Los modelos de semántica de marcos para la representación del conocimiento jurídico en el Derecho Comparado: el caso de la responsabilidad del Estado

En aquest article s'analitza en profunditat i es realitza una proposta de representació del coneixement jurídic subjacent al concepte de responsabilitat de l'Estat des d'una perspectiva multilingüe i juscomparativa. Per a això, es proposa d'augmentar la informació dels marcs semàntics (a partir d'ara, marcs) a través dels semantic types en el sistema FrameNet, amb el doble objectiu de servir com a representació interlingua del coneixement jurídic i de formalitzar les causes del desajust lèxic i conceptual dels sistemes jurídics. S'estudia el principi de responsabilitat de l'Estat en els models espanyol, anglès, francès i italià i es demostra com una descripció més detallada del coneixement jurídic, a través de la vinculació dels frame elements (a partir d'ara designats amb l'acrònim FE) dels marcs amb els tipus semàntics [±sentient], possibilita no només la utilització d'aquests com a representació interlingua, sinó, a més, procura explicar les divergències/convergències dels diferents plantejaments del concepte de responsabilitat de l'Estat, ancorats en contextos socioculturals de diferent tradició. La present proposta evidencia els avantatges de l'esmentada formalització com a model explicatiu del procés dinàmic de vaig donar/convergència en la jurisprudència del Tribunal de Justícia de la Unió Europea (a partir d'ara designat amb la sigla TJUE).This article offers an in-depth analysis, and proposes a representation of the legal knowledge underlying the concept of State responsibility from a multilingual and comparative law perspective. To this end, it recommends increasing information on frame semantics (hereinafter, frames) through the semantic types in the FrameNet system, with the double purpose of acting as an interlingual representation of legal knowledge and formalising the causes for lexical and conceptual imbalances in legal systems. The article studies the principle of State responsibility in the Spanish, English, French and Italian models and shows how a more detailed description of legal knowledge through the linking of the frame elements (hereinafter designed by the acronym FE) of the frames with the semantic types [±sentient], makes it feasible not just to use these as an interlingual representation, but also to try to explain the divergences/convergences of the various approaches to the concept of the State responsibility that are rooted in sociocultural contexts of a different tradition. This proposal demonstrates the advantages of this formalisation as a model to explain the dynamic process of divergence/convergence in the case law of the Court of Justice of the European Union (referred to hereinafter by the acronym CJEU).En este artículo se analiza en profundidad y se realiza una propuesta de representación del conocimiento jurídico subyacente al concepto de responsabilidad del Estado desde una perspectiva multilingüe y juscomparativa. Para ello, se propone aumentar la información de los marcos semánticos (a partir de ahora, marcos) a través de los semantic types en el sistema FrameNet, con el doble objetivo de servir como representación interlingüe del conocimiento jurídico y de formalizar las causas del desajuste léxico y conceptual de los sistemas jurídicos. Se estudia el principio de responsabilidad del Estado en los modelos español, inglés, francés e italiano y se demuestra cómo una descripción más detallada del conocimiento jurídico, a través de la vinculación de los frame elements (a partir de ahora designados con el acrónimo FE) de los marcos con los tipos semánticos [±sentient], posibilita no solo la utilización de estos como representación interlingüe, sino, además, procura explicar las divergencias/convergencias de los distintos planteamientos del concepto de responsabilidad del Estado, anclados en contextos socioculturales de diferente tradición. La presente propuesta evidencia las ventajas de dicha formalización como modelo explicativo del proceso dinámico de di/convergencia en la jurisprudencia del Tribunal de Justicia de la Unión Europea (a partir de ahora designado con la sigla TJUE)

Specificul molecular genetic în Fenilcetonurie în Republica Moldova (anii 2018-2019)

