经验提出对我国高校公共书法教学改革的具体建议和措施,设计并实践一种具备学科视野的、以学生为主体的公共书法教学的新模式。论文共分为五部分。引言扼要介绍论文写...Chinese calligraphy is a treasure of Chinese traditional culture. It not only is an art, but also has profound culture contents. Opening the public calligraphy course to the university students is in accordance with the concept that advocated by the current quality- oriented education, which aims to cultivate the students’ creative talent and comprehensive development. This paper concentrates on a...学位:文学硕士院系专业:艺术教育学院美术系_美术学学号:20042201

    Assessing the Effectiveness of Tradable Landuse Rights for Biodiversity Conservation: An Application to Canada's Boreal Mixedwood Forest

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