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Sample records for fragil em vaso

  1. Discontinuous finite element formulation for bodies of revolution with application in the prevention of fragile fracture in pressure vessel of PWR reactors; Formulacao de elementos finitos descontinuos para corpos de revolucao com aplicacao na prevencao de fratura fragil em vaso de pressao de reatores PWR

    Energy Technology Data Exchange (ETDEWEB)

    Benitez Alvarez, Gustavo

    1999-08-15

    In this work, a hybrid formulation is established for bodies of revolution, based on the equation of Fourier series for the discontinuous finite element method, analogous to the one that exists in the classical finite element method. Furthermore, a methodology to analyse the prevention of fragile fracture in pressure vessel of pressurized water reactors is presented. The results obtained suggest that careful analysis must be made for non symmetric refrigeration. (author)

  2. Cultivo de girassol ornamental (Helianthus annuus L. em vaso sob diferentes doses de paclobutrazol

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    JOSÉ GERALDO BARBOSA

    2008-07-01

    Full Text Available O girassol apresenta facilidade de propagação – tempo curto para colheita, e uma inflorescência atrativa, o que aumenta sua procura para a confecção de arranjos e vasos. O grande crescimento natural tem sido um dos entraves para que o girassol seja explorado como planta ornamental. O paclobutrazol é um regulador de crescimento que inibe a biossíntese de giberelina e reduz o alongamento da haste, podendo ser usado em diversas espécies ornamentais. Assim, para verificar a resposta de plantas de girassol, variedade Golden, dobrado, cultivado em vaso com diferentes substratos à aplicação de paclobutrazol, instalou-se experimento, utilizando-se o delineamento experimental em blocos casualizados em arranjo fatorial 4 x 4 com quatro substratos (solo:areia:casca de arroz carbonizada 2:0,5:2 v/v; solo:areia:carvão de cana, 2:0,5:2 v/v; solo:areia:carvão vegetal 2:0,5:2 v/v e substrato comercial e quatro doses de paclobutrazol (0; 2; 4; 6 mg i.a./vaso com 3 repetições. Avaliaram-se os parâmetros altura de planta (cm, número de folhas, concentração de clorofila em unidades SPAD. Verificou-se melhor qualidade nas plantas cultivadas nos substratos que continham casca de arroz carbonizada e carvão de cana. Houve aumento da unidade SPAD com o aumento das doses de paclobutrazol, sendo o máximo estimado na dose de 5,08 mg i.a./vaso. Observou-se uma relação inversa entre a dosagem de paclobutrazol e a altura da planta, o que permite constatar que a aplicação da dose de 6 mg i.a./vaso reduziu o porte das plantas de girassol,possibilitando boa harmonia de vaso, sendo, portanto, a mais recomendada.

  3. Detecção de vasos sanguineos em imagens de fundo de olhos

    OpenAIRE

    Sérgio de Sá Leitão Paiva Júnior

    2006-01-01

    Descrevemos neste trabalho um método automático para localizar e segmentar vasos sanguíneos em imagens de fundo de olhos. Este método pode ser usado por um software especialista em imagens de retina para diagnosticar automaticamente doenças como retinopatia diabética entre out r a s , para avaliação da evolução do tratamento, e para estudos clínicos. O método proposto é baseado na combinação do procedimento 2d matched fi l t e r ( usado na localização dos vasos) com o algoritmo de Hoshen-Kope...

  4. Crescimento de girassol como flor em vaso em função de doses de paclobutrazol

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    Christina da Silva Wanderley

    2014-02-01

    Full Text Available O girassol (Helianthus annuus L., que é comumente cultivado para produção de sementes e óleo, também apresenta potencial de uso como planta ornamental. Entretanto, seu porte elevado dificulta essa utilização, principalmente como flor em vaso. Objetivou-se neste experimento avaliar os efeitos do regulador de crescimento paclobutrazol sobre o crescimento de plantas de girassol em vaso, cultivadas em sistema hidropônico, dentro de estufa, em vasos preenchidos com sílica moída. Os dois genótipos de girassol utilizados foram BRS Oásis, com capítulo marrom e altura média de 1,85 m, e Helio 358, com capítulo amarelo e altura média de 1,45 m. As doses de paclobutrazol avaliadas foram 0; 0,5; 1; 2; 4; e 6 mg L-1 , aplicadas diretamente no substrato de sílica moída. A solução nutritiva utilizada foi a de Hoagland, e as plantas foram mantidas sob aeração constante. O delineamento experimental foi o de blocos casualizados, em esquema fatorial com cinco repetições, totalizando 60 parcelas. Avaliaram-se a altura das plantas e a massa de matéria seca de raiz, caule, folhas e de capítulos. Houve efeito do paclobutrazol sobre o crescimento das plantas. O uso do paclobutrazol na dose de 2mgL-1 reduz a altura do girassol, porém não afeta a qualidade da inflorescência, o que pode viabilizar o seu uso na produção de flores de girassol como ornamentais. A sensibilidade ao paclobutrazol é maior para o genótipo Helio 358 em relação ao genótipo BRS Oásis.

  5. Estenose aórtica supravalvar em adulto com anomalia de vasos da base e insuficiência aórtica

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    Acrisio Sales Valente

    2013-12-01

    Full Text Available A estenose aórtica supravalvar é uma rara cardiopatia congênita, bastante incomum em adultos. Apresentamos um caso de estenose aórtica supravalvar em adulto com anomalia de vasos do arco aórtico, já com presença de insuficiência aórtica importante, tratado com êxito por meio de plastia da aorta ascendente e troca valvar aórtica.

  6. Efeitos do boro em cana-de-açúcar cultivada em vasos contendo solo Effects of boron on sugarcane cultivated in pots with soil

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    A. Espironelo

    1976-07-01

    Full Text Available Em ensaios em vasos foram estudados os efeitos do boro na cana-de-açúcar var. CB 41-14 (cana-planta e cana-soca, através da administração de doses crescentes do elemento à cana-planta cultivada em solo da série Ibitiruna, do Município de Piracicaba, com teor muito baixo de boro solúvel em água quente. Ao solo dos vasos (80 litros de capacidade foram administradas as doses de 0, 0,5, 1, 2, 3, 4 e 8 ppm B, sob a forma de ácido bórico. Foram obtidos dados de produção de cana (planta e soca e do teor de boro em folhas de três posições na planta (+3, +1 e -1, em amostragens feitas aos cinco e oito meses de idade da cana-planta, e calcularam-se dados de açúcar-provável (cana-planta e soca. O boro aplicado ao solo não proporcionou aumentos nas produções de cana e de açúcar-provável, tanto em cana-planta como em cana-soca, induzindo, entretanto, efeito depressivo em ambos os casos, pela dose mais elevada (8 ppm B na cana-planta. Os teores de B nas folhas ( + 3, +1 e -1 de cana-planta, aos cinco meses de idade, cresceram com as doses administradas ao solo, alcançando níveis muito elevados (289 ppm no tratamento 8 ppm B, com a média geral atingindo 124,4 ppm B. Aos oito meses, os valores foram bem mais baixos (média geral 61,4 ppm B e não seletivos em relação à dose do boro aplicada. A melhor época de amostragem estaria em torno de cinco meses. As três posições de folha estudadas mostraram-se eficientes em relação às doses de boro aplicadas ao solo, sendo que +3 e +1 deram valores mais altos do que -1.The effects of boron on the behaviour of sugarcane (plant-crop and first ratton crop var. CB 41-14 were studied in a trial pot with increasing doses of this micronutrient to Ibitiruna soil from Piracicaba, SP, considered poor in boron content. The boron applied did not increase cane growth nor sugar content, but 8 ppm B reduced both cane and sugar production. Chemical analysis with leaves 5 months old revealed

  7. Avaliação de substratos para cultivo de crisântemo(Crysanthemum morifolium Ramat. - Compositae) 'White Polaris' em vasos.

    OpenAIRE

    Marília Maia de Souza; Luiz Eduardo F. Fontes; Luiz Carlos Lopes

    1995-01-01

    Com intuito de selecionar substratos que proporcionem melhor crescimento e floração do crisântemo, em vaso, foram cultivadas mudas de crisântemo 'White Polaris' em substratos solo:areia:casca de arroz carbonizada; solo:areia:casca de arroz não carbonizada; solo:areia:casca de café; solo:areia: serragem; solo:areia:vermiculita, em quatro proporções volumétricas (1:0,5:1, 1:0,5:2, 1:1:2 e 2:0,5:2), e em solo: areia, na proporção volumétrica de 1:0,5, com densidade variando de 0,6-0,7g/cm3. Aval...

  8. Efeito da aplicação de calcário em estacas de Populus deltoides Bartr. ex Marsh cultivadas em vaso.

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    Jean Gabriel da Silva Medeiros

    2010-08-01

    Full Text Available Com este trabalho estudou-se o comportamento do Populus deltoides referente ao seu desenvolvimento, quando submetido a diferentes doses de calcário. A pesquisa foi realizada no Centro Tecnológico de Silvicultura, na Universidade Federal de Santa Maria, município de Santa Maria, RS. O experimento constituiu-se de 48 unidades experimentais ordenadas em delineamento inteiramente casualizado, tendo seis tratamentos com oito repetições. Cada unidade experimental era constituída por um vaso de três litros contendo uma única estaca de Populus deltoides. Os tratamentos constituíram-se em cinco doses diferentes de calagem (T2: 7 toneladas de calcário/ha; T3: 14 toneladas de calcário/ha; T4: 21 toneladas de calcário/ha; T5: 29 toneladas de calcário/ha; T6: 38 toneladas de calcário/ha mais a testemunha, determinados com base na análise do solo utilizado no experimento. As mudas foram avaliadas, após 120 dias da data de plantio, quanto ao seu crescimento em altura, diâmetro do colo e peso seco da parte aérea. As melhores médias foram alcançadas pelos tratamentos sem calagem e com sete toneladas de calcário por ha em todos os parâmetros observados. Os demais tratamentos apresentaram médias inferiores, eles não possuem diferença significativa ao nível de 5% entre si, para todos os parâmetros observados.

  9. Substratos para cultivo de feijoeiro em vasos com fertirrigação Substrates for common bean grown in flowerpots with fertirrigation

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    Leo Hoffmann Junior

    2007-01-01

    Full Text Available Considerando a necessidade de se identificar um substrato apropriado para o cultivo de feijoeiro em vasos - objetivo deste trabalho - foram desenvolvidos dois experimentos em casa-de-vegetação, durante o ano de 2005, com a cultivar de feijoeiro TPS Nobre. Sete substratos foram avaliados: Plantimax®, casca de arroz carbonizada, vermiculita, areia, casca de arroz carbonizada + 20% de Plantimax®, casca de arroz carbonizada + 20% de vermiculita e casca de arroz carbonizada + 20% de areia. O delineamento experimental utilizado foi inteiramente casualizado, com cinco repetições. Os nutrientes foram fornecidos semanalmente por fertirrigação e a água foi adicionada de maneira a ser reposto o volume consumido. Nos substratos avaliados constataram-se características químicas e físicas diferenciadas. Caracterizou-se o substrato comercial Plantmax® pela elevada capacidade de troca de cátions (30,6 cmol c L-1 e maior capacidade de armazenamento de água por unidade de volume, o que contribuiu para a superioridade na produção de fitomassa seca das plantas e dos componentes do rendimento de grãos de feijão. O substrato comercial Plantmax® é apropriado para o cultivo de feijoeiro em vasos com o uso de fertirrigação.The objective of this paper was to identify an appropriate substrate for common bean grown in flowerpots. The experiments were carried out in a green house during 2005, with the TPS Nobre common bean cultivar. Seven substrates were evaluated: Plantimax®, carbonized rice husks, vermiculite, sand, carbonized rice husks + 20% of Plantimax®, carbonized rice husks + 20% of vermiculite and carbonized rice husks + 20% of sand. The experimental design was completely randomized with five replications. The nutrients were supplied weekly to fertigation and the water was added to refill the consumed volume. The evaluated substrates had different chemical and physical characteristics. The commercial substrate Plantmax® was characterized

  10. Efeito de diferentes fertilizantes nitrogenados na produção do trigo em vasos Effect of different nitrogen fertilizers on wheat production

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    Hermano Gargantini

    1972-01-01

    Full Text Available Estudou-se a influência do sulfato de amônio, do salitre-do-chile e da uréia no desenvolvimento e na produção do trigo, em vasos, em casa de vegetação, empregando-se um Latossolo Vermelho Escuro orto, do município de Itapetininga, Estado de São Paulo. Os resultados indicaram que, para produção de matéria seca, não houve diferença entre os fertilizantes utilizados, porém para produção de grãos o sulfato de amônio foi o mais eficiente.The influence of ammonium sulphate, Chilean nitrate, and urea on wheat production was studied in a greenhouse pot experiment using a ortho Dark Red Latosol. The results showed that in the dry matter production any difference was noted among the fertilizers compared; in grain production, however, ammonium sulphate gave the best results.

  11. SISTEMA AUTOMÁTICO DE FORNECIMENTO DE SOLUÇÃO NUTRITIVA PARA CULTIVO HIDROPÔNICO DE PLANTAS EM VASOS

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    Maia Nilson Borlina

    1999-01-01

    Full Text Available Desenvolveu-se um sistema automático de fornecimento de solução nutritiva com o objetivo de melhorar os resultados obtidos em trabalhos de pesquisa envolvendo a nutrição de plantas cultivadas em hidroponia. O sistema consiste de um conjunto de reservatórios equipados com moto-bombas individuais, dotados de bóias para controle dos níveis das soluções nutritivas nos vasos e um temporizador. O cultivo de plantas de hortelã (Mentha crispa mostrou que a produção de folhas obtida com o sistema automático foi cerca de três vezes maior que a produção obtida com o sistema convencional. Constatou-se variação de concentrações dos nutrientes na parte aérea e raízes quando se comparou o sistema automático e convencional.

  12. Análise de crescimento de gérbera de vaso conduzida em diferentes substratos Growth analysis of potted gerbera grown in different substrates

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    Fernanda Ludwig

    2010-03-01

    Full Text Available O cultivo e o comércio de gérbera em vaso são recentes e há uma grande demanda quanto à seleção de substratos adequados à sua produção. O presente trabalho foi desenvolvido com o objetivo de avaliar o crescimento de duas cultivares de gérbera de vaso, conduzidas com diferentes substratos. O experimento foi realizado em casa de vegetação entre setembro e novembro de 2008, em Botucatu-SP. O delineamento experimental utilizado foi de blocos ao acaso em esquema fatorial 5x2 (substratos x cultivares e quatro repetições. Plantas de gérbera, cultivares Cherry e Red, foram avaliadas quinzenalmente (1, 15, 29 e 43 dias após aclimatação (DAA quanto à área foliar e a fitomassa seca da parte aérea, as quais foram ajustadas em relação ao tempo com o programa ANACRES para obtenção da razão da área foliar (RAF, taxa de crescimento relativo (TCR e taxa de assimilação líquida (TAL. As plantas de gérbera apresentaram melhor desenvolvimento quando conduzidas no substrato 4 (30% casca de pinus, 30% C1, 20% fibra de coco granulada, 20% fibra de coco mista. A seleção de substratos adequados ao desenvolvimento de gérbera deve considerar, principalmente, as características químicas e físicas dos mesmos. Para as cultivares estudadas, valores de pH do substrato inferiores a 5,5 e superiores a 7,0 foram inadequados para o desenvolvimento, bem como densidades úmidas superiores a 530 kg m-3.Cultivation and commercialization of potted gerbera are recent issues and there is a great demand for adequate substrates for its production. This work aimed to evaluate the growth of two potted gerbera cultivars (Cherry and Red grown in different substrates. The experiment was carried out in a greenhouse from September to November 2008 at Botucatu, São Paulo State, Brazil. Experimental design was randomized blocks in a 5x2 factorial arrangement (substrates x cultivars. Gerbera plants leaf area and dry phytomass were evaluated fortnightly (1, 15

  13. Manejo da irrigação na cultura do crisântemo em vaso, cultivar rage, cultivado em ambiente protegido Irrigation schedule in pot chrysanthemum, cultivar rage, grown in greenhouse

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    Maryzélia F. de Farias

    2004-04-01

    Full Text Available Dentre as dificuldades que os produtores têm encontrado ao adotarem o cultivo em ambiente protegido, destaca-se a falta de dados específicos sobre o uso racional da água e o desconhecimento da quantidade e do momento de irrigar. Esta pesquisa foi desenvolvida na propriedade de um produtor, no Distrito de Holambra II, região de Paranapanema - SP, em cultivos rotineiramente desenvolvidos pelo produtor, buscando melhor representatividade dos dados obtidos. O objetivo principal deste experimento foi identificar a tensão de água no solo que pudesse resultar em melhor qualidade comercial da cultivar de crisântemo "Rage", cultivado em vaso e mantido em ambiente protegido. O delineamento experimental foi o inteiramente casualizado, com duas repetições. Os tratamentos foram definidos por seis níveis de tensão de água: -2; -3; -4; -6; -10 e -30 kPa. Para cada tensão, foram calculadas a altura correspondente na coluna de mercúrio do tensiômetro, as lâminas e o tempo de irrigação. Os resultados obtidos demonstraram que a cultivar Rage obteve a maior porcentagem de vasos de alta qualidade (A1 no tratamento irrigado com a tensão de -4 kPa. O tratamento irrigado, quando atingia -30 kPa, resultou na menor porcentagem de vasos A1.The most important difficulty that the producers have been found to adopt the cultivation in plastic greenhouses is the lack of specific data on the rational use of the water and informations about when and how much water apply. This research was developed in a farm in Paranapanema - SP, Brazil, with tradition in pot chrysanthemum grow. The main objective was to identify the soil water tension that could result in better commercial quality of chrysanthemum Rage, cultivated in pot and maintained in greenhouse. The treatments were defined for six levels of substrate water tension: -2; -3; -4; -6; -10 and -30 kPa. The irrigation time was defined for each tension and also the equivalent height int the mercury column of

  14. Morfometria do coração e dos vasos da base e sua implicação no mergulho em Chelonia mydas

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    Janine K.F.S. Braz

    2013-12-01

    Full Text Available Objetivou-se caracterizar a morfologia das câmaras cardíacas e das artérias aortas e pulmonares da espécie Chelonia mydas. Foram avaliados 11 espécimes de C. mydas mortas coletadas no litoral do estado do Rio Grande do Norte, Brasil. Os animais foram necropsiados para a obtenção do coração, fragmentos das artérias aorta e pulmonares direita e esquerda. Os vasos adquiridos foram fixados em formol e submetidos ao processamento histológico de rotina e coloração com Técnica de Verhoff modificada. Enquanto, do coração, os parâmetros largura, altura base-ápice e a circunferência ventricular foram mensurados por meio do paquímetro. Nessa espécie a microscopia das artérias pulmonares e artérias aortas variaram de acordo com o antímero. A maior espessura relativa do Cavum Venosum (CV auxilia no bombeamento cardíaco durante o mergulho e sua menor espessura direita é uma vantagem para a dilatação ventricular durante a imersão profunda enquanto que a quantificação das lâminas elásticas e fibras musculares da túnica média das artérias aortas e pulmonares direita e esquerda comprovaram que a túnica média das aortas predomina o componente elástico vs. muscular, entretanto, nas artérias pulmonares o componente elástico não-predomina. Essa angioarquitetura pode estar relacionada com a capacidade de mergulho, favorecendo um maior aproveitamento do sangue oxigenado armazenado previamente durante o período de apneia.

  15. Densidade de plantas e número de drenos influenciando a produtividade de roseiras cultivadas em vaso Planting density and number of drains influencing the productivity of rose plants cultivated in pots

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    Thales VA Viana

    2008-12-01

    Full Text Available O cultivo de flores no estado do Ceará vem se ampliando nos últimos anos, principalmente nas regiões serranas que proporcionam clima favorável ao desenvolvimento de diversas culturas. Entretanto, poucos são os trabalhos desenvolvidos nessa área, fazendo com que os produtores se utilizem do empirismo no desenvolvimento dos cultivos. Por conseguinte, esse trabalho teve como objetivo avaliar os efeitos do número de plantas (2, 3 e 4 e da quantidade de drenos (1 e 8 por vaso no número de hastes por vaso da roseira, em ambiente protegido. O experimento foi conduzido na Empresa Reijers, no município de São Benedito-CE, sendo o delineamento experimental em blocos casualizados em arranjo fatorial 3 x 2, com quatro repetições. Avaliou-se o número de hastes com 35, 40, 50 e 60 cm e, o número total de hastes por vasos de 12,0 L. As maiores quantidades de plantas por vaso proporcionaram um maior número de hastes por área, mas com predomínio de hastes de menor valor comercial. A utilização de um menor número de plantas por vaso resultou em um menor número de hastes por área, mas em maior número de hastes com maior valor comercial. A utilização de vasos com maior número de drenos reduziu o número total de hastes por vaso.The cultivation of flowers in Ceará State has expanded during the last years, especially in highland areas with a favorable climate for several crops. However, there exist only limited research in this area, so that producers work empirically with those crops. Consequently, this research aimed to evaluate the effects of the number of plants (2, 3 and 4 and the amount of drains (1 and 8 per pot on the number of stems per pot, in a protected environment. The experiment was carried out in São Benedito, Ceará State, Brazil, following a 3 x 2 factorial randomized block design with four repetitions. The number of stems with 35, 40, 50, 60 cm and, the total number of stems per pots of 12.0 L were evaluated. The highest

  16. Produção de crisântemo (Dendranthema grandiflora Tzvelev.) ‘Snowdon’ em vaso II: ciclo da cultivar, comprimento, largura e área da folha

    OpenAIRE

    Mainardi,Jucelma de Cássia C. Tolotti; Bellé,Rogério Antônio; Mainardi,Leandro

    2004-01-01

    O objetivo do trabalho foi avaliar a resposta quanto ao ciclo e comprimento, largura e área da folha, ao redutor de crescimento Daminozide, pulverizado na cultivar de crisântemo (Dendranthema grandiflora Tzvelev.) de corte Snowdon, conduzida em vaso. O estudo foi composto por dois ensaios com cinco repetições, no delineamento experimental inteiramente casualizado bifatorial 4 x 2, sendo formado por quatro doses de Daminozide (0, 2000, 4000 e 6000 mg.L-1) e duas freqüências de aplicações (sema...

  17. Macronutrientes em mudas de citros cultivadas em vasos em resposta à adubação NPK Macronutrients in citrus nursery trees grown in pots in response to NPK fertilization

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    Alberto Carlos de Campos Bernardi

    2000-12-01

    Full Text Available Mudas de laranjeira 'Valência' (Citrus sinensis sobre o porta-enxerto de limoeiro 'Cravo' (C. limonia foram cultivadas em vasos com substrato de casca de Pinus, vermiculita e perlita com o objetivo de avaliar-se os efeitos do fornecimento de N, P e K sobre os teores de macronutrientes do porta-enxerto e das mudas. Utilizou-se um esquema de fatorial fracionário (1/55³ com um delineamento em blocos ao acaso. Os tratamentos consistiram em 5 doses (em g por planta de N: 1,25; 6,25; 11,25; 16,25; 21,25; 5 doses de K: 0,42; 3,75; 6,22; 9,34; 12,45; e 5 doses de P: 0,19; 0,89; 1,59; 2,29; 2,99. O N e K foram fornecidos semanalmente, sendo 37,5% da dose para o porta-enxerto. O P foi fornecido totalmente no plantio. Determinaram-se os teores totais de N, P e K, Ca, Mg e S nas folhas novas e velhas, raízes e caule e a extração de N, P e K. Os teores de N relacionaram-se diretamente e os de P e K inversamente com a adubação nitrogenada. Os teores de Ca, Mg e S relacionaram-se positivamente até as doses intermediárias de N utilizadas. Houve efeito inibitório do fertilizante potássico sobre a absorção de Ca e Mg. A acumulação de NPK pelos porta-enxertos foi em torno de 30% pelas raízes e 70% pela parte aérea.'Valencia' sweet orange nursery trees (Citrus sinensis budded on Rangpur lime rootstocks (C. limonia were grown in containers using pine bark, vermiculite and perlite as substracts with the objective of evaluating the NPK fertilization effect on macronutrient levels. The experiment was a complete (1/5 5³ factorial randomized block design. Treatments comprised five concentrations in g per plant of N: 1.25; 6.25; 11.25; 16.25; 21.25; five concnetrations of K: 0.42; 3.75; 6.22; 9.34; 12.45; and five concentration of P: 0.19; 0.89; 1.59; 2.29; 2.99. Nitrogen and K were applieded weekly, and 37.5% of the total fertilization was applied to rootstocks. All P was supplied at planting. Young and old leaves, root and stem total nutrient

  18. Análise de crescimento do crisântemo de vaso, cultivar Puritan, irrigado em diferentes tensões de água em ambiente protegido

    OpenAIRE

    Farias,Maryzélia Furtado de; Saad,João Carlos Cury

    2011-01-01

    A irrigação é prática fundamental para o cultivo de crisântemo, porém seu manejo adequado tem sido negligenciado pelos produtores, resultando em prejuízos no crescimento vegetal e conseqüentes decréscimos na produtividade e na qualidade do produto final. Para melhorar a representatividade dos dados obtidos, o experimento foi desenvolvido na propriedade de um produtor tradicional, no Distrito de Holambra II, município de Paranapanema-SP (23º4'S e 49º00'W). O objetivo principal do trabalho foi ...

  19. Vasculites dos médios e grandes vasos

    OpenAIRE

    Brandt,Hebert Roberto Clivati; Arnone,Marcelo; Valente,Neusa Yuriko Sakai; Sotto,Mirian Nacagami; Criado,Paulo Ricardo

    2009-01-01

    As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal); pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumátic...

  20. Desenvolvimento de mudas de citros cultivadas em vaso em resposta à adubação NPK Development of citrus nursery trees grown in pots in response to NPK fertilization

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    Alberto Carlos de Campos Bernardi

    2000-12-01

    Full Text Available O objetivo deste trabalho foi avaliar o efeito da adubação NPK sobre o desenvolvimento de mudas de laranjeira 'Valência' (Citrus sinensis sobre o porta-enxerto de limoeiro 'Cravo' (C. limonia em vasos com substrato de casca de Pinus, vermiculita e perlita. Utilizou-se um esquema de fatorial fracionário (1/55³ com um delineamento em blocos ao acaso. Os tratamentos consistiram em 5 doses (em g por planta de N: 1,25; 6,25; 11,25; 16,25; 21,25; 5 doses de K: 0,42; 3,75; 6,22; 9,34; 12,45; e 5 doses de P: 0,19; 0,89; 1,59; 2,29; 2,99. O N e o K foram fornecidos semanalmente, sendo 37,5% da dose para o porta-enxerto. O P foi fornecido totalmente no plantio. Avaliaram-se a produção de material seco das folhas, raízes, caule, parte aérea e total; área foliar, relação raízes/parte aérea; altura de plantas; volume radicular, relação área foliar/material seco para os porta-enxertos e as mudas, e o diâmetro do caule para os porta-enxertos. Funções de resposta foram ajustadas e os níveis de nutrientes foram calculados. Os resultados mostraram que as doses de fertilizantes que levaram às melhores respostas para produção de mudas de laranjeira 'Valência'/'Cravo' sobre o porta-enxertos de limoeiro 'Cravo' foram: N, 9,85; P, 2,86; K, 7,99 g por plantas.The objective of this research was to evaluate the NPK fertilizer effect on 'Valencia' sweet orange nursery tree (Citrus sinensis development, budded on Rangpur lime rootstocks (C. limonia in a protected environment using containers with Pinus bark, vermiculite and perlite substrates. The experiment consisted of a complete (1/5 5³ factorial randomized block design. Treatments comprised five concentrations in g per plant of N: 1.25; 6.25; 11.25; 16.25; 21.25; five concnetrations of K: 0.42; 3.75; 6.22; 9.34; 12.45; and five concentration of P: 0.19; 0.89; 1.59; 2.29; 2.99. Nitrogen and K were applieded weekly, and 37.5% of the total fertilization was applied to rootstocks. All P was

  1. Estratégias para redução de girassol ornamental para comercialização em vaso Strategies for reducing the height of potted ornamental sunflower plants

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    Caroline de Moura D'Andréa Mateus

    2009-09-01

    Full Text Available O objetivo do trabalho foi avaliar o uso de tela plástica fotosseletiva e de doses do regulador de crescimento paclobutrazol (0; 0,25; 0,50; 0,75 e 1,0 mg L-1 de substrato no desenvolvimento de plantas de girassol ornamental cv. Sunbright Supreme em ambiente protegido, visando à comercialização em vasos. Dois abrigos foram cobertos com filme de polietileno transparente de 0,1 mm de espessura e, em um deles, o filme plástico foi aplicado sobre tela plástica de cor azul, com 30% de sombreamento. Foram realizadas avaliações de microclima e fitotécnicas. A radiação solar global foi reduzida em 27,5% com o uso da tela fotosseletiva, porém, houve pouca influência sobre a temperatura e umidade relativa do ar. Ainda, o uso de tela fotosseletiva não reduziu o porte das plantas e não influenciou significativamente o diâmetro do capítulo, o número de folhas por planta e o tempo para a floração. Todavia, o uso de paclobutrazol reduziu significativamente a altura das plantas, de modo que permitisse sua comercialização em vasos, sem prejuízos ao seu aspecto visual, visto que não diminuiu o diâmetro do capítulo. O uso do regulador de crescimento retardou a floração, por um tempo que variou com a dose empregada, mas que não ultrapassou uma semana.The objective of the research was to evaluate the use of photoselective plastic shadenet and the effect of plant growth retardant paclobutrazol drench at different concentrations (0; 0.25; 0.50; 0.75 e 1.0 mg L-1 of substrate on potted plants of sunflower cv Sunbright Supreme grown under protected environment. Two greenhouses were covered with transparent polyethylene film, 0,1 mm thick, and at one of them, the plastic film was applied over a blue photoselective plastic shadenet (30% of shading. Microclimatic and agronomic parameters were evaluated. The solar radiation was reduced by 27.5 % when the shadenet was used, which resulted in a very low influence on the air temperature and

  2. Crescimento, desenvolvimento e retardamento da senescência foliar em girassol de vaso (Helianthus annuus L.: fontes e doses de nitrogênio Growth, development and delay of leaf senescence in pot-grown sunflower (Helianthus annuus L.: sources and rates of nitrogen

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    Joelma Dutra Fagundes

    2007-08-01

    Full Text Available O girassol é a quarta oleaginosa em produção de grãos no mundo e alguns genótipos são usados com finalidade ornamental para flor de corte e de vaso (girassol de vaso. O objetivo do trabalho foi avaliar o efeito de diferentes fontes e doses de nitrogênio sobre alguns parâmetros de crescimento, desenvolvimento e no retardamento da senescência das folhas basais em girassol de vaso. Um experimento foi conduzido em casa de vegetação, em Santa Maria, RS. Os tratamentos foram: uréia, nitrato de amônio e nitrato de cálcio nas doses de 0, 50, 100 e 150mg L-1 de N na solução de fertirrigação, com duas aplicações semanais. O experimento foi um bi-fatorial (fontes e doses de N no delineamento inteiramente casualizado, com seis repetições. Cada repetição foi um vaso no 15 (1,3L, 15cm de altura com uma planta por vaso. As variáveis analisadas foram: número final de folhas, altura final de plantas, porcentagem de folhas senescentes no ponto de venda, porcentagem de folhas senescentes no final de vida de vaso, área foliar total da planta, filocrono e a soma térmica acumulada da emergência ao botão visível e da emergência ao ponto de venda. A fonte de N tem influência sobre a área foliar do girassol de vaso, sendo a uréia recomendável para o maior crescimento das folhas. A dose de N em torno de 100mg L-1 aplicada duas vezes por semana via fertirrigação favorece características desejáveis para a comercialização, como precocidade e retardamento da senescência das folhas.Sunflower is the fourth oil grain crop grown worldwide and some genotypes are used with ornamental purpose as cut and pot-grown flower. The objective of this study was to evaluate the effect of different sources and rates of nitrogen on some growth and developmental parameters, and on the delay of leaf senescence in pot-grown sunflower. An experiment was carried out inside a greenhouse in Santa Maria, RS, Brazil. Treatments were: urea, ammonium nitrate and

  3. DISTRIBUIÇÃO ARTERIAL DOS PRINCIPAIS VASOS DA CAVIDADE CELOMÁTICA EM Kinosternon sorpioides ARTERIAL DISTRIBUTION OF THE PRINCIPAL VASES OF THE CAVITY CELOMATIC IN Kinosternon scorpioides

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    Antônia Santos Oliveira

    2009-09-01

    Full Text Available

    Descreveu-se a distribuição arterial dos principais vasos da cavidade celomática de seis exemplares de Kinosternon scorpioides scorpioides, adultos. O circuito arterial foi preenchido com látex corado e os espécimes dissecados foram fixados em solução de formaldeído a 10%. Evidenciou-se que a irrigação dos principais órgãos da cavidade celomática, a partir das aortas, esquerda e direita, formam a aorta dorsal. A aorta esquerda dividiu-se em ramos celíaco e o mesentérico do ramo celíaco derivou duas artérias gástricas. A artéria mesentérica respondeu pela irrigação dos segmentos dos intestinos delgado e grosso, e contribuiu na irrigação do baço através da artéria esplênica. A aorta direita anastomosa-se à esquerda formando a aorta dorsal. Esta, por sua vez, irrigou os rins e órgãos genitais femininos e masculinos e finaliza-se como artérias ilíacas comuns direita e esquerda. Concluiu-se que, em Kinosternon s. scorpioides, a origem e o trajeto das aortas apresentaram semelhança com outras espécies de quelônios, com variações no que se refere à distribuição dos seus ramos primários.

    PALAVRAS-CHAVES: Anatomia, artérias, Kinosternon scorpioides scorpioides.

    Were described the arterial distribution of the vessels in the celomatic cavity of six Kinosternon scorpioides scorpioides adults. The arterial circuit was filled out with red-faced latex, fixation in solution formaldehyd to 10% and dissection subsequent. It was evidenced that the irrigation of the organs of the cavity celomatic happens starting from two aorta, the left and the right aorta that form the dorsal aorta. The left aorta becomes separated in two branches the celiac and the mesenteric artery. The celiac branch originate two gastric arteries. While the mesenteric artery answers for the irrigation of the segments

  4. Esplenectomia subtotal, em cães, com preservação do pólo inferior(PI suprido por vasos do ligamento gastroesplênico

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    Danilo Nagib Salomão Paulo

    Full Text Available O objetivo desse trabalho foi estudar a viabilidade do pólo inferior (PI do baço de cães, após a ligadura e secção da artéria e veia esplênicas. Foram operados 24 cães, mestiços, machos, com peso variando entre 12kg e 14kg. Os animais anestesiados foram submetidos a laparotomia mediana supra e infra-umbilical, com 12cm de comprimento. Nos do grupo 1 fez-se a ligadura e secção da porção superior do ligamento gastroesplênico, ligadura e secção da artéria e veia esplênicas. Após a ligadura do ramo descendente dos vasos esplênicos, o baço foi seccionado transversalmente, a superfície de corte do PI foi suturada e a peça enviada para estudo microscópico. A parede abdominal foi suturada por planos. Os cães foram mantidos vivos e sacrificados no sétimo (subgrupo I A - quatro cães, 15° (subgrupo 18 - cinco cães, trigésimo (subgrupo I C - quatro cães e septuagésimo dia (subgrupo I D - três cães. Nessa ocasião, o Pl foi retirado para estudo. No grupo 2, três cães foram submetidos a laparotomia e manipulação do baço (controle 2 - simulação, para controle morfológico. Esse procedimento foi feito no 15° dia (subgrupo 2A - dois cães e no sexagésimo dia (subgrupo 28 - um cão. Dos 24 cães operados, cinco foram a óbito. A causa foi evisceração (dois cães, hemorragia intraperitoneal (um cão, hemorragia digestiva baixa de causa não esclarecida (um cão e indeterminada (um caso. O exame macroscópico do PI comparado àquele dos controles I e 2 mostrou aspecto duvidoso em apenas dois casos, onde o PI apresentava-se aderido firmemente à parede abdominal e alças intestinais. Não houve, no entanto, diferença estatisticamente significante (p>O.O5 - teste exato de Fisher no número de casos viáveis entre os grupos controles e grupo I. O exame microscópico do PI, comparado àqueles do restante do baço (controle I e ao controle 2 (simulação, mostrou que a referida porção apresentou alterações morfol

  5. Prensa isostática de vasos gêmeos: projeto

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    C. A. Fortulan

    2014-06-01

    Full Text Available O projeto e a fabricação de uma prensa isostática wetbag com dois vasos de diferentes capacidades volumétricas são apresentados. A prensa isostática, amplamente utilizada na conformação de cerâmicas técnicas desde o início do século passado ainda representa importante equipamento laboratorial, onde são requeridas pressões de até 500 MPa. As maiores dificuldades dos usuários destes equipamentos estão na operação e manutenção. Este trabalho apresenta o projeto de uma prensa com dois diferentes vasos em termos de capacidade volumétrica, assim, em função das dimensões das amostras em processo é possível realizar a seleção apropriada de um vaso ou dos dois vasos simultaneamente. Foi escolhida a pressão hidrostática de 210 MPa e projetados dois vasos de diâmetros internos de 95 mm e 55 mm com fechamentos da tampa por clover leaf e por rosca, respectivamente. O resultado obtido é um equipamento compacto, seguro, econômico e de manutenção otimizada, que acelera o processo e a produção de corpos de prova.