Background. Phenylketonuria (PKU) is an autosomal recessive disorder, caused by phenylanine hydroxylase deficiency, leading to phenylalanine(Phe) accumulation in blood, that is responsible for progressive mental retardation. Objective of the study. The aim of the study was to diagnose PKU, on the basis of neonatal screening, organized in 2018-2019 and to review the cases by molecular methods and identify the mutations that caused the disease. Material and Method. In 2018-2019, 62943 of newborns were subjected to neonatal screening in Moldova (screening rate 96.16%), based on the fluorometric method. There were 17 children diagnosed with PKU, from which 11 were tested by PCR/RFLP on 6 frequent mutations in PAH gene (R408W, P281L, L48S, R158Q, R252W, R261Q) and sequencing of the exons 2,3,5,6-8,10-13 by Sanger method. Results. As a result of neonatal screening, there were identified 376 suspects for PKU, showing Phe blood levels >3 mg/dL, from which only 17 were confirmed with PKU. The DNA samples were collected from 11 PKU subjects. The molecular analysis by PCR/RFLP method lead to identification of 45.45% of mutations. The most frequent genotype was R408W/L48S, established in 3 patients. The replenishment of the study with Sanger sequencing results enabled detection of complete genotype by following: splicing (IVS7+4A>G, IVS11+7T>C, IVS12+1G>A), missense (A342T), and nonsense (R111X) mutations. Conclusion. To conclude, there was identified the spectrum of mutations specific to Moldovan PKU cohort diagnosed in 2018-2019, grace to efficient organization of neonatal screening and diagnostics of PKU. Introducere. Fenilcetonuria (PKU) este o patologie autosomal recesivă, cauzată de deficiența enzimei fenilalanin hidroxilaza, care duce la acumularea fenilalaninei (Phe) serice, cauzând retard mental. Scopul lucrării. Scopul studiului a fost de a diagnostica PKU în baza screeningului neonatal, organizat în perioada 2018-2019, precum și a cerceta la nivel molecular genetic pacienții identificați pentru stabilirea mutațiilor, cauzatoare de boală. Material și Metode. În perioada 2018-2019 au fost supuși screeningului 62943 de nou-născuți din Republica Moldova (rata de screening 96,16%), în baza metodei fluorometrice. La 17 copii a fost detectată PKU, iar 11 au fost testați genetic prin metoda PCR/RFLP la 6 mutații în gena PAH (R408W, P281L, L48S, R158Q, R252W, R261Q) și secvențierea exonilor 2,3,5,6-8,10-13 ai genei PAH după metoda Sanger. Rezultate. În urma screening-ului, s-au identificat 376 de suspecți la PKU, prezentând valori ale Phe>3 mg/dL, dintre care doar 17 au fost confirmați cu PKU, ADN- ul a fost obținut de la 11 pacienți. Analiza molecular-genetică prin PCR/RFLP a permis detectarea mutațiilor în 45,45% de cazuri. Cel mai frecvent genotip a fost R408W/L48S, stabilit la 3 pacienți. Suplinirea analizei prin metoda Sanger a permis detectarea genotipului complet în restul cazurilor, determinând mutații de tip splicing (IVS7+4A>G, IVS11+7T>C, IVS12+1G>A), missense (A342T) și nonsense (R111X). Concluzii. În urma studiului, a fost detectat un spectru de mutații specifice cohortei PKU din Republica Moldova, datorită metodelor eficiente de screening și de diagnostic

Biologia reprodutiva de Davilla kunthii A. St-Hil. (Dilleniaceae) na Amazônia Central

