Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment

Mucopolysaccharidoses (MPS) are rare genetic diseases caused by the deficiency of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG) breakdown pathway. This metabolic block leads to the accumulation of GAG in various organs and tissues of the affected patients, resulting in a multisystemic clinical picture, sometimes including cognitive impairment. Until the beginning of the XXI century, treatment was mainly supportive. Bone marrow transplantation improved the natural course of the disease in some types of MPS, but the morbidity and mortality restricted its use to selected cases. The identification of the genes involved, the new molecular biology tools and the availability of animal models made it possible to develop specific enzyme replacement therapies (ERT) for these diseases. At present, a great number of Brazilian medical centers from all regions of the country have experience with ERT for MPS I, II, and VI, acquired not only through patient treatment but also in clinical trials. Taking the three types of MPS together, over 200 patients have been treated with ERT in our country. This document summarizes the experience of the professionals involved, along with the data available in the international literature, bringing together and harmonizing the information available on the management of these severe and progressive diseases, thus disclosing new prospects for Brazilian patients affected by these conditions

Proyecto, investigación e innovación en urbanismo, arquitectura y diseño industrial

Actas de congresoLas VII Jornadas de Investigación “Encuentro y Reflexión” y I Jornadas de Investigación de becarios y doctorandos. Proyecto, investigación e innovación en Urbanismo, Arquitectura y Diseño Industrial se centraron en cuatro ejes: el proyecto; la dimensión tecnológica y la gestión; la dimensión social y cultural y la enseñanza en Arquitectura, Urbanismo y Diseño Industrial, sustentados en las líneas prioritarias de investigación definidas epistemológicamente en el Consejo Asesor de Ciencia y Tecnología de esta Universidad Nacional de Córdoba. Con el objetivo de afianzar continuidad, formación y transferencia de métodos, metodología y recursos se incorporó becarios y doctorandos de los Institutos de investigación. La Comisión Honoraria la integraron las tres Secretarias de Investigación de la Facultad, arquitectas Marta Polo, quien fundó y María del Carmen Franchello y Nora Gutiérrez Crespo quienes continuaron la tradición de la buena práctica del debate en la cotidianeidad de la propia Facultad. Los textos que conforman las VII Jornadas son los avances y resultados de las investigaciones realizadas en el bienio 2016-2018.Fil: Novello, María Alejandra. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Repiso, Luciana. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Mir, Guillermo. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Brizuela, Natalia. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Herrera, Fernanda. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Períes, Lucas. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Romo, Claudia. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Gordillo, Natalia. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; ArgentinaFil: Andrade, Elena Beatriz. Universidad Nacional de Córdoba. Facultad de Arquitectura, Urbanismo y Diseño; Argentin

The Strategic Plan and Local Economic Development of Cordoba, Argentina

The Strategic Plan of Cordoba (SPC) is one of the few strategic urban development plans in Latin America, which has actually been implemented in the majority of its components. The SPC was conceived as a collective and global project of the city as a whole without excessive conflicting interests or special ownership by a few. The SPC integrated a policy of public works – oriented at the “social debt” which existed in the city – with a work on urban norms and directions and economic development. The SPC as a space for articulation achieved to organise a great number of representatives from civil society organisations, achieving legitimacy and representation in the work of the SPC. The SPC has given a new orientation and increased the municipal capacity to manage the city. The SPC has developed innovative and participatory forms of management (e.g. the Follow-Up and Monitoring Groups, with their annual meetings for presenting audit reports known as “presenting the bills”). The population has perceived the incentive to participate in the planning process of the SPC since it was possible to propose concrete projects, as long as these were feasible. The SPC has p

Administración Pública y Sociedad (ApyS). Núm. 11, Diciembre 1998

La Revista Administración Pública y Sociedad (APyS) es una publicación del Instituto de Investigación y Formación en Administración Pública (IIFAP), unidad académica perteneciente a la Facultad de Ciencias Sociales de la Universidad Nacional de Córdoba (UNC).publishedVersio

Yield stability in maize (Zea mays L.) and correlations among the parameters of the Eberhart and Russell, Lin and Binns and Huehn models