  6. Nodulação e fixação biológica de nitrogênio de Adesmia latifolia e Lotus corniculatus em vasos de Leonard Nodulation and biological nitrogen fixation of Adesmia latifolia and Lotus corniculatus in Leonard jars

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    Simone Meredith Scheffer-Basso

    2001-06-01

    Full Text Available Adesmia latifolia, que é uma leguminosa forrageira nativa do Sul do Brasil, destaca-se pelo seu hábito de crescimento estolonífero e por produzir forragem durante a estação fria. O Lotus corniculatus (cornichão é uma forrageira comercialmente utilizada na formação de pastagens hibernais em regiões subtropicais e temperadas. Para ambas as espécies, no entanto, são limitados os trabalhos científicos relacionados ao padrão de nodulação e à fixação biológica de nitrogênio (FBN. Este trabalho foi desenvolvido com o objetivo de analisar tais processos nessas espécies, bem como comparar o seu desenvolvimento morfológico sob diferentes fontes de N: N-mineral (nitrato de amônio 5%, N-simbiótico (inoculação e na ausência dessas fontes (testemunha. O ensaio foi conduzido em casa de vegetação, utilizando-se vasos de Leonard com solução nutritiva; o substrato constou de uma mistura de areia + vermiculita + carvão. O delineamento foi completamente casualizado, com quatro repetições. Ao final de 65 dias, as plantas foram colhidas e avaliadas quanto ao comprimento e volume de raízes, número e peso de nódulos, acúmulo de MS e FBN. A. latifolia destacou-se pelo maior número de nódulos (126/vaso e peso total de nódulos (82,22 mg MS/vaso em relação ao cornichão, com 82 nódulos/vaso e 20,25 mg MS/vaso. A FBN foi mais efetiva em A. latifolia, cujas plantas inoculadas produziram, em média, 37% de MS em relação às plantas supridas com N-mineral; no cornichão esse percentual foi de apenas 15%. A quantidade de N fixado simbioticamente foi de 43,12 mg/vaso em Adesmia e de 9,92 mg N/vaso em cornichão.Adesmia latifolia is a forage legume native from Southern Brazil which is outstanding due to stoloniferous growth habit and for forage production during the cool season. The Lotus corniculatus (birdsfoot trefoil is a forage usually utilized as cool season species in subtropical and temperate regions. For both species, however

  7. Histologia e ultra-estrutura do vaso dorsal de Scaptotrigona postica (Hymenoptera, Apidae em operárias e rainhas de diferentes idades Histology and ultrastructure of the dorsal vessel of Scaptotrigona postica (Hymenoptera, Apidae in workers and queens of various ages

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    Silvana B. Poiani

    2006-03-01

    Full Text Available O vaso dorsal é um órgão tubular localizado na região mediano-dorsal do corpo dos insetos, abaixo do tegumento. Fez-se um estudo de microscopia de luz e eletrônica de transmissão da porção abdominal do vaso dorsal, o coração, em uma espécie de abelha indígena. Foram estudadas operárias e rainhas em diferentes idades. O coração está localizado no sinus pericárdico. A parede cardíaca é formada por fibras musculares estriadas e apresenta aberturas ou ostíolos providos de válvulas. A fibra cardíaca contém miofibrilas arranjadas irregularmente, núcleos alongados ou redondos, mitocôndrias grandes e numerosas, e depósitos de glicogênio. Em operárias e rainhas longevas, as fibras encontram-se em degeneração, evidenciada por vacúolos autofágicos, alterações mitocondriais e acúmulo de corpos mielínicos. Em conclusão, o coração de Scaptotrigona postica é semelhante ao de outros insetos estudados. As alterações encontradas estão relacionadas ao processo de envelhecimento e mantêm relação temporal com a expectativa de vida da casta.The dorsal vessel is a tubular organ located in the mid-dorsal region of the insects body, just below the tegument. This work is a light and electron microscopy study of the abdominal part of the vessel, the heart, in a stingless bee species. Workers and queens of several ages were studied. The heart is located inside the pericardial sinus. Its wall presents openings or ostia provided with valves and is composed by special striated muscular fibers. The cardiac fiber presents myofibrils irregularly arranged, round or elongated nuclei, numerous and large mitochondria, and glycogen deposits. Old workers and queens present fibers degeneration, represented by autophagic vacuoles, mitochondria alterations and accumulation of myelinic bodies. In conclusion, the heart of Scaptotrigona postica Latreille, 1807 is similar to other insects. The changes found are related to aging progress and

  8. MORFOLOGIA DO VASO DEFERENTE DE Callinectes ornatus (Crustacea: Portunoidea COLETADOS NO LITORAL SUL CAPIXABA

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    Marina Lopes Marinho

    2017-02-01

    Full Text Available O siri azul (Callinectes ornatus é um importante recurso pesqueiro, logo conhecer as estruturas que colaboram para seu sucesso reprodutivo é essencial. Este estudo objetivou descrever o vaso deferente dos machos desta espécie de siri, por meio da microscopia de luz. A estrutura do vaso deferente é constituída por três regiões distintas: anterior (VDA revestido por epitélio cilíndrico simples e, em seu lúmen, é possível notar uma secreção acidófila; média (VDM possui epitélio cúbico simples e um grande volume de secreção em seu interior; posterior (VDP que apresenta uma camada muscular bem desenvolvida e vários espermatóforos, encontrados em vários estágios.

  9. Vias de acesso aos grandes vasos mediastinais no trauma torácico

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    Roberto Gonçalves

    Full Text Available O traumatismo é a causa mais comum de mortes na população economicamente ativa e o trauma torácico é responsável direta ou indiretamente em um quarto destas mortes, aos quais as lesões de grandes vasos torácicos relacionam-se às mortes imediatas ou precoces no ambiente hospitalar. Dentro dos doentes que chegam vivos, pode-se dividi-los em estáveis ou instáveis, a via de acesso para a abordagem destes vasos irá depender desta situação, bem como de suas particularidades anatômicas, o que poderá implicar em incisões combinadas para uma adequada exposição. Neste artigo serão revisadas e discutidas as lesões destas estruturas e as vias acesso às mesmas.

  10. Vasculites dos médios e grandes vasos Medium and large vessel vasculitis

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    Hebert Roberto Clivati Brandt

    2009-02-01

    Full Text Available As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal; pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas; e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia. Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis; medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases; and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia. This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.

  11. Vias de acesso aos grandes vasos mediastinais no trauma torácico

    OpenAIRE

    Gonçalves,Roberto; Saad Júnior,Roberto

    2012-01-01

    O traumatismo é a causa mais comum de mortes na população economicamente ativa e o trauma torácico é responsável direta ou indiretamente em um quarto destas mortes, aos quais as lesões de grandes vasos torácicos relacionam-se às mortes imediatas ou precoces no ambiente hospitalar. Dentro dos doentes que chegam vivos, pode-se dividi-los em estáveis ou instáveis, a via de acesso para a abordagem destes vasos irá depender desta situação, bem como de suas particularidades anatômicas, o que poderá...

  12. Produção de crisântemo (Dendranthema grandiflora Tzvelev. 'Snowdon' em vaso I: doses e freqüências de aplicação de daminozide Production of chrysanthemum (Dendranthema grandiflora Tzvelev., cv. 'Snowdon', in pots I: daminozide's concentrations and times of application

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    Jucelma de Cássia Câmara Tolotti

    2003-12-01

    Full Text Available O trabalho teve como objetivo avaliar a resposta de crisântemo através da análise de parâmetros fenométricos, ao redutor de crescimento Daminozide, pulverizado na cultivar de crisântemo de corte "Snowdon", cultivada em vaso. O estudo foi formado por dois ensaios com cinco repetições, usando delineamento experimental inteiramente casualizado em esquema fatorial 4 x 2, sendo formado por quatro doses de Daminozide (0, 2.000, 4.000 e 6.000 mg.L-1 e duas freqüências de aplicações (semanal e bissemanal. Os resultados evidenciaram que as diferentes doses e freqüências de aplicações reduziram a altura da planta, o número de nós, o comprimento de entrenós, o diâmetro da inflorescência e o comprimento do pedúnculo floral. Entretanto, constatou-se aumento dos diâmetros da haste e do pedúnculo floral. Na dose 4.000 mg.L-1 aplicada semanalmente, produziram-se os vasos de melhor qualidade, com altura da planta nos padrões recomendados para comercialização.The aim of this study was to investigate the response of potted chrysanthemum cv. Snowdon, to the growth retardant Daminozide, in terms of fenometric parameters. The study consisted of two experiments with five repetitions. The experiments were bifactorial 4 x 2, with four concentrations of Daminozide (0, 2,000, 4,000 and 6,000 mg.L-1 and two times of application (weekly and bi-weekly of the product. Plant height, number of nodes, length of internodes, diameter of stems and inflorescences, length and diameter of floral peduncle were determinated. All concentrations and times of applications were able to reduce plant height, length of internodes, flower diameter and floral peduncle length. However, the diameter of the stem and floral peduncle were increased. The weekly application of 4,000 mg.L-1 Daminozide produced plants with the best quality within the commercial standards.

  13. Crescimento em vasos, de cultivares de soja e de trigo em função da saturação de alumínio Tolerance of soybean and wheat varieties in sub-soil high in exchangeable aluminum

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    H.A.A. Mascarenhas

    1995-08-01

    Full Text Available Um dos problemas que a acidez do solo causa às plantas é concentrar alguns elementos químicos em nível tóxico, como por exemplo de alumínio (Al e manganês. Visando estudar esse aspecto, foi conduzido um experimento em casa de vegetação, utilizando-se amostra de subsolo, classificado como Latossolo Vermelho Amarelo fase rasa, de extrema acidez e alto teor de Al trocáveL A acidez do substrato foi neutralizada com doses de hidróxido de cálcio, no intuito de reduzir a saturação de Al originalmente de 67 para 45, 30, e 15%. As incubações e os cultivos foram feitos em vasos contendo 6 kg daquele subsolo. Inicialmente foi testada a soja variedades Biloxi, Davis e Santa Rosa, com medições de: altura das plantas, número de nós, tamanho dos internódios, número de folhas trifoliadas, produção de matéria seca da parte aérea e das raízes. Imediatamente após, o sub-solo foi cultivado com trigo variedades BH-1146 e Siete Cerros, sendo medido o comprimento dos seus sistemas radiculares. Verificou-se para a soja que não houve diferenças significativas dentro de cada variedade para as saturações de Al estudadas, evidenciando que todas foram tolerantes ao AL As raízes do trigo BH-1146 não mostraram redução no desenvolvimento em quaisquer saturações de Al. Por outro lado, as da v. Siete Cerros mostraram menor comprimento à medida que se aumentou a saturação de Al, provando sua suscetibilidade a esse elemento.A pot experiment was installed in a greenhouse using an acid sub-soil, classified as a Red Yellow Latosol, shallow phase, high hi aluminum and very low in phosphorus availabilities. The soil acidity was neutralized using calcium hidroxide to reduce the original aluminum saturation of 67 to 45, 30 and 15%. The quantity of subsoil used per pot was 6 kg and 60 kg/ha of P2O5 were applied hi the form of superphosphate. The varieties of soybeans utilized were Biloxi, Davis and Santa Rosa. The results showed that whichever

  14. Osmotic fragility test

    Science.gov (United States)

    Spherocytosis - osmotic fragility; Thalassemia - osmotic fragility ... done to detect conditions called hereditary spherocytosis and thalassemia . Hereditary spherocytosis makes red blood cells more fragile ...

  15. ASPECTOS MORFO-HISTOQUÍMICOS DO VASO DEFERENTE DE Callichirus major (CRUSTACEA: AXIIDEA

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    Tugstênio Lima Souza

    2017-01-01

    Full Text Available Callichirus major é um decápode encontrado na região entre-marés de ambientes costeiros, em especial praias com areia de baixa granulometria. Esta espécie tem sido amplamente explorada para ser usada como isca viva para pesca. Devido à inexistência de informação microscópica acerca de seu aparelho reprodutor, o presente estudo teve como objetivo caracterizar o vaso deferente como forma de contribuir para o entendimento de sua reprodução. Os animais, coletados ao longo da Praia do Corujão, em Piúma/ES, e, em laboratório, tiveram seus vasos deferentes dissecados para o processamento histológico de rotina. O vaso deferente foi caracterizado em três regiões: proximal (VDP, de epitélio cilíndrico com função secretora; média (VDM e distal (VDD, com epitélio variando de pavimentoso a cúbico. No lúmen destas estruturas foi verificada a presença de matriz espermatofórica entre os espermatozoides, sendo esta intensamente reativa para os testes histoquímicos. Sendo assim, com base nas características observadas, foi possível compreender o papel funcional deste órgão na formação do conteúdo espermático desta espécie.

  16. Fragile Elite

    DEFF Research Database (Denmark)

    Bregnbæk, Susanne

    China's One Child Policy and its rigorous national focus on educational testing are well known. But what happens to those "lucky" few at the very top of the pyramid? Fragile Elite explores the contradictions of being an elite student through ethnographic research conducted at two top universities...... shifts in educational policy and definition of what a "quality" student, child, and citizen is in contemporary China....

  17. Pathophysiological aspects of sickle cell vaso-occlusion

    International Nuclear Information System (INIS)

    Nagel, R.L.

    1987-01-01

    This book contains over 30 selections. Some of the titles are: An Animal Model for Sickle Cell Vaso-Occlusion: A Study Using NMR and Technetium Imaging; Sickle-Cell Vaso-Occlusion in an Animal Model: Intravital Microscopy and Radionuclide Imaging of Selective Sequestration of Dense Cells; Magnetic Resonance Imaging, Percentage of Dense Cells, and Serum Prostanoids as Tools for Objective Assessment of Pain Crisis: A Preliminary Report; and Painful Crisis and Dense Echinocytes: Effects of Hydration and Vasodilators

  18. Nitrogênio e saturação por bases no desempenho do capim-pojuca (Paspalum atratum Swalen cv. Pojuca cultivado em vasos Nitrogen and base saturation in the performance of pojuca grass (Paspalum atratum Swalen cv. Pojuca grown in pots

    Directory of Open Access Journals (Sweden)

    Ana Cristina Wyllie Elyas

    2006-06-01

    Full Text Available Objetivou-se avaliar o crescimento e a produção de MS do capim-Pojuca cultivado em Gleissolo háplico. O experimento foi conduzido em casa-de-vegetação do Departamento de Ciência do Solo da UFLA, Lavras MG. A semeadura foi realizada em vasos plásticos, sem furos, com capacidade para 4 dm³ de solo cada. Os tratamentos consistiram de quatro doses de N (0, 100, 200 e 400 mg/dm³ e quatro níveis de saturação por bases do solo (40, 50, 60 e 80%. Os tratamentos foram dispostos em um delineamento de blocos ao acaso, em esquema fatorial 4 x 4, com quatro repetições. Foram efetuados três cortes da forragem a cerca de 4 cm de altura do solo. Antes da realização do primeiro corte, foram avaliadas as taxas de surgimento de perfilhos (TSP, de alongamento de perfilhos (TAlP e de aparecimento de folhas (TApF do capim-Pojuca. Também foram avaliadas a densidade de perfilhos (DP e a produção de matéria seca (MS, nos três cortes. As TSP e TAlP responderam de maneira significativa ao aumento das doses de N, enquanto a TApF foi indiferente ao suprimento de N. A adubação nitrogenada proporcionou aumentos na DP e na produção de MS da gramínea nos três cortes efetuados. Não foi observada resposta significativa das variáveis estudadas ao aumento dos níveis de saturação por bases do solo. Conclui-se que a aplicação de 200 mg/dm³ de N é indispensável para um bom crescimento e boa produção de MS do capim-Pojuca, sendo desnecessário elevar o nível de saturação por bases do solo acima de 40% em Gleissolo háplico.This work aimed to evaluate the growth and dry matter production of Pojuca grass grown in haplic Gleysol. The experiment was carried out in greenhouse of the Soil Science Department of the Universidade Federal de Lavras, Lavras, MG, Brazil. The sowing of seeds was performed in plastic pots without holes, with a capacity of 4 dm³ each of soil. The treatments consisted of four doses of N (0, 100, 200 and 400 mg/dm³ and four

  19. Mechanism of vaso-occlusion in sickle cell anemia

    Science.gov (United States)

    Lei, Huan; Karniadakis, George

    2012-11-01

    Vaso-occlusion crisis is one of the key hallmark of sickle cell anemia. While early studies suggested that the crisis is caused by blockage of a single elongated cell, recent experimental investigations indicate that vaso-occlusion is a complex process triggered by adhesive interactions among different cell groups in multiple stages. Based on dissipative particle dynamics, a multi-scale model for the sickle red blood cells (SS-RBCs), accounting for diversity in both shapes and cell rigidities, is developed to investigate the mechanism of vaso-occlusion crisis. Using this model, the adhesive dynamics of single SS-RBC was investigated in arterioles. Simulation results indicate that the different cell groups (deformable SS2 RBCs, rigid SS4 RBCs, leukocytes, etc.) exhibit heterogeneous adhesive behavior due to the different cell morphologies and membrane rigidities. We further simulate the tube flow of SS-RBC suspensions with different cell fractions. The more adhesive SS2 cells interact with the vascular endothelium and further trap rigid SS4 cells, resulting in vaso-occlusion in vessels less than 15 μm . Under inflammation, adherent leukocytes may also trap SS4 cells, resulting in vaso-occlusion in even larger vessels. This work was supported by the NSF grant CBET-0852948 and the NIH grant R01HL094270.

  20. EFECTO DE LA BIOFERTILIZACIÓN SOBRE EL CRECIMIENTO EN MACETA DE PLANTAS DE CAÑA DE AZÚCAR (Saccharum officinarum EFEITO DA BIOFERTILIZAÇÃO NO CRESCIMENTO DE PLANTAS EM VASOS açúcar de cana (Saccharum officinarum EFFECT OF BIOFERTILIZATION ON THE GROWTH OF POTTED SUGARCANE PLANTS (Saccharum officinarum

    Directory of Open Access Journals (Sweden)

    LILIANA SERNA-COCK

    2011-12-01

    Full Text Available El uso de microorganismos como fertilizante, ha demostrado tener efectos benéficos sobre el crecimiento de plantas y son una alternativa al uso de fertilizantes guímicos, sin embargo, cada microorganismo difiere en sus efectos benéficos. En este trabajo se evaluó el efecto de la aplicación de microorganismos fertilizantes, Azospirillum brasilense, Azotobacter chroccocum y Trichoderma lignorum sobre el crecimiento en maceta de plantas de caña de azúcar variedad CC 934418. El crecimiento de las plantas se midió en términos de diámetro del tallo, longitud de tallo y raíces, y número de hojas y raíces a los 15, 30 y 45 días de la siembra. El crecimiento de las plantas mostró diferencias estadísticamente significativas entre los tratamientos. Los microorganismos fertilizantes mostraron efecto positivo sobre el crecimiento de plantas de caña de azúcar, siendo Azospirillum brasilense y Trichoderma lignorum los microorganismos gue ejercieron mayor efecto sobre el diámetro del tallo y los sistemas radical y foliar de la planta. Se observaron los efectos beneficiosos de Trichoderma lignorum sobre el crecimiento de la hoja. Este es un nuevo aporte científico, ya que esta especie no ha sido reportada como promotora de crecimiento vegetal.0 uso de microrganismos como fertilizante, tem sido demonstrado gue têm efeitos benéficos no crescimento das plantas e são considerados uma alternativa ao uso de fertilizantes guímicos, no entanto, cada microrganismo possui diferentes efeitos benéficos. Neste estudo foi avaliado o efeito da aplicação de microorganismos fertilizantes, Azospirillum brasilense, Azotobacter chroccocum e Trichoderma lignorum no crescimento de cana-de-açucar da variedade CC 934418 plantadas em vasos. 0 crescimento das plantas foi medido em termos do diâmetro do caule, comprimento de caule e da raiz e número de folhas e raízes nos dias 15, 30 e 45 após a semeadura. 0 crescimento da planta mostrou diferen

  1. Sickle cell vaso-occlusion in an animal model

    International Nuclear Information System (INIS)

    Kurantsin-Mills, J.; Jacobs, H.M.; Lessin, L.S.

    1987-01-01

    Sickle cell disease is clinically characterized with vaso-occlusive painful crisis which is pleomorphic in terms of frequency of occurrence. The intracellular polymerization of deoxygenated hemoglobin S increases the internal viscosity of the sickle cells exponentially, concurrent with binding of hemoglobin S to the membrane and discocyte-drepanocyte transformation. As a result, the red cells in sickle cell disease are heterogenous with cells of varying density and mean corpuscular hemoglobin concentration which alter the rheological features of the blood in the microcirculation. The cellular, physiological, biochemical and rheological factors that contribute to the vaso-occlusive events are not completely understood. Nonetheless, recent clinical studies have demonstrated that a certain fraction of the dense cells disappear during sickle cell painful crisis. In an attempt to elucidate some of the cellular and rheological factors involved in the initiation of vaso-occlusion, the authors have employed intravital videomicroscopy and radionuclide imaging of indium-III labeled sickle cells to determine the dynamics and sites of vaso-occlusion using the rat exchanged-transfused with sickle (HbSS) erythrocytes as a model

  2. Fragile Pluralism

    Directory of Open Access Journals (Sweden)

    Hans-Georg Soeffner

    2016-01-01

    A shorter version of the essay was published in Hans-Georg Soeffner, and Thea D. Boldt, eds. Fragiler Pluralismus, Wiesbaden: VS Springer (2014, 207–24. The present translation for Entangled Religions – Interdisciplinary Journal for the Study of Religious Contact and Transfer is by Nicola Morris. The article describes the emergence of pluralism within the process of globalization and the impact of this development upon individuals communication and the definitions of the ‘self’ and the ‘Other’. The author illustrates the pitfalls of the human tendency to view the world from an ethnocentric perspective and with the corresponding attitude. He argues that in ‘open societies’, successful citizens will be capable of recognising and articulating distinctions between individuals, as well as between groups, beliefs, lifestyles and attitudes. These citizens must also be aware and capable of adapting for their purposes the full repertoire of language games and role games in their social world, in order to perceive and utilise comprehensive systems such as frameworks for cooperation. These skills will help them implement ‘maxims of communication’ and ‘existential hypotheses’.

  3. Níveis de lípides plasmáticos em ratos submetidos à esplenectomia total, ligadura simultânea dos vasos esplênicos e à esplenectomia subtotal com preservação do pólo inferior

    Directory of Open Access Journals (Sweden)

    Isabel Cristina Andreatta Lemos Paulo

    Full Text Available OBJETIVO: Verificar o efeito da esplenectomia total, da ligadura dos vasos esplênicos principais e da esplenectomia subtotal com preservação do pólo inferior, nos lípides plasmáticos de ratos alimentados com dieta-controle ou dieta acrescida com 2,5% de colesterol. MÉTODO: Foram utilizados 111 ratos Wistar, machos, pesando entre 273g e 427g, com idade aproximada de 12 semanas, assim distribuídos: Grupo 1, controle (N = 20, não operado; Grupo 2 (N = 20 submetido à manipulação do baço; Grupo 3 (N = 31 submetido à esplenectomia total; Grupo 4 (N = 20, ligadura simultânea da artéria e veia esplênicas; Grupo 5 (N = 20, esplenectomia subtotal com preservação do pólo inferior. Foram dosados os lípides plasmáticos, e os ratos foram distribuídos em dois subgrupos, de acordo com a dieta (Subgrupo A- dieta-controle; Subgrupo B - dieta acrescida com 2,5% de colesterol. Todos os animais foram submetidos à nova colheita de sangue após 90 dias do início do experimento. RESULTADOS: Os animais submetidos à esplenectomia total, independentemente do tipo de dieta, apresentaram aumento significante (p 0,05. Os animais submetidos à ligadura simultânea da artéria e veia esplênicas e à esplenectomia subtotal com preservação do pólo inferior e alimentados com dieta-controle, não apresentaram alterações nos níveis de lípides plasmáticos, exceto pelo aumento da HDL (p < 0,05 observado no grupo da esplenectomia subtotal. Nos animais desses grupos, alimentados com dieta acrescida com 2,5% de colesterol, houve aumento significante de colesterol total, LDL, e VLDL e triglicérides. CONCLUSÕES: A esplenectomia total produz aumento significante do colesterol total, das frações LDL e VLDL e dos triglicérides, tanto nos ratos alimentados com dieta-controle quanto nos alimentados com dieta acrescida com 2,5% de colesterol. O aumento é mais significante nos animais alimentados com dieta acrescida com 2,5% de colesterol. A ligadura

  4. National Fragile X Foundation

    Science.gov (United States)

    ... assist and educate individuals and families living with Fragile X in all stages of life, through outreach and ... Awareness – To increase public and professional awareness of Fragile X to influence public policy, support legislative advocacy and ...

  5. Vasculite cutânea de pequenos vasos: subtipos e tratamento - Parte II

    OpenAIRE

    Brandt, Hebert Roberto Clivati; Arnone, Marcelo; Valente, Neusa Yuriko Sakai; Criado, Paulo Ricardo; Sotto, Mirian Nacagami

    2007-01-01

    Vasculite cutânea de pequenos vasos refere-se a grupo de doenças geralmente caracterizado por púrpura palpável, causada por vasculite leucocitoclástica das vênulas pós-capilares. Vasculites podem variar em gravidade, podendo manifestar-se como doença autolimitada, com acometimento de único órgão, ou como doença sistêmica, acometendo múltiplos órgãos, e evoluir para quadro de falência de múltiplos órgãos e sistemas. Esse grupo de doenças apresenta-se como desafio para o dermatologista, incluin...

  6. Níveis de lípides plasmáticos em ratos submetidos à esplenectomia total, ligadura simultânea dos vasos esplênicos e à esplenectomia subtotal com preservação do pólo inferior

    OpenAIRE

    Paulo,Isabel Cristina Andreatta Lemos; Paulo,Danilo Nagib Salomão; Silva,Alcino Lázaro da; Foletto,Rodrigo Moulin; Colnago,Geraldo Luiz; Vargas,Paulo Merçon

    2005-01-01

    OBJETIVO: Verificar o efeito da esplenectomia total, da ligadura dos vasos esplênicos principais e da esplenectomia subtotal com preservação do pólo inferior, nos lípides plasmáticos de ratos alimentados com dieta-controle ou dieta acrescida com 2,5% de colesterol. MÉTODO: Foram utilizados 111 ratos Wistar, machos, pesando entre 273g e 427g, com idade aproximada de 12 semanas, assim distribuídos: Grupo 1, controle (N = 20), não operado; Grupo 2 (N = 20) submetido à manipulação do baço; Grupo ...

  7. Fragile X Syndrome: Other FAQs

    Science.gov (United States)

    ... Other FAQs Share Facebook Twitter Pinterest Email Print Fragile X Syndrome: Other FAQs Basic information for topics, such ... Are there specific disorders or conditions associated with Fragile X syndrome? Among the other conditions associated with Fragile ...

  8. Fragile X Syndrome

    Science.gov (United States)

    Schwarte, Andrea R.

    2008-01-01

    This article provides an overview of current research on Fragile X Syndrome, and how that knowledge can be used to guide successful intervention. The genetic etiology of Fragile X is reviewed and the physical, cognitive, adaptive, behavioral, and emotional phenotypes of children with the disorder are described, highlighting the differences in…

  9. Equipment fragility testing

    International Nuclear Information System (INIS)

    Holman, G.S.; Chou, C.K.; Cummings, G.E.

    1985-01-01

    Current probabilistic risk assessment (PRA) methods for nuclear power plants utilize component fragilities which are for the most part based on a limited data base and engineering judgement. The seismic design of components is based on code limits and NRC requirements that do not reflect the actual capacity of a component to resist failure. In order to improve the present component fragility data base and establish component seismic design margins, the NRC has commissioned a projected three-year program to compile existing fragilities data and at the same time independently perform fragilities tests on selected mechanical and electrical components. This paper presents the planning and technical approach being taken by LLNL in the NRC Component Fragility Program

  10. Trio Fragile / Olga Kaljundi

    Index Scriptorium Estoniae

    Kaljundi, Olga, 1941-2001

    1998-01-01

    Tallinna Vene Draamateatri galeriis esinenud trupi "Trio Fragile" vernissaazhist. Trio loomingust ja osalejatest : kahe muusiku seltskonnas esineb ka 1984.a. Kunstiülikooli lõpetanud kunstnik Tõnu Talve.

  11. Transposición corregida de grandes vasos

    Directory of Open Access Journals (Sweden)

    José Rafael Escalona Aguilera

    2012-09-01

    Full Text Available Se presenta una paciente de sexo femenino, de 9 años de edad, que acude a su médico de familia y se le auscultó un soplo al examen físico y sintomatología que se comportaba como una comunicación interventricular. Luego de los estudios correspondientes, se permitió hacer el diagnóstico de transposición corregida de grandes vasos. La paciente tenía asociada una anomalía de Ebstein, comunicación interauricular, una comunicación interventricular, y una persistencia del conducto arterioso, todo lo cual agravaba su cuadro clínico. Primero es intervenida quirúrgicamente, y se cierra el ducto; de manera espontánea, cierra la comunicación interauricular, y la anomalía de Ebstein parchea la comunicación interventricular. La niña desarrolla una insuficiencia cardiaca e hipertensión pulmonar, a consecuencia de las anomalías asociadas. El caso es de interés, ya que son pocos los publicados en la literatura internacional con esta cardiopatía congénita asociada a otras anomalías estructurales del corazón, que modifican su historia natural.

  12. Equipment fragility data base

    International Nuclear Information System (INIS)

    Cover, L.E.

    1982-03-01

    Part of the effort of the Seismic Safety Margins Research Program (SSMRP) has been directed at generating a fragility data base for equipment used in control and safety systems in commercial nuclear power plants. Component fragility data exist in various forms, depending on their content, intended use, and level of reduction. Th data are stored in a relational data base on the LLNL CDC 7600 computers; this provides easy accessibility for LLNL computer users. This report describes the present structure of the data base and presents its contents through the use of tables

  13. Incarceration in Fragile Families

    Science.gov (United States)

    Wildeman, Christopher; Western, Bruce

    2010-01-01

    Since the mid-1970s the U.S. imprisonment rate has increased roughly fivefold. As Christopher Wildeman and Bruce Western explain, the effects of this sea change in the imprisonment rate--commonly called mass imprisonment or the prison boom--have been concentrated among those most likely to form fragile families: poor and minority men with little…

  14. Component fragility research program

    International Nuclear Information System (INIS)

    Tsai, N.C.; Mochizuki, G.L.; Holman, G.S.

    1989-11-01

    To demonstrate how ''high-level'' qualification test data can be used to estimate the ultimate seismic capacity of nuclear power plant equipment, we assessed in detail various electrical components tested by the Pacific Gas ampersand Electric Company for its Diablo Canyon plant. As part of our Phase I Component Fragility Research Program, we evaluated seismic fragility for five Diablo Canyon components: medium-voltage (4kV) switchgear; safeguard relay board; emergency light battery pack; potential transformer; and station battery and racks. This report discusses our Phase II fragility evaluation of a single Westinghouse Type W motor control center column, a fan cooler motor controller, and three local starters at the Diablo Canyon nuclear power plant. These components were seismically qualified by means of biaxial random motion tests on a shaker table, and the test response spectra formed the basis for the estimate of the seismic capacity of the components. The seismic capacity of each component is referenced to the zero period acceleration (ZPA) and, in our Phase II study only, to the average spectral acceleration (ASA) of the motion at its base. For the motor control center, the seismic capacity was compared to the capacity of a Westinghouse Five-Star MCC subjected to actual fragility tests by LLNL during the Phase I Component Fragility Research Program, and to generic capacities developed by the Brookhaven National Laboratory for motor control center. Except for the medium-voltage switchgear, all of the components considered in both our Phase I and Phase II evaluations were qualified in their standard commercial configurations or with only relatively minor modifications such as top bracing of cabinets. 8 refs., 67 figs., 7 tabs

  15. Topografia dos vasos sanguíneos arteriais e venosos nos rins de Cavalos Puro Sangue Inglês

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio de Moraes Barros

    2015-04-01

    Full Text Available As doenças renais em equinos, geralmente estão associadas a problemas respiratórios e digestórios, entretanto não são frequentes as intervenções cirúrgicas neste órgão para estes animais. O estudo dos vasos sanguíneos relativos aos rins nos animais domésticos e selvagens é de fundamental importância para o entendimento de diversos aspectos de sua fisiologia e nutrição. Neste trabalho, foram utilizados 30 pares de rins de equinos Puro Sangue Inglês, dos quais 15 machos e 15 fêmeas, de diferentes faixas etárias, provenientes de doação do Jockey Club de São Paulo. Durante a necropsia dos animais, os rins unidos por meio de suas artérias e veias renais foram retirados em bloco. Em seguida, foram fixados em solução de formol a 10%, por aproximadamente 48h, para a dissecação e individualização dos componentes dos pedículos renais. Para a análise dos resultados foram demarcados os quadrantes cranioventral, craniodorsal, caudoventral e caudodorsal, com duas linhas uma craniocaudal e a outra dorsoventral. Os resultados obtidos revelaram que: os ramos arteriais da região hilar são observados no rim direito em número de dois em 50%, no esquerdo em número de dois (33,3%, os ramos arteriais na região justahilar são computados no rim direito em número de quatro a cinco em 20% e no rim esquerdo em número de seis (16,7%, e nos ramos dispostos na região extrahilar são registrados no rim direito em número de três (26,7% e no esquerdo em número de três (20%.

  16. A fragilidade/resistência da vida humana em comunidades rurais do Pantanal Mato-grossense, MT, Brasil The fragility/strength of human life in rural communities of the Pantanal wetlands, Mato Grosso State, Brazil

    Directory of Open Access Journals (Sweden)

    Marta Gislene Pignatti

    2008-02-01

    Full Text Available A fragilidade/resistência da vida humana tomada no sentido ambíguo/complexo sugere o olhar para a dinâmica da vida de grupos populacionais específicos. O estudo de uma comunidade no entorno da Reserva Particular do Patrimônio Natural do Serviço Social do Comércio - RPPN SESC Pantanal, Distrito Rural de Joselândia, município de Barão de Melgaço (MT, no período de 2003-2005, buscou aprender o modo de vida dessa população, articulando a explicação do processo saúde-doença com os aspectos históricos, econômicos e sociais da comunidade em questão e a singularidade das práticas de cura ocorridas no local. Utilizou-se de registros documentais históricos, depoimentos de moradores, inquérito populacional, entrevistas e observações. Verificou-se que houve mudanças nos laços de solidariedade entre os moradores no trabalho e na produção de alimentos. As práticas de cura populares são alternativas utilizadas para abrandar os sintomas e doenças. A fragilidade/resistência deste grupo específico se configura enquanto resistência baseada nas relações de solidariedade e familiares e a fragilidade na dificuldade do acesso ao trabalho, serviços de saúde e equipamentos públicos.From an ambiguous/complex standpoint, the fragility/strength of human life suggests an approach based on observations of the life-style dynamics of specific population groups. Conducted between 2003 and 2005, this study of a community living alongside the Pantanal Wetlands Private Natural Heritage Reserve established by the Social Enterprise Support Centre (RPPN/ SESC in the Joselândia Rural District, Barão de Melgaço Municipality, Mato Grosso State, Brazil, explores its life-style, linking explanations of the health/disease process to historical, economic and social aspects of this community and the singularity of local healing practices, using historical documentary records, statements from local residents, a population poll, interviews and

  17. A fragilidade/resistência da vida humana em comunidades rurais do Pantanal Mato-Grossense (MT, Brasil The human life fragility/resistance in rurals communities of Pantanal (MT, Brazil

    Directory of Open Access Journals (Sweden)

    Marta Gislene Pignatti

    2010-10-01

    Full Text Available A fragilidade/resistência da vida humana tomada no sentido ambíguo/complexo sugere o olhar para a dinâmica da vida de grupos populacionais específicos. O estudo de uma comunidade no entorno da Reserva Particular do Patrimônio Natural do Serviço Social do Comércio (RPPN Sesc Pantanal, Distrito Rural de Joselândia, município de Barão de Melgaço (MT, no período de 2003 a 2005, buscou apreender o modo de vida dessa população, articulando a explicação do processo saúde doença com os aspectos históricos, econômicos e sociais da comunidade em questão e a singularidade das práticas de cura ocorridas no local. Utilizou-se de registros documentais históricos, depoimentos de moradores, inquérito populacional, entrevistas e observações. Verificou-se que houve mudanças nos laços de solidariedade entre os moradores no trabalho e na produção de alimentos. As práticas de cura populares são alternativas utilizadas para abrandar os sintomas e doenças. A fragilidade/resistência desse grupo específico se configura como resistência baseada nas relações de solidariedade e familiares e a fragilidade na dificuldade do acesso ao trabalho, serviços de saúde e equipamentos públicos.The human life fragility/resistance taken in the ambiguous/complexity sense suggests an eye to the dynamics of life of specific groups. The study of the community around the Private Reserve of Natural Patrimony of the Social Service Commerce (RPPN Sesc Pantanal, situated in Joselândia rural district, municipality of Barão de Melgaço - Mato Grosso state - Brasil, 2003 at 2005, used qualitative methods to apprehend the lifestyle of this population, articulating the explanation of the health-ill process with historical, economic and social aspects of the respective community and the singularity of the cure practices that happened on site. It was used historical records, testimonials of residents, population survey, interviews and observations. The

  18. Fragile granular jamming

    Energy Technology Data Exchange (ETDEWEB)

    Rivera, Michael K [Los Alamos National Laboratory; Ecke, Robert E [Los Alamos National Laboratory; Bandi, Mahesh M [HARVARD; Kzakala, Florent [CNRS & ESPCI PARIS TECH

    2009-01-01

    We demonstrate experimentally that the route to a jammed state for a set of bi-dispersed frictional disks, subjected to uni-axial compression from a random initial unjammed state, consists of a consolidation state, a fragile jammed state, and finally a rigid jammed state. In the consolidation regime, the pressure on the sides increases very slowly with the packing fraction {phi} and there are no detectable stress chains. In the fragile jammed state, stress chains are visible, the pressure increases exponentially with {phi}, and the fraction of moving disks drops exponentially. Eventually, a final regime where particle displacements are below our resolution and the pressure varies approximately linearly with {phi} is reached. We argue that this scenario is generic for ather mal frictional compressed particles.

  19. Fragile X syndrome.

    Science.gov (United States)

    Saldarriaga, Wilmar; Tassone, Flora; González-Teshima, Laura Yuriko; Forero-Forero, Jose Vicente; Ayala-Zapata, Sebastián; Hagerman, Randi

    2014-01-01

    Fragile X Syndrome (FXS) is a genetic disease due to a CGG trinucleotide expansion, named full mutation (greater than 200 CGG repeats), in the fragile X mental retardation 1 gene locus Xq27.3; which leads to an hypermethylated region in the gene promoter therefore silencing it and lowering the expression levels of the fragile X mental retardation 1, a protein involved in synaptic plasticity and maturation. Individuals with FXS present with intellectual disability, autism, hyperactivity, long face, large or prominent ears and macroorchidism at puberty and thereafter. Most of the young children with FXS will present with language delay, sensory hyper arousal and anxiety. Girls are less affected than boys, only 25% have intellectual disability. Given the genomic features of the syndrome, there are patients with a number of triplet repeats between 55 and 200, known as premutation carriers. Most carriers have a normal IQ but some have developmental problems. The diagnosis of FXS has evolved from karyotype with special culture medium, to molecular techniques that are more sensitive and specific including PCR and Southern Blot. During the last decade, the advances in the knowledge of FXS, has led to the development of investigations on pharmaceutical management or targeted treatments for FXS. Minocycline and sertraline have shown efficacy in children.