This survey aimed at describing the interactions of floral visitors and Davilla kunthii A. St.-Hil. as well as characteristics of its reproductive biology in Itacoatiara, state of Amazonas, Brazil. Tests of the breeding system were performed. The guild of visitors was described according to richness, abundance, relative frequency and constancy. The breeding system tests indicated that D. kunthii is self-compatible. The pollination system was characterized as generalist, with 39 visitor species, from three different orders. Bees were the main group of pollinators, thus some behavioural aspects were described. Th e period of highest foraging activity was between 7 and 10 am. Some species presented agonistic and monopolistic behaviour. Given the behaviour and destructive potential, the Curculionidae seem to have a greater impact as seed predators than pollinators.Este trabalho descreve as relações entre visitantes florais e Davilla kunthii A. St.-Hil., bem como características de sua biologia reprodutiva em Itacoatiara (Amazonas). Foram realizados os testes referentes ao estudo do sistema reprodutivo. A guilda de visitantes foi caracterizada quanto à riqueza, abundância, frequência relativa e constância. Os testes para sistema reprodutivo indicaram que D. kunthii possui auto-compatibilidade. O mecanismo de polinização configurou-se como generalista com 39 espécies de visitantes florais, provenientes de três diferentes ordens. Abelhas corresponderam ao principal grupo de polinizadores, desta forma, alguns aspectos comportamentais foram descritos. O horário de maior forrageamento ocorreu entre 7 e 10 horas. Algumas espécies apresentaram interações agonísticas e comportamento monopolizador da fonte de alimento. Dado o comportamento e o potencial destrutivo, embora possa ocasionalmente atuar como polinizadores, Curculionidae parecem possuir maior impacto como predadores de sementes.487496Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq

Finite element modelling of shear bands in porous media by means of non-local viscoplasticity

This work presents a consistent numerical approach in the framework of analyzing the localization process in a slope stability problem. An already existing model, embedded in Mechanics of multiphase porous media, is enhanced with local and non-local elasto-viscoplastic constitutive models to obtain regularized numerical solutions. The initiation and the propagation of the shear band are effectively described by means of FEM analysis, regardless of the mesh size adopted. The strain localization process realistically occurs within the shear band failure mode and its size is governed by the internal length variable, which can be directly estimated by experimental approaches

Relaxin family peptide receptors Rxfp1 and Rxfp2: mapping of the mRNA and protein distribution in the reproductive tract of the male rat

<p>Abstract</p> <p>Background</p> <p>Relaxin is the endogenous ligand of the G-protein coupled receptor RXFP1, previously known as LGR7. In humans relaxin can also activate, but with lower affinity, the closely related receptor for the insulin-like peptide from Leydig cells, RXFP2, previously known as LGR8. The lack of relaxin impairs male fertility but the precise distribution and the function of relaxin receptors in the male reproductive tract is not known. We investigated the distribution of Rxfp1 and Rxfp2 in the reproductive tract of the male rat and the function of relaxin in the vas deferens, a tissue with high expression of both receptors.</p> <p>Methods</p> <p>The presence of mRNA for Rxfp1 and Rxfp2 was investigated in testes, cultured Sertoli cells, epididymis, vas deferens, seminal vesicle, prostate, and spermatozoa by RT-PCR and Southern blot. Protein expression in the testis, vas deferens, primary culture of Sertoli cells, and spermatozoa was assessed by immunohistochemistry and immunofluorescence. The role of relaxin in the vas deferens was evaluated by contractility studies and radioimmunoassay of cAMP production. The effect of relaxin on mRNA levels for metalloproteinase-7 was measured by Northern blot.</p> <p>Results</p> <p>Transcripts for Rxfp1 and Rxfp2 were present in almost all parts of the male reproductive tract, with high levels in testis and vas deferens. Both receptors were immunolocalized in late stage germ cells but not in mature spermatozoa, although mRNAs for both receptors were also present in mature spermatozoa. Rxfp1 but not Rxfp2 was detected in cultured Sertoli cells. Strong immunostaining for Rxfp1 and Rxfp2 was seen in muscular and epithelial layers of the vas deferens and in arteriolar walls. Relaxin did not affect contractility and cyclic AMP production of the vas deferens, but increased the levels of mRNA for metalloproteinase-7.</p> <p>Conclusion</p> <p>Rxfp1 and Rxfp2 are widely and similarly distributed throughout the male reproductive tract. Our results suggest that Rxfp1 on spermatids and Sertoli cells may be important in spermatogenesis. Relaxin in the vas deferens does not affect contractility, but may affect vascular compliance and collagen and matrix remodeling.</p

