109 research outputs found

    Primary peripheral PNET/Ewing's sarcoma arising in the meninges, confirmed by the presence of the rare translocation t(21;22) (q22;q12)

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    Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (ES) (pPNET/ES) of intracranial origin are very rare. These tumors are characterized by specific translocations involving a gene on chromosome 22q12, the most common being t(11;22) (q24;q12). We report a case of 37-year-old man with pPNET/ES arising in the meninges and bearing the rare translocation t(21;22) (q22;q12). The tumor was composed of sheets and nests of monotonous small cells with round to oval nuclei, finely dispersed chromatin, small nucleolus and scant cytoplasm. We discuss the importance of the differential diagnosis with central primitive neuroectodermal tumors (cPNET). © 2011 Japanese Society of Neuropathology

    Previously Unreported TMEM38B Variant in Osteogenesis Imperfecta Type XIV: A Case Report and Systematic Review of the Literature

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    Osteogenesis imperfecta (OI) type XIV is a rare recessive disorder caused by TMEM38B pathogenic variants that disrupt an endoplasmic reticulum protein essential for calcium homeostasis and bone mineralization. This leads to severe bone fragility, early-onset fractures, skeletal deformities, low bone mass, scoliosis, and variable features like blue sclerae or dental abnormalities. We present a case report of a 21-year-old Italian male with a novel homozygous TMEM38B splice variant (c.112 + 1G > T), detailing the clinical presentation, genetic findings, and therapeutic outcomes. The patient exhibited multiple skeletal deformities and showed a moderate response to bisphosphonate therapy (neridronate). In addition, a systematic review of PubMed and Scopus identified 12 relevant studies from an initial set of 82 publications, encompassing data from 56 patients diagnosed with OI type XIV. Unlike classical collagen-related OI, TMEM38B-related OI necessitates genetic screening beyond classical collagen genes (COL1A1 and COL1A2). While bisphosphonates provide some clinical benefit, persistent fractures underscore the need for long-term management and innovative therapies. This case report and systematic review enhance understanding of OI type XIV and underscore the clinical importance of TMEM38B variants in bone fragility disorders

    Acqua Fatua

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    Progetto realizzato in collaborazione con Confartigianato, Rimini e realizzato domenica 4 luglio in Piazza Tre Martiri a Rimini nel contesto delle manifestazioni programmate per “La Notte Rosa”. ZoneModa partecipa all'iniziativa presentando gli esiti del workshop “Acqua fatua”, un lavoro di styling realizzato per questo evento dagli studenti del Corso di laurea in Culture e tecniche della moda, con il contributo di Uni.Rimini. “Acqua fatua” riflette sulle deformazioni che l’acqua provoca su tessuti e silhoutte modellandone colori e forme. Tinte e liquidi si fondono sui corpi e sugli abiti delle modelle con effetti di implosioni ed esplosioni. Il progetto trae ispirazione dal celebre bagno notturno di Anita Ekberg e Marcello Mastroianni nella fontana di Trevi. Il titolo “Acqua Fatua” riprende le intenzioni oniriche del regista e reinventa lo scenario magico del film. Il progetto si è potuto realizzare grazie all’entusiasmo degli studenti del Corso di laurea con il coordinamento di Francesca Iaconisi, sotto la supervisione di Alessandra Vaccari. Le fotografie e il video sono di Girolamo Lanzafame. La musica è di Antonio Pagano. All’organizzazione hanno lavorato Cristiana Curreli, Raffaela Marinetti e Mariaelena Schiavo. Gli studenti che hanno partecipato al workshop sono: Gaia Arrigoni, Giulia Antonaz, Carolina Benedetti, Maria Fabbri, Federico Gigli, Anisa Memishaj, Chiara Pepe, Serena Pifferi, Serena Salvi, Nicole Sebastiani e Giovanni Tesse

    Analýza rakouské teorie hospodářského cyklu z pohledu komplexní ekonomie

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    This thesis analyzes the Austrian business cycle theory (ABCT) from the Complexity Economics approach. Complexity Economics is an approach which comes from the complexity science. It has started to develop since 1980s' in Santa Fe Institute, the leading institution engaging in the research of complexity. In the thesis I mention the terms which are bound to Complexity Economics, for example emergence, the "bottom-up", self-organization, bounded rationality etc. The evolution of the markets, networks and nonlinear dynamics are included in Complexity Economics as well. Complexity Economics views an economy as a dynamical system which never reaches the equilibrium unlike the Traditional Economics approach which main focus is on the general equilibrium of the economy. Another difference would be the way of modelling economies in which Complexity Economics is leaving the unrealistic assumptions because it utilizes the agent-based simulations which enable to do that. In the thesis I also focus on the similarities between Austrian and Complexity Economics, for example the heterogeneity of the agents or the emergence of the price system. These and others similarities are then applied in the analysis of ABCT which serves as an example of demonstration that Austrian Economics could provide the theoretical framework for Complexity Economics which aspires to become a new paradigm in economics

    Pobreza en áreas centrales urbanas: Métodos de análisis e intervenciones: Manual de consulta para profesionales

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    Las áreas centrales de una ciudad contienen una mezcla de problemas y oportunidades. Permiten a sus residentes y empresas la opción de vivir y producir cerca de sus trabajos y mercados, y ofrecen a los gobiernos la posibilidad de reducir los costos de transporte y promover un uso más intensivo de la infraestructura, los espacios públicos y los servicios urbanos existentes. También presentan complejos desafíos de política urbana, incluida la solución de los problemas sociales que afectan a los pobres en esas áreas. Muchas ciudades en el mundo están afrontando activamente estos problemas. En este libro se analizan estas oportunidades y estos retos desde la perspectiva de la práctica del urbanismo.