  20. Seismic fragility analyses

    International Nuclear Information System (INIS)

    Kostov, Marin

    2000-01-01

    In the last two decades there is increasing number of probabilistic seismic risk assessments performed. The basic ideas of the procedure for performing a Probabilistic Safety Analysis (PSA) of critical structures (NUREG/CR-2300, 1983) could be used also for normal industrial and residential buildings, dams or other structures. The general formulation of the risk assessment procedure applied in this investigation is presented in Franzini, et al., 1984. The probability of failure of a structure for an expected lifetime (for example 50 years) can be obtained from the annual frequency of failure, β E determined by the relation: β E ∫[d[β(x)]/dx]P(flx)dx. β(x) is the annual frequency of exceedance of load level x (for example, the variable x may be peak ground acceleration), P(fI x) is the conditional probability of structure failure at a given seismic load level x. The problem leads to the assessment of the seismic hazard β(x) and the fragility P(fl x). The seismic hazard curves are obtained by the probabilistic seismic hazard analysis. The fragility curves are obtained after the response of the structure is defined as probabilistic and its capacity and the associated uncertainties are assessed. Finally the fragility curves are combined with the seismic loading to estimate the frequency of failure for each critical scenario. The frequency of failure due to seismic event is presented by the scenario with the highest frequency. The tools usually applied for probabilistic safety analyses of critical structures could relatively easily be adopted to ordinary structures. The key problems are the seismic hazard definitions and the fragility analyses. The fragility could be derived either based on scaling procedures or on the base of generation. Both approaches have been presented in the paper. After the seismic risk (in terms of failure probability) is assessed there are several approaches for risk reduction. Generally the methods could be classified in two groups. The

  1. Contagem numérica em estudantes com síndromes de X-Frágil e Prader-Willi Numerical counting in students with fragile X and Prader-Willi Syndromes

    Directory of Open Access Journals (Sweden)

    Telma Regina França Rosso

    2012-06-01

    Full Text Available as dificuldades de aprendizagem matemática constituem um campo bastante complexo e ainda relativamente pouco explorado. Pesquisas com síndromes associadas ao baixo desempenho matemático indicam que há diferentes perfis cognitivos subjacentes às dificuldades na matemática e nos mostram que nem toda dificuldade de aprendizagem matemática é originada da mesma maneira. Este estudo teve como objetivo investigar as características dos princípios da contagem numérica em dois estudantes: um com síndrome do X-Frágil (SXF e outro com síndrome de Prader-Willi (SPW. Foram feitas observações em sala de aula e no espaço escolar e investigaram-se os princípios numéricos em cinco situações matemáticas diferentes com intenção de observar os seguintes princípios numéricos: correspondência um a um, ordem constante, cardinalidade, abstração e irrelevância da ordem. Concluiu-se que o princípio da correspondência um a um (termo a termo mostra-se em desenvolvimento mais lento do que nas crianças da mesma faixa etária, enquanto que os princípios da abstração e irrelevância da ordem são os menos desenvolvidos nas duas crianças estudadas. Crianças com estas síndromes estão em salas de aulas regulares e o conhecimento sobre suas habilidades cognitivas e sua apropriação de conceitos e conteúdos matemáticos se torna necessário para garantir uma inclusão que favoreça o sucesso matemático.Difficulties in learning mathematics are a complex issue that is still relatively unexplored. Research into syndromes associated with low mathematical performance indicate that different cognitive profiles underlie learning difficulties in math and show that not all such difficulties have the same origins. This study aimed to investigate characteristics of the principles of numerical counting in two students: one with Fragile X syndrome (FXS and another with Prader-Willi syndrome (PWS. Observations were made in the classroom and on the

  2. VASO FERMENTADOR DE BAJO COSTO PARA LA MICROPROPAGACIÓN MASIVA DE JENGIBRE

    OpenAIRE

    Alejandro Hern\\u00E1ndez-Soto; Andr\\u00E9s Gatica-Arias; Silvana Alvarenga-Venutolo

    2008-01-01

    La presente investigación se realizó en el Centro de Investigación en Biotecnología del Instituto Tecnológico de Costa Rica, durante el primer semestre del 2003 y tuvo como objetivo desarrollar un vaso fermentador de bajo costo para la micropropagación masiva de jengibre. El vaso fermentador propuesto en este trabajo tuvo un costo aproximado de $4 USD, con capacidad de utilizar de 50 a 75 brotes como material de partida en 350 ml de medio de cultivo líquido. Cada brote inicial generó alrededo...

  3. Genetics Home Reference: fragile X syndrome

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Fragile X syndrome Fragile X syndrome Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Fragile X syndrome is a genetic condition that causes a ...

  4. Management of Medically Fragile Infants.

    Science.gov (United States)

    Ueda, Dawn; Caulfield, Rick

    2001-01-01

    Discusses the role of the Child Life Specialist in helping to meet the special needs of medically fragile children. Argues that since many child care professionals may come into contact with medically fragile children at some point in their careers, it is important to examine child life as a specialization in the health care profession. (SD)

  5. Manipulation of a fragile object.

    Science.gov (United States)

    Gorniak, Stacey L; Zatsiorsky, Vladimir M; Latash, Mark L

    2010-04-01

    We investigated strategies of adjustments in kinetic and kinematic patterns, and in multi-digit synergies during quick vertical transport of an instrumented handle that collapsed when the grasping force exceeded a certain magnitude (quantified with a fragility index). The collapse threshold of the object was set using a novel electromagnetic device. Moving a fragile object is viewed as a task with two constraints on the grip force defined by the slipping and crushing thresholds. When moving more fragile objects, subjects decreased object peak acceleration, increased movement time, showed a drop in the safety margin (SM) (extra force over the slipping threshold), and showed a tendency toward violating the minimum-jerk criterion. Linear regression analysis of grip force against load force has shown tight coupling between the two with a decline in the coefficient of determination with increased fragility index. The SM was lower in bimanual tasks, compared to unimanual tasks, for both fragile and non-fragile objects. Two novel indices have been introduced and studied, the SM due to fragility and the drop-crush index. Both indices showed a decrease with increased object fragility. Changes in the drop-crush index showed that the subjects would rather crush the fragile objects as opposed to dropping them, possibly reflecting the particular experimental procedure. We did not find differences between the performance indices of the dominant and non-dominant hand thus failing to support the recently formulated dominance hypothesis. The synergies stabilizing grip force were quantified at two levels of an assumed two-level control hierarchy using co-variation indices between elemental variables across trials. There were strong synergies at the upper level of the hierarchy (the task is shared between the opposing groups of digits) that weakened with an increase in object fragility. At the lower level (action of an effector is shared among the four fingers), higher fragility led

  6. Fragile Thermodynamic Order

    International Nuclear Information System (INIS)

    Bernhoeft, N.; Lander, G.H.; Colineau, E.

    2003-01-01

    An asymmetric shift in the position of the magnetic Bragg peak with respect to the fiducial lattice has been observed by resonant X-ray scattering in a diverse series of antiferromagnetic compounds. This apparent violation of Bragg's law is interpreted in terms of a dynamically phased order parameter. We demonstrate the use of this effect as a novel probe of fragile or dynamic thermodynamic order in strongly correlated electronic systems. In particular, fresh light is shed on the paradoxical situation encountered in URu 2 Si 2 where the measured entropy gain on passing through T Neel is incompatible with the ground state moment estimated by neutron diffraction. The intrinsic space-time averaging of the probe used to characterise the thermodynamic macroscopic state may play a crucial and previously neglected role. In turn, this suggests the further use of resonant X-ray scattering in investigations of systems dominated by quantum fluctuations. (author)

  7. Flooding Fragility Experiments and Prediction

    Energy Technology Data Exchange (ETDEWEB)

    Smith, Curtis L. [Idaho National Lab. (INL), Idaho Falls, ID (United States); Tahhan, Antonio [Idaho National Lab. (INL), Idaho Falls, ID (United States); Muchmore, Cody [Idaho National Lab. (INL), Idaho Falls, ID (United States); Nichols, Larinda [Idaho National Lab. (INL), Idaho Falls, ID (United States); Bhandari, Bishwo [Idaho National Lab. (INL), Idaho Falls, ID (United States); Pope, Chad [Idaho National Lab. (INL), Idaho Falls, ID (United States)

    2016-09-01

    This report describes the work that has been performed on flooding fragility, both the experimental tests being carried out and the probabilistic fragility predictive models being produced in order to use the text results. Flooding experiments involving full-scale doors have commenced in the Portal Evaluation Tank. The goal of these experiments is to develop a full-scale component flooding experiment protocol and to acquire data that can be used to create Bayesian regression models representing the fragility of these components. This work is in support of the Risk-Informed Safety Margin Characterization (RISMC) Pathway external hazards evaluation research and development.

  8. Flooding Fragility Experiments and Prediction

    International Nuclear Information System (INIS)

    Smith, Curtis L.; Tahhan, Antonio; Muchmore, Cody; Nichols, Larinda; Bhandari, Bishwo; Pope, Chad

    2016-01-01

    This report describes the work that has been performed on flooding fragility, both the experimental tests being carried out and the probabilistic fragility predictive models being produced in order to use the text results. Flooding experiments involving full-scale doors have commenced in the Portal Evaluation Tank. The goal of these experiments is to develop a full-scale component flooding experiment protocol and to acquire data that can be used to create Bayesian regression models representing the fragility of these components. This work is in support of the Risk-Informed Safety Margin Characterization (RISMC) Pathway external hazards evaluation research and development.

  9. Learning about Fragile X Syndrome

    Science.gov (United States)

    Skip to main content Learning About Fragile X Syndrome Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research News Features Funding Divisions ...

  10. VASO FERMENTADOR DE BAJO COSTO PARA LA MICROPROPAGACIÓN MASIVA DE JENGIBRE

    Directory of Open Access Journals (Sweden)

    Alejandro Hern\\u00E1ndez-Soto

    2008-01-01

    Full Text Available La presente investigación se realizó en el Centro de Investigación en Biotecnología del Instituto Tecnológico de Costa Rica, durante el primer semestre del 2003 y tuvo como objetivo desarrollar un vaso fermentador de bajo costo para la micropropagación masiva de jengibre. El vaso fermentador propuesto en este trabajo tuvo un costo aproximado de $4 USD, con capacidad de utilizar de 50 a 75 brotes como material de partida en 350 ml de medio de cultivo líquido. Cada brote inicial generó alrededor de 2,9±1,4 brotes, con una altura de 3,1±0,1cm, un peso fresco de 1,2±0,5 g y un peso seco de 0,24±0,27 por brote.

  11. SINDROME DE INMUNODEFICIENCIA ADQUIRIDA. COMPROMISO DEL CORAZÓN Y DE LOS VASOS

    Directory of Open Access Journals (Sweden)

    Dr. Jorge Yovanovich S.

    2015-03-01

    Full Text Available Las complicaciones cardiovasculares del SIDA, son a menudo asintomáticas, aunque en ocasiones pueden constituir la causa directa de muerte. Puede comprometerse el pericardio, miocardio, endocardio y los vasos, ya sea como manifestación de la enfermedad de base o como resultado de la terapia antirretroviral y su efecto sobre los factores de riesgo, en el contexto de una patología que es actualmente de evolución crónica y con mejores expectativas de sobrevida gracias a las nuevas drogas empleadas para su control y tratamiento. El objetivo de este artículo es presentar una revisión de los aspectos más relevantes del SIDA que comprometen al corazón y los vasos.

  12. International Companies in Fragile States

    DEFF Research Database (Denmark)

    Patey, Luke; Kragelund, Peter

    Denmark must not fail to promote corporate social responsibility in fragile states. International companies remain active in these environments, and often worsen rather than alleviate poor governance. Financial transparency and human rights initiatives offer the first step in ensuring that this d......Denmark must not fail to promote corporate social responsibility in fragile states. International companies remain active in these environments, and often worsen rather than alleviate poor governance. Financial transparency and human rights initiatives offer the first step in ensuring...

  13. Internal dosimetry for blood vessels radiotherapy; Dosimetria interna para terapia com radiacao em vasos sanguineos

    Energy Technology Data Exchange (ETDEWEB)

    Campos, Laelia Pumilla Botelho [Pernambuco Univ., Recife, PE (Brazil). Dept. de Energia Nuclear] E-mail: lpbcampos@uol.com.br; Stabin, Michael Gregory [Vanderbilt Univ., Nashville, TN (United States). Dept. of Radiology and Radiological Sciences] E-mail: Michael.Stabin@mcmail.vanderbilt.edu

    2001-07-01

    Among the cardiovascular diseases, the most common is acute myocardial infarction, which occurs because of the occlusion of one or more coronary arteries. Balloon angioplasty has been a popular treatment which is less invasive than surgeries involving revascularization of the myocardium, thus promising a better quality of life for patients. Unfortunately, the rate of restenosis (re-closing of the vessel) after balloon angioplasty is high (approximately 30-50% within the first year after treatment). Known as Intravascular Brachytherapy, the technique has been used with several radiation sources, and researchers have obtained success in decreasing the rate of restenosis. In order to study the radiation dosimetry in the patient and radiological protection for this therapy, radiation dose distributions for monoenergetic electrons and photons (at nine discrete energies) were calculated for blood vessels of diameter 0.15, 0.30 and 0.45 cm with balloon and wire sources using the radiation transport code MCNP4B. Specific calculations were carried out for several radionuclides. Advantages and disadvantages of the radionuclides and source geometries are discussed and the dosimetry developed here will aid in the realization of the benefits obtained in patients. (author)

  14. Crescimento e qualidade de crisântemo cultivado em vaso sob ambiente protegido

    OpenAIRE

    Farias,Maryzélia F. de; Saad,João Carlos C.

    2005-01-01

    O crisântemo (Dendranthema grandiflora) é uma planta ornamental com grande demanda pelo mercado consumidor. Apesar de haver poucas informações sobre a sua exigência hídrica, sabe-se que irrigações deficitárias ou excessivas prejudicam seu desenvolvimento, reduzindo a produtividade e a qualidade. Considerando-se a importância da qualidade para a venda dos produtos florícolas o objetivo desse trabalho foi identificar a tensão de água no substrato (potencial matricial) com melhor crescimento e d...

  15. Three Faces of Fragile X.

    Science.gov (United States)

    Lieb-Lundell, Cornelia C E

    2016-11-01

    Fragile X syndrome (FXS) is the first of 3 syndromes identified as a health condition related to fragile X mental retardation (FMR1) gene dysfunction. The other 2 syndromes are fragile X-associated primary ovarian insufficiency syndrome (FXPOI) and fragile X-associated tremor/ataxia syndrome (FXTAS), which together are referred to as fragile X-associated disorders (FXDs). Collectively, this group comprises the 3 faces of fragile X. Even though the 3 conditions share a common genetic defect, each one is a separate health condition that results in a variety of body function impairments such as motor delay, musculoskeletal issues related to low muscle tone, coordination limitations, ataxia, tremor, undefined muscle aches and pains, and, for FXTAS, a late-onset neurodegeneration. Although each FXD condition may benefit from physical therapy intervention, available evidence as to the efficacy of intervention appropriate to FXDs is lacking. This perspective article will discuss the genetic basis of FMR1 gene dysfunction and describe health conditions related to this mutation, which have a range of expressions within a family. Physical therapy concerns and possible assessment and intervention strategies will be introduced. Understanding the intergenerational effect of the FMR1 mutation with potential life-span expression is a key component to identifying and treating the health conditions related to this specific genetic condition. © 2016 American Physical Therapy Association.

  16. Sarcopenia and fragility fractures.

    Science.gov (United States)

    Cederholm, T; Cruz-Jentoft, A J; Maggi, S

    2013-02-01

    Sarcopenia, a reduction in muscle mass and muscle function, is considered one of the hallmarks of the aging process. Current views consider sarcopenia as the consequence of multiple medical, behavioural and environmental factors that characterize aged individuals. Likewise bone fragility is known to depend on several pathogenetic mechanisms leading to bone mass loss and reduction of bone strength. Muscle weakness, fear of falls, falls and subsequent fractures are associated to concurrent sarcopenia and osteoporosis and lead to restricted mobility, loss of autonomy and reduced life expectancy. The skeletal and the muscular organ systems are tightly intertwined: the strongest mechanical forces applied to bones are, indeed, those created by muscle contractions that condition bone density, strength, and microarchitecture. Not surprising, therefore, the decrease in muscle strength leads to lower bone strength. The degenerative processes leading to osteoporosis and sarcopenia show many common pathogenic pathways, like the sensitivity to reduced anabolic hormone secretion, increased inflammatory cytokine activity and reduced physical activity. Thus they may also respond to the same kind of treatments. Basic is life-style interventions related to exercise and nutrition. Sufficient vitamin D levels are of importance for both bone and muscle, primarily provided by sun exposure at younger age, and by supplementation at older age. Resistance training several times per week is crucial, and to be effective adequate access to energy and proteins is necessary.

  17. Dynamics of von Willebrand factor reactivity in sickle cell disease during vaso-occlusive crisis and steady state

    NARCIS (Netherlands)

    Sins, J. W. R.; Schimmel, M.; Luken, B. M.; Nur, E.; Zeerleder, S. S.; van Tuijn, C. F. J.; Brandjes, D. P. M.; Kopatz, W. F.; Urbanus, R. T.; Meijers, J. C. M.; Biemond, B. J.; Fijnvandraat, K.

    2017-01-01

    Background: Endothelial activation plays a central role in the pathophysiology of vaso-occlusion in sickle cell disease (SCD), facilitating adhesive interactions with circulating blood cells. Upon activation, various adhesive molecules are expressed, including von Willebrand factor (VWF). Increased

  18. Vasculite cutânea de pequenos vasos: subtipos e tratamento - Parte II Small vessel cutaneous vasculitis: subtypes and treatment - Part II

    OpenAIRE

    Hebert Roberto Clivati Brandt; Marcelo Arnone; Neusa Yuriko Sakai Valente; Paulo Ricardo Criado; Mirian Nacagami Sotto

    2007-01-01

    Vasculite cutânea de pequenos vasos refere-se a grupo de doenças geralmente caracterizado por púrpura palpável, causada por vasculite leucocitoclástica das vênulas pós-capilares. Vasculites podem variar em gravidade, podendo manifestar-se como doença autolimitada, com acometimento de único órgão, ou como doença sistêmica, acometendo múltiplos órgãos, e evoluir para quadro de falência de múltiplos órgãos e sistemas. Esse grupo de doenças apresenta-se como desafio para o dermatologista, incluin...

  19. The genetics of skin fragility.

    Science.gov (United States)

    Has, Cristina; Bruckner-Tuderman, Leena

    2014-01-01

    Genetic skin fragility manifests with diminished resistance of the skin and mucous membranes to external mechanical forces and with skin blistering, erosions, and painful wounds as clinical features. Skin fragility disorders, collectively called epidermolysis bullosa, are caused by mutations in 18 distinct genes that encode proteins involved in epidermal integrity and dermal-epidermal adhesion. The genetic spectrum, along with environmental and genetic modifiers, creates a large number of clinical phenotypes, spanning from minor localized lesions to severe generalized blistering, secondary skin cancer, or early demise resulting from extensive loss of the epidermis. Laboratory investigations of skin fragility have greatly augmented our understanding of genotype-phenotype correlations in epidermolysis bullosa and have also advanced skin biology in general. Current translational research concentrates on the development of biologically valid treatments with therapeutic genes, cells, proteins, or small-molecule compounds in preclinical settings or human pilot trials.

  20. Resilience and the Fragile City

    Directory of Open Access Journals (Sweden)

    John de Boer

    2015-04-01

    Full Text Available Humanitarian, security, and development actors are witnessing two distinct but intertwined trends that will have a dramatic impact on their operations. The first relates to the fact that the locus of global poverty and vulnerability to disaster are increasingly concentrated in fragile and conflict affected states. The second trend is associated with the notion that the world has entered a period of unprecedented urbanization. For the first time in history, more people live inside urban centres than outside of them. As the world continues to urbanize, global emergencies will increasingly be concentrated in cities, particularly in lower income and fragile countries where the pace of urbanization is fastest. Yet, despite the growing risks facing urban populations living in fragile and conflict affected countries, there is very little understanding of what can be done to reduce the risks posed to these cities and their populations.

  1. Seismic fragility capacity of equipment

    International Nuclear Information System (INIS)

    Iijima, Toru; Abe, Hiroshi; Suzuki, Kenichi

    2006-01-01

    Seismic probabilistic safety assessment (PSA) is an available method to evaluate residual risks of nuclear plants that are designed on definitive seismic conditions. From our preliminary seismic PSA analysis, horizontal shaft pumps are important components that have significant influences on the core damage frequency (CDF). An actual horizontal shaft pump and some kinds of elements were tested to evaluate realistic fragility capacities. Our test results showed that the realistic fragility capacity of horizontal shaft pump would be at least four times as high as a current value, 1.6 x 9.8 m/s 2 , used for our seismic PSA. We are going to incorporate the fragility capacity data that were obtained from those tests into our seismic PSA analysis, and we expect that the reliability of seismic PSA should increase. (author)

  2. Estresse e autoconceito em pais e mães de crianças com a síndrome do X-frágil Stress and self-concept in parents of children with fragile X-syndrome

    Directory of Open Access Journals (Sweden)

    Zuleika Ana Cherubini

    2008-01-01

    Full Text Available A síndrome do x-frágil é a principal causa de deficiência mental herdada, suplantada apenas pela síndrome de Down, sendo a mulher a principal transmissora. Indivíduos com a síndrome do X-frágil apresentam um conjunto de características que pode causar um impacto no sistema familiar. Este estudo investiga o estresse e o autoconceito em 15 pais e mães de meninos com a síndrome do X-frágil, 15 de meninos com Síndrome de Down e 15 de meninos com desenvolvimento típico, baseado no modelo biopsicossocial de Bradford. Os resultados revelaram uma diferença significativa no autoconceito (self pessoal entre as mães de crianças com a Síndrome do X-frágil e as mães de crianças com desenvolvimento típico, mas não entre aquelas e o grupo S. de Down, sugerindo que o fator da transmissão por si só não explica as diferenças no autoconceito. Não houve diferença entre os grupos na medida de estresse.The Fragile X Syndrome is the major inherited intellectual disability, superseded only by Down Syndrome, and it is transmitted mostly by females. Patients with Fragile X Syndrome show a set of physical, clinical, behavioral and cognitive features that may impact the family structures, especially the parental figures. This study investigates the stress and self-concept of 15 parents of boys with Fragile X Syndrome, 15 parents of boys with Down syndrome and 15 with typical development accordingly to the biopsychosocial model of Bradford. The results showed a significant difference of self-concept between mothers of children with Fragile X Syndrome and typical developmental groups, but not in those with Down Syndrome, suggesting that the transmission factor solely does not explain differences in self-concept.

  3. Autism Spectrum Disorder and Fragile X Syndrome

    Science.gov (United States)

    ... only after another family member has been diagnosed. Autism Spectrum Disorder and Fragile X Syndrome Fragile X syndrome is ... known single gene cause of ASD What Is Autism Spectrum Disorder? Autism spectrum disorder (ASD) is a behavioral diagnosis. ...

  4. Learning to deliver education in fragile states

    Directory of Open Access Journals (Sweden)

    Martin Greeley

    2006-07-01

    Full Text Available The Fragile States Group within the Development AssistanceCommittee (DAC of the Organisation for EconomicCooperation and Development is working to advise donors onprovision of education (and other services in ‘fragile states’.

  5. Fragile X syndrome and fragile X-associated disorders

    Science.gov (United States)

    Rajaratnam, Akash; Shergill, Jasdeep; Salcedo-Arellano, Maria; Saldarriaga, Wilmar; Duan, Xianlai; Hagerman, Randi

    2017-01-01

    Fragile X syndrome (FXS) is caused by a full mutation on the FMR1 gene and a subsequent lack of FMRP, the protein product of FMR1. FMRP plays a key role in regulating the translation of many proteins involved in maintaining neuronal synaptic connections; its deficiency may result in a range of intellectual disabilities, social deficits, psychiatric problems, and dysmorphic physical features. A range of clinical involvement is also associated with the FMR1 premutation, including fragile X-associated tremor ataxia syndrome, fragile X-associated primary ovarian insufficiency, psychiatric problems, hypertension, migraines, and autoimmune problems. Over the past few years, there have been a number of advances in our knowledge of FXS and fragile X-associated disorders, and each of these advances offers significant clinical implications. Among these developments are a better understanding of the clinical impact of the phenomenon known as mosaicism, the revelation that various types of mutations can cause FXS, and improvements in treatment for FXS. PMID:29259781

  6. Systems fragility: The sociology of chaos.

    Science.gov (United States)

    Hodges, Lori R

    2016-01-01

    This article examines the concept of community fragility in emergency management from a systems perspective. Using literature that addresses fragility in four areas of complex systems, including ecosystems, social systems, sociotechnical systems, and complex adaptive systems, a theoretical framework focused on the emergency management field is created. These findings illustrate how community fragility factors can be used in the emergency management field to not only improve overall outcomes after disaster but also build less fragile systems and communities in preparation for future disasters.

  7. APLICAÇÃO DO MÉTODO DE CLASSIFICAÇÃO CONTÍNUA FUZZY PARA O MAPEAMENTO DA FRAGILIDADE DO TERRENO EM RELAÇÃO À OCORRÊNCIA DE RAVINAS NO PARQUE NACIONAL DA SERRA DA CANASTRA - APPLICATION OF CONTINUOUS FUZZY CLASSIFICATION METHOD FOR MAPPING TERRAIN FRAGILITY REGARDING THE OCCURRENCE OF GULLIES IN THE SERRA DA CANASTRA NATIONAL PARK

    Directory of Open Access Journals (Sweden)

    Cassiano Gustavo Messias

    2017-04-01

    Full Text Available Este trabalho tem como objetivo realizar o mapeamento da fragilidade ambiental à ocorrência de ravinas no Parque Nacional da Serra da Canastra, por meio de um modelo espacial baseado em lógica fuzzy, desenvolvido para SIG, que combina mapas das seguintes variáveis geoambientais: índice de vegetação, densidade de lineamentos estruturais, densidade de vias de circulação e declividade do terreno. Este modelo utiliza parâmetros do teste estatístico Kolmogorov-Smirnov (KS, extraídos a partir da avaliação do grau de aderência entre a distribuição dos valores das variáveis ambientais e a distribuição espacial real das ravinas observadas no parque. Os pesos são utilizados em um processo de álgebra de mapas, baseado na soma ponderada das variáveis geoambientais convertidas em escala fuzzy. O mapa de fragilidade ambiental produzido é representado segundo o método de classificação contínua fuzzy, indicando o grau de afinidade de cada pixel à ocorrência de ravinas. Os resultados mostraram que a ordem de importância das variáveis na ocorrência de ravinas, nesta Unidade de Conservação, é a seguinte: (1 densidade de lineamentos estruturais; (2 índice de vegetação; (3 densidade de estradas; (4 declividades. As áreas de maior fragilidade concentram-se em bacias localizadas a noroeste e na porção central do Chapadão da Babilônia. Os graus menores de fragilidade são observados, sobretudo, no Chapadão da Canastra e em pontos a sudeste do Chapadão da Babilônia, em bacias que apresentam densidades de ravinas abaixo da média do parque. ABSTRACT The aim of this work was to map environmental fragility of Serra da Canastra National Park, located in Southwest region of Minas Gerais state, Brazil, using ravine sites identified on high spatial resolution images, as terrain true controls points. The methodology used for mapping was based on fuzzy logic model and GIS techniques. The model combines four environmental variables

  8. Peso do útero após ligadura no ramo ovariano do vaso uterino Weight of uterus after ligation of ovarian branches of the uterine vessel

    Directory of Open Access Journals (Sweden)

    Roberto Adelino de Almeida Prado

    2007-04-01

    Full Text Available OBJETIVO: O presente estudo teve por objetivo principal avaliar se o bloqueio do fluxo sangüíneo aos ovários, proveniente dos vasos uterinos, pode alterar a função gonadal, resultando na diminuição da secreção de estrogênio e do peso do útero. MÉTODOS: Foi criado um modelo experimental no qual 30 ratas adultas e virgens da cepa Wistar foram incluídas. Após pesagem, os animais foram separados em dois grupos: o grupo ligadas, com 20 animais submetidos a laparotomia e ligadura dos ramos ováricos dos vasos uterinos, e o grupo controle, com dez ratas nas quais praticou-se apenas a laparotomia. Após 16 semanas, as ratas foram pesadas e submetidas à eutanásia, tendo seus úteros dissecados e pesados. Calculou-se o quociente entre o peso do útero e o peso do animal. RESULTADOS: Significância estatística não foi observada quando as médias dos pesos uterinos e dos índices entre o peso uterino e o peso dos animais foram comparadas entre os grupos. CONCLUSÃO: A interrupção da irrigação dos ovários, proveniente dos ramos ováricos dos vasos uterinos, não produziu alteração no peso dos úteros dos animais estudados.OBJECTIVE: This study aims to evaluate whether the blockage of ovarian irrigation from uterine vessels can change the gonadal function resulting in diminished estrogen secretion and weight of uterus. METHODS: An experimental model was created including 30 virgin female Wistar rats. After weight determination, animals were separated into two groups: the ligation group with 20 animals, submitted to laparotomy and ligation of the ovarian branches of the uterine blood vessels, and the control group with 10 animals submitted only to laparotomy. Sixteen weeks later the rats were weighed and euthanized, and their uteri dissected and weighed. The index between animal and uteri weights was calculated. RESULTS: Statistical significance was not observed when the mean uterus weights and the indexes between weight of the uterus

  9. Análise dos Vasos do Trato Urinário Inferior de Ratas Durante e Após a Prenhez Analysis of Urinary Tract Vessels during and after Pregnancy in Rats

    Directory of Open Access Journals (Sweden)

    José Vicente Kosmiskas

    2002-05-01

    Full Text Available Objetivos: estudar as variações da anatomia vascular de ratas, avaliando o número de vasos da uretra proximal e distal, da junção vésico-uretral e da bexiga, durante e após a prenhez. Métodos: trinta ratas, com teste positivo de acasalamento, foram divididas aleatoriamente em três grupos contendo 10 animais cada, a saber: GI - ratas no 10º dia de prenhez; GII - ratas no 20º dia de prenhez; GIII - ratas no 5º dia de puerpério; um grupo controle (GIV era composto de 10 ratas na fase de estro. Foram contados os vasos sangüíneos em quatro lâminas, por animal, coradas pelo método do tricrômico de Masson, com ocular de integração de 25 pontos, acoplada a microscopia de luz, com objetiva de 40 X. As regiões estudadas foram uretra proximal e distal, junção vésico-uretral e bexiga. Resultados: não houve variação significativa no número de vasos da bexiga, da junção vésico-uretral e da uretra proximal nas ratas durante a gestação ou em relação ao grupo controle. Na uretra distal, o número de vasos no grupo controle foi 13,7, sendo significativamente menor do que nos grupos com prenhez (20,5 a 24,4 vasos. Conclusões: as ratas prenhes apresentaram maior número de vasos na uretra distal do que aquelas em estro. Nos demais locais estudados não houve diferenças entre os grupos.Purpose: to evaluate the variations in vascular anatomy by assessing the number of vessels of the proximal and distal urethra, of the vesicourethral canal and of the bladder, during and after pregnancy in rats. Method: thirty female rats, with a positive test for pregnancy, were divided into three groups of 10 animals each: GI - rats on the 10th day of pregnancy; GII - rats on the 20th day of pregnancy; GIII - rats on the 5th day of puerperium; a control group (GIV composed of 10 rats in the estrous phase. The vessels were stained by the method of Masson and counted with a 25-dot integration ocular, coupled to a light microscope, with an objective

  10. Premature ovarian failure (POF in Brazilian fragile X carriers

    Directory of Open Access Journals (Sweden)

    Angela M. Vianna-Morgante

    1999-12-01

    Full Text Available The gynecological and reproductive histories of 193 women from fragile X families were surveyed. Among the 101 carriers of the premutation, 14 experienced premature menopause, contrarily to their 37 fully mutated and 55 noncarrier female relatives. Although premature menopause showed a tendency to cluster in certain fragile X families, as a group, the premutated women experienced menopause earlier than noncarriers. This suggests that premature menopause may be the extreme effect of a spectrum of ovarian anomalies associated with the fragile X premutation.Entrevistamos 193 mulheres de famílias com afetados pela síndrome do cromossomo X frágil, quanto a sua história ginecológica e reprodutiva. Entre as 101 portadoras da pré-mutação, 14 tiveram menopausa precoce, mas nenhuma das 37 portadoras da mutação completa ou das 55 não portadoras apresentaram esta anomalia. Observamos uma tendência para a concentração da menopausa precoce em certas famílias, o que poderia significar uma peculiariedade de certas pré-mutações. Entretanto, o fato de as mulheres pré-mutadas tenderem a entrar em menopausa mais cedo do que as não portadoras sugere que a menopausa precoce seja o extremo do espectro de efeitos ovarianos da pré-mutação.

  11. Severe Vaso-occlusive Retinopathy as the Primary Manifestation in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Tsung-Yu Ho

    2008-07-01

    Full Text Available Severe vaso-occlusive retinopathy as the initial manifestation of systemic lupus erythematosus (SLE is rare. We report a 16-year-old female who developed bilateral visual impairment. Fundus examinations showed bilateral “cherry-red spot” appearance, multiple confluent cotton wool spots, and widespread arterial occlusion. Laboratory studies showed leukopenia, antinuclear antibody (+, and anti-double-stranded DNA antibody (+. Malar rashes, oral ulcers, and bilateral knee joint tenderness were noted during physical examination. SLE was diagnosed and pulse therapy started immediately. Best corrected visual acuity of the left eye improved to 6/10 after treatment. However, there was no visual improvement in the right eye. Four months later, bilateral panretinal laser photocoagulation was performed due to retinal neovascularization. However, tractional retinal detachment of the right eye and vitreous hemorrhage of the left eye still occurred. After undergoing cryoretinopexy of the right eye and intravitreous tissue plasminogen activator injection of the left eye, the visual acuity of the patient's right eye remained hand movement only at 10 cm, but that of the left eye returned to 6/10. The ocular and systemic conditions were stable in the follow-up period of more than 2 years. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.

  12. Severe vaso-occlusive retinopathy as the primary manifestation in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Ho, Tsung-Yu; Chung, Yu-Mei; Lee, An-Fei; Tsai, Chang-Youh

    2008-07-01

    Severe vaso-occlusive retinopathy as the initial manifestation of systemic lupus erythematosus (SLE) is rare. We report a 16-year-old female who developed bilateral visual impairment. Fundus examinations showed bilateral "cherry-red spot" appearance, multiple confluent cotton wool spots, and widespread arterial occlusion. Laboratory studies showed leukopenia, antinuclear antibody (+), and anti-double-stranded DNA antibody (+). Malar rashes, oral ulcers, and bilateral knee joint tenderness were noted during physical examination. SLE was diagnosed and pulse therapy started immediately. Best corrected visual acuity of the left eye improved to 6/10 after treatment. However, there was no visual improvement in the right eye. Four months later, bilateral panretinal laser photocoagulation was performed due to retinal neovascularization. However, tractional retinal detachment of the right eye and vitreous hemorrhage of the left eye still occurred. After undergoing cryoretinopexy of the right eye and intravitreous tissue plasminogen activator injection of the left eye, the visual acuity of the patient's right eye remained hand movement only at 10 cm, but that of the left eye returned to 6/10. The ocular and systemic conditions were stable in the follow-up period of more than 2 years. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.

  13. Reinterpretando la información de los vasos dinásticos de los Kaan ajawo’ob.

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    Pablo Alberto Mumary Farto

    2015-12-01

    Full Text Available Los conocidos como vasos dinásticos forman parte de una colección de alrededor de 17 vasos cerámicos caracterizados por la narrativa epigráfica que contienen en la cual se disponen listados de supuestos gobernantes de la dinastía Kaan desde su hipotético fundador. Tomando los trabajos de finales del siglo XX realizados por los investigadores Robicsek, Hales y Martin, reanalizaremos la información epigráfica contenida en dos vasos seleccionados proponiendo nuevas lecturas y, consecuentemente, presentando información que no había sido tenida en cuenta.

  14. Ectodermal Dysplasia Skin Fragility Syndrome

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    Ayça Alan Atalay

    2014-06-01

    Full Text Available Ectodermal dysplasia-skin fragility syndrome (EDSFS is a rare autosomal recessive genodermatosis first described in 1997 by Mc Grath. EDSFS results from loss of function mutations in plakophilin-1 (PKP1. PKP1 is a structural component of desmosomes, cellcell adhesion complexes. It is also found as a nuclear protein in several cell types that are lack of desmosomes. In skin, however, PKP1 expression is confined mainly to suprabasal keratinocytes and the outer root sheath of hair follicules. Loss of function mutation in PKP1 leads to extensive skin fragility, bullae and erosions following minor trauma, focal keratoderma with painful fissures, alopecia, and nail dystrophy. In some patients hypohidrosis may also be seen. EDSFS is now considered as a specific suprabasal form of epidermolysis bullosa simplex. In this report we describe a 20 year old EDSFS case.

  15. Preserved entropy and fragile magnetism.

    Science.gov (United States)

    Canfield, Paul C; Bud'ko, Sergey L

    2016-08-01

    A large swath of quantum critical and strongly correlated electron systems can be associated with the phenomena of preserved entropy and fragile magnetism. In this overview we present our thoughts and plans for the discovery and development of lanthanide and transition metal based, strongly correlated systems that are revealed by suppressed, fragile magnetism, quantum criticality, or grow out of preserved entropy. We will present and discuss current examples such as YbBiPt, YbAgGe, YbFe2Zn20, PrAg2In, BaFe2As2, CaFe2As2, LaCrSb3 and LaCrGe3 as part of our motivation and to provide illustrative examples.