O cenário do surgimento e o impacto do teorema da incompletude de Gödel na matemática

Este artigo trata do panorama das discussões matemáticas mantidas entre os matemáticos à época em que Gödel apresentou à comunidade matemática seu teorema da incompletude. Argumenta-se que o teorema da incompletude de Gödel (TIG) é um teorema mais para a alma do que para as mãos dos matemáticos. Afirma-se ser ele importante porque mostra que a matemática não pode comunicar (provar) todas as suas verdades. Porém, as provas de que a aritmética básica dos naturais é incompleta e incompletável e da impossibilidade de demonstrar a sua não contradição não impossibilita que a matemática continue sendo produzida. A linha de argumentação exposta segue apresentando: o cenário matemático vigente no momento da publicação do TIG; o ponto de incidência deste resultado na matemática, o impacto deste teorema nesta ciência, bem como, como ele foi compreendido e acolhido pelos matemáticos

Молекулярно-генетическая специфика фенилкетонурии в Республике Молдова (2018- 2019)

Institute of Mother and Child, Chisinau, Republic of MoldovaIntroducere: Fenilcetonuria (PKU) este o patologie autosomal recesivă, cauzată de deficienţa enzimei fenilalanin hidroxilaza, care duce la acumularea fenilalaninei (Phe) serice, cauzând retard mental. Scopul lucrării: Diagnosticarea PKU în baza screeningului neonatal, organizat în perioada 2018-2019, precum şi cercetarea, la nivel molecular genetic, a pacienţilor identificaţi pentru a stabili mutaţiile cauzatoare de boală. Material şi metode: În perioada 2018-2019 au fost supuşi screeningului 62943 de nou-născuţi din Moldova (rata de screening 96,16%), în baza metodei fluorometrice. La 17 copii a fost detectată PKU, iar 11 au fost testaţi genetic prin metoda PCR/RFLP la 6 mutaţii în gena PAH (p.R408W, p.P281L, p.L48S, .p.R158Q, p.R252W, p.R261Q) şi secvenţierea exonilor 2,3,5,6-8,10-13 ai genei PAH după metoda Sanger. Rezultate: Analiza molecular-genetică prin PCR/RFLP a permis detectarea mutaţiilor în 45,45% de cazuri. Cel mai frecvent genotip a fost p.R408W/p.L48S, stabilit la 3 pacienţi. Analiza suplimentară prin metoda Sanger a permis detectarea genotipului complet în restul cazurilor, determinând mutaţii de tip splicing (IVS7+4A>G, IVS11+7T>C, IVS12+1G>A), missense (p.A342T) şi nonsense (p.R111*). Concluzii: În urma studiului a fost detectat un spectru de mutaţii specifice cohortei PKU din Moldova, datorită metodelor eficiente de screening şi diagnostic.Введение: Фенилкетонурия (ФКУ) – это генетическое заболевание с аутосомно рецессивной моделью наследования обусловленное дефицитом фенилаланин- гидроксилазы. Дисфункция данного энзима приводит к накоплению фенилаланина в крови и последующим дегенеративным процессам в нервной системе. Цель: Целью исследования была диагностика пациентов с ФКУ с помощью неонатального скрининга, в период 2018-2019 годов, а так же проведение молекулярно- генетического изучения пациентов для идентификации мутаций в гене PAH. Материалы и методы: В период 2018-2019 годов, неонатальному скринингу подверглись 62943 новорождённых (доля 96,16%), по средством флуорометрического метода. У 17 детей была выявлена ФКУ, 11 из которых были подвержены ПЦР/ПДРФ анализу с целью выявления 6 частых мутаций в гене PAH (p.R408W, p.P281L, p.L48S, p.R158Q, p.R252W, p.R261Q) и секвенированию экзонов 2,3,5,6-8,10-13 гена PAH при помощи капилярного электрофореза по методике Sanger. Результаты: В результате проведения скрининга, были выявлены 376 подозреваемых случаев ФКУ, у которых показатели фенилаланина были больше 3 мг/дЛ, Образцы ДНК были получены у 11 из 17 подтвержденных пациентов. Молекулярно- генетическое исследование при помощи ПЦР/ПДРФ, позволило выявить мутации в 45,45% случаев. Самым распространённым генотипом оказался p.R408W/ p.L48S, выявленный у 3 пациентов. Пополнение исследования результатами секвенирования кодирующей части гена PAH позволило определить полный генотип в остальных случаях