Assessment of the stability and adaptability of a genotype to different environments is useful for recommending cultivars for known conditions of cultivation and should be a requirement in breeding programs. Twenty maize (Zea mays L.) cultivars were tested at eight locations in Minas Gerais by the National Center for Maize and Sorghum Research (CNPMS) of the Brazilian Enterprise for Agricultural Research (EMBRAPA) for two years. The experiments involved a randomized complete block design in which three procedures were used to analyze cultivar stability and adaptability. The level of association among the parameters obtained by the three methods was assessed using Spearman's rank correlation. Hybrids 'DINA 170', 'G-96C', 'C 505', 'DINA 70' and 'C 435' had a mean yield greater than 6,000 kg/ha. Eberhart and Russell's regression coefficient (betai) was negative and correlated significantly (P < 0.01) with Lin and Binn's superiority index (Pi), indicating that the most responsive cultivars tended to have smaller Pi. Pi did not correlate with Huehn's nonparametric measurements Si(2) and Si(3) nor with Eberhart and Russell's sigmadi² (P > or = 0.05), but correlated positively with Si(1) (P < 0.05), indicating that superior genotypes (with lower Pi) could also be stable, a finding not commonly reported in the literature. The stability parameters, Si(1), Si(2), Si(3) and sigmadi², correlated positively among each other (P < 0.01), indicating that the stability estimates of the Huehn's nonparametric model did not add important information to those obtained by the Eberhart and Russell's method. Estimates from the Huehn's method, however, showed that stability estimates from nonparametric models are useful alternatives to parametric models. 'DINA 170', which had a greater general mean, was characterized as a cultivar adapted to favorable environments, and was among the most productive in the different environments assessed. The cultivar 'G-96C' showed medium adaptation to all environments (ideal cultivar) and had good stability. Cultivars 'C 505' and 'C435' were alternatives for 'G-96C'. 'DINA 70' showed good adaptability but had low stability.<br>O conhecimento sobre a estabilidade e adaptabilidade de comportamento de genótipos contém informações muito úteis para a recomendação de cultivares para condições de cultivo conhecidas a priori, de modo que a avaliação da resposta dos genótipos às variações ambientais deve ser etapa obrigatória em programas de melhoramento. Para caracterizar 20 cultivares de milho, foram realizados dez ensaios (oito localidades do Estado de Minas Gerais, em dois anos) no delineamento de blocos ao acaso, pelo Centro Nacional de Pesquisa de Milho e Sorgo (CNPMS) da Empresa Brasileira de Pesquisa Agropecuária (Embrapa). Três procedimentos estatísticos foram adotados para a análise da estabilidade e adaptabilidade dos cultivares e avaliou-se o grau de associação entre os parâmetros dos três métodos por meio da correlação classificatória de Spearman. 'DINA 170', 'G-96C', 'C 505', 'DINA 70' e 'C 435' destacaram-se com produtividades médias superiores a 6.000 kg/ha. O coeficiente de regressão (betai) de Eberhart e Russell foi negativo e significativamente correlacionado (P < 0,01) com o índice de superioridade (Pi) de Lin e Binns, indicando que os cultivares mais responsivos tenderam a apresentar menor Pi. Pi não se correlacionou com as medidas não-paramétricas Si(2) e Si(3) de Huehn e com sigma²di de Eberhart e Russell (P > ou = 0,05), mas correlacionou-se positiva e significativamente com Si(1) (P < 0,05), indicando que genótipos mais produtivos e responsivos (com menor Pi) também podem ser estáveis (com menor Si(1)), embora tal situação não seja comumente observada na literatura. Huehn afirma que as estimativas de Si(1) indicam somente estabilidade, quando os dados são corrigidos. Os parâmetros de estabilidade Si(1), Si(2), Si(3) e sigma²di correlacionaram-se positiva e significativamente entre si (P < 0,01), indicando que as estimativas de estabilidade do modelo não-paramétrico de Huehn não acrescentaram maiores informações, além das obtidas pelo método de Eberhart e Russell, ao mesmo tempo que mostram que estimativas de estabilidade de modelos não-paramétricos são alternativas úteis às estimativas de modelos paramétricos. 'DINA 170', com maior média geral, caracterizou-se como um cultivar adaptado para ambientes favoráveis e permaneceu entre os mais produtivos no conjunto de ambientes avaliados. 'G-96C' comportou-se como um cultivar de média adaptação a todos os ambientes (cultivar ideal) e estável, enquanto 'C 505' e 'C 435' podem ser considerados alternativos para a 'G-96C'. 'DINA 70', também de adaptabilidade geral, caracterizou-se como de baixa estabilidade