  16. Autism and fragile X syndrome.

    Science.gov (United States)

    Yu, Timothy W; Berry-Kravis, Elizabeth

    2014-07-01

    Autistic spectrum disorders (ASDs) are characterized by impairments in language, social skills, and repetitive behaviors, often accompanied by intellectual disability. Advances in the genetics of ASDs are providing new glimpses into the underlying neurobiological mechanisms disrupted in these conditions. These glimpses on one hand reinforce the idea that synapse development and plasticity are one of the major pathways disrupted in autism, but beyond that are providing fresh molecular support to the idea of mechanistic parallels between idiopathic ASD and specific syndromic neurodevelopmental disorders like fragile X syndrome (FXS). Fragile X syndrome is already recognized as the most common identifiable genetic cause of intellectual disability and ASDs, with many overlapping phenotypic features. Fragile X syndrome is associated with a variety of cognitive, behavioral, physical, and medical problems, which are managed through supportive treatment. Recent major advances in the understanding of the underlying neurobiology in FXS have led to the discovery of agents that rescue phenotypes in the FXS mouse model, and early clinical trials of targeted treatments in humans with FXS. Thus translational strategies in FXS may be poised to serve as models for ASD and other cognitive disorders. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  17. Procalcitonin as a biomarker of bacterial infection in sickle cell vaso-occlusive crisis.

    Science.gov (United States)

    Patel, Dilip Kumar; Mohapatra, Manoj Kumar; Thomas, Ancil George; Patel, Siris; Purohit, Prasanta

    2014-01-01

    Sickle cell anaemia (SCA) patients with vaso-occlusive crisis (VOC) have signs of inflammation and it is often difficult to diagnose a bacterial infection in them. This study was undertaken to evaluate the role of serum procalcitonin (PCT) as a biomarker of bacterial infection in acute sickle cell vaso-occlusive crisis. Hundred homozygous SCA patients were studied at Sickle Cell Clinic and Molecular Biology Laboratory, V.S.S. Medical College, Burla, Odisha, India. All the patients were divided into three categories namely category-A (VOC/ACS with SIRS but without evidence of bacterial infection - 66 patients), category-B (VOC/ACS with SIRS and either proven or suspected bacterial infection - 24 patients) and category-C (SCA patients in steady state without VOC/ACS or SIRS - 10 patients). Complete blood count, C-reactive protein (CRP) estimation and PCT measurement were done in all the patients. There was no significant difference in TLC and CRP values between category-A and B. In category-A, the PCT level was value >0.5 ng/mL with 87.5% of patients having >2 ng/mL. In category-C, PCT value was value (100%) for bacterial infection at a cutoff value of 0.5 ng/mL; whereas the specificity is excellent at a cut-off value of 2 ng/mL. SCA patients with VOC/ACS and SIRS having a PCT level of value of >2 ng/mL is indicative of bacterial infection necessitating early antimicrobial therapy.

  18. Pharmacotherapeutical strategies in the prevention of acute, vaso-occlusive pain in sickle cell disease: a systematic review

    NARCIS (Netherlands)

    Sins, Joep W. R.; Mager, David J.; Davis, Shyrin C. A. T.; Biemond, Bart J.; Fijnvandraat, Karin

    2017-01-01

    Sickle-cell disease (SCD) is characterized by frequent and painful vaso-occlusive crises (VOCs). Various treatments have been evaluated over the years. However, a clear overview is lacking. The objective of this study was to systematically review all pharmacotherapeutical strategies in the

  19. Dynamics of von Willebrand factor reactivity in sickle cell disease during vaso-occlusive crisis and steady state

    NARCIS (Netherlands)

    Sins, J. W.R.; Schimmel, Marein; Luken, Brenda M.; Nur, Erfan; Zeerleder, S.; van Tuijn, Charlotte F. J.; Brandjes, Dees P. M.; Kopatz, W. F.; Urbanus, R. T.; Meijers, Joost C. M.; Biemond, B. J.; Fijnvandraat, K.

    2017-01-01

    Essentials The role of von Willebrand Factor (VWF) in the pathophysiology of sickle cell disease is unclear. We assessed markers of VWF during admission for vaso-occlusive crisis (VOC) and steady state. VWF reactivity was higher during VOC and was associated with inflammation and neutrophil

  20. Significance of the analytical forms of fragility curves

    International Nuclear Information System (INIS)

    Goodman, J.; Hadjian, A.H.

    1985-01-01

    Sensitivity analysis of the core melt probability to the analytical forms of the plant fragility is provided. Major parameters of the fragility curve affecting the core damage probability are discussed. Using algebra of fragilities it is demonstrated how analytical forms of component fragilities shape up the plant fragility and determine its parameters

  1. Seismic fragility of nuclear power plant components (Phase 2): A fragility handbook on eighteen components

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.K.; Hofmayer, C.H.; Kassir, M.K.; Shteyngart, S.

    1991-06-01

    Fragility estimates of seven equipment classes were published in earlier reports. This report presents fragility analysis results from eleven additional equipment categories. The fragility levels are expressed in probabilistic terms. For users' convenience, this concluding report includes a summary of fragility results of all eighteen equipment classes. A set of conversion factors based on judgment is recommended for use of the information for early vintage equipment. The knowledge gained in conducting the Component Fragility Program and similar other programs is expected to provide a new direction for seismic verification and qualification of equipment. 15 refs., 12 tabs

  2. Fragile X Speech in Finnish: Phonological Observations.

    Science.gov (United States)

    Niemi, Jussi; And Others

    Analysis of the phonological patterns of two physically normal boys, aged 5 and 8 years, with fragile X syndrome, an X-chromosomal abnormality usually connected with severe to moderate mental retardation, found language features similar to those found in other studies of fragile X speech. Some of these language features are: repetition of initial…

  3. Speech Fluency in Fragile X Syndrome

    Science.gov (United States)

    Van Borsel, John; Dor, Orianne; Rondal, Jean

    2008-01-01

    The present study investigated the dysfluencies in the speech of nine French speaking individuals with fragile X syndrome. Type, number, and loci of dysfluencies were analysed. The study confirms that dysfluencies are a common feature of the speech of individuals with fragile X syndrome but also indicates that the dysfluency pattern displayed is…

  4. Vasculite cutânea de pequenos vasos: etiologia, patogênese, classificação e critérios diagnósticos - Parte I Small vessel cutaneous vasculitis: etiology, pathogenesis, classification and diagnostic criteria - Part I

    Directory of Open Access Journals (Sweden)

    Hebert Roberto Clivati Brandt

    2007-10-01

    Full Text Available Vasculite é a inflamação da parede dos vasos. Pode variar em gravidade desde doença autolimitada de um único órgão até doença grave com risco de morte por falência de múltiplos órgãos. Existem várias causas, embora só se apresente por poucos padrões histológicos de inflamação vascular. Vasos de qualquer tipo e em qualquer órgão podem ser afetados, resultando em ampla variedade de sinais e sintomas. Diferentes vasculites com apresentações clínicas indistinguíveis têm evolução e tratamento muito diferentes. Essa condição representa desafio para o médico, incluindo classificação, diagnóstico, exames laboratoriais pertinentes, tratamento e seguimento adequado. Neste artigo são revistos a classificação, a etiologia, a patogênese e os critérios diagnósticos das vasculites cutâneas.Vasculitis is an inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple-organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. This article reviews the classification, etiology, pathology and diagnostic criteria of cutaneous vasculitis.

  5. Un vaso de alabastro procedente de Adra conservado en el Museo de Almería

    Directory of Open Access Journals (Sweden)

    García Alfonso, Eduardo

    1998-12-01

    Full Text Available A previously unpublished alabaster jar from Adra, a coastal site some 50 km to the west of Almería is yet another example of a numerous class of Egyptian containers of the 8th-7th centuries BC discovered on the southern coast of the Iberian Peninsula. A casual find, the funerary character of this piece suggests that there may have been a cemetery of the Early Phoenician Period in the immediate neighbourhood of the colonial settlement of Abdera.Se estudia un vaso de alabastro inédito procedente de la localidad almeriense de Adra, pieza que se añade a la ya numerosa serie de recipientes de origen egipcio conocida en las costas meridionales de la Península durante los siglos VIII-VII a.C. El principal interés de este hallazgo casual radica en su carácter funerario, siendo indicio de una posible necrópolis de época fenicia arcaica en las inmediaciones del asentamiento colonial de Abdera.

  6. State of the Art Management of Acute Vaso-occlusive Pain in Sickle Cell Disease.

    Science.gov (United States)

    Puri, Latika; Nottage, Kerri A; Hankins, Jane S; Anghelescu, Doralina L

    2018-02-01

    Acute vaso-occlusive crisis (VOC) is a hallmark of sickle cell disease (SCD). Multiple complex pathophysiological processes can result in pain during a VOC. Despite significant improvements in the understanding and management of SCD, little progress has been made in the management of pain in SCD, although new treatments are being explored. Opioids and non-steroidal anti-inflammatory drugs (NSAIDs) remain the mainstay of treatment of VOC pain, but new classes of drugs are being tested to prevent and treat acute pain. Advancements in the understanding of the pathophysiology of SCD and pain and the pharmacogenomics of opioids have yet to be effectively utilized in the management of VOC. Opioid tolerance and opioid-induced hyperalgesia are significant problems associated with the long-term use of opioids, and better strategies for chronic pain therapy are needed. This report reviews the mechanisms of pain associated with acute VOC, describes the current management of VOC, and describes some of the new therapies under evaluation for the management of acute VOC in SCD.

  7. Nuclear Magnetic Resonance structural studies of peptides and proteins from the vaso-regulatory System

    International Nuclear Information System (INIS)

    Sizun, Philippe

    1991-01-01

    The aim of the present work is to show how Nuclear Magnetic Resonance (NMR) allows to determine the 3D structure of peptides and proteins in solution. A comparative study of peptides involved in the vaso-regulatory System (form small hormonal peptide to the 65 amido-acid protein hirudin) has allowed to design most efficient NMR 1D and 2D strategies. It rapidly appeared that the size of the peptide plays a key role in the structuration of the molecule, smallest peptides being weakly structured owing to the lack of cooperative effects. As the molecular size increases or if conformational locks are present (disulfide bridges) the probability of stable secondary structure increases. For the protein hirudin, a combination of ail available NMR parameters deduced form dedicated experiments (chemical shifts, coupling constants, overhauser effects, accessibility of amide protons) and molecular modelling under constraints allows a clear 3D structure to be proposed for this protein in solution. Finally, a comparative study of the experimental structures and of those deduced form prediction rules has shed light on the concept of structural predisposition, the latter being of high value for a better understanding of structure-activity relationships. (author) [fr

  8. Genome Organization Drives Chromosome Fragility.

    Science.gov (United States)

    Canela, Andres; Maman, Yaakov; Jung, Seolkyoung; Wong, Nancy; Callen, Elsa; Day, Amanda; Kieffer-Kwon, Kyong-Rim; Pekowska, Aleksandra; Zhang, Hongliang; Rao, Suhas S P; Huang, Su-Chen; Mckinnon, Peter J; Aplan, Peter D; Pommier, Yves; Aiden, Erez Lieberman; Casellas, Rafael; Nussenzweig, André

    2017-07-27

    In this study, we show that evolutionarily conserved chromosome loop anchors bound by CCCTC-binding factor (CTCF) and cohesin are vulnerable to DNA double strand breaks (DSBs) mediated by topoisomerase 2B (TOP2B). Polymorphisms in the genome that redistribute CTCF/cohesin occupancy rewire DNA cleavage sites to novel loop anchors. While transcription- and replication-coupled genomic rearrangements have been well documented, we demonstrate that DSBs formed at loop anchors are largely transcription-, replication-, and cell-type-independent. DSBs are continuously formed throughout interphase, are enriched on both sides of strong topological domain borders, and frequently occur at breakpoint clusters commonly translocated in cancer. Thus, loop anchors serve as fragile sites that generate DSBs and chromosomal rearrangements. VIDEO ABSTRACT. Published by Elsevier Inc.

  9. Fragile X syndrome: Current insight

    Directory of Open Access Journals (Sweden)

    Deepika Delsa Dean

    2016-10-01

    Full Text Available Fragile X syndrome (FXS is a multigenerational disorder having massive adverse effect not only on the individuals but also on their families. It is the most common type of intellectual disability after Down’s syndrome. Over two decades have passed since the discovery of FMR1, the causal gene for FXS, but still little is known about the pathophysiology of this disease. This lack of knowledge presents the major barrier encountered by the scientific community for early diagnosis and effective treatment. Since early diagnosis has important implication in determining the disease status among members of the family tree so the genetic counseling and supportive therapy get hampered in larger perspective. The present review emphasizes on the recent findings in FXS pathophysiology, therapeutics and technical challenges in molecular diagnosis.

  10. BDNF in fragile X syndrome.

    Science.gov (United States)

    Castrén, Maija L; Castrén, Eero

    2014-01-01

    Fragile X syndrome (FXS) is a monogenic disorder that is caused by the absence of FMR1 protein (FMRP). FXS serves as an excellent model disorder for studies investigating disturbed molecular mechanisms and synapse function underlying cognitive impairment, autism, and behavioral disturbance. Abnormalities in dendritic spines and synaptic transmission in the brain of FXS individuals and mouse models for FXS indicate perturbations in the development, maintenance, and plasticity of neuronal network connectivity. However, numerous alterations are found during the early development in FXS, including abnormal differentiation of neural progenitors and impaired migration of newly born neurons. Several aspects of FMRP function are modulated by brain-derived neurotrophic factor (BDNF) signaling. Here, we review the evidence of the role for BDNF in the developing and adult FXS brain. This article is part of the Special Issue entitled 'BDNF Regulation of Synaptic Structure, Function, and Plasticity'. Copyright © 2013 Elsevier Ltd. All rights reserved.

  11. Component fragilities - data collection, analysis and interpretation

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.K.; Hofmayer, C.H.

    1986-01-01

    As part of the component fragility research program sponsored by the US Nuclear Regulatory Commission, BNL is involved in establishing seismic fragility levels for various nuclear power plant equipment with emphasis on electrical equipment, by identifying, collecting and analyzing existing test data from various sources. BNL has reviewed approximately seventy test reports to collect fragility or high level test data for switchgears, motor control centers and similar electrical cabinets, valve actuators and numerous electrical and control devices of various manufacturers and models. Through a cooperative agreement, BNL has also obtained test data from EPRI/ANCO. An analysis of the collected data reveals that fragility levels can best be described by a group of curves corresponding to various failure modes. The lower bound curve indicates the initiation of malfunctioning or structural damage, whereas the upper bound curve corresponds to overall failure of the equipment based on known failure modes occurring separately or interactively. For some components, the upper and lower bound fragility levels are observed to vary appreciably depending upon the manufacturers and models. An extensive amount of additional fragility or high level test data exists. If completely collected and properly analyzed, the entire data bank is expected to greatly reduce the need for additional testing to establish fragility levels for most equipment

  12. Seismic fragility analysis for NPP structural components

    International Nuclear Information System (INIS)

    Casciati, F.; Faravelli, L.

    1984-01-01

    Relationship between probability of failure and seismic intensity (fragility curves) summarize the results of the vulnerability analysis of a NPP structural component. The appropriate operative procedures for the evaluation of these fragility curves are discussed. The paper illustrates a general purpose computer code which includes two different probabilistic models for two different levels of sophistication in the fragility analysis. Attention is then focussed (i) on the mechanical aspects associated with the local strength estimation (ii) on the simulation of suitable artificial ground motions and (iii) on the definition of an appropriate measure of the seismic intensity. (Author) [pt

  13. Behavioral Phenotype of Fragile X Syndrome in Adolescence and Adulthood

    Science.gov (United States)

    Smith, Leann E.; Barker, Erin T.; Seltzer, Marsha Mailick; Abbeduto, Leonard; Greenberg, Jan S.

    2012-01-01

    The present study explored the behavioral profile of individuals with fragile X syndrome during adolescence and adulthood. Individuals with both fragile X syndrome and autism (n = 30) were compared with (a) individuals diagnosed with fragile X syndrome (but not autism; n = 106) and (b) individuals diagnosed with autism (but not fragile X syndrome;…

  14. Low-molecular-weight heparins for managing vaso-occlusive crises in people with sickle cell disease.

    Science.gov (United States)

    van Zuuren, Esther J; Fedorowicz, Zbys

    2015-12-18

    Sickle cell disease is one of the most common and severe genetic disorders in the world. It can be broadly divided into two distinct clinical phenotypes characterized by either haemolysis or vaso-occlusion. Pain is the most prominent symptom of vaso-occlusion, and hypercoagulability is a well-established pathogenic phenomenon in people with sickle cell disease. Low-molecular-weight heparins might control this hypercoagulable state through their anticoagulant effect. This is an update of a previously published version of this review. To assess the effects of low-molecular-weight heparins for managing vaso-occlusive crises in people with sickle cell disease. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register comprising references identified from comprehensive electronic database searches. We also searched abstract books of conference proceedings and several online trials registries for ongoing trials.Date of the last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register: 28 September 2015. Randomised controlled clinical trials and controlled clinical trials that assessed the effects of low-molecular-weight heparins in the management of vaso-occlusive crises in people with sickle cell disease. Study selection, data extraction, assessment of risk of bias and analyses were carried out independently by the two review authors. Two studies comprising 287 participants were included. One study (with an overall unclear to high risk of bias) involved 253 participants and the quality of the evidence for most outcomes was very low. This study, reported that pain severity at day two and day three was lower in the tinzaparin group than in the placebo group (P quality of evidence rated very low. The most important reasons for downgrading the quality of evidence were serious risk of bias and imprecision (due to low sample size or low occurrence of events). Based on the results

  15. Predictors of patency after two-stitch invagination vaso-epididymal anastomosis for idiopathic obstructive azoospermia

    Directory of Open Access Journals (Sweden)

    G Gautam

    2005-01-01

    Full Text Available Objectives: Anastomotic patency with return of sperm in the ejaculate following microsurgical vasoepididymostomy (VEA is not universal and may be delayed. The ability to predict the result of VEA based on preoperative or intra-operative parameters would enable the surgeon to offer the best treatment to the infertile couple. We used the two-stitch invagination technique of VEA in patients of idiopathic obstructive azoospermia and prospectively analyzed factors that could predict a patent anastomosis. While such studies have previously been done for patients undergoing VEA for secondary infertility following a vasectomy, to the best of our knowledge this is the first study analyzing these parameters for patients with primary infertility and idiopathic obstruction. Methods and materials: Over a 2-year period, 29 men underwent the 2-suture invagination VEA for idiopathic obstructive azoospermia. Twenty-four patients provided at least one postoperative semen sample. Preoperative and intra-operative parameters were compared between patients with a patent anastomosis with sperm in ejaculate (n = 12 and those with no sperm in the ejaculate (n = 12 using the t-test, Fisher′s exact test or chi-square test, as appropriate and a multivariate statistical analysis to determine any significant difference. Results: The mean follow up of the 24 patients was 7.6 months (2-30 months. A significantly greater number of patients with patent anastomosis had motile epididymal sperms (P = 0.034 and higher surgeon′s technical satisfaction with the procedure (P = 0.034. However, this difference was seen only on a univariate analysis and did not persist when a multivariate analysis was used. Conclusions: The presence of motile sperms in the epididymal fluid and a high level of technical satisfaction with the anastomosis may indicate a higher likelihood of success following a vaso-epididymal anastomosis for idiopathic obstruction. However, these parameters are not

  16. A note on families of fragility curves

    International Nuclear Information System (INIS)

    Kaplan, S.; Bier, V.M.; Bley, D.C.

    1989-01-01

    In the quantitative assessment of seismic risk, uncertainty in the fragility of a structural component is usually expressed by putting forth a family of fragility curves, with probability serving as the parameter of the family. Commonly, a lognormal shape is used both for the individual curves and for the expression of uncertainty over the family. A so-called composite single curve can also be drawn and used for purposes of approximation. This composite curve is often regarded as equivalent to the mean curve of the family. The equality seems intuitively reasonable, but according to the authors has never been proven. The paper presented proves this equivalence hypothesis mathematically. Moreover, the authors show that this equivalence hypothesis between fragility curves is itself equivalent to an identity property of the standard normal probability curve. Thus, in the course of proving the fragility curve hypothesis, the authors have also proved a rather obscure, but interesting and perhaps previously unrecognized, property of the standard normal curve

  17. Fragile-X Syndrome and Education.

    Science.gov (United States)

    Gibb, Charles; Holliday, Judith

    1992-01-01

    This article summarizes the major cognitive, behavioral, and language characteristics of the Fragile X syndrome and considers their educational implications. This common heritable cause of mental disability has implications for teaching style, curriculum, family counseling, and speech therapy. (DB)

  18. Courting a cure for fragile X.

    Science.gov (United States)

    Dölen, Gül; Bear, Mark F

    2005-03-03

    Fragile X syndrome is the most common heritable cause of mental retardation. Previous work has suggested that overactive signaling by group I metabotropic glutamate receptors (mGluRs) may be a mechanism underlying many of the disease symptoms. As a test of this theory, McBride et al. show that in a Drosophila model for Fragile X syndrome, treatment with mGluR antagonists can rescue short-term memory, courtship, and mushroom body defects.

  19. Avaliação de cultivares de alface adubadas com silicato de cálcio em casa-de-vegetação.

    OpenAIRE

    FERREIRA, R. L. F.; SOUZA, R. J. de; CARVALHO, J. G. de; ARAÚJO NETO, S. E. de; MENDONÇA, V.; WADT, P. G. S.

    2011-01-01

    O experimento foi conduzido no Departamento de Ciência do Solo da Universidade Federal de Lavras - UFLA, no período de junho a agosto de 2002. Objetivou-se avaliar a produtividade, o estado nutricional e a qualidade (classe de tamanho) de cultivares de alface cultivadas com doses de silicato de cálcio em vasos sob casa-de-vegetação. O delineamento experimental foi em blocos casualizados, com quatro repetições, com 1 planta/vaso, em esquema fatorial 3 x 4 + 3, sendo três cultivares de alface (...

  20. Retratos da informalidade: a fragilidade dos sistemas de proteção social em momentos de infortúnio Informality portraits: the fragility of social protectionist systems in misfortune situations

    Directory of Open Access Journals (Sweden)

    Fernanda Flávia Cockell

    2011-03-01

    Full Text Available A pesquisa teve como objetivo estudar as estratégias e redes sociais utilizadas por trabalhadores informais em situações de infortúnio. Foram entrevistados dezesseis trabalhadores da construção de edificações de São Carlos (SP. Procuramos entender como esses trabalhadores lidam com a perda da capacidade de trabalho diante do contexto de precariedade e como percebem tais situações. As análises qualitativas apontam que a maior parte dos entrevistados encontra dificuldades financeiras para adquirir formas privadas de proteção. Os resultados mostram que as redes sociais informais são fundamentais no enfrentamento dos períodos de incapacidade laboral, prevalecendo os apoios da família nuclear e extensa e das redes formadas na identificação religiosa.The research has as the objective to study the strategies and social networks used by informal workers in misfortune situations. We interviewed sixteen workers of the buildings' construction sector of São Carlos (São Paulo, Brazil. We tried to understand how they deal with working capacity reduction inside a precariousness context and how they perceive these situations. The qualitative analysis shows that most of them have financial difficulties to acquire private forms of protection. We could infer that informal social networks are essential for them deal to periods of working incapacity. Moreover the nuclear and extended family supports and religious networks are predominating.

  1. Transposición corregida de grandes vasos Corrected transposition of the great arteries

    Directory of Open Access Journals (Sweden)

    José Rafael Escalona Aguilera

    2012-09-01

    Full Text Available Se presenta una paciente de sexo femenino, de 9 años de edad, que acude a su médico de familia y se le auscultó un soplo al examen físico y sintomatología que se comportaba como una comunicación interventricular. Luego de los estudios correspondientes, se permitió hacer el diagnóstico de transposición corregida de grandes vasos. La paciente tenía asociada una anomalía de Ebstein, comunicación interauricular, una comunicación interventricular, y una persistencia del conducto arterioso, todo lo cual agravaba su cuadro clínico. Primero es intervenida quirúrgicamente, y se cierra el ducto; de manera espontánea, cierra la comunicación interauricular, y la anomalía de Ebstein parchea la comunicación interventricular. La niña desarrolla una insuficiencia cardiaca e hipertensión pulmonar, a consecuencia de las anomalías asociadas. El caso es de interés, ya que son pocos los publicados en la literatura internacional con esta cardiopatía congénita asociada a otras anomalías estructurales del corazón, que modifican su historia natural.This paper presented a 9 years-old female patient, who went to the family's doctor and was physically examined to detect heart murmur and symptoms of ventricular septal defect. After the corresponding studies, the diagnosis was the corrected transposition of the great arteries. The patient also suffered Ebstein anomaly, atrial septal defect, ventricular septal defect and persistence of arteriose duct, all of which worsened her clinical picture. First, she was operated on and the duct was closed. Later the atrial septal defect closed spontaneously, and the Ebstein anomaly patched the ventricular septal defect. The girl developed heart failure and pulmonary hypertension, as a result of associated anomalies. The case is interesting since few cases like this one, associated with other structural heart anomalies that change its natural course, are presented in the international literature.

  2. Sensitivity to food additives, vaso-active amines and salicylates: a review of the evidence.

    Science.gov (United States)

    Skypala, Isabel J; Williams, M; Reeves, L; Meyer, R; Venter, C

    2015-01-01

    Although there is considerable literature pertaining to IgE and non IgE-mediated food allergy, there is a paucity of information on non-immune mediated reactions to foods, other than metabolic disorders such as lactose intolerance. Food additives and naturally occurring 'food chemicals' have long been reported as having the potential to provoke symptoms in those who are more sensitive to their effects. Diets low in 'food chemicals' gained prominence in the 1970s and 1980s, and their popularity remains, although the evidence of their efficacy is very limited. This review focuses on the available evidence for the role and likely adverse effects of both added and natural 'food chemicals' including benzoate, sulphite, monosodium glutamate, vaso-active or biogenic amines and salicylate. Studies assessing the efficacy of the restriction of these substances in the diet have mainly been undertaken in adults, but the paper will also touch on the use of such diets in children. The difficulty of reviewing the available evidence is that few of the studies have been controlled and, for many, considerable time has elapsed since their publication. Meanwhile dietary patterns and habits have changed hugely in the interim, so the conclusions may not be relevant for our current dietary norms. The conclusion of the review is that there may be some benefit in the removal of an additive or a group of foods high in natural food chemicals from the diet for a limited period for certain individuals, providing the diagnostic pathway is followed and the foods are reintroduced back into the diet to assess for the efficacy of removal. However diets involving the removal of multiple additives and food chemicals have the very great potential to lead to nutritional deficiency especially in the paediatric population. Any dietary intervention, whether for the purposes of diagnosis or management of food allergy or food intolerance, should be adapted to the individual's dietary habits and a suitably

  3. Tratamento cirúrgico sem circulação extracorpórea de arteriopatia relacionada com arterite de Takayasu envolvendo aorta e vasos da base Surgical treatment without extracorporeal circulation of artery disease related to Takayasu's arteritis involving the aorta and great vessels

    OpenAIRE

    Rodrigo Milani; Paulo Brofman; Tayse Sandri; Alexandre Varela; José Augusto Souza; Luiz Gustavo Emed; Stefan da Silveira; Marcelo Dantas; Maximiliano Guimarães; Rafael Pontarolli; Francisco Maia

    2005-01-01

    Relatamos o caso de duas pacientes portadoras de arterite de Takayasu, encaminhadas ao nosso serviço onde as lesões acometiam a aorta torácica descendente e os vasos da base e, em uma delas, lesão obstrutiva crítica do óstio da coronária esquerda. Ambas as pacientes foram operadas sem circulação extracorpórea, com heparinização plena e auxílio de autotransfusão.We report the cases of 2 female patients with Takayasu's arteritis referred to our service with lesions affecting the descending thor...

  4. Stress and self-concept in parents of children with fragile X-syndrome / Estresse e autoconceito em pais e mães de crianças com a síndrome do X-frágil

    Directory of Open Access Journals (Sweden)

    Zuleika Ana Cherubini

    2008-01-01

    Full Text Available The Fragile X Syndrome is the major inherited intellectual disability, superseded only by Down Syndrome, and it is transmitted mostly by females. Patients with Fragile X Syndrome show a set of physical, clinical, behavioral and cognitive features that may impact the family structures, especially the parental figures. This study investigates the stress and self-concept of 15 parents of boys with Fragile X Syndrome, 15 parents of boys with Down syndrome and 15 with typical development accordingly to the biopsychosocial model of Bradford. The results showed a significant difference of self-concept between mothers of children with Fragile X Syndrome and typical developmental groups, but not in those with Down Syndrome, suggesting that the transmission factor solely does not explain differences in self-concept.

  5. Prototipo de material polimérico compuesto a base de vasos de cartón para bebidas calientes

    OpenAIRE

    Jiménez Moreno, Luz Adriana

    2013-01-01

    La transformación de un residuo como vasos de cartón para bebidas calientes en un material compuesto polimérico requiere un análisis de las propiedades de la materia prima inicial y de los procesos que pueden llevarse a cabo de acuerdo a los recursos disponibles para la manufactura del mismo. Por medio de la selección de procedimientos experimentales, casos de estudio relevantes en el área y el uso de normas internacionales que provean un marco experimental adecuado, se realiza la caracteriza...

  6. Fragile X syndrome and fragile X-associated tremor ataxia syndrome.

    Science.gov (United States)

    Hall, Deborah A; Berry-Kravis, Elizabeth

    2018-01-01

    Fragile X-associated disorders encompass several conditions, which are caused by expansion mutations in the fragile X mental retardation 1 (FMR1) gene. Fragile X syndrome is the most common inherited etiology of intellectual disability and results from a full mutation or >200 CGG repeats in FMR1. It is associated with developmental delay, autism spectrum disorder, and seizures. Fragile X-associated tremor/ataxia syndrome is a progressive neurodegenerative disease that occurs in premutation carriers of 55-200 CGG repeats in FMR1 and is characterized by kinetic tremor, gait ataxia, parkinsonism, executive dysfunction, and neuropathy. Fragile X-associated primary ovarian insufficiency also occurs in premutation carrier women and manifests with infertility and early menopause. The diseases constituting fragile X-associated disorders differ mechanistically, due to the distinct molecular properties of premutation versus full mutations. Fragile X syndrome occurs when there is a lack of fragile X mental retardation protein (FMRP) due to FMR1 methylation and silencing. In fragile X-associated tremor ataxia syndrome, a toxic gain of function is postulated with the production of excess CGG repeat-containing FMR1 mRNA, abnormal translation of the repeat sequence leading to production of polyglycine, polyalanine, and other polypeptides and to outright deficits in translation leading to reduced FMRP at larger premutation sizes. The changes in underlying brain chemistry due to FMR1 mutations have led to therapeutic studies in these disorders, with some progress being made in fragile X syndrome. This paper also summarizes indications for testing, genetic counseling issues, and what the future holds for these disorders. Copyright © 2018 Elsevier B.V. All rights reserved.

  7. Effects of Ground Motion Input on the Derived Fragility Functions: Case study of 2010 Haiti Earthquake

    Science.gov (United States)

    Hancilar, Ufuk; Harmandar, Ebru; Çakti, Eser

    2014-05-01

    Empirical fragility functions are derived by statistical processing of the data on: i) Damaged and undamaged buildings, and ii) Ground motion intensity values at the buildings' locations. This study investigates effects of different ground motion inputs on the derived fragility functions. The previously constructed fragility curves (Hancilar et al. 2013), which rely on specific shaking intensity maps published by the USGS after the 2010 Haiti Earthquake, are compared with the fragility functions computed in the present study. Building data come from field surveys of 6,347 buildings that are grouped with respect to structural material type and number of stories. For damage assessment, the European Macroseismic Scale (EMS-98) damage grades are adopted. The simplest way to account for the variability in ground motion input could have been achieved by employing different ground motion prediction equations (GMPEs) and their standard variations. However, in this work, we prefer to rely on stochastically simulated ground motions of the Haiti earthquake. We employ five different source models available in the literature and calculate the resulting strong ground motion in time domain. In our simulations we also consider the local site effects by published studies on NEHRP site classes and micro-zoning maps of the city of Port-au-Prince. We estimate the regional distributions from the waveforms simulated at the same coordinates that we have damage information from. The estimated spatial distributions of peak ground accelerations and velocities, PGA and PGV respectively, are then used as input to fragility computations. The results show that changing the ground motion input causes significant variability in the resulting fragility functions.

  8. Component fragilities. Data collection, analysis and interpretation

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.K.; Hofmayer, C.H.

    1985-01-01

    As part of the component fragility research program sponsored by the US NRC, BNL is involved in establishing seismic fragility levels for various nuclear power plant equipment with emphasis on electrical equipment. To date, BNL has reviewed approximately seventy test reports to collect fragility or high level test data for switchgears, motor control centers and similar electrical cabinets, valve actuators and numerous electrical and control devices, e.g., switches, transmitters, potentiometers, indicators, relays, etc., of various manufacturers and models. BNL has also obtained test data from EPRI/ANCO. Analysis of the collected data reveals that fragility levels can best be described by a group of curves corresponding to various failure modes. The lower bound curve indicates the initiation of malfunctioning or structural damage, whereas the upper bound curve corresponds to overall failure of the equipment based on known failure modes occurring separately or interactively. For some components, the upper and lower bound fragility levels are observed to vary appreciably depending upon the manufacturers and models. For some devices, testing even at the shake table vibration limit does not exhibit any failure. Failure of a relay is observed to be a frequent cause of failure of an electrical panel or a system. An extensive amount of additional fregility or high level test data exists

  9. Fragile X syndrome associated with tic disorders.

    Science.gov (United States)

    Schneider, Susanne A; Robertson, Mary M; Rizzo, Renata; Turk, Jeremy; Bhatia, Kailash P; Orth, Michael

    2008-06-15

    Movement disorders other than late onset tremor-ataxia in association with fragile X syndrome, the most common identifiable cause of inherited mental retardation, seem to be rare. Here we describe five male patients from three unrelated families with fragile X syndrome that presented with motor and phonic tics. Clinically, 4 patients fulfilled diagnostic criteria for Gilles de la Tourette syndrome (GTS) while 1 patient would have been diagnosed with an adult onset tic disorder. However, in all patients onset of tics was considerably later than in typical GTS. Three patients had atypical tics and two patients reported waxing and waning of tic intensity over time. Four of the 5 patients showed clinical signs typical of fragile X syndrome, in particular dysmorphic features, learning difficulties and speech and language problems that required special treatment. All patients had co-morbidities common to both GTS and fragile X syndrome. We suggest considering fragile X syndrome in GTS complicated by co-morbidity with late onset of atypical tics, in particular when learning disability and dysmorphic features are present. (c) 2008 Movement Disorder Society

  10. Recomendações em auditoria operacional: uma prospecção de fragilidades, com base na inteligência competitiva RECOMMENDATIONS IN OPERATIONAL AUDITORSHIP: A PROSPECTION OF FRAGILITIES, ON THE BASIS OF COMPETITIVE INTELLIGENCE

    Directory of Open Access Journals (Sweden)

    Alan José de Moura Silva

    2010-08-01

    Full Text Available A sociedade brasileira vem se apercebendo da importância de exigir a utilização responsável dos recursos públicos, postos à disposição de seus representantes eleitos, revertendo-os em benefícios que atendam às necessidades dos cidadãos. Os órgãos de controle externo começam a se preocupar com o atingimento de metas e melhoria de padrões de desempenho das políticas públicas e passam a realizar auditorias de natureza operacional, que buscam avaliar a gestão do ponto de vista da economicidade, eficiência e eficácia, bem como da efetividade das despesas governamentais. Esta pesquisa verificou se era possível identificar fragilidades que comprometessem a eficácia das recomendações emitidas pelas auditorias operacionais realizadas pelo Tribunal de Contas do Estado de PE, a partir do conceito de inteligência competitiva e com vistas a subsidiar uma atuação preventiva e/ou contingencial. Utilizou-se do método dedutivo com pesquisa bibliográfica e documental, com a análise de todas as recomendações das auditorias operacionais realizadas e julgadas pelo TCE/PE de 2003-2007. Concluiu-se, com base na inteligência competitiva, que há fragilidades que podem comprometer a eficácia da implementação das recomendações emitidas pela auditoria operacional, que uma vez identificadas permitirão o estabelecimento de uma estratégia preventiva e uma estratégia contingencial, para fazer face aos fatores de risco, caso se concretizem.The Brazilian company has been aware of the importance of requiring the responsible use of public resources, made available to their elected representatives, reverting them into benefits that meet the needs of citizens. The bodies of external control are beginning to worry about achievement of targets and improvement of the performance of public policy and start to perform audits of an operational nature, seeking to evaluatethe management in terms of economy, efficiency and effectiveness, and the

  11. Fragile Families in the American Welfare State

    Science.gov (United States)

    Garfinkel, Irwin; Zilanawala, Afshin

    2016-01-01

    The proportion of children born out of wedlock is now over 40 percent. At birth, about half of these parents are co-habiting. This paper examines data from the Fragile Families and Child Wellbeing study (N = 4,271) to describe for the first time the role of welfare state benefits in the economic lives of married, cohabiting, and single parent families with young children. Surprisingly, total welfare state benefits received by the three family types are relatively similar. Nearly half of the full incomes of fragile families come from welfare state transfers. For single parent families the proportion is slightly more than two thirds. Though aggregate welfare state transfers are approximately equal across family type and thus change very little as marital status changes, these transfers and the taxes required to finance them cushion family status changes and substantially narrow the gap in full income between married and fragile families. PMID:27114616

  12. Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void.

    Science.gov (United States)

    Solomon, Lawrence R

    2008-02-01

    Pain due to vaso-occlusive crisis is the major cause of hospital use in sickle cell disease. Although available guidelines provide recommendations for opioid administration in this setting, only 4 (21%) of 19 medical textbooks present treatment regimens that are consistent with them. Moreover, only 7 texts (37%) note that addiction is infrequent in this population, while 11 (92%) of 12 texts provide such reassurance for cancer-related pain (P occlusive crises is completely defined only in 2 textbooks. Thus, most medical texts provide neither adequate information for the treatment or prevention of pain due to vaso-occlusive crisis in sickle cell disease nor reassurance of the unlikelihood of addiction in this population. In contrast, treatment recommendations for less common hematologic disorders are consistent with current standards in 53% to 84% of appropriate texts (P training on pain control in sickle cell disease are needed to parallel studies of environmental and genetic factors contributing to the known clinical heterogeneity of this disorder.