Hemophagocytic syndrome associated with hepatitis A: case report and literature review

Virus-Associated Hemophagocytic Syndrome (VAHS) is a severe hematological disorder related to some viral infections. It is an illness characterized by persistent fever, pancytopenia, splenomegaly, hyperferritinemia and, the most important, hemophagocytosis observed in the bone marrow, liver and/or lymph nodes. VAHS associated with hepatitis A virus infection is rarely described, despite the high incidence of this viral infection in the population in general. There is no consensus in the literature regarding the optimal treatment of VAHS. In this article the clinical features, presumed pathogenesis, diagnostic criteria and treatment of VAHS are discussed, including description of cases of VAHS related to hepatitis A virus infection found in the medical literature.A síndrome hemofagocitária associada a vírus é uma doença hematológica grave relacionada com algumas síndromes virais. É doença caracterizada por febre persistente, pancitopenia, esplenomegalia, hiperferritinemia e hemofagocitose na medula óssea, fígado e/ou linfonodos. A síndrome hemofagocitária associada ao vírus da hepatite A é raramente descrita, apesar da alta incidência desta infecção viral na população como um todo. Não existem consensos na literatura a respeito do tratamento desta morbidade. Neste artigo, os aspectos clínicos, patogênese, critérios diagnósticos e tratamento da síndrome hemofagocitária associada a vírus, incluindo a descrição de casos publicados da síndrome associada ao vírus da hepatite A

Receptores e função do estrógeno no sistema reprodutor masculino

A substantial advance in our understanding on the estrogen signaling occurred in the last decade. Estrogens interact with two receptors, ESR1 and ESR2, also known as ERα and ERβ, respectively. ESR1 and ESR2 belong to the nuclear receptor family of transcription factors. In addition to the well established transcriptional effects, estrogens can mediate rapid signaling, triggered within seconds or minutes. These rapid effects can be mediated by ESRs or the G protein-coupled estrogen receptor GPER, also known as GPR30. The effects of estrogen on cell proliferation, differentiation and apoptosis are often mediated by growth factors. The understanding of the cross-talk between androgen, estrogen and growth factors signaling pathways is therefore essential to understand the physiopathological mechanisms of estrogen action. In this review we focused on recent discoveries about the nature of the estrogen receptors, and on the signaling and function of estrogen in the male reproductive system.Durante a última década, ocorreu um avanço substancial no conhecimento da sinalização do estrógeno. Estrógenos interagem com dois receptores, ESR1 e ESR2, também conhecidos como ERα e ERβ, respectivamente. ESR1 e ESR2 pertencem à família de receptores nucleares, que funcionam como fatores de transcrição. Além dos bem estabelecidos efeitos transcricionais, os estrógenos medeiam a sinalização rápida, desencadeada dentro de segundos ou minutos. Esses efeitos rápidos podem ser mediados por ESRs ou pelo receptor de estrógeno acoplado à proteína G, GPER, também conhecido como GPR30. Os efeitos de estrógenos sobre a proliferação celular, diferenciação e apoptose são, muitas vezes, mediados por fatores de crescimento. Portanto, a compreensão da interação entre as vias de sinalização de andrógeno, estrógeno e fatores de crescimento é essencial para entender os mecanismos fisiopatológicos envolvidos na ação estrogênica. Nesta revisão, foram abordadas descobertas recentes sobre a estrutura dos receptores, a sinalização e a função do estrógeno no sistema reprodutor masculino.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Departamento de FarmacologiaUNIFESP, EPM, Depto. de FarmacologiaSciEL