The Rizzoli Multiple Osteochondromas Classification revised: describing the phenotype to improve clinical practice

Multiple osteochondromas (MO) is a rare disorder, characterized by benign osteocartilaginous tumors (osteochondromas), arising from the perichondrium of bones. The osteochondromas increase during growth, frequently causing deformities and limitations. Our study aims to analyze the data captured by the Registry of Multiple Osteochondromas, to refine Istituto Ortopedico Rizzoli (IOR) Classification, providing a representative picture of the phenotypic manifestations throughout the lifespan. We conducted a single-institution cross-sectional study. Patients were categorized according to IOR Classification, which identifies three patients' classes on the presence/absence of deformities and/or limitations. The present dataset was compared with our previously published data, to refine the classification. Nine hundred sixty-eight patients were included: 243 children (<10 years), 136 adolescents (10–15 years), and 589 adults. Of the entire population, half patients presented at least one deformity, and one quarter reported at least one limitation. Compared with our previous study, the amount of children was more than doubled and the percentage of mild/moderate cases was notably increased, giving a better disease overview throughout the lifespan and suggesting a different cut-off for dividing Class II in subclasses. We confirmed that MO is characterized by phenotypic heterogeneity, suggesting that an early classification of the disease may offer a useful tool to follow disease pattern and evolution, to support clinical practice, and to propose timely interventions

Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival

Background: Secondary peripheral chondrosarcomas arising in solitary osteochondromas is an unusual complication, reported in small series. In this study, we aimed to present our experience with this rare variant of chondrosarcoma and compare results with already published data in order to determine prognostic factors for overall and disease-free survival. Methods: The case study includes retrospective data from patients diagnosed at a single institution from 1943 to 2019. Clinical data were collected reviewing all available medical records from first to last follow-up visits. To exclude the presence of the Multiple Osteochondroma Hereditary Syndrome, few patients, with a suspect of a familial form of the disease, were evaluated for the presence of germline heterozygous variants in EXT1 and EXT2 genes. Results were summarized using descriptive statistics and statistical analysis were performed to reveal associations between variables. Results: Two hundred and fourteen secondary peripheral chondrosarcomas that arose exclusively from solitary osteochondromas diagnosed in a multidisciplinary setting at the IRCCS Istituto Ortopedico Rizzoli were retrospectively identified, 66.4% males and 33.6% females with a median age at diagnosis of 38 years. The local recurrence rate was 17.3%, while the metastases one was 5.1%. Besides age, a high histologic grade is the only factor associated with worse 5-year and 10-year overall survival (log-rank p = 0.0005, HR = 3.74; 95% CI 1.69–8.26). Moreover, high histological grade (HR = 3.75; 95% CI = 1.69–8.34; p = 0.001) and surgical debulking (HR = 3.71; 95% CI = 1.57–8.79; p = 0.003) were associated with a significantly worse disease-free survival. Conclusions: Our study confirm the low-grade behavior of secondary peripheral chondrosarcomas and demonstrate that the best choice of treatment for those arising in solitary osteochondromas is the wide surgical excision, when possible. Location per se is not a factor that affects prognosis, while the accurate histological grade assessment is correlated with the tumor aggressiveness and a long term follow up is necessary for this rare variant of chondrosarcoma

The experience of educational research at doctoral level in the region of Cuyo, Argentina

[EN] In this paper are reported the results of an exploratory-analytical research of a qualitative nature, whose intention is to approach the comprehension of the experiences, their peculiarities and problems, of a sample of students and graduates from the three doctorates in Education existing in the region of Cuyo, Argentina, which –though they reach a certain maturity– also show in these years some specific difficulties, among which the most relevant is the low rate of completion (close to 20%). The administered questionnaire, elaborated ad hoc, by means of open-ended questions, inquires on three dimensions that can explain the experience of the PhD student: 1) educational research methodology training, 2) practice in scientific research and 3) doctoral research process. The results show that curricular paths of doctorate’s research methodology, in dependence of their approach, play an important role in the process of theses, in synergy with other factors: integration into a research team, a combination of intrinsic and extrinsic motives in the choice of the topic, a more elaborated conceptualization of educational research, conceptual changes of greater importance. Among the peculiarities, one warns the primacy of the experience and/or teacher workplace in decisions concerning doctoral research; among the difficulties, stand out the lack of time and the major frequency of intrinsic difficulties to the thesis in delayed students.[ES] En este trabajo se informan los resultados de una investigación exploratorio-analítica de carácter cualitativo, cuyo propósito es acercarnos a la comprensión de las experiencias, sus particularidades y problemáticas, de una muestra de alumnos y egresados de los tres doctorados en Educación existentes en la región de Cuyo, Argentina, los que –si bien alcanzan una cierta madurez– también muestran en estos años algunas dificultades específicas, entre las cuales la más relevante es la baja tasa de finalización (cercana al 20%). El cuestionario que se administra, elaborado ad hoc, mediante preguntas abiertas, indaga sobre tres dimensiones que pueden explicar la experiencia del doctorando: 1) formación en metodología de la investigación educativa, 2) práctica en investigación científica y 3) proceso de investigación doctoral. Los resultados muestran que los trayectos curriculares de metodología de la investigación del doctorado, en dependencia de su enfoque, desempeñan un papel relevante en el proceso de tesis, en sinergia con otros factores: la inserción en un equipo de investigación, una combinación de motivos intrínsecos y extrínsecos en la elección del tema, una conceptualización más elaborada de investigación educativa, cambios conceptuales de mayor envergadura. Entre las peculiaridades, se advierte la primacía de la experiencia y/o desempeño docente en decisiones relativas a la investigación doctoral; entre las dificultades, destaca la falta de tiempo y la mayor frecuencia de dificultades intrínsecas a la tesis en los estudiantes demorados.Difabio De Anglat, H.; Portela De Nieto, A.; Gelonch Villarino, S.; Muscará, F.; Boarini De Dutto, MG. (2018). La experiencia de investigación educativa de nivel doctoral en la región de Cuyo, Argentina. 11-32. doi:10.4995/redu.2018.5690SWORD113