  13. How Do Health Care Providers Diagnose Fragile X Syndrome?

    Science.gov (United States)

    ... Email Print How do health care providers diagnose Fragile X syndrome? Health care providers often use a blood sample ... information helps families and providers to prepare for Fragile X syndrome and to intervene as early as possible. Possible ...

  14. Fragile X-Associated Tremor and Ataxia Syndrome (FXTAS)

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Fragile X Syndrome Condition Information NICHD Research Information Find a Study ... have the intellectual or developmental disabilities common in fragile X syndrome. NICHD-supported researchers were the first to identify ...

  15. A Trial of Metformin in Individuals With Fragile X Syndrome

    Science.gov (United States)

    2018-04-10

    Fragile X Syndrome; Fragile X Mental Retardation Syndrome; Mental Retardation, X Linked; Genetic Diseases, X-Linked; Trinucleotide Repeat Expansion; Fra(X) Syndrome; Intellectual Disability; FXS; Neurobehavioral Manifestations; Sex Chromosome Disorders

  16. Treatment of Fragile X Syndrome with a Neuroactive Steroid

    Science.gov (United States)

    2015-08-01

    anxiety, inattention, and impulsivity problems in children with fragile X syndrome. We planned to enroll 60 children, ages 6-17 years, with fragile X...enhances GABAA activity. We hypothesized that ganaxolone will significantly improve behavioral problems such as anxiety, inattention, and impulsivity ...problems in children with fragile X syndrome. We planned to enroll 60 children, ages 6–17 years, with fragile X syndrome over a 4-year period and they

  17. Desenvolvimento de cultivares de antúrio IAC como plantas de vaso no norte do Paraná Performance of Anthurium's cultivars as an ornamental vessel plant in northern Paraná

    Directory of Open Access Journals (Sweden)

    Lúcia Sadayo Assari Takahashi

    2009-09-01

    Full Text Available O gênero Anthurium Schott. (Araceae compreende mais de 600 espécies. Todas são ornamentais e se destacam pela beleza da folhagem. A espécie Anthurium andraeanum Lind. tem a preferência dos consumidores como planta ornamental devido ao tamanho e colorido de suas espatas. O objetivo do trabalho foi avaliar o desenvolvimento, em vaso, de cinco cultivares de A. andraeanum em Londrina, Norte do Paraná. As cultivares 'Parakanã', 'Terena', 'Apalai', 'Ianomami' e 'Rubi' foram produzidas por meio de micropropagação e selecionadas pelo Instituto Agronômico (IAC. O trabalho foi desenvolvido em estufa tipo arco com 80% de sombreamento obtido por tela de polipropileno de cor preta. Foi plantada uma muda por vaso plástico com volume de quatro litros usando como substrato fibra de coco tipo 80. A avaliação do desempenho comparativo foi iniciada, a partir do transplante das mudas. O comprimento do pecíolo, o número de folhas, a largura e o comprimento das espatas e o comprimento da haste floral foram avaliados mensalmente. Houve aumento na altura e no número de folhas das plantas nos três anos de cultivo. As cultivares 'Parakanã', 'Rubi', 'Terena' e 'Apalai' floresceram e tiveram maior desenvolvimento vegetativo sendo recomendadas para o cultivo em vasos no Norte do Paraná.The genus Anthurium Schott. (Araceae comprehends more than 600 species. All species are ornamental and are distinguished by the beauty of their foliage. The Anthurium andraeanum Lind. has the preference of the public as an ornamental plant because of the color and size of its spathes. The objective of this work was to evaluate of five cultivars of A. andraeanum, as vessel plant, in the northern region of Paraná State. The cultivars 'Parakanã', 'Terena', 'Apalai', 'Ianomami' and 'Rubi' produced by micropropagation by Instituto Agronômico (IAC were analyzed. The study was conducted at greenhouse with 80% of shade given by a black colored polypropylene screen. One seedling

  18. Novel CFTR missense mutations in Brazilian patients with congenital absence of vas deferens: counseling issues Mutações novas no gene CFTR de pacientes brasileiros portadores de agenesia dos vasos deferentes: dificuldades no aconselhamento

    Directory of Open Access Journals (Sweden)

    Patricia de Campos Pieri

    2007-01-01

    Full Text Available PURPOSE: Screening for mutations in the entire Cystic Fibrosis gene (CFTR of Brazilian infertile men with congenital absence of vas deferens, in order to prevent transmission of CFTR mutations to offspring with the use of assisted reproductive technologies. METHOD: Specific polymerase chain reaction (PCR primers were designed to each of the 27 exons and splicing sites of interest followed by single strand conformational polymorphism and Heteroduplex Analysis (SSCP-HA in precast 12.5% polyacrylamide gels at 7ºC and 20ºC. Fragments with abnormal SSCP migration pattern were sequenced. RESULTS: Two novel missense mutations (S753R and G149W were found in three patients (two brothers together with the IVS8-5T allele in hetrozygosis. CONCLUSION: The available screenings for CF mutations do not include the atypical mutations associated to absence of vas deferens and thus, when these tests fail to find mutations, there is still a genetic risk of affected children with the help of assisted reproduction. We recommend the screening of the whole CFTR gene for these infertile couples, as part of the work-up before assisted reproduction.OBJETIVO: Pesquisar mutações em toda a extensão do gene que causa a Fibrose Cística (CFTR de homens brasileiros inférteis por agenesia congênita dos vasos deferentes, com a finalidade de prevenir a transmissão de mutações em CFTR à prole com o uso das tecnologias de reprodução assistida. MÉTODOS: Foram desenhados oligonucleotídeos específicos para realização de reação de polimerização em cadeia (PCR para cada um dos 27 exons e sítios de processamento de interesse no gene CFTR. O PCR foi seguido pela técnica de SSCP-HA (polimorfismos de conformação no DNA de fita simples e na formação de heteroduplexes em géis pré-fabricados de poliacrilamida a 12,5% em duas temperaturas, 7ºC e 20ºC. Os fragmentos com padrão alterado na migração do SSCP foram submetidos a seqüenciamento automatizado

  19. Displasia fibro-muscular de vasos cerebrais: relato de sete casos

    Directory of Open Access Journals (Sweden)

    Ápio Cláudio M. Antunes

    1974-06-01

    Full Text Available Os autores apresentam sete casos de displasia fibro-muscular (DFM, sendo seis deles na artéria carótida e um na artéria vertebral, estudados pelas correspondentes angiografias. São relatadas as histórias clínicas que, em cinco pacientes, são de acidente vascular cerebral isquêmico e, em dois, de hemorragia sub-aracnóide por rotura de aneurisma. Dois pacientes foram à cirurgia: um deles sofreu ligadura de carótida primitiva e outro sofreu endarterectomia para retirada de placa de ateroma da artéria carótida contralateral a aquela com DFM. Finalmente é feita breve discussão sobre a referida patologia, quadro clínico, dignóstico e tratamento, sendo ressaltada a importância do estudo angiográfico carotídeo e vertebral quando encontrada DFM em outro território vascular.

  20. Conversational Characteristics of Children with Fragile X Syndrome: Tangential Language.

    Science.gov (United States)

    Sudhalter, Vicki; Belser, Richard C.

    2001-01-01

    The production of tangential language during conversations was studied with people with fragile X syndrome (n=10), autism (n=10), and mental retardation not caused by fragile X (n=10). Tangential language was found to be more prevalent among those with fragile X compared to the control groups, especially within unsolicited comments. (Contains…

  1. Determinants of Banking System Fragility : A Regional Perspective

    NARCIS (Netherlands)

    Degryse, H.A.; Elahi, M.A.; Penas, M.F.

    2012-01-01

    Abstract: Banking systems are fragile not only within one country but also within and across regions. We study the role of regional banking system characteristics for regional banking system fragility. We find that regional banking system fragility reduces when banks in the region jointly hold more

  2. What Are the Symptoms of Fragile X Syndrome?

    Science.gov (United States)

    ... Pinterest Email Print What are the symptoms of Fragile X syndrome? People with Fragile X do not all have the same signs and ... in males. Intelligence and learning. Many people with Fragile X have problems with intellectual functioning. These problems can ...

  3. Multipartnered Fertility and Depression among Fragile Families

    Science.gov (United States)

    Turney, Kristin; Carlson, Marcia J.

    2011-01-01

    We used data from the Fragile Families and Child Wellbeing Study to examine the association between multipartnered fertility (MPF)--when parents have children with more than one partner--and depression. Random-effects models suggested that MPF is associated with a greater likelihood of depression, net of family structure and other covariates.…

  4. Brief Report: Acamprosate in Fragile X Syndrome

    Science.gov (United States)

    Erickson, Craig A.; Mullett, Jennifer E.; McDougle, Christopher J.

    2010-01-01

    Glutamatergic dysfunction is implicated in the pathophysiology of fragile X syndrome (FXS). We report on the first trial of acamprosate, a drug with putative mGluR5 antagonism, in three adults with FXS and autism. Medical records describing open-label treatment with acamprosate in 3 patients with FXS and a comorbid diagnosis of autistic disorder…

  5. The Greeting Behavior of Fragile X Males.

    Science.gov (United States)

    Wolff, Peter H.; And Others

    1989-01-01

    The study found that mentally retarded males (N=18) above 12 years of age with a fragile site on the X chromosome exhibited a highly idiosyncratic stereotypic form of gaze avoidance during greeting ceremonies. Results suggest this aberrant greeting behavior may be uniquely associated with this syndrome of mental retardation. (Author/DB)

  6. Language in Boys with Fragile X Syndrome

    Science.gov (United States)

    Levy, Yonata; Gottesman, Riki; Borochowitz, Zvi; Frydman, Moshe; Sagi, Michal

    2006-01-01

    The current paper reports of language production in 15 Hebrew-speaking boys, aged 9;0-13;0, with fully methylated, non-mosaic fragile X syndrome and no concomitant diagnosis of autism. Contrary to expectations, seven children were non-verbal. Language production in the verbal children was studied in free conversations and in context-bound speech.…

  7. Attention and Language in Fragile X

    Science.gov (United States)

    Cornish, Kim; Sudhalter, Vicki; Turk, Jeremy

    2004-01-01

    Fragile X syndrome (FXS) is a well-recognized cause of mental retardation and developmental delay in males. Alongside the well-documented clinical characteristics of the condition, recent advances in technology and methodology have begun to define FXS at a number of different levels: genetic, brain structure and function, cognition, and behavior.…

  8. Emotion Potentiated Startle in Fragile X Syndrome

    Science.gov (United States)

    Ballinger, Elizabeth C.; Cordeiro, Lisa; Chavez, Alyssa D.; Hagerman, Randi J.; Hessl, David

    2014-01-01

    Social avoidance and anxiety are prevalent in fragile X syndrome (FXS) and are potentially mediated by the amygdala, a brain region critical for social behavior. Unfortunately, functional brain resonance imaging investigation of the amygdala in FXS is limited by the difficulties experienced by intellectually impaired and anxious participants. We…

  9. Fragile X Syndrome: An Aging Perspective

    Science.gov (United States)

    Schneider, Andrea; Ligsay, Andrew; Hagerman, Randi J.

    2013-01-01

    Cognitive and behavioral correlates of molecular variations related to the FMR1 gene have been studied rather extensively, but research about the long-term outcome in individuals with fragile X spectrum disorders remains sparse. In this review, we present an overview of aging research and recent findings in regard to cellular and clinical…

  10. Modeling Family Adaptation to Fragile X Syndrome

    Science.gov (United States)

    Raspa, Melissa; Bailey, Donald, Jr.; Bann, Carla; Bishop, Ellen

    2014-01-01

    Using data from a survey of 1,099 families who have a child with Fragile X syndrome, we examined adaptation across 7 dimensions of family life: parenting knowledge, social support, social life, financial impact, well-being, quality of life, and overall impact. Results illustrate that although families report a high quality of life, they struggle…

  11. Fragile X Syndrome: Genetic Predisposition to Psychopathology.

    Science.gov (United States)

    Bregman, Joel D.; And Others

    1988-01-01

    Psychiatric evaluation of 14 males (ages 3-27 years) with the fragile X syndrome found pervasive hyperactivity, impulsivity, and attentional deficits, and a significant degree of anxiety. However, diagnostic criteria for persistent pervasive developmental disorder and autism were not met. (Author/DB)

  12. Comparative Erythrocytes Osmotic Fragility Test and some ...

    African Journals Online (AJOL)

    Erythrocytes osmotic fragility and haematological parameters of subjects with HbAS (sickle cell trait) and HbSS (sickle cell anaemia) were determined and compared with subjects with HbAA (normal adult haemoglobin), which acted as control. They were divided into three groups of 40 subjects for HbAA, 35 subjects for ...

  13. Employment Impact and Financial Burden for Families of Children with Fragile X Syndrome: Findings from the National Fragile X Survey

    Science.gov (United States)

    Ouyang, L.; Grosse, S.; Raspa, M.; Bailey, D.

    2010-01-01

    Background: The employment impact and financial burden experienced by families of children with fragile X syndrome (FXS) has not been quantified in the USA. Method: Using a national fragile X family survey, we analysed data on 1019 families with at least one child who had a full FXS mutation. Out-of-pocket expenditures related to fragile X were…

  14. Fragility and hazard aspects of the Chinshan seismic PRA

    International Nuclear Information System (INIS)

    Hardy, G.S.; Tong, W.H.; Griffin, M.J.; Han, L.C.

    1989-01-01

    This paper provides an overview of the methodology and the results of the fragility and hazard portions of the seismic probabilistic risk assessment (PRA) and the containment overpressure fragilities at the Chinshan Nuclear Power Plant in Taiwan. The Chinshan seismic PRA involves the generation and combination of the seismic hazard at the site, the fragilities of equipment and structures and the dominant accident sequences leading to core damage. The seismic fragilities and the hazard study and the containment overpressure fragilities have been completed and are presented in this paper

  15. Fragility of epidermis and its consequence in dermatology.

    Science.gov (United States)

    Stalder, J F; Tennstedt, D; Deleuran, M; Fabbrocini, G; de Lucas, R; Haftek, M; Taieb, C; Coustou, D; Mandeau, A; Fabre, B; Hernandez-Pigeon, H; Aries, M F; Galliano, M F; Duplan, H; Castex-Rizzi, N; Bessou-Touya, S; Mengeaud, V; Rouvrais, C; Schmitt, A M; Bottino, R; Cottin, K; Saint Aroman, M

    2014-06-01

    The skin is the largest organ of the body, providing a protective barrier against bacteria, chemicals and physical insults while maintaining homeostasis in the internal environment. Such a barrier function the skin ensures protection against excessive water loss. The skin's immune defence consists of several facets, including immediate, non-specific mechanisms (innate immunity) and delayed, stimulus-specific responses (adaptive immunity), which contribute to fending off a wide range of potentially invasive microorganisms. This article is an overview of all known data about 'fragile skin'. Fragile skin is defined as skin with lower resistance to aggressions. Fragile skin can be classified into four categories up to its origin: physiological fragile skin (age, location), pathological fragile skin (acute and chronic), circumstantial fragile skin (due to environmental extrinsic factors or intrinsic factors such as stress) and iatrogenic fragile skin. This article includes the epidemiologic data, pathologic description of fragile skin with pathophysiological bases (mechanical and immunological role of skin barrier) and clinical description of fragile skin in atopic dermatitis, in acne, in rosacea, in psoriasis, in contact dermatitis and other dermatologic pathologies. This article includes also clinical cases and differential diagnosis of fragile skin (reactive skin) in face in adult population. In conclusion, fragile skin is very frequent worldwide and its prevalence varies between 25% and 52% in Caucasian, African and Asian population. © 2014 European Academy of Dermatology and Venereology.

  16. Acúmulo de micronutrientes em tango em função do número de hastes cultivadas

    OpenAIRE

    Muniz, Moisés A; Yumbla-Orbes, Maria; Barbosa, José Geraldo; Oliveira, Luma G

    2013-01-01

    O cultivo de tango no país é recente, sendo importante o conhecimento sobre suas exigências nutricionais para obtenção de melhor manejo da cultura. O objetivo deste trabalho foi avaliar o acúmulo de micronutrientes em plantas de tango submetidas a diferentes sistemas de condução. O experimento foi desenvolvido em delineamento de blocos casualizados, disposto em esquema fatorial (3x7), com três repetições. Cada unidade experimental foi constituída por um vaso contendo três plantas. Os tratamen...

  17. Fragile X-associated tremor/ataxia syndrome: another phenotype of the fragile X gene.

    Science.gov (United States)

    Hessl, David; Grigsby, Jim

    2016-08-01

    Neuropsychologists have an important role in evaluating patients with fragile X-associated disorders, but most practitioners are unaware of the recently identified neurodegenerative movement disorder known as fragile X-associated tremor ataxia syndrome (FXTAS). The objective of this editorial is to orient the reader to FXTAS and highlight the importance of clinical neuropsychology in describing the fragile X premutation phenotype and the role practitioners may have in assessing and monitoring patients with or at risk for neurodegeneration. We issued a call for papers for the special issue, highlighting the primary objective of familiarizing clinical neuropsychologists with FXTAS, and with the neuropsychological phenotype of both male and female asymptomatic carriers. Eight papers are included, including an overview of the fragile X-associated disorders (Grigsby), a review of the neuroradiological and neurological aspects of FXTAS and how the disorder compares to other movement disorders (O'Keefe et al.), a perspective on the prominence of white matter disease and dementia in FXTAS (Filley), and a review of mouse models of FXTAS (Foote). There are four research papers, including one on self-reported memory problems in FXTAS (Birch et al.), and three papers focused on the neuropsychiatric aspects of the fragile X premutation, a review (Bourgeois), an examination of autism-related traits (Schneider), and a research paper on executive functioning and psychopathology (Grigsby). The issue highlights the importance of awareness of fragile X-associated disorders for neuropsychologists, an awareness that must reach beyond neurodevelopmental aspects related to fragile X syndrome into the realm of neurodegenerative disease and aging.

  18. Breastfeeding of a medically fragile foster child.

    Science.gov (United States)

    Gribble, Karleen D

    2005-02-01

    A case is presented in which a medically fragile baby was breastfed by her foster mother. As a result, the child's physical and emotional health were improved. The mechanisms whereby human milk improves health are well known. The act of breastfeeding may also have an analgesic and relaxant effect as a result of hormonal influences and skin-to-skin contact. Many foster babies may benefit from human milk or breastfeeding. However, the risk of disease transmission must be minimized. Provision of human milk to all medically fragile foster babies is desirable. Breastfeeding by the foster mother may be applicable in cases in which the child is likely to be in long-term care, the child has been previously breastfed, or the child's mother expresses a desire that the infant be breastfed. However, social barriers must be overcome before breastfeeding of foster babies can become more common.

  19. Seismic fragility evaluation of unreinforced masonry walls

    International Nuclear Information System (INIS)

    Park, Y.J.; Hofmayer, C.H.; Reich, M.; Lee, S.K.

    1991-01-01

    A practical analysis scheme to evaluate the seismic fragility of unreinforced masonry walls which are used at various places in older reactor facilities is presented. Among the several failure modes for such walls, the out-of-plane bending failure is considered to be a major risk contributor in seismic PRA studies. In order to evaluate this failure mode, the use of an equivalent linear approximation method is examined based on comparisons with available test data and nonlinear time history analyses. (author)

  20. The highly fragile glass former Decalin

    International Nuclear Information System (INIS)

    Eibl, Stefan

    2009-01-01

    Systems exhibiting the glass transition can be classified by fragility. In this work we studied structural and dynamical aspects of highly fragile C 10 H 18 Decalin. Trans Decalin is locked into a pseudo-flat centrosymmetric conformation, while cis Decalin interchanges dynamically between chiral, pseudo-spherical ground states. On investigation of the phase behaviour trans Decalin was found to crystallise rapidly and cleanly; its crystal structure could be determined. From the crystal structure the dynamics of crystalline trans Decalin could be calculated using ab-initio lattice energy calculations and compared to measurements. Using neutron diffraction and molecular dynamics simulations the amorphous structure of Decalin was investigated. The difference in structure to the common molecular liquid Cumene is significant. The features of the amorphous structure of sphere-like cis Decalin show strong resemblance to the ones of Argon and metallic glasses. The dynamics of Decalin were investigated in the slightly supercooled liquid range. Using neutron scattering and optical spectroscopy, data was collected for a wide spectral range and several temperatures. The data suggests high fragility for the generic Decalin mixture, which is in agreement with the reported results. By contrast to previous estimations, an extrapolation of our data indicates cis Decalin to be only slightly less fragile than the generic mixture. Finally a lower limit to the four point susceptibility function χ 4 could be calculated and the number of correlated molecules determined. The evolution of this value as a function of T g /T and relaxation time are in agreement with literature. (author) [fr

  1. Modeling Fragile X Syndrome in Drosophila

    Science.gov (United States)

    Drozd, Małgorzata; Bardoni, Barbara; Capovilla, Maria

    2018-01-01

    Intellectual disability (ID) and autism are hallmarks of Fragile X Syndrome (FXS), a hereditary neurodevelopmental disorder. The gene responsible for FXS is Fragile X Mental Retardation gene 1 (FMR1) encoding the Fragile X Mental Retardation Protein (FMRP), an RNA-binding protein involved in RNA metabolism and modulating the expression level of many targets. Most cases of FXS are caused by silencing of FMR1 due to CGG expansions in the 5′-UTR of the gene. Humans also carry the FXR1 and FXR2 paralogs of FMR1 while flies have only one FMR1 gene, here called dFMR1, sharing the same level of sequence homology with all three human genes, but functionally most similar to FMR1. This enables a much easier approach for FMR1 genetic studies. Drosophila has been widely used to investigate FMR1 functions at genetic, cellular, and molecular levels since dFMR1 mutants have many phenotypes in common with the wide spectrum of FMR1 functions that underlay the disease. In this review, we present very recent Drosophila studies investigating FMRP functions at genetic, cellular, molecular, and electrophysiological levels in addition to research on pharmacological treatments in the fly model. These studies have the potential to aid the discovery of pharmacological therapies for FXS.

  2. Dynamic fragility concepts for equipment design and qualification

    International Nuclear Information System (INIS)

    Kana, D.D.; Pomerening, D.J.

    1985-01-01

    Fragility concepts are explored for use in design and qualification of nuclear plant equipment, and for relating equipment ultimate capability to that of the overall plant. In the most general sense, the fragility level of a device may depend on several different types of environmental stress or challenge factors, (i.e., heat, nuclear radiation, vibration, etc.) that influence its operation. However, emphasis is concentrated on the dynamic, and particularly seismic fragility levels of equipment. A general definition of dynamic fragility and various methods for its measurement are described. The state of published data on nuclear equipment fragility is discussed, and limitations on its use are noted. From there, the concept of a standardized seismic fragility data base and its potential uses are considered. Various gaps in the methodology are identified, and recommendations for further research are suggested

  3. Dynamic fragility concepts for equipment design and qualification

    International Nuclear Information System (INIS)

    Kana, D.D.; Pomerening, D.J.

    1986-01-01

    Fragility concepts are explored for use in the design and qualification of nuclear plant equipment and for relating the ultimate capability of equipment to that of the overall plant. In the most general sense, the fragility level of a device may depend on several different types of environmental stress or challenge factors (i.e., heat, nuclear radiation, vibration, etc.) that influence its operation. However, emphasis is concentrated on the dynamic and particularly the seismic fragility levels of equipment. A general definition of dynamic fragility and various methods for its measurement are described. The state of published data on nuclear equipment fragility is discussed, and limitations on its use are noted. From there, the concept of a standardized seismic fragility data base and its potential uses are considered. Various gaps in the methodology are identified, and recommendations for further research are suggested. (orig.)

  4. Efeito da aplicação de calcário em estacas de Populus deltoides Bartr. ex Marsh cultivadas em vaso

    Directory of Open Access Journals (Sweden)

    Jean Gabriel Medeiros da Silva

    2002-01-01

    Full Text Available This work intended to study the behavior of Populus deltoides regarding its development when submitted to different doses of lime. The research was carried out in the Silviculture Tecnology Center, at Universidade Federal de Santa Maria, Santa Maria - RS. The experiment was constituted by forty eight (48 experimental units completely randomized, with six (06 treatments and eight repetitions (08. Each experimental unit was constituted by a vase of three liters containing only one shoot cutting of Populus deltoides. The treatments were constituted by five different doses (T2: 7 tons of lime/ha; T3: 14 tons of lime/ha; T4: 21 tons of lime/ha; T5: 29 tons of lime/ha; T6: 38 tons of lime/ha plus the testimony, determined with base in the soil analysis used in the experiment. The plants were evaluated, after 120 days of the plantation date, considering its growth in height, its diameter of the neck and its weigh dry of the aerial part. The best average were reached by the treatments without lime and with seven tons of lime for ha in all the parameters observed. The other treatments presented inferior averages, which do not possess significant difference to each other at the level of 5%, for all the observed parameters.

  5. Nuclear Power Plant Mechanical Component Flooding Fragility Experiments Status

    Energy Technology Data Exchange (ETDEWEB)

    Pope, C. L. [Idaho State Univ., Pocatello, ID (United States); Savage, B. [Idaho State Univ., Pocatello, ID (United States); Johnson, B. [Idaho State Univ., Pocatello, ID (United States); Muchmore, C. [Idaho State Univ., Pocatello, ID (United States); Nichols, L. [Idaho State Univ., Pocatello, ID (United States); Roberts, G. [Idaho State Univ., Pocatello, ID (United States); Ryan, E. [Idaho State Univ., Pocatello, ID (United States); Suresh, S. [Idaho State Univ., Pocatello, ID (United States); Tahhan, A. [Idaho State Univ., Pocatello, ID (United States); Tuladhar, R. [Idaho State Univ., Pocatello, ID (United States); Wells, A. [Idaho State Univ., Pocatello, ID (United States); Smith, C. [Idaho National Lab. (INL), Idaho Falls, ID (United States)

    2017-07-24

    This report describes progress on Nuclear Power Plant mechanical component flooding fragility experiments and supporting research. The progress includes execution of full scale fragility experiments using hollow-core doors, design of improvements to the Portal Evaluation Tank, equipment procurement and initial installation of PET improvements, designation of experiments exploiting the improved PET capabilities, fragility mathematical model development, Smoothed Particle Hydrodynamic simulations, wave impact simulation device research, and pipe rupture mechanics research.

  6. Enhanced chromosomal fragility in neuroblastoma: correlation with poor prognosis.

    Science.gov (United States)

    Vernole, P; Tedeschi, B; Melino, G; Pianca, C; Nicoletti, B

    1992-01-01

    In a previous study we demonstrated spontaneous fragility and hypersensitivity to fragile site induction by aphidicolin in lymphocytes from some neuroblastoma patients and their parents. Here we report data based on a total of 40 patients and 37 families. Possible correlations between higher sensitivity to aphidicolin and a variety of personal and clinical characteristics were verified. Patients with a poor prognosis generally proved to be more susceptible to fragile site induction.

  7. Fragility of epidermis in newborns, children and adolescents.

    Science.gov (United States)

    Blume-Peytavi, U; Tan, J; Tennstedt, D; Boralevi, F; Fabbrocini, G; Torrelo, A; Soares-Oliveira, R; Haftek, M; Rossi, A B; Thouvenin, M D; Mangold, J; Galliano, M F; Hernandez-Pigeon, H; Aries, M F; Rouvrais, C; Bessou-Touya, S; Duplan, H; Castex-Rizzi, N; Mengeaud, V; Ferret, P J; Clouet, E; Saint Aroman, M; Carrasco, C; Coutanceau, C; Guiraud, B; Boyal, S; Herman, A; Delga, H; Biniek, K; Dauskardt, R

    2016-05-01

    Within their first days of life, newborns' skin undergoes various adaptation processes needed to accommodate the transition from the wet uterine environment to the dry atmosphere. The skin of newborns and infants is considered as a physiological fragile skin, a skin with lower resistance to aggressions. Fragile skin is divided into four categories up to its origin: physiological fragile skin (age, location), pathological fragile skin (acute and chronic), circumstantial fragile skin (due to environmental extrinsic factors or intrinsic factors such as stress) and iatrogenic fragile skin. Extensive research of the past 10 years have proven evidence that at birth albeit showing a nearly perfect appearance, newborn skin is structurally and functionally immature compared to adult skin undergoing a physiological maturation process after birth at least throughout the first year of life. This article is an overview of all known data about fragility of epidermis in 'fragile populations': newborns, children and adolescents. It includes the recent pathological, pathophysiological and clinical data about fragility of epidermis in various dermatological diseases, such as atopic dermatitis, acne, rosacea, contact dermatitis, irritative dermatitis and focus on UV protection. © 2016 European Academy of Dermatology and Venereology.

  8. microRNAs and Fragile X Syndrome.

    Science.gov (United States)

    Lin, Shi-Lung

    2015-01-01

    Fragile X syndrome (FXS) is one of the major causes for autism and mental retardation in humans. The etiology of FXS is linked to the expansion of the CGG trinucleotide repeats, r(CGG), suppressing the fragile X mental retardation 1 (FMR1) gene on the X chromosome, resulting in a loss of fragile X mental retardation protein (FMRP) expression, which is required for regulating normal neuronal connectivity and plasticity. Recent studies have further identified that microRNAs are involved in the mechanisms underlying FXS pathogenesis at three different developmental stages. During early embryogenesis before the blastocyst stage, an embryonic stem cell (ESC)-specific microRNA, miR-302, interferes with FMR1 mRNA translation to maintain the stem cell status and inhibit neural development. After blastocyst, the downregulation of miR-302 releases FMRP synthesis and subsequently leads to neuronal development; yet, in FXS, certain r(CGG)-derived microRNAs, such as miR-fmr1s, are expressed and accumulated and then induce DNA hypermethylation on the FMR1 gene promoter regions, resulting in transcriptional inactivation of the FMR1 gene and the loss of FMRP. In normal neuronal development, FMRP is an RNA-binding protein responsible for interacting with miR-125 and miR-132 to regulate the signaling of Group 1 metabotropic glutamate receptor (mGluR1) and N-methyl-D-aspartate receptor (NMDAR), respectively, and consequently affecting synaptic plasticity. As a result, the loss of FMRP impairs these signaling controls and eventually causes FXS-associated disorders, such as autism and mental retardation. Based on these current findings, this chapter will summarize the etiological causes of FXS and further provides significant insights into the molecular mechanisms underlying microRNA-mediated FXS pathogenesis and the related therapy development.

  9. Germline mosaicism at the fragile X locus

    Energy Technology Data Exchange (ETDEWEB)

    Papp, A.C.; Snyder, P.J.; Sedra, M.S. [Ohio State Univ., Columbus, OH (United States)] [and others

    1994-09-01

    The fragile X full mutation, which is associated with the phenotypic expression of the disorder, is characterized by an expansion of CGG repeat and hypermethylation of the CpG island adjacent to the FMR1 gene. New mutations leading to amplification of the CGG repeat have not been reported. We have identified a fragile X syndrome pedigree where the disorder is associated with a molecular deletion. The deletion was present in the DNA of two affected sons but was absent in the mother`s somatic cell (lymphocyte) DNA. This was confirmed by dosage analysis of the Southern blot using StB12-3 and an additional probe against the dystrophin gene and by PCR analysis of DXS548 alleles. The results are consistent with the deletion arising as a postzygotic event in the mother, who therefore is germinally mosaic. The case reported here clearly demonstrates that FMR1 deletions, unlike the expansions, are not always inherited and the finding of heterozygosity or normal dosage from lymphocyte DNA in the mother of a deletion case does not necessarily rule out the possibility of having a second affected child. The deletion of FMR1 gene may be responsible for a small but significant number of fragile X cases. Therefore, it is imperative that those involved in genetic counseling recognize this diagnostic pitfall. Since it depends upon the size of the mutant clone in the mosaic mother, the exact recurrence risk in germline carriers is unknown. However, prenatal and carrier testing should be performed independently of the outcome of the mother. Furthermore, it is possible that the deletion may not be restricted to the germline, and therefore the mother may actually be a somatic mosaic.

  10. Fragile X syndrome and fragile X-associated disorders [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Akash Rajaratnam

    2017-12-01

    Full Text Available Fragile X syndrome (FXS is caused by a full mutation on the FMR1 gene and a subsequent lack of FMRP, the protein product of FMR1. FMRP plays a key role in regulating the translation of many proteins involved in maintaining neuronal synaptic connections; its deficiency may result in a range of intellectual disabilities, social deficits, psychiatric problems, and dysmorphic physical features. A range of clinical involvement is also associated with the FMR1 premutation, including fragile X-associated tremor ataxia syndrome, fragile X-associated primary ovarian insufficiency, psychiatric problems, hypertension, migraines, and autoimmune problems. Over the past few years, there have been a number of advances in our knowledge of FXS and fragile X-associated disorders, and each of these advances offers significant clinical implications. Among these developments are a better understanding of the clinical impact of the phenomenon known as mosaicism, the revelation that various types of mutations can cause FXS, and improvements in treatment for FXS.

  11. Self-Injurious Behavior and Fragile X Syndrome: Findings from the National Fragile X Survey

    Science.gov (United States)

    Symons, Frank J.; Byiers, Breanne J.; Raspa, Melissa; Bishop, Ellen; Bailey, Donald B., Jr.

    2010-01-01

    We used National Fragile X Survey data in order to examine reported self-injurious behavior (SIB) to (a) generate lifetime and point prevalence estimates, (b) document detailed features of SIB (frequency, types, location, severity) in relation to gender, and (c) compare comorbid conditions between matched pairs (SIB vs. no SIB). Results indicate…

  12. Chromosome fragility at FRAXA in human cleavage stage embryos at risk for fragile X syndrome.

    Science.gov (United States)

    Verdyck, Pieter; Berckmoes, Veerle; De Vos, Anick; Verpoest, Willem; Liebaers, Inge; Bonduelle, Maryse; De Rycke, Martine

    2015-10-01

    Fragile X syndrome (FXS), the most common inherited intellectual disability syndrome, is caused by expansion and hypermethylation of the CGG repeat in the 5' UTR of the FMR1 gene. This expanded repeat, also known as the rare fragile site FRAXA, causes X chromosome fragility in cultured cells from patients but only when induced by perturbing pyrimidine synthesis. We performed preimplantation genetic diagnosis (PGD) on 595 blastomeres biopsied from 442 cleavage stage embryos at risk for FXS using short tandem repeat (STR) markers. In six blastomeres, from five embryos an incomplete haplotype was observed with loss of all alleles telomeric to the CGG repeat. In all five embryos, the incomplete haplotype corresponded to the haplotype carrying the CGG repeat expansion. Subsequent analysis of additional blastomeres from three embryos by array comparative genomic hybridization (aCGH) confirmed the presence of a terminal deletion with a breakpoint close to the CGG repeat in two blastomeres from one embryo. A blastomere from another embryo showed the complementary duplication. We conclude that a CGG repeat expansion at FRAXA causes X chromosome fragility in early human IVF embryos at risk for FXS. © 2015 Wiley Periodicals, Inc.

  13. Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome

    NARCIS (Netherlands)

    R.F. Berman (Robert); R.A.M. Buijsen (Ronald); K. Usdin (Karen); E. Pintado (Elizabeth); F. Kooy (Frank); D. Pretto (Dalyir); I.N. Pessah (Isaac); D.L. Nelson (David); Z. Zalewski (Zachary); N. Charlet-Bergeurand (Nicholas); R. Willemsen (Rob); R.K. Hukema (Renate)

    2014-01-01

    textabstractCarriers of the fragile X premutation (FPM) have CGG trinucleotide repeat expansions of between 55 and 200 in the 5′-UTR of FMR1, compared to a CGG repeat length of between 5 and 54 for the general population. Carriers were once thought to be without symptoms, but it is now recognized

  14. MUS81 promotes common fragile site expression

    DEFF Research Database (Denmark)

    Ying, Songmin; Minocherhomji, Sheroy; Chan, Kok Lung

    2013-01-01

    the faithful disjunction of sister chromatids in mitosis. However, the mechanisms by which CFSs express their fragility, and the cellular factors required to suppress CFS instability, remain largely undefined. Here, we report that the DNA structure-specific nuclease MUS81-EME1 localizes to CFS loci in early......, CFS cleavage by MUS81-EME1 promotes faithful sister chromatid disjunction. Our findings challenge the prevailing view that CFS breakage is a nonspecific process that is detrimental to cells, and indicate that CFS cleavage actually promotes genome stability....

  15. Folic acid for fragile X syndrome.

    Science.gov (United States)

    Rueda, José-Ramón; Ballesteros, Javier; Guillen, Virginia; Tejada, Maria-Isabel; Solà, Ivan

    2011-05-11

    It has been argued that individuals with fragile X syndrome could have low folate levels in their bodies and that supplementing their dietary intake might remediate the adverse developmental and behavioural effects of the condition. To review the efficacy and safety of folic acid in the treatment of people with fragile X syndrome. We searched four databases in November 2010: CENTRAL, PubMed, EMBASE and PsycINFO. Randomised controlled trials. Two review authors independently extracted data and assessed risk of bias using the Cochrane 'Risk of bias' tool. We included five trials, which were published between 1986 and 1992. Overall, they included 67 patients, all male, with ages ranging from one to 54 years. Intellectual disability in participants varied from borderline to severe and some studies included patients with an additional diagnosis of autism or autistic behaviour. Four of the studies were placebo-controlled cross-over trials and one study was a parallel design. The duration of follow-up ranged from two months to 12 months and the period on folic acid or placebo ranged from two to eight months. Doses of folic acid ranged from 10 mg to 250 mg per day, 10 mg per day being the most common. Most of the younger patients involved were also taking part in special education programmes (usually involving language and occupational therapy).We were not able to perform meta-analysis to combine results but none of the individual studies found evidence of clinical benefit with the use of folic acid medication in fragile X syndrome patients on any of the areas of interest, either psychological and learning capabilities or behaviour and social performance, as measured with standardised tools. Separate analysis of evidence for patients of different age groups, i.e. prepubertal children and postpubertal young people, found some statistically significant results, but did not show clear evidence of benefit for either group. Adverse effects of folic acid treatment were rare, not

  16. Seismic fragility evaluation of unreinforced masonry walls

    International Nuclear Information System (INIS)

    Park, Y.J.; Hofmayer, C.H.; Reich, M.; Lee, S.K.