    City Development: Experiences in the Preservation of Ten World Heritage Sites

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    The IDB undertakes multiple activities in support of urban heritage preservation, including the provision of investment loans and reimbursable and nonreimbursable technical cooperation. The IDB also organizes policy debates, seminars, and technical exchanges among cities, and produces technical publications. The present study is intended to increase available knowledge on factors contributing to the long-term sustainability of urban heritage preservation efforts. In particular, the IDB seeks to expand the understanding of the economic aspects of sustainable preservation that are complementary to the social, environmental, and institutional aspects of heritage preservation that are better understood. The studies presented in this publication are intended to fill a knowledge gap and improve the overall effectiveness of urban heritage preservation and development efforts in the Latin American and Caribbean region, as well as in other regions around the world

    City Development: Experiences in the Preservation of Ten World Heritage Sites

    No full text
    The IDB undertakes multiple activities in support of urban heritage preservation, including the provision of investment loans and reimbursable and nonreimbursable technical cooperation. The IDB also organizes policy debates, seminars, and technical exchanges among cities, and produces technical publications. The present study is intended to increase available knowledge on factors contributing to the long-term sustainability of urban heritage preservation efforts. In particular, the IDB seeks to expand the understanding of the economic aspects of sustainable preservation that are complementary to the social, environmental, and institutional aspects of heritage preservation that are better understood. The studies presented in this publication are intended to fill a knowledge gap and improve the overall effectiveness of urban heritage preservation and development efforts in the Latin American and Caribbean region, as well as in other regions around the world.

    Cultural Industries in Latin America and the Caribbean: Challenges and Opportunities

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    Over the past decade, the new creative-cultural industries have increasingly become one of the main sources of greater production and employment in many countries. Although the growth of cultural industries has been mostly limited to the world\u27s richest countries, a number of low- and middle-income countries have come to recognize that they too can participate in the cultural economy. In this regard, Latin American and Caribbean (LAC) countries have begun to identify new opportunities and are actively seeking sustainable solutions to maintain these industries as an integral part of cultural and economic life. The objective of this paper is to assess the strengths and weaknesses of cultural industries in LAC and offer lessons learned through this analysis to policymakers and donors. This study demonstrates the value and the potential for cultural industries in the LAC economy and also identifies the need for a comprehensive approach, structured policies, and targeted interventions aimed at improving the conditions and prospects of these industries

    Epidermal growth factor receptor and caveolin-1 coexpression identifies adult supratentorial ependymomas with rapid unfavorable outcomes

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    Supratentorial ependymomas account for a minority of intracranial ependymomas, which still have uncertain prognostic markers. Among them, epidermal growth factor receptor (EGFR) overexpression correlates with a poor prognosis. In glioblastoma cells, EGFR function has been reported to be regulated by its migration from cell membrane infoldings called caveolae and by its colo-calization with the caveolae-associated protein caveolin-1 (cav-1). Therefore, we decided to investigate cav-1 expression and coexpression with EGFR in a series of adult intracranial ependymomas. We analyzed 22 adult supratentorial ependymomas and compared tumor grades as determined by the WHO classification and patient survival rates with the expression of EGFR, cav-1, and p53 and the values of the proliferation marker Ki-67, all tested by immunohistochemistry; in addition, we investigated the mutational profile of cav-1. The results demonstrate that the tumor grade is directly correlated with EGFR, Ki-67, and cav-1 expression only, whereas (by univariate analysis) the expression of all the studied markers, as well as the tumor histological grade, significantly correlated with the patient's overall survival (OS). By multivariate analysis using the Cox proportional hazards model, among all variables considered, cav-1 was the only independent prognostic marker related to OS (relative risk = 13.92; P =.013). Among grade II epen-dymomas, only cav-1 correlated with poor OS (P =.011), distinguishing 2 distinct subgroups of tumors with different outcomes despite sharing identical grading. All the patients studied carried wild-type cav-1 sequences, demonstrating that cav-1 overexpression is not driven by activating mutations, as previously reported in other tumor types. Interestingly, after stratifying all cases into 4 distinct groups according to cav-1 and EGFR expression (cav-1+/EGFR+, cav-1+/EGFR+, cav-1+/EGFR+, and cav-1+/EGFR+), the coexpres-sion of cav-1 and EGFR identified a subset of patients with definitively poor prognoses. Further studies are needed to support this evidence on a larger scale and to clarify how cav-1 and EGFR interaction can influence tumor aggressiveness. © The Author(s) 2010
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