Derecho aduanero. Derecho del turismo

Como ya se expresara, casi todas las aristas que toca nuestra disciplina han sido afectadas por la aparición de esta nueva enfermedad que ha trastocado el estilo de vida en el mundo entero. Incertidumbre generalizada, paralización completa de actividades, pérdidas, reprogramaciones inciertas, suspensiones y despidos están a la orden del día desde principios de este particular año 2020. Este nuevo virus se ha extendido a lo largo y a lo ancho de todo el planeta. Ninguna actividad, industria, Estado, ha quedado al margen de la cadena de infección. En consecuencia, los Jefes de Estado han ido adoptando medidas para evitar que la población contraiga dicha patología. En resumen, ante la situación excepcional generada por la irrupción de la COVID-19, la posterior declaración de pandemia que realizara la OMS en fecha 11/03/2020 y las normativas de crisis dictadas por la mayoría de los Estados para contener la situación, el abordaje jurídico adquiere carácter extraordinario. Ya en condiciones normales la intermediación que realiza el agente de viajes entre los prestadores de servicios turísticos y consumidores es un tópico complejo. Es por ello, que el lector se encontrará con una solución de justicia contributiva cuya argumentación conjuga normas, principios y valores jurídicos. Su razonabilidad viene dada por el equilibrio que revela y dispensa, tanto a los operadores turísticos, agencias de viajes y consumidores. El término “cooperación” emerge como decisorio en orden a allanar la dificultad, el caos.Fil: Pesqueira Nozikovsky, M. Soledad. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Frontera Villamil, Ernesto. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Cinalli, Juan Marcelo. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Rivarola, Hugo. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Baigorria, Nelly. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Cevallos, Diego. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Ferronato, Victoria. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Freijo, Maricel. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Giubergia, M. Victoria. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: González Boarini, Paula. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Hidalgo, Guadalupe. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Latini Marramá, Ignacio. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Luna, Iván. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Mana, Noelia I. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Núñez, Dante Ariel. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Oliver, Lucía. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina.Fil: Saimandi, Erika. Universidad Nacional de Córdoba. Facultad de Derecho; Argentina

Wellbeing indicators affecting female entrepreneurship in OECD countries

[EN] The objective of this research is to know which wellbeing indicators, such as work-life balance, educational level, income or job security, are related to the rate of female entrepreneurship in 29 OECD countries. In addition, these countries have been classified according to the motivation of the entrepreneur either by necessity or by opportunity. The empiric study is focused on 29 OECD countries covering the different geographic areas (Western Europe, Central and Eastern Europe, Middle East, etc.) Due to the fact that the sample is relatively small, it is essential to use a selective approach when selecting the causal conditions. To this end, fsQCA is the most appropriate methodology for such a small data set. A total of 5 variables have been used: an independent variable (female TEA ratio), and four dependent variables (work life balance, educational level, sustainable household income and job security). Data measuring female TEA ratio have been obtained from Global Entrepreneur Monitor (GEM in Global report, 2015) data base, while data measuring wellbeing dimensions were taken from the Better Life Index (OECD in How¿s life? Measuring wellbeing, 2015. http://www.oecdbetterlifeindex.org). The results of this piece of research show that countries with high sustainable household income together with high level of education achieves high female entrepreneurship ratio with both, a good work-life balance (despite of a high unemployment probability), or a high labour-personal imbalance (in this latter, with a low probability of unemployment).This work has been funded by the R + D project for emerging research groups with reference (GVA) GV/2016/078.Ribes-Giner, G.; Moya Clemente, I.; Cervelló Royo, RE.; Perelló Marín, MR. (2019). Wellbeing indicators affecting female entrepreneurship in OECD countries. 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Caracterização da queixa escolar no Centro de Psicologia Aplicada da Universidade Federal de Juiz de Fora

A queixa escolar vem se constituindo, historicamente, como um dos motivos mais frequentes de encaminhamentos de crianças para os serviços-escola de Psicologia, traduzindo, pela sua complexidade, um desafio para a formação e práticas no âmbito da psicologia escolar/educacional. Diante da necessidade de aperfeiçoar o atendimento à clientela e o processo de triagem, o presente estudo objetivou analisar os prontuários referentes à queixa escolar de pacientes atendidos no Centro de Psicologia Aplicada da Universidade Federal de Juiz de Fora, Minas Gerais, Brasil, num total de 1.590 documentos, no período de 1996 a 2009. Os resultados quanto à clientela evidenciaram a prevalência de meninos na faixa entre 9 e 13 anos de idade, com predomínio de encaminhamentos oriundos de escolas de periférias da cidade. Quanto à queixa, constatou-se expressiva coocorrência de problemas comportamentais e de aprendizagem. Os dados permitem uma reflexão crítica acerca da queixa escolar e de alguns fatores contextuais, bem como a necessidade de aperfeiçoamento da ficha de triagem