    1991-01-01

    A practical analysis scheme to evaluate the seismic fragility of unreinforced masonry walls which are used to various places in older reactor facilities is presented. Among the several failure modes for such walls, the out-of-plane bending failure is considered to be a major risk contributor in seismic PRA studies. In order to evaluate this failure mode, the use of an equivalent linear approximation method is examined based on comparisons with available test data and nonlinear time history analyses. 6 refs., 4 figs., 3 tabs

  17. Estudio preoperatorio de vasos receptores en reconstrucción mamaria con colgajo DIEP Preoperative planning of receiver vessels in breast reconstruction with DIEP flap

    Directory of Open Access Journals (Sweden)

    C. Tejerina Botella

    2011-09-01

    Full Text Available El estudio preoperatorio vascular de cualquier colgajo microquirúrgico es esencial para el buen desarrollo del mismo. En el colgajo DIEP (colgajo de perforante de arteria epigástrica inferior profunda aplicado a la reconstrucción mamaria, se han establecido durante los últimos años diferentes técnicas para la planificación preoperatoria de la anatomía de los vasos perforantes de la arteria epigástrica inferior. Sin embargo, pensamos, que también es muy importante la planificación anatómica preoperatoria de los vasos receptores, que en la mayoría de los casos son la arteria y vena mamarias internas. Estos vasos sufren en ocasiones variaciones anatómicas, llegando incluso a no existir en algunos pacientes. El conocimiento exacto de la localización, permeabilidad y calibre de estos vasos receptores hará que la intervención sea mas rápida y sencilla.An accurate preoperative vascular evaluation of microsurgical flaps is essential for a good procedure. In DIEP (deep inferior epigastric perforator flap, in breast reconstruction, have been described different preoperative techniques to study the anatomy of the deep inferior epigastric perforators. We think that preoperative planning of the receiver vessels, (internal mammary artery and vein, in most of cases is very important too. These vessels have anatomic changes in some patients. To know the exact location, permeability, and calibre of these vessels will help us to have a more easy and quick operation.

  18. The kinetic fragility of natural silicate melts

    International Nuclear Information System (INIS)

    Giordano, Daniele; Dingwell, Donald B

    2003-01-01

    Newtonian viscosities of 19 multicomponent natural and synthetic silicate liquids, with variable contents of SiO 2 (41-79 wt%), Al 2 O 3 (10-19 wt%), TiO 2 (0-3 wt%), FeO tot (0-11 wt%); alkali oxides (5-17 wt%), alkaline-earth oxides (0-35 wt%), and minor oxides, obtained at ambient pressure using the high-temperature concentric cylinder, the low-temperature micropenetration, and the parallel plates techniques, have been analysed. For each silicate liquid, regression of the experimentally determined viscosities using the well known Vogel-Fulcher-Tammann (VFT) equation allowed the viscosity of all these silicates to be accurately described. The results of these fits, which provide the basis for the subsequent analysis here, permit qualitative and quantitative correlations to be made between the VFT adjustable parameters (A VFT , B VFT , and T 0 ). The values of B VFT and T 0 , calibrated via the VFT equation, are highly correlated. Kinetic fragility appears to be correlated with the number of non-bridging oxygens per tetrahedrally coordinated cation (NBO/T). This is taken to infer that melt polymerization controls melt fragility in liquid silicates. Thus NBO/T might form an useful ingredient of a structure-based model of non-Arrhenian viscosity in multicomponent silicate melts

  19. Translational endpoints in fragile X syndrome.

    Science.gov (United States)

    de Esch, Celine E F; Zeidler, Shimriet; Willemsen, Rob

    2014-10-01

    Fragile X syndrome (FXS) occurs in less than 10% of the intellectually disabled (ID) population. The cause of FXS is a CGG trinucleotide repeat longer than 200 CGG units within the first exon of the FMR1 gene, which leads to hypermethylation and consequently silencing of the FMR1 gene. The lack of FMR1's gene product, the fragile X mental retardation protein (FMRP) in neurons is the cause of the ID in patients with FXS. FMRP plays an important role in local protein synthesis at the synapse including modulation of synaptic plasticity. The advancing knowledge about the cellular function of FMRP has led to the identification of translational endpoints for future therapeutic intervention strategies. This review highlights the challenging routes to the identification of reliable outcome measures in preclinical studies using both cellular models and Fmr1 knockout mice. Finally, clinical studies carried out to correct intellectual and behavioral deficits in patients with FXS, using a variety of existing and new drugs, are discussed. Copyright © 2013 Elsevier Ltd. All rights reserved.

  20. Erythrocyte osmotic fragility of pigs administered ascorbic acid and ...

    African Journals Online (AJOL)

    Blood samples for erythrocyte osmotic fragility determination which was done using standard procedure, were taken early in the morning a day before transportation, immediately after and a week after transportation. Erythrocyte osmotic fragility decreased significantly (P < 0.05) at NaCl concentration of 0.85, 0.80 and 0.70% ...

  1. Study on the Progress of Ecological Fragility Assessment in China

    Science.gov (United States)

    Chen, Pei; Hou, Kang; Chang, Yue; Li, Xuxiang; Zhang, Yunwei

    2018-02-01

    The basic elements of human survival are based on the ecological environment. The development of social economic and the security of the ecological environment are closely linked and interact with each other. The fragility of the environment directly affects the stability of the regional ecosystem and the sustainable development of the ecological environment. As part of the division of the national ecological security, the assessment of ecological fragility has become a hot and difficult issue in environmental research, and researchers at home and abroad have systematically studied the causes and states of ecological fragility. The assessment of regional ecological fragility is a qualitative and quantitative analysis of the unbalanced distribution of ecological environment factors caused by human socio-economic activities or changes in ecosystems. At present, researches on ecological fragility has not formed a complete and unified index assessment system, and the unity of the assessment model has a direct impact on the accuracy of the index weights. Therefore, the discussion on selection of ecological fragility indexes and the improvement of ecological fragility assessment model is necessary, which is good for the improvement of ecological fragility assessment system in China.

  2. Dilemmas in counselling females with the fragile X syndrome

    NARCIS (Netherlands)

    B.B.A. de Vries (Bert); H.M. van den Boer-van den Berg; M.F. Niermeijer (Martinus); A. Tibben (Arend)

    1999-01-01

    textabstractThe dilemmas in counselling a mildly retarded female with the fragile X syndrome and her retarded partner are presented. The fragile X syndrome is an X linked mental retardation disorder that affects males and, often less severely, females. Affected females

  3. Promoting Leadership for Economic Policy in Fragile and Post ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Promoting Leadership for Economic Policy in Fragile and Post-Conflict States in Africa. This project provides three-year funding to the African Economic Research Consortium (AERC), to strengthen capacity for research and policy analysis in post-conflict and fragile states in Africa. No guarantees economic growth is ...

  4. Financial Inclusion and Financial Fragility: An Empirical Note

    OpenAIRE

    Ghosh, Saibal

    2008-01-01

    Using data on Indian state-owned banks for 1997-2007, the article tests the relationship between financial inclusion and financial fragility. The findings reveal that these variables are intertwined, with each tending to reinforce the other. More importantly, financial fragility is a non-linear determinant of both availability and use of banking services.

  5. Quantitative Prediction of Stone Fragility From Routine Dual Energy CT

    DEFF Research Database (Denmark)

    Ferrero, Andrea; Montoya, Juan C; Vaughan, Lisa E

    2016-01-01

    RATIONALE AND OBJECTIVES: Previous studies have demonstrated a qualitative relationship between stone fragility and internal stone morphology. The goal of this study was to quantify morphologic features from dual-energy computed tomography (CT) images and assess their relationship to stone fragil...

  6. Molecular characterization of X chromosome fragility in idiopathic ...

    African Journals Online (AJOL)

    Background: Fragile X syndrome (FXS) is the most common form of inherited mental retardation. Frequency of fragile X syndrome among male siblings and relatives of mentally retarded patients is relatively high. Cytogenetic diagnosis of FXS is unreliable since it is ineffective for the diagnosis of premutated males or ...

  7. The Detection and Analysis of Chromosome Fragile Sites

    DEFF Research Database (Denmark)

    Bjerregaard, Victoria A; Özer, Özgün; Hickson, Ian D

    2018-01-01

    A fragile site is a chromosomal locus that is prone to form a gap or constriction visible within a condensed metaphase chromosome, particularly following exposure of cells to DNA replication stress. Based on their frequency, fragile sites are classified as either common (CFSs; present in all...

  8. Attentional Set-Shifting in Fragile X Syndrome

    Science.gov (United States)

    Van der Molen, M. J. W.; Van der Molen, M. W.; Ridderinkhof, K. R.; Hamel, B. C. J.; Curfs, L. M. G.; Ramakers, G. J. A.

    2012-01-01

    The ability to flexibly adapt to the changing demands of the environment is often reported as a core deficit in fragile X syndrome (FXS). However, the cognitive processes that determine this attentional set-shifting deficit remain elusive. The present study investigated attentional set-shifting ability in fragile X syndrome males with the…

  9. Diagnostic, carrier and prenatal genetic testing for fragile X ...

    African Journals Online (AJOL)

    Background. Fragile X syndrome (FXS), the most common inherited cause of intellectual disability (ID) worldwide, is caused by the expansion of a CGG repeat in the fragile X mental retardation gene (FMR-1) gene. Objectives. To review, retrospectively, the genetic services for FXS and other FMR-1-related disorders ...

  10. Osmotic Fragility in Stored Non-Detergent Washed Human ...

    African Journals Online (AJOL)

    Osmotic fragility (OF) of non-detergent washed erythrocytes was evaluated in Nigerian human male erythrocytes stored for 0h, 12h, 24h and 48h. Storage of these human erythrocytes for up to 24h did not significantly alter their membrane characteristics. Mean corpuscular fragility (MCF) diminished and correlated negatively ...

  11. What Should You Know about Fragile X Syndrome (FXS)?

    Science.gov (United States)

    ... Fragile X Syndrome is Inherited Video: Causes of Fragile X Associated Disorders Related Concerns Data & Statistics Articles & Key Findings Pediatrics Supplement Free Materials My Story Links to Other Websites Our Work Language: English (US) Español (Spanish) File Formats Help: How ...

  12. Fragile X Syndrome in Males: Diagnostic, Behavioral, and Educational Implications.

    Science.gov (United States)

    Bellinger, Diane; And Others

    This paper reviews the research on fragile X syndrome, the second most common cause of mental retardation related to chromosomal anomaly. It notes that far more males than females are affected by the fragile X syndrome, which typically results in craniofacial changes, delays in growth and development, speech/language difficulties, and cognitive…

  13. Aberrant Behaviors of Young Boys with Fragile X Syndrome.

    Science.gov (United States)

    Lachiewicz, Ave M.; And Others

    1993-01-01

    Caregivers of 55 boys with fragile X syndrome (ages 3-12) and 57 matched controls completed five behavioral questionnaires. Principal components analysis yielded 5 behavioral clusters: abnormal language, tactile defensiveness, poor self-control, poor eye contact/shyness, and hand flapping. Boys with fragile X were four times more likely to have…

  14. Improved Erythrocyte Osmotic Fragility and Packed Cell Volume ...

    African Journals Online (AJOL)

    Improved Erythrocyte Osmotic Fragility and Packed Cell Volume following administration of Aloe barbadensis Juice Extract in Rats. ... Abstract. Aloe barbadensis is a popular house plant that has a long history of a multipurpose folk remedy. ... Keywords: osmotic fragility, packed cell volume, haemoglobin, Aloe vera ...

  15. The Social Consequences of Raising Medically Fragile and ...

    African Journals Online (AJOL)

    ... achieved, families were likely to limit social activities so that the child who was medically fragile and or developmentally challenged could be cared for at home, a secured environment in the view of parents. Keywords: Social Situation; Medically Fragile Child; Developmentally Challenged; Chronically Ill Child, Disability.

  16. Cities could hold the key to understanding fragility | CRDI - Centre ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Cities are engines of economic growth and the primary sites of basic service delivery. Yet weak governance, along with inequalities related to income, social class, religion, and gender, may lead to a breakdown of systems and structures, and eventually to "fragile cities." Although the fragile cities concept is relatively new, ...

  17. Usng subjective percentiles and test data for estimating fragility functions

    International Nuclear Information System (INIS)

    George, L.L.; Mensing, R.W.

    1981-01-01

    Fragility functions are cumulative distribution functions (cdfs) of strengths at failure. They are needed for reliability analyses of systems such as power generation and transmission systems. Subjective opinions supplement sparse test data for estimating fragility functions. Often the opinions are opinions on the percentiles of the fragility function. Subjective percentiles are likely to be less biased than opinions on parameters of cdfs. Solutions to several problems in the estimation of fragility functions are found for subjective percentiles and test data. How subjective percentiles should be used to estimate subjective fragility functions, how subjective percentiles should be combined with test data, how fragility functions for several failure modes should be combined into a composite fragility function, and how inherent randomness and uncertainty due to lack of knowledge should be represented are considered. Subjective percentiles are treated as independent estimates of percentiles. The following are derived: least-squares parameter estimators for normal and lognormal cdfs, based on subjective percentiles (the method is applicable to any invertible cdf); a composite fragility function for combining several failure modes; estimators of variation within and between groups of experts for nonidentically distributed subjective percentiles; weighted least-squares estimators when subjective percentiles have higher variation at higher percents; and weighted least-squares and Bayes parameter estimators based on combining subjective percentiles and test data. 4 figures, 2 tables

  18. Finiteness Marking in Boys with Fragile X Syndrome

    Science.gov (United States)

    Sterling, Audra M.; Rice, Mabel L.; Warren, Steven F.

    2012-01-01

    Purpose: The current study investigated finiteness marking (e.g., he walk "s", he walk "ed") in boys with fragile X syndrome (FXS); the boys were grouped based on receptive vocabulary (i.e., borderline, impaired). Method: Twenty-one boys with the full mutation of fragile X, between the ages of 8 and 16 years participated. The…

  19. Whole-of-Government Approaches to Fragile States in Africa

    DEFF Research Database (Denmark)

    Olsen, Gorm Rye

    2013-01-01

    For a number of years fragile states have been high on the foreign policy agendas of the USA and the EU. Both actors look upon fragile states with great concern and consider them as security threats. Officially they give priority to ‘whole-of-government approaches’ (wga) when addressing the threats...

  20. Cities could hold the key to understanding fragility | IDRC ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Cities are engines of economic growth and the primary sites of basic service delivery. Yet weak governance, along with inequalities related to income, social class, religion, and gender, may lead to a breakdown of systems and structures, and eventually to "fragile cities." Although the fragile cities concept is relatively new, ...

  1. Recurring genomic breaks in independent lineages support genomic fragility

    Directory of Open Access Journals (Sweden)

    Hannenhalli Sridhar

    2006-11-01

    Full Text Available Abstract Background Recent findings indicate that evolutionary breaks in the genome are not randomly distributed, and that certain regions, so-called fragile regions, are predisposed to breakages. Previous approaches to the study of genomic fragility have examined the distribution of breaks, as well as the coincidence of breaks with segmental duplications and repeats, within a single species. In contrast, we investigate whether this regional fragility is an inherent genomic characteristic and is thus conserved over multiple independent lineages. Results We do this by quantifying the extent to which certain genomic regions are disrupted repeatedly in independent lineages. Our investigation, based on Human, Chimp, Mouse, Rat, Dog and Chicken, suggests that the propensity of a chromosomal region to break is significantly correlated among independent lineages, even when covariates are considered. Furthermore, the fragile regions are enriched for segmental duplications. Conclusion Based on a novel methodology, our work provides additional support for the existence of fragile regions.

  2. Seismic fragility levels of nuclear power plant equipment

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.K.; Hofmayer, C.H.

    1987-01-01

    Seismic fragility levels of safety-related electrical and mechanical equipment used in nuclear power plants are discussed. The fragility level is defined as the vibration level corresponding to initiation of equipment malfunctions. The test response spectrum is used as a measure of this vibration level. The fragility phenomenon of an equipment is represented by a number of response spectra corresponding to various failure modes. Analysis methods are described for determination of the fragility level by use of existing test data. Useful conversion factors are tabulated to transform test response spectra from one damping value to another. Results are presented for switch-gears and motor control centers. The capacity levels of these equipment assemblies are observed to be limited by malfunctioning of contactors, motor starters, relays and/or switches. The applicability of the fragility levels, determined in terms of test response spectra, to Seismic Margin Studies and Probabilistic Risk Assessments is discussed and specific recommendations are provided

  3. Trench Foot or Non-Freezing Cold Injury As a Painful Vaso-Neuropathy: Clinical and Skin Biopsy Assessments

    Directory of Open Access Journals (Sweden)

    Praveen Anand

    2017-09-01

    Full Text Available BackgroundTrench foot, or non-freezing cold injury (NFCI, results from cold exposure of sufficient severity and duration above freezing point, with consequent sensory and vascular abnormalities which may persist for years. Based on observations of Trench foot in World War II, the condition was described as a vaso-neuropathy. While some reports have documented nerve damage after extreme cold exposure, sensory nerve fibres and vasculature have not been assessed with recent techniques in NFCI.ObjectiveTo assess patients with chronic sensory symptoms following cold exposure, in order to diagnose any underlying small fibre neuropathy, and provide insight into mechanisms of the persistent pain and cold hypersensitivity.MethodsThirty soldiers with cold exposure and persistent sensory symptoms (>4 months were assessed with quantitative sensory testing, nerve conduction studies, and skin biopsies. Immunohistochemistry was used to assess intraepidermal (IENF and subepidermal (SENF nerve fibres with a range of markers, including the pan-neuronal marker protein gene product 9.5 (PGP 9.5, regenerating fibres with growth-associated protein 43 (GAP43, and nociceptor fibres with transient receptor potential cation channel subfamily V member 1 (TRPV1, sensory neuron-specific receptor (SNSR, and calcitonin gene-related peptide (CGRP. von Willebrand factor (vWF, endothelial nitric oxide synthase (eNOS, and vascular endothelial growth factor (VEGF were used for assessing blood vessels, and transient receptor potential cation channel, subfamily A member 1 (TRPA1 and P2X purinoceptor 7 (P2X7 for keratinocytes, which regulate nociceptors via release of nerve growth factor.ResultsClinical examination showed pinprick sensation was abnormal in the feet of 20 patients (67%, and between 67 and 83% had abnormalities of thermal thresholds to the different modalities. 7 patients (23% showed reduced sensory action potential amplitude of plantar nerves. 27 patients (90% had

  4. Expressão de caracteres de tubérculos em função do tamanho de recipiente usado no cultivo de batata na geração de plântulas

    Directory of Open Access Journals (Sweden)

    Mario Alvaro Aloisio Verissimo

    2012-12-01

    Full Text Available No melhoramento genético de batata, para alguns caracteres, é possível eliminar genótipos inferiores já nas gerações iniciais, o que permite reduzir o número de genótipos avaliados a campo, proporcionando redução de custos. O objetivo deste trabalho foi verificar a influência do tamanho de vaso na expressão de caracteres de tubérculo, em famílias de batata, na geração de plântula. Em casa de vegetação, dez famílias de batata foram avaliadas em dois tamanhos de recipientes, vaso grande (1 L e vaso pequeno (250 ml. Os caracteres de tubérculos avaliados foram: cor, aspereza, profundidade de olho, sobrancelha, formato, uniformidade de formato, apontamento, curvatura, achatamento, aparência geral, massa por planta, massa média e número de tubérculos. O vaso grande proporciona maior expressão da variação genética para os caracteres profundidade de olho, sobrancelha, curvatura, achatamento, aparência geral e todos os componentes de rendimento. As maiores médias de produção de tubérculos em número, tamanho e massa foram obtidas no vaso grande, enquanto o maior coeficiente de variação genético para os caracteres formato, uniformidade de formato e apontamento de tubérculo foi obtido no vaso pequeno.

  5. Addressing challenges of clinical trials in acute pain: The Pain Management of Vaso-occlusive Crisis in Children and Young Adults with Sickle Cell Disease Study.

    Science.gov (United States)

    Nottage, Kerri A; Hankins, Jane S; Faughnan, Lane G; James, Dustin M; Richardson, Julie; Christensen, Robbin; Kang, Guolian; Smeltzer, Matthew; Cancio, Maria I; Wang, Winfred C; Anghelescu, Doralina L

    2016-08-01

    Neuropathic pain is a known component of vaso-occlusive pain in sickle cell disease; however, drugs targeting neuropathic pain have not been studied in this population. Trials of acute pain are complicated by the need to obtain consent, to randomize participants expeditiously while optimally treating pain. We describe the challenges in designing and implementing the Pain Management of Vaso-occlusive Crisis in Children and Young Adults with Sickle Cell Disease Study (NCT01954927), a phase II, randomized, double-blind, placebo-controlled trial to determine the effect of gabapentin for vaso-occlusive crisis. In the Pain Management of Vaso-occlusive Crisis in Children and Young Adults with Sickle Cell Disease Study, we aim to assess the analgesic effect of gabapentin during vaso-occlusive crisis. Difficulties we identified included avoiding delay of notification of study staff of potential participants which we resolved by automated notification. Concern for rapid randomization and drug dispensation was addressed through careful planning with an investigational pharmacy and a single liquid formulation. We considered obtaining consent during well-visits to avoid the time constraints with acute presentations, but the large number of patients and limited duration that consent is valid made this impractical. In all, 79% of caregivers/children approached have agreed to participate. The trial is currently active, and enrollment is at 45.8% of that targeted (76 of 166) and expected to continue for two more years. Maintaining staff availability after-hours remains problematic, with 8% of screened patients missed for lack of available staff. Lessons learned in designing a trial to expedite procedures in the acute pain setting include (1) building study evaluations upon a standard-of-care backbone; (2) implementing a simple study design to facilitate consent and data capture; (3) assuring ample, well-trained study staff; and (4) utilizing technology to automate procedures

  6. The fragile X syndrome: Isolation of the FMR-1 gene and characterization of the fragile X mutation

    NARCIS (Netherlands)

    B.A. Oostra (Ben); A. Verkerk

    1992-01-01

    markdownabstractConclusion Rapid progress has been made in the analysis of the fragile X syndrome during 1991. Different groups have discovered that fragile X chromosomes are preferentially methylated. In these X chromosomes an insertion has been found in the methylated region. The FMR-1 gene,

  7. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study.

    Directory of Open Access Journals (Sweden)

    Deepika S Darbari

    Full Text Available BACKGROUND: Frequent painful vaso-occlusive crises (VOCs were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD over twenty years ago. Modern therapies for sickle cell anemia (SCA like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort. METHODS: Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation. RESULTS: Higher baseline hematocrit (p = 0.0008, ferritin (p = 0.005, and HDL cholesterol (p = 0.01 were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03. Higher tricuspid regurgitatant jet velocity (TRV (RR 2.41, 95% CI 1.5-3.9, p < 0.0001, elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001 and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001 were also independent risk factors for mortality. CONCLUSIONS: Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies. TRIAL REGISTRATION: ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/

  8. Hospitalist management of vaso-occlusive pain crisis in patients with sickle cell disease using a pathway of care.

    Science.gov (United States)

    Allen Liles, Edmund; Kirsch, Jonathan; Gilchrist, Michael; Adem, Mukhtar

    2014-04-01

    Patients with sickle cell disease (SCD) suffer from intermittent vaso-occlusive pain crises (VOCs). These crises lead to frequent hospitalizations, significant morbidity, and increased mortality risk. Care pathways can enhance efficiency and quality of care. Our study sought to evaluate the development and implementation of a care pathway for patients with SCD experiencing VOCs. The University of North Carolina (UNC) Comprehensive Sickle Cell Program provides all levels of care for a large population of patients with sickle cell anemia. All patients admitted to UNC Hospitals with SCD VOCs from January 2009 through June 2011 were evaluated. During this time period, we also assessed sequential prospective cohorts during progressive phases of developing and implementing a quality improvement and pathway of care program for this patient population in our study. The developed pathway entailed geographic localization for VOC patients, a single group of faculty physicians caring for these patients, and early use of patient-controlled analgesia (PCA) to achieve pain control. Physicians from the UNC Hospital Medicine Program were responsible for the initiatives. Cohorts were compared to a baseline historical control. Outcomes of interest included patient length of stay (LOS) in the hospital, 30-day readmission rate, need for transfusion, incidence of acute chest syndrome, use of naloxone, and use of PCA. Compared with an historical baseline cohort, the development and implementation of a VOC care pathway for patients with SCD led to reduction in average hospital LOS by 1.44 days (P management of patients with SCD VOCs using a care pathway that emphasizes early, aggressive PCA-based pain control is associated with reduced hospital LOS. The LOS reduction seen in our study is clinically meaningful. Notably, other measures of patient outcomes and quality of care metrics did not change significantly, and some trended towards improvement.

  9. A model of blood pressure, heart rate, and vaso-vagal responses produced by vestibulo-sympathetic activation

    Directory of Open Access Journals (Sweden)

    Theodore eRaphan

    2016-03-01

    Full Text Available Blood Pressure (BP, comprised of recurrent systoles and diastoles, is controlled by central mechanisms to maintain blood flow. Periodic behavior of BP was modeled to study how peak amplitudes and frequencies of the systoles are modulated by vestibular activation. The model was implemented as a relaxation oscillator, driven by a central signal related to Desired BP. Relaxation oscillations were maintained by a second order system comprising two integrators and a threshold element in the feedback loop. The output signal related to BP was generated as a nonlinear function of the derivative of the first state variable, which is a summation of an input related to desired BP, feedback from the states, and an input from the vestibular system into one of one of the feedback loops. This nonlinear function was structured to best simulate the shapes of systoles and diastoles, the relationship between BP and Heart Rate (HR as well as the amplitude modulations of BP and Pulse Pressure. Increases in threshold in one of the feedback loops produced lower frequencies of HR, but generated large pulse pressures to maintain orthostasis, without generating a VasoVagal Response (VVR. Pulse pressures were considerably smaller in the anesthetized rats than during the simulations, but simulated pulse pressures were lowered by including saturation in the feedback loop. Stochastic changes in Threshold maintained the compensatory Baroreflex Sensitivity. Sudden decreases in Desired BP elicited non-compensatory VVRs with smaller pulse pressures, consistent with experimental data. The model suggests that the Vestibular Sympathetic Reflex modulates BP and HR of an oscillating system by manipulating parameters of the baroreflex feedback and the signals that maintain the oscillations. It also shows that a VVR is generated when the vestibular input triggers a marked reduction in Desired BP.

  10. Severe Painful Vaso-Occlusive Crises and Mortality in a Contemporary Adult Sickle Cell Anemia Cohort Study

    Science.gov (United States)

    Darbari, Deepika S.; Wang, Zhengyuan; Kwak, Minjung; Hildesheim, Mariana; Nichols, James; Allen, Darlene; Seamon, Catherine; Peters-Lawrence, Marlene; Conrey, Anna; Hall, Mary K.; Kato, Gregory J.; Taylor VI, James G.

    2013-01-01

    Background Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort. Methods Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation. Results Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality. Conclusions Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies. Trial Registration ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/ PMID:24224021

  11. High functioning male with fragile X syndrome and fragile X-associated tremor/ataxia syndrome.

    Science.gov (United States)

    Basuta, Kirin; Schneider, Andrea; Gane, Louise; Polussa, Jonathan; Woodruff, Bryan; Pretto, Dalyir; Hagerman, Randi; Tassone, Flora

    2015-09-01

    Fragile X syndrome (FXS) affects individuals with more than 200 CGG repeats (full mutation) in the fragile X mental retardation 1 (FMR1) gene. Those born with FXS experience cognitive and social impairments, developmental delays, and some features of autism spectrum disorders. Carriers of a premutation (55-200 CGG repeats) are generally not severely affected early in life; however, are at high risk of developing the late onset neurodegenerative disorder, Fragile X-associated Tremor/Ataxia Syndrome (FXTAS), or Fragile X-associated Primary Ovarian Insufficiency (FXPOI), and may have other medical conditions such as developmental delay, autism spectrum disorders, hypertension, anxiety, and immune-mediated disorders. Here we present a case of a 58-year-old man with a borderline IQ, average memory skills, and executive function deficits. He met criteria for multiple psychiatric diagnoses and presented with tremor and ataxia, meeting criteria for FXTAS. Molecular testing unveiled a completely unmethylated FMR1 full mutation in peripheral blood mononucleated cells with elevated FMR1 mRNA and premutation alleles of different sizes in two other tissues (primary fibroblasts and sperm), indicating the presence of allele instability based on both inter- and intra-tissue mosaicism. The observation of FXTAS in this case of a full mutation mosaic man suggests that the pathogenic mechanism underlying this disorder is not observed exclusively in premutation carriers as it was originally thought. The concomitant presence of features of FXS and late onset neurological deterioration with probable FXTAS likely result from a combined molecular pathology of elevated FMR1 mRNA levels, a molecular hallmark of FXTAS and low FMRP expression that leads to FXS. © 2015 Wiley Periodicals, Inc.

  12. Pathological Plasticity in Fragile X Syndrome

    Directory of Open Access Journals (Sweden)

    Brandon S. Martin

    2012-01-01

    Full Text Available Deficits in neuronal plasticity are common hallmarks of many neurodevelopmental disorders. In the case of fragile-X syndrome (FXS, disruption in the function of a single gene, FMR1, results in a variety of neurological consequences directly related to problems with the development, maintenance, and capacity of plastic neuronal networks. In this paper, we discuss current research illustrating the mechanisms underlying plasticity deficits in FXS. These processes include synaptic, cell intrinsic, and homeostatic mechanisms both dependent on and independent of abnormal metabotropic glutamate receptor transmission. We place particular emphasis on how identified deficits may play a role in developmental critical periods to produce neuronal networks with permanently decreased capacity to dynamically respond to changes in activity central to learning, memory, and cognition in patients with FXS. Characterizing early developmental deficits in plasticity is fundamental to develop therapies that not only treat symptoms but also minimize the developmental pathology of the disease.

  13. Fragile X Syndrome in a Colombian Family

    Directory of Open Access Journals (Sweden)

    Saldarriaga Gil, Wilmar

    2018-01-01

    Full Text Available A study was performed on a family from Cali, Colombia in which nine patients were evaluated, three of which presented with intellectual disability with no previous etiological diagnosis. The proband was diagnosed with Fragile X syndrome by DNA molecular testing and, cascade testing, performed on all available family members, identifying two additional individuals with the full mutation and four carriers of a premutation allele. With this report we seek to contribute to Colombian epidemiology of the syndrome and emphasize the importance of diagnosis to provide a comprehensive and specific treatment to those affected. Further we seek to identify premutation carriers in their families or women with a full mutation without the classic phenotype for genetic counseling and education about potential associated pathologies.

  14. Nematoides fitoparasitos em áreas de cultivo de antúrio dos estados de São Paulo, Paraná e Santa Catarina

    Directory of Open Access Journals (Sweden)

    Claudio Marcelo Gonçalves de Oliveira

    2015-08-01

    Full Text Available O objetivo do presente trabalho foi identificar os nematoides associados a plantas de antúrio (Anthurium andraeanum cultivadas como flor de corte (13 amostras ou plantas em vaso (41 amostras nos estados de São Paulo, Paraná e Santa Catarina. Nematoides foram extraídos de 10g de raízes e 250cm3 de solo ou substrato e identificados sob microscópio de luz. A espécie mais frequente foi Radopholus similis, detectada em 54% das amostras de antúrio cultivado como flor de corte. Constituíram novas ocorrências em antúrio no Brasil: Helicotylenchus erythrinae, H. californicus, H. multicinctus, Meloidogyne paranaensis e R. similis. Entretanto, nenhum nematoide parasito de plantas foi encontrado nos antúrios cultivados em vaso, com substrato de fibra de coco.

  15. Divergent effects of obesity on fragility fractures

    Directory of Open Access Journals (Sweden)

    Caffarelli C

    2014-09-01

    Full Text Available Carla Caffarelli, Chiara Alessi, Ranuccio Nuti, Stefano Gonnelli Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy Abstract: Obesity was commonly thought to be advantageous for maintaining healthy bones due to the higher bone mineral density observed in overweight individuals. However, several recent studies have challenged the widespread belief that obesity is protective against fracture and have suggested that obesity is a risk factor for certain fractures. The effect of obesity on fracture risk is site-dependent, the risk being increased for some fractures (humerus, ankle, upper arm and decreased for others (hip, pelvis, wrist. Moreover, the relationship between obesity and fracture may also vary by sex, age, and ethnicity. Risk factors for fracture in obese individuals appear to be similar to those in nonobese populations, although patterns of falling are particularly important in the obese. Research is needed to determine if and how visceral fat and metabolic complications of obesity (type 2 diabetes mellitus, insulin resistance, chronic inflammation, etc are causally associated with bone status and fragility fracture risk. Vitamin D deficiency and hypogonadism may also influence fracture risk in obese individuals. Fracture algorithms such as FRAX® might be expected to underestimate fracture probability. Studies specifically designed to evaluate the antifracture efficacy of different drugs in obese patients are not available; however, literature data may suggest that in obese patients higher doses of the bisphosphonates might be required in order to maintain efficacy against nonvertebral fractures. Therefore, the search for better methods for the identification of fragility fracture risk in the growing population of adult and elderly subjects with obesity might be considered a clinical priority which could improve the prevention of fracture in obese individuals. Keywords: bone mineral density, BMI

  16. Seismic fragility of nuclear power plant components (Phase II)

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.K.; Hofmayer, C.H.; Kassir, M.K.; Pepper, S.E.

    1990-02-01

    As part of the Component Fragility Program which was initiated in FY 1985, three additional equipment classes have been evaluated. This report contains the fragility results and discussions on these equipment classes which are switchgear, I and C panels and relays. Both low and medium voltage switchgear assemblies have been considered and a separate fragility estimate for each type is provided. Test data on cabinets from the nuclear instrumentation/neutron monitoring system, plant/process protection system, solid state protective system and engineered safeguards test system comprise the BNL data base for I and C panels (NSSS). Fragility levels have been determined for various failure modes of switchgear and I ampersand C panels, and the deterministic results are presented in terms of test response spectra. In addition, the test data have been evaluated for estimating the respective probabilistic fragility levels which are expressed in terms of a median value, an uncertainty coefficient, a randomness coefficient and an HCLPF value. Due to a wide variation of relay design and the fragility level, a generic fragility level cannot be established for relays. 7 refs., 13 figs., 12 tabs

  17. Fragility and cooperativity concepts in hydrogen-bonded organic glasses

    Energy Technology Data Exchange (ETDEWEB)

    Delpouve, N., E-mail: delpouve.nicolas@gmail.com [AMME-LECAP EA 4528 International Laboratory, University of Rouen, Avenue de l' Universite BP 12, 76801 Saint Etienne du Rouvray (France); Vuillequez, A.; Saiter, A.; Youssef, B.; Saiter, J.M. [AMME-LECAP EA 4528 International Laboratory, University of Rouen, Avenue de l' Universite BP 12, 76801 Saint Etienne du Rouvray (France)

    2012-09-01

    Molecular dynamics at the glass transition of three lactose/oil glassy systems have been investigated according to the cooperativity and fragility approaches. From Donth's approach, the cooperativity length is estimated by modulated temperature calorimetric measurements. Results reveal that modification of the disaccharide by oil leads to increase the disorder degree in the lactose, the size of the cooperative domains and the fragility index. These particular hydrogen-bonded organic glasses follow the general tendency observed on organic and inorganic polymers: the higher the cooperativity length, the higher the value of the fragility index at T{sub g}.

  18. Fragility and cooperativity concepts in hydrogen-bonded organic glasses

    International Nuclear Information System (INIS)

    Delpouve, N.; Vuillequez, A.; Saiter, A.; Youssef, B.; Saiter, J.M.

    2012-01-01

    Molecular dynamics at the glass transition of three lactose/oil glassy systems have been investigated according to the cooperativity and fragility approaches. From Donth's approach, the cooperativity length is estimated by modulated temperature calorimetric measurements. Results reveal that modification of the disaccharide by oil leads to increase the disorder degree in the lactose, the size of the cooperative domains and the fragility index. These particular hydrogen-bonded organic glasses follow the general tendency observed on organic and inorganic polymers: the higher the cooperativity length, the higher the value of the fragility index at T g .

  19. Seismic fragility analysis of structural components for HFBR facilities

    International Nuclear Information System (INIS)

    Park, Y.J.; Hofmayer, C.H.

    1992-01-01

    The paper presents a summary of recently completed seismic fragility analyses of the HFBR facilities. Based on a detailed review of past PRA studies, various refinements were made regarding the strength and ductility evaluation of structural components. Available laboratory test data were analysed to evaluate the formulations used to predict the ultimate strength and deformation capacities of steel, reinforced concrete and masonry structures. The biasness and uncertainties were evaluated within the framework of the fragility evaluation methods widely accepted in the nuclear industry. A few examples of fragility calculations are also included to illustrate the use of the presented formulations

  20. Clinical neurogenetics: fragile x-associated tremor/ataxia syndrome.

    Science.gov (United States)

    Hall, Deborah A; O'Keefe, Joan A

    2013-11-01

    This article summarizes the clinical findings, genetics, pathophysiology, and treatment of fragile X-associated tremor ataxia syndrome. The disorder occurs from a CGG repeat (55-200) expansion in the fragile X mental retardation 1 gene. It manifests clinically in kinetic tremor, gait ataxia, and executive dysfunction, usually in older men who carry the genetic abnormality. The disorder has distinct radiographic and pathologic findings. Symptomatic treatment is beneficial in some patients. The inheritance is X-linked and family members may be at risk for other fragile X-associated disorders. This information is useful to neurologists, general practitioners, and geneticists. Copyright © 2013. Published by Elsevier Inc.

  1. Subsystem fragility: Seismic Safety Margins Research Program (Phase I)

    Energy Technology Data Exchange (ETDEWEB)

    Kennedy, R. P.; Campbell, R. D.; Hardy, G.; Banon, H.

    1981-10-01

    Seismic fragility levels of safety related equipment are developed for use in a seismic oriented Probabilistic Risk Assessment (PRA) being conducted as part of the Seismic Safety Margins Research Program (SSMRP). The Zion Nuclear Power Plant is being utilized as a reference plant and fragility descriptions are developed for specific and generic safety related equipment groups in Zion. Both equipment fragilities and equipment responses are defined in probabilistic terms to be used as input to the SSMRP event tree/fault tree models of the Zion systems. 65 refs., 14 figs., 11 tabs.

  2. Invertible chaotic fragile watermarking for robust image authentication

    International Nuclear Information System (INIS)

    Sidiropoulos, Panagiotis; Nikolaidis, Nikos; Pitas, Ioannis

    2009-01-01

    Fragile watermarking is a popular method for image authentication. In such schemes, a fragile signal that is sensitive to manipulations is embedded in the image, so that it becomes undetectable after any modification of the original work. Most algorithms focus either on the ability to retrieve the original work after watermark detection (invertibility) or on detecting which image parts have been altered (localization). Furthermore, the majority of fragile watermarking schemes suffer from robustness flaws. We propose a new technique that combines localization and invertibility. Moreover, watermark dependency on the original image and the non-linear watermark embedding procedure guarantees that no malicious attacks will manage to create information leaks.

  3. Anestesia en la transposición de los grandes vasos ANESTHESIA IN THE TRANSPOSITION OF THE GREAT VESSELS

    Directory of Open Access Journals (Sweden)

    Lincoln de la Parte Pérez

    2005-03-01

    Full Text Available La transposición de los grandes vasos es una cardiopatía compleja que se acompaña de una elevada mortalidad. Se conoce que el 45 % de los pacientes fallece durante el primer mes de vida y alrededor del 90 % antes del año. Las principales causas de la muerte son la hipoxia y la insuficiencia cardíaca. El desarrollo de las especialidades que trabajan en cirugía cardiovascular pediátrica ha permitido aumentar la supervivencia de estos niños, especialmente con el uso de prostaglandinas para mantener el ductus permeable, la técnica de Raskind-Miller ( septostomía de balón y una tendencia cada vez mayor a la corrección anatómica temprana (operación de Jatene en lugar de los clásicos procedimientos paliativos y las técnicas de corrección fisiológica de Senning y Mustard . Se presenta una revisión bibliográfica sobre los factores a tener en cuenta en el manejo anestésico de estos pacientes.The transposition of the great vessels is a complex heart disease accompanied of a high mortality. It is known that 45 % of the patients die during the first month of life and at about 90 % do it before being one year old. The main causes of death are hypoxia and heart failure. The development of the specialties working in pediatric cardiovascular surgery has allowed to increase the survival of these children, specially with the use of prostaglandins to maintain the ductus permeable, the Raskind-Miller's technique (balloon septostomy, and an increasing trend towards an early anatomical correction (Jatene's operation to replace the classical palliative procedures and Senning and Mustartd's techniques of physiological correction. A bibliographic review of the factors to be taken into account in the anesthetic management of these patients is presented.

  4. What Are the Treatments for Fragile X Syndrome?

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Preeclampsia and Eclampsia About NICHD Research Information Find a ... Facebook Twitter Pinterest Email Print What are the treatments for Fragile X syndrome? There is no single ...

  5. Conducting Research With an Adolescent Diagnosed With Fragile X Syndrome

    Directory of Open Access Journals (Sweden)

    Chantel Lynette Weber

    2017-03-01

    Full Text Available In this article I address the reflexive nature of research undertaken when I investigated the presence of resilience found in an adolescent girl diagnosed with fragile X syndrome. The highlights of this article include specific challenges I experienced when conducting research with this adolescent and how I have adapted the process accordingly. These challenges involve the planning and preparation before data commenced; the influence of sensory integration, behavioral, cognitive and language characteristics of fragile X syndrome on an adolescent girl; and the aspects of ethical and rigorous research. I have also included recommendations such as guidelines for other researchers interested in conducting a similar study with adolescents affected by fragile X syndrome. I hope that with this article, other researchers would be provided with a better understanding of how to proceed with research that involves individuals with disabilities and more specifically, individuals diagnosed with fragile X syndrome. URN: http://nbn-resolving.de/urn:nbn:de:0114-fqs1702102

  6. Establishing local government in fragile states: experimental evidence from Afghanistan

    NARCIS (Netherlands)

    Jochem, T.; Murtazashvili, I.; Murtazashvili, J.

    2016-01-01

    International and domestic policy makers often promote elections to establish village government in fragile states. However, two additional options are available in such countries: formalization of self-governing village councils and formalization of community development councils (CDCs). We

  7. Pesquisa anatômica sobre o funículo umbilical em bovinos azebuados

    Directory of Open Access Journals (Sweden)

    Antonio Augusto Coppi Maciel Ribeiro

    1997-12-01

    Full Text Available Mediante esta pesquisa, obtivemos dados sobre os aspectos morfológicos da disposição e da constituição das artérias e das veias do funículo umbilical em bovinos azebuados. Utilizamos para isto 30 fetos (13 machos e 17 fêmeas, com idades variando de 2 a 7 meses, cujos vasos umbilicais foram injetados com solução de látex-Neoprene 650 corada e posteriormente dissecados. O comprimento do funículo umbilical variou de 3 a 43 cm em fetos de 2 a 7 meses de idade. Quatro vasos umbilicais (duas artérias e duas veias e um dueto alantóide constituíam o funículo umbilical desses ruminantes, sendo que existia entre eles uma grande quantidade de gelatina de Wharton e pequenos vasos (vasa vasorum. Todas as estruturas estavam envolvidas pelo epitélio amniótico. Em 28 (93,33% ± 4,5 dos 30 fetos examinados, observamos uma anastomose interarterial umbilical.

  8. Manejo quirúrgico del trauma de grandes vasos abdominales en el servicio de emergencias del Hospital General Luis Vernaza, enero 2007 - diciembre 2010

    OpenAIRE

    Rocha Galecio, Adriano Bernardo

    2013-01-01

    Las lesiones vasculares intraabdominales se encuentran entre las más letales que puede sufrir un paciente traumático. La mortalidad hospitalaria es casi la misma que la prehospitalaria. El manejo de dichas lesiones constituye un desafío para los cirujanos y determinar las lesiones vasculares más frecuentes por trauma atendidas en el Servicio de Emergencias. 2. Describir el manejo quirúrgico de las lesiones de grandes vasos intraabdominales. 3. Determinar la morbimortalidad de las lesio...

  9. Fragile X testing in obstetrics and gynaecology in Canada.

    Science.gov (United States)

    Chitayat, David; Wyatt, Philip R

    2008-09-01

    To provide Canadian family physicians, genetic counsellors, medical geneticists, midwives, and obstetrician-gynaecologists with recommendations regarding screening for fragile X in the obstetrical and gynaecological population. Medline, the Cochrane Library, journals, and textbooks were searched for English-language articles, published between 1966 and March 2008, relating to fragile X testing outcomes. Search terms included fragile X, screening, prenatal testing, pregnancy outcome, premutation, trinucleotide repeats, and ovarian failure. All study types were reviewed. Randomized controlled trial results were considered evidence of the highest quality, followed by results of cohort studies. Key individual studies on which the recommendations are based are referenced. Supporting data for each recommendation are summarized with evaluative comments and references. This document represents an abstraction of the information. The quality of evidence reported in this document has been described using the criteria outlined in the report of the Canadian Task Force on Preventive Health Care. 1. Any testing for fragile X syndrome must occur only following thorough counselling and with the informed consent of the woman to be tested. (III-A) 2. Fragile X testing is indicated for a woman with a family history of fragile X syndrome, fragile X tremor/ataxia syndrome, or premature ovarian failure (in more than one family member) if the pedigree structure indicates that she is at risk of inheriting the mutated gene. Referral to a medical geneticist for counselling and assessment should be considered in these cases. (II-2A) 3. Fragile X testing is indicated for women who have a personal history of autism or mental retardation/developmental delay of an unknown etiology or who have at least one male relative with these conditions within a three-generation pedigree. (II-2A) 4. Fragile X testing is indicated for women who have reproductive or fertility problems associated with an

  10. Thermalization as an Invisibility Cloak for Fragile Quantum Superpositions

    OpenAIRE

    Hahn, Walter; Fine, Boris V.

    2017-01-01

    We propose a method for protecting fragile quantum superpositions in many-particle systems from dephasing by external classical noise. We call superpositions "fragile" if dephasing occurs particularly fast, because the noise couples very differently to the superposed states. The method consists of letting a quantum superposition evolve under the internal thermalization dynamics of the system, followed by a time reversal manipulation known as Loschmidt echo. The thermalization dynamics makes t...

  11. Bayesian tsunami fragility modeling considering input data uncertainty

    OpenAIRE

    De Risi, Raffaele; Goda, Katsu; Mori, Nobuhito; Yasuda, Tomohiro

    2017-01-01

    Empirical tsunami fragility curves are developed based on a Bayesian framework by accounting for uncertainty of input tsunami hazard data in a systematic and comprehensive manner. Three fragility modeling approaches, i.e. lognormal method, binomial logistic method, and multinomial logistic method, are considered, and are applied to extensive tsunami damage data for the 2011 Tohoku earthquake. A unique aspect of this study is that uncertainty of tsunami inundation data (i.e. input hazard data ...

  12. Molecular characterization of X chromosome fragility in idiopathic mental retardation

    Directory of Open Access Journals (Sweden)

    Heba Alla Hosny Omar

    2016-04-01

    Conclusions: A simplified checklist of fragile X should be used for patients with idiopathic MR and those patients above score 3 should be tested for FXS. The diagnostic assay may be used as a screening method for fragile X syndrome being rapid and cost effective compared to other techniques. In addition, screening of all relatives of proven patients should be performed to detect clinically unidentified cases for provision of proper counseling and optimal management of detected cases.

  13. Environmental Change & Fragile States Early Warning and Intervention

    Science.gov (United States)

    2011-05-12

    Fragile States Early Warning and Intervention 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) 5d. PROJECT NUMBER 5e...position of the Department of the Army, Department of Defense, or the U.S. government Environmental Change & Fragile States Early Warning and... Intervention Steven Hearne, P.E., Senior Fellow Army Environmental Policy Institute (AEPI) Jeremey Alcorn, Research Fellow Logistics Management Institute (LMI

  14. Treatment of Fragile X Syndrome with a Neuroactive Steroid

    Science.gov (United States)

    2012-08-01

    has shown its efficacy in the treatment of partial seizures, infantile spasms and catamenial epilepsy [18,42]. However, this is the first time that...AD_________________ Award Number: W81XWH-11-1-0626 TITLE: Treatment of Fragile X Syndrome with a...2012 4. TITLE AND SUBTITLE Treatment of Fragile X Syndrome with a Neuroactive Steroid 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-11-1-0626 5c

  15. Fragility curves for bridges under differential support motions

    DEFF Research Database (Denmark)

    Konakli, Katerina

    2012-01-01

    This paper employs the notion of fragility to investigate the seismic vulnerability of bridges subjected to spatially varying support motions. Fragility curves are developed for four highway bridges in California with vastly different structural characteristics. The input in this analysis consists...... of simulated ground motion arrays with temporal and spectral nonstationarities, and consistent with prescribed spatial variation patterns. Structural damage is quantified through displacement ductility demands obtained from nonlinear time-history analysis. The potential use of the ‘equal displacement’ rule...

  16. Longitudinal profiles of adaptive behavior in fragile X syndrome.

    Science.gov (United States)

    Klaiman, Cheryl; Quintin, Eve-Marie; Jo, Booil; Lightbody, Amy A; Hazlett, Heather Cody; Piven, Joseph; Hall, Scott S; Chromik, Lindsay C; Reiss, Allan L

    2014-08-01

    To examine longitudinally the adaptive behavior patterns in fragile X syndrome. Caregivers of 275 children and adolescents with fragile X syndrome and 225 typically developing children and adolescents (2-18 years) were interviewed with the Vineland Adaptive Behavior Scales every 2 to 4 years as part of a prospective longitudinal study. Standard scores of adaptive behavior in people with fragile X syndrome are marked by a significant decline over time in all domains for males and in communication for females. Socialization skills are a relative strength as compared with the other domains for males with fragile X syndrome. Females with fragile X syndrome did not show a discernible pattern of developmental strengths and weaknesses. This is the first large-scale longitudinal study to show that the acquisition of adaptive behavior slows as individuals with fragile X syndrome age. It is imperative to ensure that assessments of adaptive behavior skills are part of intervention programs focusing on childhood and adolescence in this condition. Copyright © 2014 by the American Academy of Pediatrics.

  17. A systematic review of population screening for fragile X syndrome.

    Science.gov (United States)

    Hill, Melissa K; Archibald, Alison D; Cohen, Jonathan; Metcalfe, Sylvia A

    2010-07-01

    To conduct a systematic review of literature regarding population-based screening for fragile X syndrome in newborns and women of reproductive age, either before or during pregnancy. Seven electronic databases were searched for English language studies published between January 1991 and November 2009. Data extraction was performed for all included studies. Results were synthesized using a narrative approach. One article that examined offering newborn screening for fragile X syndrome and 10 that examined the offer of fragile X syndrome screening to women of reproductive age were identified. Two of these articles also addressed psychosocial aspects of population screening for fragile X syndrome such as attitudes to screening and experiences of screening, and a further nine addressed these issues alone. Studies exploring psychosocial issues demonstrated challenges for counseling arising from a lack of awareness or personal experience with fragile X syndrome in the general population. Targeted counseling and educational strategies will be essential to support women from the general population. It is crucial that future studies offering screening for fragile X syndrome explore a range of psychosocial aspects in addition to looking at uptake of testing and mutation frequency.

  18. A Case Report of Fragile X Syndrome with Fingers Anomaly

    Directory of Open Access Journals (Sweden)

    F. Ashrafzadeh

    2003-04-01

    Full Text Available Fragile X Syndrome, the most common cause of inherited mental retardation, results from mutation in fragile X mental retardation gene (FMR1 on long arm of X chromosome, Xq27.3. Clinical features include moderate to severe mental retardation without neurologic deficit, long face, large ears, prominent jaw, macro-orchidism, attention deficit, behavior disorder and occasionally autistic behavior . Hyperextensible joints may also be seen in these patients. There is no other congenital malformation in fragile X syndrome. In this article we presented a 6 years old boy with fragile X syndrome who in addition to his characteristic clinical features had large thumbs and toes, which was disproportionate in size to his thumb nails and toe nails. In a case report from division of medical Genetics, Newyork Hospital in Cornell University, a twin brothers with fragile X syndrome also had cleft palate and ventricular septal defect . The diagnosis of this case was performed by culturing the patient’s peripheral blood lymphocytes in media lacking folate which expose the fragile X sites. The chromosomal analysis was performed using G-banding.

  19. Longitudinal Profiles of Adaptive Behavior in Fragile X Syndrome

    Science.gov (United States)

    Quintin, Eve-Marie; Jo, Booil; Lightbody, Amy A.; Hazlett, Heather Cody; Piven, Joseph; Hall, Scott S.; Reiss, Allan L.

    2014-01-01

    OBJECTIVE: To examine longitudinally the adaptive behavior patterns in fragile X syndrome. METHOD: Caregivers of 275 children and adolescents with fragile X syndrome and 225 typically developing children and adolescents (2–18 years) were interviewed with the Vineland Adaptive Behavior Scales every 2 to 4 years as part of a prospective longitudinal study. RESULTS: Standard scores of adaptive behavior in people with fragile X syndrome are marked by a significant decline over time in all domains for males and in communication for females. Socialization skills are a relative strength as compared with the other domains for males with fragile X syndrome. Females with fragile X syndrome did not show a discernible pattern of developmental strengths and weaknesses. CONCLUSIONS: This is the first large-scale longitudinal study to show that the acquisition of adaptive behavior slows as individuals with fragile X syndrome age. It is imperative to ensure that assessments of adaptive behavior skills are part of intervention programs focusing on childhood and adolescence in this condition. PMID:25070318

  20. Abordagem neurologica na sindrome do X fragil

    OpenAIRE

    Marilisa Mantovani Guerreiro

    1993-01-01

    Resumo: Com o propósito de realizar a abordagem neurológica na Síndrome do X Frágil (SXF), estudaram-se 11 pacientes com este diagnóstico citogenético. A idade variou de 8 a 19 anos. Nove pacientes eram do sexo masculino e dois do feminino. Todos os indivíduos apresentaram pavilhões auriculares grandes e faces alongadas. Observou-se fronte proeminente em 63% dos casos e macrorquidia em 33% dentre aqueles do sexo masculino. Atraso na aquisição da fala, fala repetitiva, perseverativa, impulsiva...

  1. Interim Stabilisation in Fragile Security Situations

    Directory of Open Access Journals (Sweden)

    Nat J. Colletta

    2012-09-01

    Full Text Available For more than two decades a conventional approach to security promotion has been widely applied by multilateral and bilateral agencies during war-to-peace transitions. Advocates of this approach typically recommend a combination of disarmament, demobilisation and reintegration (DDR and security sector reform (SSR to consolidate peace-making and peace-building processes (Colletta et al 2009, Muggah 2006. Notwithstanding the broad acceptance of such activities – and the theory that underlies them – there is little evidence that such interventions have contributed to any enduring solution to conflict and fragility (Muggah 2009. Indeed, analysts have come to recognise that the political, economic and social pre-conditions for DDR and SSR – including a relatively functional government, a reasonably stable labour market and a minimum level of social trust – are seldom in place. Even when these ambitious pre-requisites have been achieved, it is not clear that they are sufficient for DDR and SSR to take hold. Nevertheless, these orthodoxies persist in security promotion policy and practice.

  2. Extracellular histones induce erythrocyte fragility and anemia.

    Science.gov (United States)

    Kordbacheh, Farzaneh; O'Meara, Connor H; Coupland, Lucy A; Lelliott, Patrick M; Parish, Christopher R

    2017-12-28

    Extracellular histones have been shown to play an important pathogenic role in many diseases, primarily through their cytotoxicity toward nucleated cells and their ability to promote platelet activation with resultant thrombosis and thrombocytopenia. In contrast, little is known about the effect of extracellular histones on erythrocyte function. We demonstrate in this study that histones promote erythrocyte aggregation, sedimentation, and using a novel in vitro shear stress model, we show that histones induce erythrocyte fragility and lysis in a concentration-dependent manner. Furthermore, histones impair erythrocyte deformability based on reduced passage of erythrocytes through an artificial spleen. These in vitro results were mirrored in vivo with the injection of histones inducing anemia within minutes of administration, with a concomitant increase in splenic hemoglobin content. Thrombocytopenia and leukopenia were also observed. These findings suggest that histones binding to erythrocytes may contribute to the elevated erythrocyte sedimentation rates observed in inflammatory conditions. Furthermore, histone-induced increases in red blood cell lysis and splenic clearance may be a significant factor in the unexplained anemias seen in critically ill patients. © 2017 by The American Society of Hematology.

  3. The Brazilian Pampa: A Fragile Biome

    Directory of Open Access Journals (Sweden)

    Valdir Marcos Stefenon

    2009-12-01

    Full Text Available Biodiversity is one of the most fundamental properties of Nature. It underpins the stability of ecosystems, provides vast bioresources for economic use, and has important cultural significance for many people. The Pampa biome, located in the southernmost state of Brazil, Rio Grande do Sul, illustrates the direct and indirect interdependence of humans and biodiversity. The Brazilian Pampa lies within the South Temperate Zone where grasslands scattered with shrubs and trees are the dominant vegetation. The soil, originating from sedimentary rocks, often has an extremely sandy texture that makes them fragile—highly prone to water and wind erosion. Human activities have converted or degraded many areas of this biome. In this review we discuss our state-of-the-art knowledge of the diversity and the major biological features of this regions and the cultural factors that have shaped it. Our aim is to contribute toward a better understanding of the current status of this special biome and to describe how the interaction between human activities and environment affects the region, highlighting the fragility of the Brazilian Pampa.

  4. Fragile X Mental Retardation Syndrome: Structure of the KH1-KH2 Domains of Fragile X Mental Retardation Protein

    Energy Technology Data Exchange (ETDEWEB)

    Valverde,R.; Poznyakova, I.; Kajander, T.; Venkatraman, J.; Regan, L.

    2007-01-01

    Fragile X syndrome is the most common form of inherited mental retardation in humans, with an estimated prevalence of about 1 in 4000 males. Although several observations indicate that the absence of functional Fragile X Mental Retardation Protein (FMRP) is the underlying basis of Fragile X syndrome, the structure and function of FMRP are currently unknown. Here, we present an X-ray crystal structure of the tandem KH domains of human FMRP, which reveals the relative orientation of the KH1 and KH2 domains and the location of residue Ile304, whose mutation to Asn is associated with a particularly severe incidence of Fragile X syndrome. We show that the Ile304Asn mutation both perturbs the structure and destabilizes the protein.

  5. Influência de idade e da posição radial nas dimensões das fibras e dos vasos da madeira de Eucalyptus grandis Hill ex. Maiden Influence of age and radial position on fiber and vessel dimensions of Eucalyptus grandis Hill ex. Maiden wood

    Directory of Open Access Journals (Sweden)

    José de Castro Silva

    2007-12-01

    Full Text Available Objetivou-se neste trabalho avaliar a variação das dimensões das fibras e dos vasos da madeira de Eucalyptus grandis com quatro diferentes idades (10, 14, 20 e 25 anos, proveniente de talhões comerciais. A amostragem do material na árvore foi feita através da coleta de três discos, retirados da base e das extremidades das duas primeiras toras, ambas com o comprimento comercial de 3 m. De cada disco, retiraram-se cinco amostras, de dimensões 1,0 x 1,0 x 1,0 cm, tomadas de pontos eqüidistantes, correspondentes a 0; 25; 50; 75; e 100% da seção, no sentido radial medula-casca, fazendo-se a medição das fibras e dos vasos. Verificou-se, em todos os parâmetros, o efeito da idade e da variação radial, no sentido medula-casca, à exceção da largura e do diâmetro do lume das fibras; todos os demais parâmetros apresentaram correlação positiva.The objective of this work was to evaluate the variability of wood quantitative anatomical characteristics (fibers and vessels of Eucalyptus grandis of four different ages (10, 14, 20 and 25 years, obtained from commercial stands. Tree material sampling was collected from three disks in the bottom and top of the first two logs, both three m long. Five samples were collected from each disc at the pith-to-bark direction at 0, 25, 50, 75 and 100% of the radii. In all cases, age and pith-to-bark direction were observed to have a marked influence on all the assays, except for fiber lumen diameter and width; all the other parameters showed a positive correlation.

  6. Grupos de edad y género en un nuevo vaso del Tossal de Sant Miquel de Llíria (Valéncia

    Directory of Open Access Journals (Sweden)

    Isabel Izquierdo Peraile

    2005-01-01

    Full Text Available Presentamos en este trabajo una tinaja de grandes dimensiones inédita con decoración figurada procedente de la ciudad ibérica del Tossal de Sant Miquel de Llíria (Valencia. Con un elevado índice de fragmentación. el vaso muestra interesantes figuraciones como una escena femenina de hilado y tejido, joven a caballo, así como damas estantes. Su iconografía nos aproxima al análisis de los grupos de edad y género en la cultura ibérica: ternas como la naturaleza o los rituales de tránsito a la edad adulta. La dimensión simbólica de las escenas plasmadas en este vaso de encargo se integra en el programa de imágenes de la antigua Edeta, en un marco urbano y aristocrático, de tradición mediterránea.

  7. Does e-pain plan improve management of sickle cell disease associated vaso-occlusive pain crisis? a mixed methods evaluation.

    Science.gov (United States)

    Kato-Lin, Yi-Chin; Krishnamurti, Lakshmanan; Padman, Rema; Seltman, Howard J

    2014-11-01

    There is limited application and evaluation of health information systems in the management of vaso-occlusive pain crises in sickle cell disease (SCD) patients. This study evaluates the impact of digitization of paper-based individualized pain plans on process efficiency and care quality by examining both objective patient data and subjective clinician insights. Retrospective, before and after, mixed methods evaluation of digitization of paper documents in Children's Hospital of Pittsburgh of UPMC. Subjective perceptions are analyzed using surveys completed by 115 clinicians in emergency department (ED) and inpatient units (IP). Objective effects are evaluated using mixed models with data on 1089 ED visits collected via electronic chart review 28 months before and 22 months after the digitization. Surveys indicate that all clinicians perceived the digitization to improve the efficiency and quality of pain management. Physicians overwhelmingly preferred using the digitized plans, but only 44% of the nurses had the same response. Analysis of patient records indicates that adjusted time from analgesic order to administration was significantly reduced from 35.50 to 26.77 min (pmanagement. This study highlights the important role of health information technology (HIT) on vaso-occlusive pain management for pediatric patients with sickle cell disease and the critical challenges in accommodating human factor considerations in implementing and evaluating HIT effects. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  8. Atividade de enzimas fermentativas em raízes e nódulos de soja sob condições de hipóxia e pós-hipóxia

    OpenAIRE

    Borella,Junior; Durigon,Marcel Angelo; Amarante,Luciano do; Emygdio,Beatriz Marti

    2013-01-01

    O objetivo deste trabalho foi avaliar a atividade de enzimas fermentativas em raízes e nódulos de soja (Glycine max) sob condições de hipóxia e pós-hipóxia. Os genótipos Fundacep 53 RR e BRS Macota foram cultivados em vasos de 3L contendo vermiculita como substrato em associação com Bradyrhizobium elkanii em casa de vegetação. No estádio R2, o sistema radicular das plantas foi submetido à hipóxia pelo encaixe de um segundo vaso sem perfurações e inundado com solução nutritiva diluída a 1/3 da...

  9. Study on model of seismic fragility analysis for nuclear power plant

    International Nuclear Information System (INIS)

    Fu Zhiwei; Zhang Chunming; Wang Zhe; Zheng Jiye; Zhang Donghui

    2013-01-01

    After the Fukushima nuclear accident, the impact evaluation of nuclear power plants resulted from external events is required in China, and the 12th Five-Year Plan for Nuclear Safety also demands that the probability safety assessment of external events shall be developed before 2015. The earthquake is one of the most important external events which are essentially evaluated, and the seismic fragility is a key element in seismic PSA, while the fragility model is the basis of seismic fragility analysis. In this paper, the concept of seismic fragility was introduced, the seismic fragility model recommended by NRC and EPRI was studied, and the fragility model was derived. Then, the application example of the fragility model was given, and the effects of fragility due to randomness and uncertainty were discussed. The results show that a wealth of knowledge and experience is useful for the fragility analysis, which would reduce the uncertainty and gain access to practice. (authors)

  10. Aberrant face and gaze habituation in fragile x syndrome.

    Science.gov (United States)

    Bruno, Jennifer Lynn; Garrett, Amy S; Quintin, Eve-Marie; Mazaika, Paul K; Reiss, Allan L

    2014-10-01

    The authors sought to investigate neural system habituation to face and eye gaze in fragile X syndrome, a disorder characterized by eye-gaze aversion, among other social and cognitive deficits. Participants (ages 15-25 years) were 30 individuals with fragile X syndrome (females, N=14) and a comparison group of 25 individuals without fragile X syndrome (females, N=12) matched for general cognitive ability and autism symptoms. Functional MRI (fMRI) was used to assess brain activation during a gaze habituation task. Participants viewed repeated presentations of four unique faces with either direct or averted eye gaze and judged the direction of eye gaze. Four participants (males, N=4/4; fragile X syndrome, N=3) were excluded because of excessive head motion during fMRI scanning. Behavioral performance did not differ between the groups. Less neural habituation (and significant sensitization) in the fragile X syndrome group was found in the cingulate gyrus, fusiform gyrus, and frontal cortex in response to all faces (direct and averted gaze). Left fusiform habituation in female participants was directly correlated with higher, more typical levels of the fragile X mental retardation protein and inversely correlated with autism symptoms. There was no evidence for differential habituation to direct gaze compared with averted gaze within or between groups. Impaired habituation and accentuated sensitization in response to face/eye gaze was distributed across multiple levels of neural processing. These results could help inform interventions, such as desensitization therapy, which may help patients with fragile X syndrome modulate anxiety and arousal associated with eye gaze, thereby improving social functioning.

  11. Desenvolvimento do tomate em diferentes níveis de irrigação e de doses de salinidade

    OpenAIRE

    Silva Junior, Josué Ferreira [UNESP

    2012-01-01

    O tomate é uma das hortaliças mais importante da agroindústria brasileira. Neste estudo, foram testados os efeitos de três doses de salinidade da água de irrigação (0, 3 e 5 dS m-1) e três níveis mínimos de irrigação (-10, -30 e -60 kPa) sobre o desenvolvimento de tomateiro tipo industrial, Kátia. As mudas foram transplantadas em vasos e o cultivo foi em casa de vegetação até a maturação dos frutos. As irrigações foram procedidas por pesagem dos vasos quando atingiam os valores mínimos aproxi...

  12. Fatores que afetam a iniciação de raizes adventicias em caules de Lycopersicon Esculentum mill

    OpenAIRE

    Marlene Aparecida Schiavinato Massei

    1982-01-01

    Resumo: A propagação vegetativa de plantas tem um papel fundamental em horticultura. A capacidade que diferentes espécies ou variedades de plantas tem de desenvolver raízes adventícias varia consideravelmente. O objetivo dessa pesquisa foi analisar a influência de fatores ambientais, nutricionais e hormonais na formação de raízes adventícias em uma região localizada do caule, exposta a alta umidade (alporque). Foram utilizados tomateiros com aproximadamente um mês de idade, plantados em vasos...

  13. Synthesizing seismic fragility of components by use of existing data

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.K; Hofmayer, C.H.

    1987-01-01

    Brookhaven National Laboratory has completed Phase I of the Component Fragility Program and is now performing Phase II. The results of Phase I have been published in NUREG/CR-4659. In both Phases, existing test data for various models are utilized to determine the seismic fragilities of different equipment categories. This represents the first large scale attempt to assemble, compile and interpret the very large heretofore fragmented data base. In Phase I, a methodology has been established to compile the test data for variations of testing methods, vibration inputs, damping values, etc. Test response spectra have been used as a measure of the test vibration inputs. Fragility data have been collected and stored in a computerized data bank at BNL for many electrical and control equipment pieces. The data for motor control centers (MCC) and switchgears have been analyzed in Phase I for determination of the respective fragility levels. Additional test data are being collected in Phase II for these two equipment pieces, as well as other equipment categories. Statistical analyses are also being performed to estimate a single fragility descriptor for each equipment family

  14. Fragile X premutation carriers: A systematic review of neuroimaging findings.

    Science.gov (United States)

    Brown, Stephanie S G; Stanfield, Andrew C

    2015-05-15

    Expansion of the CGG repeat region of the FMR1 gene from less than 45 repeats to between 55 and 200 repeats is known as the fragile X premutation. Carriers of the fragile X premutation may develop a neurodegenerative disease called fragile X-associated tremor/ataxia syndrome (FXTAS). Recent evidence suggests that premutation carriers experience other psychiatric difficulties throughout their lifespan. Medline, EMBASE and PsychINFO were searched for all appropriate English language studies published between January 1990 and December 2013. 419 potentially relevant articles were identified and screened. 19 articles were included in the analysis. We discuss key structural magnetic resonance imaging (MRI) findings such as the MCP sign and white matter atrophy. Additionally, we discuss how functional MRI results have progressed our knowledge of how FXTAS may manifest, including reduced brain activation during social and memory tasks in multiple regions. This systematic review may have been limited by the search for articles on just 3 scientific databases. Differing techniques and methods of analyses between research groups and primary research articles may have caused differences in results between studies. Current MRI studies into the fragile X premutation have been important in the diagnosis of FXTAS and identifying potential pathophysiological mechanisms. Associations with blood based measures have also demonstrated that neurodevelopmental and neurodegenerative aspects of the fragile X premutation could be functionally and pathologically separate. Larger longitudinal studies will be required to investigate these conclusions. Copyright © 2015 Elsevier B.V. All rights reserved.

  15. Developing empirical collapse fragility functions for global building types

    Science.gov (United States)

    Jaiswal, K.; Wald, D.; D'Ayala, D.

    2011-01-01

    Building collapse is the dominant cause of casualties during earthquakes. In order to better predict human fatalities, the U.S. Geological Survey’s Prompt Assessment of Global Earthquakes for Response (PAGER) program requires collapse fragility functions for global building types. The collapse fragility is expressed as the probability of collapse at discrete levels of the input hazard defined in terms of macroseismic intensity. This article provides a simple procedure for quantifying collapse fragility using vulnerability criteria based on the European Macroseismic Scale (1998) for selected European building types. In addition, the collapse fragility functions are developed for global building types by fitting the beta distribution to the multiple experts’ estimates for the same building type (obtained from EERI’s World Housing Encyclopedia (WHE)-PAGER survey). Finally, using the collapse probability distributions at each shaking intensity level as a prior and field-based collapse-rate observations as likelihood, it is possible to update the collapse fragility functions for global building types using the Bayesian procedure.

  16. Laeliocattleya 'Brazilian Girl Rosa': cultivar de orquídea para cultivo em vaso Laeliocattleya 'Brazilian Girl Rosa': orchid cultivar for pot growth

    Directory of Open Access Journals (Sweden)

    Jean C Cardoso

    2010-09-01

    Full Text Available A obtenção de cultivares de espécies ornamentais é estratégica para o desenvolvimento do país e possibilita obter plantas adaptadas às condições climáticas de cultivo. Aproximadamente 200 plantas de um cruzamento intergenérico entre um híbrido de Cattleya e outro de Laelia foram submetidos à seleção massal na fase de desenvolvimento vegetativo, selecionado-se aquelas com desenvolvimento mais rápido e sem sintomas de pragas e doenças. Ao final de três anos e meio, uma planta com características de crescimento vegetativo vigoroso, sem sintomas das principais pragas e doenças da cultura e boa qualidade de floração foi selecionada como nova cultivar Laeliocattleya 'Brazilian Girl Rosa'. A primeira floração demonstrou inflorescência longa e boa arquitetura com quatro flores de diâmetro médio de 11,7 cm, pétalas e sépalas firmes e proporcionais ao tamanho das flores. A coloração predominante das flores foi o rosa, com senescência aos 20 dias após a antese.The generation of cultivars of ornamental species is strategic for the country development and provides the achievement of plants which are adapted to the climatic conditions for cultivation. Nearly 200 plants from an intergeneric cross between a hybrid of Cattleya and another hybrid of Laelia were subjected to mass selection during the vegetative stage. The ones with quick development and without symptoms of pests and diseases were selected. After three and a half years, a plant presenting vigorous vegetative growth, no symptoms of the crop major pests and diseases and high flowering quality was selected as the new 'Brazilian Girl Rosa' cultivar of Laeliocattleya. The first flowering displayed good architecture and a long inflorescence with four flowers presenting an average diameter of 11.7 cm and, also, firm petals and sepals which were proportional to the flower size. Pink was the flower predominant color and the senescence occurred 20 days after anthesis.

  17. Stress analysis in a non axisymmetric loaded reactor pressure vessel; Verificacao de tensoes em um vaso de pressao nuclear com carregamentos nao-axissimetricos

    Energy Technology Data Exchange (ETDEWEB)

    Albuquerque, Levi Barcelos; Assis, Gracia Menezes V. de [Coordenadoria para Projetos Especiais (COPESP), Sao Paulo, SP (Brazil); Miranda, Carlos Alexandre J.; Cruz, Julio Ricardo B.; Mattar Neto, Miguel [Instituto de Pesquisas Energeticas e Nucleares (IPEN), Sao Paulo, SP (Brazil)

    1995-12-31

    In this work we intend to present the stress analysis of a PWR vessel under postulated concentrated loads. The vessel was modeled with Axisymmetric solid 4 nodes harmonic finite elements with the use of the ANSYS program, version 5.0. The bolts connecting the vessel flanges were modeled with beam elements. Some considerations were made to model the contact between the flanges. The perforated part of the vessel tori spherical head was modeled (with reduced properties due to its holes) to introduce its stiffness and loads but was not within the scope of this work. The loading consists of some usual ones, as pressure, dead weight, bolts preload, seismic load and some postulated ones as concentrated loads, over the vessel, modeled by Fourier Series. The results in the axisymmetric model are taken in terms of linearized stresses, obtained in some circumferential positions and for each position, in some sections along the vessel. Using the ASME Code (Section III, Division 1, Sub-section NB) the stresses are within the allowable limits. In order to draw some conclusions about stress linearization, the membrane plus bending stresses (Pl + Pb) are obtained and compared in some sections, using three different methods. (author) 4 refs., 15 figs., 7 tabs.

  18. Stress categorization in nozzle to pressure vessel connections finite elements models; Categorizacao de tensoes em modelos de elementos finitos de conexoes bocal-vaso de pressao

    Energy Technology Data Exchange (ETDEWEB)

    Albuquerque, Levi Barcelos de

    1999-07-01

    The ASME Boiler and Pressure Vessel Code, Section III , is the most important code for nuclear pressure vessels design. Its design criteria were developed to preclude the various pressure vessel failure modes throughout the so-called 'Design by Analysis', some of them by imposing stress limits. Thus, failure modes such as plastic collapse, excessive plastic deformation and incremental plastic deformation under cyclic loading (ratchetting) may be avoided by limiting the so-called primary and secondary stresses. At the time 'Design by Analysis' was developed (early 60's) the main tool for pressure vessel design was the shell discontinuity analysis, in which the results were given in membrane and bending stress distributions along shell sections. From that time, the Finite Element Method (FEM) has had a growing use in pressure vessels design. In this case, the stress results are neither normally separated in membrane and bending stress nor classified in primary and secondary stresses. This process of stress separation and classification in Finite Element (FE) results is what is called stress categorization. In order to perform the stress categorization to check results from FE models against the ASME Code stress limits, mainly from 3D solid FE models, several research works have been conducted. This work is included in this effort. First, a description of the ASME Code design criteria is presented. After that, a brief description of how the FEM can be used in pressure vessel design is showed. Several studies found in the literature on stress categorization for pressure vessel FE models are reviewed and commented. Then, the analyses done in this work are presented in which some typical nozzle to pressure vessel connections subjected to internal pressure and concentrated loads were modeled with solid finite elements. The results from linear elastic and limit load analyses are compared to each other and also with the results obtained by formulae for simple shell geometries (cylinder and sphere). Based on the results comparison, some conclusions and recommendations on the type of FEM (linear elastic or limit load) and on the stress categorization are addressed for the studied cases. (au0010th.

  19. Touch and Massage for Medically Fragile Infants

    Science.gov (United States)

    Livingston, Karen; Beider, Shay; Kant, Alexis J.; Gallardo, Constance C.; Joseph, Michael H.

    2009-01-01

    of the infants receiving massage. Massage in a tertiary urban academic NICU continues to be an area of needed study. Future studies examining infant health outcomes, such as weight gain, decreased length of hospitalization and caregiver–infant bonding, would provide greater insight into the impact of massage for medically fragile infants. PMID:18955228

  20. Aging in Fragile X Premutation Carriers.

    Science.gov (United States)

    Lozano, Reymundo; Saito, Naomi; Reed, Dallas; Eldeeb, Marwa; Schneider, Andrea; Hessl, David; Tassone, Flora; Beckett, Laurel; Hagerman, Randi

    2016-10-01

    It is now recognized that FMR1 premutation carriers (PC) are at risk to develop a range of neurological, psychiatric, and immune-mediated disorders during adulthood. There are conflicting findings regarding the incidence of hypertension, hypothyroidism, diabetes, and cancer in these patients that warrant further study. A retrospective controlled study was performed in a convenience sample of 248 controls (130 men, 118 women) and 397 FMR1 PC with and without fragile X-associated tremor ataxia syndrome (FXTAS) (176 men, 221 women); all participants were at least 45 years old (men: mean 62.4, SD 9.5; women: mean 62.8, SD 9.9; p = 0.63). Memory and cognitive assessments (Wechsler Adult Intelligence Scale (WAIS-III), Wechsler Memory Scale (WMS-III)) and molecular testing (CGG repeats and FMR1-mRNA levels) were performed. Additional data included body mass index (BMI), cholesterol levels, blood pressure, hemoglobin A1c (HbA1c) levels, and medical history. A higher percentage of PC subjects self-reported having a diagnosis of hypertension (50.0 vs. 35.0 %, p = 0.006) and thyroid problems (20.4 vs. 10.0 %, p = 0.012) than control subjects. When comparing controls versus PC with FXTAS, the association was higher for diabetes (p = 0.043); however, the effect was not significant after adjusting for demographic predictors. Blood pressure, blood glucose levels, HbA1c, and BMI values were not significantly different between the two groups. The PC with FXTAS group performed consistently lower in neuropsychological testing compared with the PC without FXTAS group, but the differences were very small for all but the WAIS full-scale IQ. Based on these findings, it appears that the risk for hypertension, thyroid problems, and diabetes may be more frequent in PC with FXTAS, which will require verification in future studies.

  1. A nonsense mutation in FMR1 causing fragile X syndrome

    DEFF Research Database (Denmark)

    Grønskov, Karen; Brøndum-Nielsen, Karen; Dedic, Alma

    2011-01-01

    Fragile X syndrome is a common cause of inherited intellectual disability. It is caused by lack of the FMR1 gene product FMRP. The most frequent cause is the expansion of a CGG repeat located in the 5'UTR of FMR1. Alleles with 200 or more repeats become hypermethylated and transcriptionally silent....... Only few patients with intragenic point mutations in FMR1 have been reported and, currently, routine analysis of patients referred for fragile X syndrome includes solely analysis for repeat expansion and methylation status. We identified a substitution in exon 2 of FMR1, c.80C>A, causing a nonsense...... mutation p.Ser27X, in a patient with classical clinical symptoms of fragile X syndrome. The mother who carried the mutation in heterozygous form presented with mild intellectual impairment. We conclude that further studies including western blot and DNA sequence analysis of the FMR1 gene should...

  2. Normal RNAi response in human fragile x fibroblasts

    DEFF Research Database (Denmark)

    Madsen, Charlotte; Grønskov, Karen; Brøndum-Nielsen, Karen

    2009-01-01

    BACKGROUND: Fragile x syndrome is caused by loss of expression of the FMRP protein involved in the control of a large number of mRNA targets. The Drosophila ortholog dFXR interacts with a protein complex that includes Argonaute2, an essential component of the RNA-induced silencing complex (RISC......). Furthermore dFXR associates with Dicer, another essential processing enzyme of the RNAi pathway. Both microRNA and microRNA precursors can co-immunoprecipitate with dFXR. Consequently it has been suggested that the Fragile x syndrome may be due to a defect in an RNAi-related apparatus. FINDINGS: We have...... investigated the RNAi response in Fragile x patient cells lacking FMRP compared with normal controls. RNAi responses were successfully detected, but no statistically significant difference between the response in normal cells compared to patients cells was found - neither one nor two days after transfection...

  3. Fragile X syndrome in two siblings with major congenital malformations

    Energy Technology Data Exchange (ETDEWEB)

    Giampietro, P.F.; Haas, B.R.; Lipper, E. [Cornell Univ. Medical Center, New York, NY (United States)] [and others

    1996-05-17

    We report on 2 brothers with both fragile X and VACTERL-H syndrome. The first sibling, age 5, had bilateral cleft lip and palate, ventricular septal defect, and a hypoplastic thumb. The second sibling, age 2{1/2}, had a trachesophageal fistula, esophageal atresia, and vertebral abnormality. High-resolution chromosome analysis showed a 46,XY chromosome constitution in both siblings. By PCR and Southern blot analysis, the siblings were found to have large triplet repeat expansions in the fragile X gene (FMR 1) and both had methylation mosaicism. Enzyme kinetic studies of iduronate sulfatase demonstrated a two-fold increase in activity in the first sib as compared to the second. Possible mechanisms through which the fragile X mutation can cause down-regulation of adjacent loci are discussed. 24 refs., 4 figs.

  4. Low Levels of HDL in Fragile X Syndrome Patients.

    Science.gov (United States)

    Lisik, Małgorzata Z; Gutmajster, Ewa; Sieroń, Aleksander L

    2016-02-01

    Fragile X syndrome (FXS) is the most common form of familial mental retardation and one of the leading known causes of autism. The mutation responsible for FXS is a large expansion of the CGG repeats in the promoter region of the FMR1 gene resulting in the transcriptional silencing of the gene in the pathophysiology of Fragile X syndrome was hypothesized. 23 male patients affected by Fragile X syndrome (full mutation in the FMR1 gene) and 24 controls were included in the study. The serum levels of HDL-C were lower in FXS patients (p < 0.001). The serum levels triacylglycerols were higher in FXS patients (p = 0.007) Further study involving larger samples are necessary to confirm the results and define the health implications for abnormal lipid levels in FXS patients.

  5. Fragile X syndrome: A review of clinical management

    Science.gov (United States)

    Lozano, Reymundo; Azarang, Atoosa; Wilaisakditipakorn, Tanaporn; Hagerman, Randi J

    2016-01-01

    Summary The fragile X mental retardation 1 gene, which codes for the fragile X mental retardation 1 protein, usually has 5 to 40 CGG repeats in the 5′ untranslated promoter. The full mutation is the almost always the cause of fragile X syndrome (FXS). The prevalence of FXS is about 1 in 4,000 to 1 in 7,000 in the general population although the prevalence varies in different regions of the world. FXS is the most common inherited cause of intellectual disability and autism. The understanding of the neurobiology of FXS has led to many targeted treatments, but none have cured this disorder. The treatment of the medical problems and associated behaviors remain the most useful intervention for children with FXS. In this review, we focus on the non-pharmacological and pharmacological management of medical and behavioral problems associated with FXS as well as current recommendations for follow-up and surveillance. PMID:27672537

  6. Biodisponibilidade de metais pesados em solos do Rio Grande do Sul

    OpenAIRE

    Daniel Scolmeister

    1999-01-01

    Com o objetivo de avaliar a biodisponibilidade dos metais Cu, Zn, Cd, Ni, Cr e Pb para as plantas de trigo (Triticum aestivum, L.) e milho (Zea mays, L.), foi conduzido em 1996 um experiment0 em vasos utilizando-se dez solos do estado do Rio Grande do Sul, com adição de diferentes níveis destes metais. As quantidades dos metais absorvidas foram correlacionadas com as extraídas do solo porHC10,l mol L-¹, DTPA 5x10-³ mol L¹", HNO3- HClO4, concentrados, resina CHELEX 100 e por um bioteste com pl...

  7. Cultivo de Oncidium baueri Lindley (Orchidaceae) em substratos a base de coco

    OpenAIRE

    Assis,Adriane Marinho de; Faria,Ricardo Tadeu de; Unemoto,Lilian Keiko; Colombo,Larissa Abgariani

    2008-01-01

    Entre os substratos utilizados no cultivo de orquídeas, a fibra de coco vem se destacando como promissor substituto do xaxim, material utilizado por muitos produtores e colecionadores de orquídeas, porém ameaçado de extinção. Objetivou-se, neste trabalho, foi avaliar a eficiência de substratos à base de coco, no cultivo da orquídea Oncidium baueri. As mudas foram cultivadas em vasos de polipropileno, permanecendo em viveiro com 50% de luminosidade. Os tratamentos constituíram-se de xaxim desf...

  8. DNA topoisomerases participate in fragility of the oncogene RET.

    Directory of Open Access Journals (Sweden)

    Laura W Dillon

    Full Text Available Fragile site breakage was previously shown to result in rearrangement of the RET oncogene, resembling the rearrangements found in thyroid cancer. Common fragile sites are specific regions of the genome with a high susceptibility to DNA breakage under conditions that partially inhibit DNA replication, and often coincide with genes deleted, amplified, or rearranged in cancer. While a substantial amount of work has been performed investigating DNA repair and cell cycle checkpoint proteins vital for maintaining stability at fragile sites, little is known about the initial events leading to DNA breakage at these sites. The purpose of this study was to investigate these initial events through the detection of aphidicolin (APH-induced DNA breakage within the RET oncogene, in which 144 APH-induced DNA breakpoints were mapped on the nucleotide level in human thyroid cells within intron 11 of RET, the breakpoint cluster region found in patients. These breakpoints were located at or near DNA topoisomerase I and/or II predicted cleavage sites, as well as at DNA secondary structural features recognized and preferentially cleaved by DNA topoisomerases I and II. Co-treatment of thyroid cells with APH and the topoisomerase catalytic inhibitors, betulinic acid and merbarone, significantly decreased APH-induced fragile site breakage within RET intron 11 and within the common fragile site FRA3B. These data demonstrate that DNA topoisomerases I and II are involved in initiating APH-induced common fragile site breakage at RET, and may engage the recognition of DNA secondary structures formed during perturbed DNA replication.

  9. Protótipo para o diagnóstico do estado dos vasos sanguíneos

    OpenAIRE

    Vale, Ana Catarina Coimbra do

    2008-01-01

    De acordo com estatísticas recentes, as doenças cardiovasculares e as principais complicações associadas, como a Aterosclerose e a Hipertensão Arterial, constituem a principal causa de morte em todo o mundo. Verifica-se ainda uma incidência crescente na população jovem, mas ainda não existem métodos suficientemente eficazes para o diagnóstico precoce de Aterosclerose. Deste modo, o objectivo principal do presente trabalho consiste no desenvolvimento da instrumentação e de um método não-invasi...

  10. Thermalization as an invisibility cloak for fragile quantum superpositions

    Science.gov (United States)

    Hahn, Walter; Fine, Boris V.

    2017-07-01

    We propose a method for protecting fragile quantum superpositions in many-particle systems from dephasing by external classical noise. We call superpositions "fragile" if dephasing occurs particularly fast, because the noise couples very differently to the superposed states. The method consists of letting a quantum superposition evolve under the internal thermalization dynamics of the system, followed by a time-reversal manipulation known as Loschmidt echo. The thermalization dynamics makes the superposed states almost indistinguishable during most of the above procedure. We validate the method by applying it to a cluster of spins ½.

  11. Dysregulation and restoration of translational homeostasis in fragile X syndrome

    Science.gov (United States)

    Richter, Joel D.; Bassell, Gary J.; Klann, Eric

    2015-01-01

    Fragile X syndrome (FXS), the most-frequently inherited form of intellectual disability and the most-prevalent single-gene cause of autism, results from a lack of fragile X mental retardation protein (FMRP), an RNA-binding protein that acts, in most cases, to repress translation. Multiple pharmacological and genetic manipulations that target receptors, scaffolding proteins, kinases and translational control proteins can rescue neuronal morphology, synaptic function and behavioural phenotypes in FXS model mice, presumably by reducing excessive neuronal translation to normal levels. Such rescue strategies might also be explored in the future to identify the mRNAs that are critical for FXS pathophysiology. PMID:26350240

  12. Skeletal stem cells and their contribution to skeletal fragility

    DEFF Research Database (Denmark)

    Aldahmash, A.

    2016-01-01

    Age-related osteoporotic fractures are major health care problem worldwide and are the result of impaired bone formation, decreased bone mass and bone fragility. Bone formation is accomplished by skeletal stem cells (SSC) that are recruited to bone surfaces from bone marrow microenvironment....... This review discusses targeting SSC to enhance bone formation and to abolish age-related bone fragility in the context of using stem cells for treatment of age-related disorders. Recent studies are presented that have demonstrated that SSC exhibit impaired functions during aging due to intrinsic senescence...

  13. Fragile X Syndrome: Keys to the Molecular Genetics of Synaptic Plasticity

    Science.gov (United States)

    Lombroso, Paul J.; Ogren, Marilee P.

    2008-01-01

    Fragile X syndrome, the most common form of inherited mental retardation is discussed. The relationship between specific impairments in synaptic plasticity and Fragile X syndrome is investigated as it strengthens synaptic contacts between neurons.

  14. Un sistema presión-volumen para la medición de propiedades mecánicas de vasos cardíacos menores

    Directory of Open Access Journals (Sweden)

    Javier Navarro Rueda

    2013-04-01

    Full Text Available Las propiedades mecánicas de vasos cardíacos menores (VM son poco estudiadas. El sistema Presión-Volumen (PV propuesto, desarrollado para evaluar comportamientos esfuerzo-deformación (σ-ε de VM, permite variar controladamente la presión interna y simular alteraciones geométricas al tubo. Se obtuvieron imágenes de las geometrías para cada valor de presión interna. Asumiendo el tubo en equilibrio, con pared elástica y presiones conocidas, se determinaron esfuerzo y deformación circunferenciales. Se obtuvieron curvas σ-ε, validadas con ensayos de tensión uniaxial (TU. Aunque se esperaban comportamientos diferentes, los datos experimentales son consistentes frente a resultados TU, con diferencias entre 5.68 y 12.60 %.

  15. Association of the SOD2 polymorphism (Val6Ala and SOD activity with vaso-occlusive crisis and acute splenic sequestration in children with sickle cell anemia

    Directory of Open Access Journals (Sweden)

    Isabela Cristina Cordeiro Farias

    2018-02-01

    Full Text Available The SOD2 polymorphism Val16Ala TàC influences the antioxidative response. This study investigated the association of the SOD2 polymorphism and superoxide dismutase (SOD activity with vaso-occlusive crisis (VOC and acute splenic sequestration (ASS in children with sickle cell anemia (SCA. One hundred ninety-five children aged 1-9 years old were analyzed. The TC and CC genotypes were associated with lower SOD activity compared with the TT genotype (p=0.0321; p=0.0253, respectively. Furthermore, TC/CC were more frequent in patients with VOC or ASS (p=0.0285; p=0.0090, respectively. These results suggest that the SOD2 polymorphism associated with low SOD activity could be involved in SCA physiopathology.

  16. Fentanyl Buccal Tablet: A New Breakthrough Pain Medication in Early Management of Severe Vaso-Occlusive Crisis in Sickle Cell Disease.

    Science.gov (United States)

    De Franceschi, Lucia; Mura, Paolo; Schweiger, Vittorio; Vencato, Elisa; Quaglia, Francesca Maria; Delmonte, Letizia; Evangelista, Maurizio; Polati, Enrico; Olivieri, Oliviero; Finco, Gabriele

    2016-07-01

    Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder. The principal clinical manifestations of SCD are the chronic hemolytic anemia and the acute vaso-occlusive crisis (VOCs), which are mainly characterized by ischemic/reperfusion tissue injury. Pain is the main symptom of VOCs, and its management is still a challenge for hematologists, requiring a multidisciplinary approach. We carried out a crossover study on adult SCD patients, who received two different types of multimodal analgesia during two separate severe VOCs with time interval between VOCs of at least 6 months. The first VOC episode was treated with ketorolac (0.86 mg/kg/day) and tramadol (7.2 mg/kg/day) (TK treatment). In the second VOC episode, fentanyl buccal tablet (FBT; 100 μg) was introduced in a single dose after three hours from the beginning of TK analgesia (TKF treatment). We focused on the first 24 hours of acute pain management. The primary efficacy measure was the time-weighted-sum of pain intensity differences (SPID24). The secondary efficacy measures included the pain intensity difference (PID), the total pain relief (TOTPAR), and the time-wighted sum of anxiety (SAID24). SPID24 was significantly higher in TKF than in TK treatment. All the secondary measures were significantly ameliorated in TKF compared to TK treatment, without major opioid side effects. Patients satisfaction was higher with TKF treatment than with TK one. We propose that VOCs might require breakthrough pain drug strategy as vaso-occlusive phenomena and enhanced vasoconstriction promoting acute ischemic pain component exacerbate the continuous pain of VOCs. FBT might be a powerful and feasible tool in early management of acute pain during VOCs in emergency departments. © 2015 World Institute of Pain.

  17. Use of a clinical pathway to improve the acute management of vaso-occlusive crisis pain in pediatric sickle cell disease.

    Science.gov (United States)

    Ender, Katherine L; Krajewski, Jennifer A; Babineau, John; Tresgallo, Mary; Schechter, William; Saroyan, John M; Kharbanda, Anupam

    2014-04-01

    The most common, debilitating morbidity of sickle cell disease (SCD) is vaso-occlusive crisis (VOC) pain. Although guidelines exist for its management, they are generally not well-followed, and research in other pediatric diseases has shown that clinical pathways improve care. The purpose of our study was to determine whether a clinical pathway improves the acute management of sickle cell vaso-occlusive crisis (VOC) pain in the pediatric emergency department (PED). Pain management practices were prospectively investigated before and after the initiation of a clinical pathway in the PED of an urban, tertiary care center with 50,000 ED visits per year and approximately 200 active sickle cell patients. The pathway included instructions for triage, monitoring, medication administration, and timing of assessments and interventions. Data were eligible from 35 pre-pathway and 33 post-pathway visits. Primary outcome was time interval to administration of first analgesic medication. Statistical analysis was by Student's t-test, using natural-log-transformed data for outcomes with skewed distribution curves. Time interval to first analgesic improved from 74 to 42 minutes (P = 0.012) and to first opioid from 94 to 46 minutes (P = 0.013). The percentage of patients who received ketorolac increased from 57% to 82% (P = 0.03). Decrease in time interval to subsequent pain score assessment was not statistically significant (110 to 72 minutes (P = 0.07)), and change in pain score was not different (P = 0.25). The use of a clinical pathway for sickle cell VOC in the PED can improve important aspects of pain management and merits further investigation and implementation. © 2013 Wiley Periodicals, Inc.

  18. Controle alternativo de nematoide das galhas (Meloidogyne incognita raça 2, com óleos essenciais em solo

    Directory of Open Access Journals (Sweden)

    Francisco José Carvalho Moreira

    2015-09-01

    Full Text Available A ação de produtos naturais sobre fitopatógenos tem sido investigada visando-se avaliar sua eficácia no controle alternativo de doenças, principalmente na agricultura orgânica. O objetivo desse trabalho foi avaliar o efeito dos óleos essenciais de alecrim pimenta (Lippia sidoides e capim citronela (Cymbopogonwinterianus no controle de Meloidogyne incognita raça 2, em tomate (Solanumlycopersicum e celósia (Celosia plicata. Para tanto, conduziu-se ensaio em esquema fatorial 6 x 2, com cinco repetições. O ensaio foi realizado em casa de vegetação do Setor de Fitossanidade do Departamento de Fitotecnia/CCA/UFC, no período de abril a junho de 2007. As mudas utilizadas neste ensaio foram transplantadas para vasos plástico contendo 2 kg de solo estéril, nos quais, 24 horas após o transplantio, foram inoculados com 4.000 ovos/J2 de M. incognita, raça 2, exceto as testemunhas negativas. Em 50% do número de vasos, aplicou-se, logo em seguida, 100 ml das soluções de cada óleo essencial em cada vaso na concentração de 2,5 ml L-1. Esperaram-se mais 48 horas para aplicação da mesma quantidade nos vasos restantes. Este volume corresponde a 60% da capacidade de campo desse substrato, que foi previamente calculada. A avaliação final do ensaio deu-se aos 45 dias após a inoculação. Analisou-se em relação ao nematoide: número de galhas (NG, número de ovos (NO, índice de massas de ovos (IMO, fator de reprodução (FR, redução no fator de reprodução (RFR. Quanto ao desenvolvimento das plantas mensurou-se: altura da planta, massa fresca e seca da parte aérea e massa fresca do sistema radicular. Verificou-se que a reprodução do nematoide, mostrou-se menos eficiente em tomate. Os óleos essenciais empregados reduziram a taxa reprodutiva do nematoide em 83 e 29%, em tomate e celósia, respectivamente. As épocas de aplicação dos óleos essenciais diferiram quanto à reprodução do nematoide, para número de galhas e fator de

  19. Estudo histopatológico de casos de eritema crônico migratório diagnosticados em Manaus

    Directory of Open Access Journals (Sweden)

    Melo Itelvina Suriadakis de

    2003-01-01

    Full Text Available FUNDAMENTOS: O estudo das alterações histopatológicas em relação ao eritema crônico migratório (doença de Lyme e a demonstração do agente etiológico em tecidos são pouco divulgados no Brasil e na Região Norte. OBJETIVOS: Descrever as principais alterações histopatológicas existentes nas biópsias cutâneas de pacientes com diagnóstico clínico de eritema crônico migratório (ECM. Demonstrar a Borrelia sp em cortes histológicos. MATERIAL E MÉTODOS: Biópsias da pele de 31 pacientes foram incluídas em parafina para obtenção de cortes histológicos que foram corados em hematoxilina eosina (HE pelo Warthin Starry e Warthin Starry modificado pH 4.0 (pirocatecol, visando investigar as principais alterações histopatológicas e demonstrar Borrelia sp respectivamente. RESULTADOS: As principais alterações verificadas foram: na epiderme, espongiose em 15 casos (44,12%; na derme, infiltrado constituído por linfócitos, histiócitos e eosinófilos em oito casos (15,69%s. O infiltrado na derme foi encontrado em torno de vasos com disposição em manguito em nove casos (29,04%. A Borrelia sp foi detectada em um caso (3,22% pela técnica de coloração de Warthin Starry. CONCLUSÕES: Os infiltrados na derme, constituídos por linfócitos, histiócitos e eosinófilos apresentavam as seguintes disposições: em torno de vasos em forma de manguito e no interstício; em torno de vasos e no interstício. Essa composição e distribuição foram consideradas de maior relevância para o diagnóstico de ECM. Pela primeira vez, na região amazônica brasileira foi demonstrada a presença de Borrelia sp em biópsia de paciente com suspeita diagnóstica de ECM em corte histológico corado pela técnica de Warthin Starry.

  20. Desempenho cognitivo em pacientes operados de aneurisma cerebral

    Directory of Open Access Journals (Sweden)

    FOBE JEAN-LUC

    1999-01-01

    Full Text Available Vinte e cinco pacientes operados de aneurisma cerebral foram submetidos a avaliação cognitiva pós operatória tardia para linguagem, praxia, orientação, lógica, compreensão, memória, depressão, demência e gnosia visual. Os resultados foram correlacionados com idade, escala de Hunt-Hess à admissão, lado, local e tamanho do aneurisma, sangue à TC (Fisher, vaso-espasmo angiográfico (George. Déficit cognitivo tardio estava ausente em 8 pacientes (32%, era leve em 5 (20%, moderado em 6 (24% e grave em 6 (24%. A função cognitiva isoladamente mais alterada foi a da lógica com acometimento moderado e grave em 7 pacientes (28%, seguida de praxia em 6 (24%, orientação em 5 (20%, linguagem e memória em 4 (16%. A alteração gnósica visual, demência e depressão foram pouco frequentes. A compreensão estava levemente alterada em 3 pacientes. Na faixa etária 25-50 anos ocorreu melhor resultado cognitivo com sequela inexistente ou leve em 9 pacientes (75%. Os aneurismas da artéria comunicante posterior direita apresentaram déficit cognitivo tardio ausente ou leve em 5 pacientes (71,42%, os da artéria cerebral média direita apresentaram resultado equivalente em 2 (66,66%. Os aneurismas de artéria cerebral média esquerda apresentaram o pior resultado com sequela cognitiva moderada e grave em 5 pacientes (71,42%. As sequelas neuropsicológicas tardias apresentaram correlação direta com a idade, Hunt-Hess, sangue à TC, vaso-espasmo angiográfico e localização anatômica do aneurisma.

  1. Frequency of fragile-x in x-linked mental retardation

    African Journals Online (AJOL)

    introduction: Fragile X syndrome (FXS) is the most common form of inher‑ ited mental retardation and accounts for about one third of all cases of X linked mental retardation (XLMR). It is inherited as an X‑linked dominant trait with a fragile site at Xq27.3 locus named fragile X mental retardation gene (FMR‑1). The FMR‑1 ...

  2. Obesity, Food Selectivity, and Physical Activity in Individuals with Fragile X Syndrome

    Science.gov (United States)

    Raspa, Melissa; Bailey, Donald B., Jr.; Bishop, Ellen; Holiday, David; Olmsted, Murrey

    2010-01-01

    National survey data from 884 families were used to examine the overall health of children and adults with fragile X syndrome. Results indicate the rate of obesity in adults with fragile X syndrome is similar to the general population (30%). Male children with fragile X syndrome, however, had higher rates of obesity (31%) when compared with…

  3. Selective Spatial Processing Deficits in an At-Risk Subgroup of the Fragile X Premutation

    Science.gov (United States)

    Hocking, Darren R.; Kogan, Cary S.; Cornish, Kim M.

    2012-01-01

    Until a decade ago, it was assumed that males with the fragile X premutation were unaffected by any cognitive phenotype. Here we examined the extent to which CGG repeat toxicity extends to visuospatial functioning in male fragile X premutation carriers who are asymptomatic for a late-onset neurodegenerative disorder, fragile X-associated…

  4. Heart Activity and Autistic Behavior in Infants and Toddlers with Fragile X Syndrome

    Science.gov (United States)

    Roberts, Jane E.; Tonnsen, Bridgette; Robinson, Ashley; Shinkareva, Svetlana V.

    2012-01-01

    The present study contrasted physiological arousal in infants and toddlers with fragile X syndrome to typically developing control participants and examined physiological predictors early in development to autism severity later in development in fragile X syndrome. Thirty-one males with fragile X syndrome (ages 8-40 months) and 25 age-matched…

  5. Family Environment and Behavior Problems in Children, Adolescents, and Adults with Fragile X Syndrome

    Science.gov (United States)

    Greenberg, Jan S.; Seltzer, Marsha Mailick; Baker, Jason K.; Smith, Leann E.; Warren, Steven F.; Brady, Nancy; Hong, Jinkuk

    2012-01-01

    We examine how the family environment is associated with aspects of the Fragile X syndrome phenotype during childhood, adolescence, and adulthood. Mothers of children (n = 48), adolescents (n = 85), and adults (n = 34) with Fragile X syndrome participated in a multisite study. For children and adults with Fragile X syndrome, the presence of warmth…

  6. Component Fragility Research Program: Phase 1 component prioritization

    International Nuclear Information System (INIS)

    Holman, G.S.; Chou, C.K.

    1987-06-01

    Current probabilistic risk assessment (PRA) methods for nuclear power plants utilize seismic ''fragilities'' - probabilities of failure conditioned on the severity of seismic input motion - that are based largely on limited test data and on engineering judgment. Under the NRC Component Fragility Research Program (CFRP), the Lawrence Livermore National Laboratory (LLNL) has developed and demonstrated procedures for using test data to derive probabilistic fragility descriptions for mechanical and electrical components. As part of its CFRP activities, LLNL systematically identified and categorized components influencing plant safety in order to identify ''candidate'' components for future NRC testing. Plant systems relevant to safety were first identified; within each system components were then ranked according to their importance to overall system function and their anticipated seismic capacity. Highest priority for future testing was assigned to those ''very important'' components having ''low'' seismic capacity. This report describes the LLNL prioritization effort, which also included application of ''high-level'' qualification data as an alternate means of developing probabilistic fragility descriptions for PRA applications

  7. Fiscal deficits, financial fragility, and the effectiveness of government policies

    NARCIS (Netherlands)

    Kirchner, M.; van Wijnbergen, S.

    2012-01-01

    Recent macro developments in the euro area have highlighted the interactions between fiscal policy, sovereign debt, and financial fragility. We take a structural macroeconomic model with frictions in the financial intermediation process, in line with recent research, but introduce asset choice and

  8. Effect of road transport stress on Erthrocyte Osmotic Fragility (EOF ...

    African Journals Online (AJOL)

    After an overnight fast, venous blood was collected from each subject for the determination of serum cortisol, glucose concentration and erythrocyte osmotic fragility. The subjects were then transported at a speed of 65 – 75Km/h covering a distance of 180km. Thereafter venous blood was again collected (within 10 minutes) ...

  9. Cellular Basis for Learning Impairment in Fragile X Syndrome

    Science.gov (United States)

    2015-08-01

    intellectual disability syndromes in general as well as learning impairments in other autism spectrum disorders . This knowledge will be necessary for the...17 4 1. INTRODUCTION Fragile X syndrome is the most common inherited form of intellectual disability. The disorder is caused by mutation in the...the leading cause of inherited intellectual disability and results in cognitive impairment, hyperactivity, attention deficits, seizure disorders

  10. The patterns of Osmotic Fragility and Thrombocytopenia in Nigerian ...

    African Journals Online (AJOL)

    Thrombocytopenia in severe malaria was significantly higher than that in uncomplicated malaria (X2 = 6.4 – 13.7; P < 0.05). At day 3 to 4 of treatment with quinine, parasitaemia became zero in all the malaria subjects but resolution of thrombocytopenia and osmotic fragility (except in uncomplicated malaria) to normalcy was ...

  11. Veteran Status, Race-Ethnicity, and Marriage among Fragile Families

    Science.gov (United States)

    Usdansky, Margaret L.; London, Andrew S.; Wilmoth, Janet M.

    2009-01-01

    We used data from the Fragile Families and Child Wellbeing Study (N = 2,679 ) to examine the impact of men's past military service on the likelihood that a couple will marry within 5 years of a nonmarital birth. Logistic regression analyses showed that men's past military service increased marriage odds by 54% for couples with Black fathers even…

  12. Fragile X syndrome: Current insight | Dean | Egyptian Journal of ...

    African Journals Online (AJOL)

    Fragile X syndrome (FXS) is a multigenerational disorder having massive adverse effect not only on the individuals but also on their families. It is the most common type of intellectual disability after Down's syndrome. Over two decades have passed since the discovery of FMR1, the causal gene for FXS, but still little is known ...

  13. Therapeutic Targets and Translational Endpoints in Fragile X Syndrome

    NARCIS (Netherlands)

    C.E.F. de Esch (Celine)

    2014-01-01

    markdownabstract__Abstract__ Fragile X syndrome is the most common inherited cause of intellectual disability. It is more common in boys (1 in 4000) than girls (1 in 6000). In addition to the intellectual disability patients often exhibit behavioral abnormalities including hyperactivity and

  14. Emerging Powers and Effective Governance in Fragile States | IDRC ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    New and emerging development organizations are providing assistance to countries experiencing tensions and challenges after periods of conflict. This research will test whether their development assistance approaches are more effective at addressing the political and social realities of fragile states than those used by ...

  15. Improved Erythrocyte Osmotic Fragility and Packed Cell Volume ...

    African Journals Online (AJOL)

    Aloe barbadensis is a popular house plant that has a long history of a multipurpose folk remedy. It has been documented to have anti-diabetic, antiseptic and anti-inflammatory effects. The effect of Aloe barbadensis juice extract on erythrocyte osmotic fragility, packed cell volume and haemoglobin concentration in Wistar rats ...

  16. Predicting The Fragility Of Renal Calculi In Response To Shock ...

    African Journals Online (AJOL)

    ray film of the kidneys, ureters and bladder (KUB) area as predictors of stone fragility during shock wave lithotripsy (SWL). Patients and Methods: This prospective study included 336 patients who had a single renal pelvic stone .20 mm and ...

  17. Molecular characterization of X chromosome fragility in idiopathic ...

    African Journals Online (AJOL)

    Heba Alla Hosny Omar

    2015-11-23

    Nov 23, 2015 ... Abstract Background: Fragile X syndrome (FXS) is the most common form of inherited mental retardation. Frequency of .... studies, without being tested for this particular genetic defect, and second, obviating the ..... spectrum disorders, repetitive and disorderly language have been found to be common in ...

  18. Erythrocyte osmotic fragility of pigs administered ascorbic acid and ...

    African Journals Online (AJOL)

    The experiment was carried out with the aim of investigating the effect of an antioxidant ascorbic acid on erythrocyte osmotic fragility of pigs transported by road for 4 h during the harmattan season. 16 pigs administered with ascorbic acid at the dose of 250 mg/kg per os and individually served as experimental animals and ...

  19. Fragile X syndrome: From protein function to therapy.

    Science.gov (United States)

    Bagni, Claudia; Oostra, Ben A

    2013-11-01

    Fragile X syndrome (FXS) is the leading monogenic cause of intellectual disability and autism. The FMR1 gene contains a CGG repeat present in the 5'-untranslated region which can be unstable upon transmission to the next generation. The repeat is up to 55 CGGs long in the normal population. In patients with fragile X syndrome (FXS), a repeat length exceeding 200 CGGs generally leads to methylation of the repeat and the promoter region, which is accompanied by silencing of the FMR1 gene. The disease is a result of lack of expression of the fragile X mental retardation protein leading to severe symptoms, including intellectual disability, hyperactivity, and autistic-like behavior. The FMR1 protein (FMRP) has a number of functions. The translational dysregulation of a subset of mRNAs targeted by FMRP is probably the major contribution to FXS. FMRP is also involved in mRNA transport to synapses where protein synthesis occurs. For some FMRP-bound mRNAs, FMRP is a direct modulator of mRNA stability either by sustaining or preventing mRNA decay. Increased knowledge about the role of FMRP has led to the identification of potential treatments for fragile X syndrome that were often tested first in the different animal models. This review gives an overview about the present knowledge of the function of FMRP and the therapeutic strategies in mouse and man. © 2013 Wiley Periodicals, Inc.

  20. Fragile X-associated tremor/ataxia syndrome.

    Science.gov (United States)

    Hoem, Gry; Koht, Jeanette

    2017-10-31

    Fragile X-associated tremor/ataxia syndrome (FXTAS) is a hereditary neurodegenerative disorder caused by a mutation on the X chromosome. The major signs and symptoms are tremor, ataxia and parkinsonism. Up to one in 2 000 persons over 50 years of age will develop the syndrome. There is reason to believe that too few individuals in Norway undergo testing for this condition.

  1. Experimental approaches towards therapeutic interventions for fragile X syndrome

    NARCIS (Netherlands)

    G.J. Levenga (Josien)

    2010-01-01

    textabstractFragile X syndrome (FXS) is one of the most common inherited forms of intellectual disability. It affects on average 1/4000 males and 1/7000 females. FXS was described for the first time in 1943 by Martin and Bell. They reported a family with an inherited form of mental retardation

  2. Attentional set-shifting in fragile X syndrome

    NARCIS (Netherlands)

    van der Molen, M.J.W.; van der Molen, M.W.; Ridderinkhof, K.R.; Hamel, B.C.J.; Curfs, L.M.G.; Ramakers, G.J.A.

    2012-01-01

    The ability to flexibly adapt to the changing demands of the environment is often reported as a core deficit in fragile X syndrome (FXS). However, the cognitive processes that determine this attentional set-shifting deficit remain elusive. The present study investigated attentional set-shifting

  3. Gender Differences in Repetitive Language in Fragile X Syndrome

    Science.gov (United States)

    Murphy, M. M.; Abbeduto, L.

    2007-01-01

    Background: Verbal perseveration (i.e. excessive self-repetition) is a characteristic of male individuals with fragile X syndrome; however, little is known about its occurrence among females or its underlying causes. This project examined the relationship between perseveration and (1) gender, (2) cognitive and linguistic ability, and (3) language…

  4. Noncomprehension Signaling in Males and Females with Fragile X Syndrome

    Science.gov (United States)

    Thurman, Angela John; Kover, Sara T.; Brown, W. Ted; Harvey, Danielle J.; Abbeduto, Leonard

    2017-01-01

    Purpose: This study used a prospective longitudinal design to evaluate the trajectory and predictors of noncomprehension signaling in male and female youth with fragile X syndrome (FXS). Method: A direction-following task in which some of the directions were inadequate was administered. Participants were 52 youth (36 boys, 16 girls) with FXS. Upon…

  5. Growth of Expressive Syntax in Children with Fragile X Syndrome

    Science.gov (United States)

    Komesidou, Rouzana; Brady, Nancy C.; Fleming, Kandace; Esplund, Amy; Warren, Steven F.

    2017-01-01

    Purpose: This research explored syntactic growth in children with fragile X syndrome (FXS) over a 5-year period, and variability in growth in relation to autism symptoms, nonverbal cognition, maternal responsivity, and gender. Method: Language samples at 4 time points from 39 children with FXS, 31 boys and 8 girls, were analyzed using the Index of…

  6. Language Development in Individuals with Fragile X Syndrome

    Science.gov (United States)

    Finestack, Lizbeth H.; Richmond, Erica K.; Abbeduto, Leonard

    2009-01-01

    Fragile X syndrome (FXS) is the leading inherited cause of intellectual disability. The syndrome is caused by a single gene mutation on the X chromosome. Although individual differences are large, most individuals with FXS display weaknesses across all language and literacy domains compared with peers of the same chronological age with typical…

  7. Auditory Brainstem Responses in Young Males with Fragile X Syndrome

    Science.gov (United States)

    Roberts, Joanne; Hennon, Elizabeth A.; Anderson, Kathleen; Roush, Jackson; Gravel, Judith; Skinner, Martie; Misenheimer, Jan; Reitz, Patricia

    2005-01-01

    Fragile X syndrome (FXS) is the most common inherited cause of mental retardation resulting in developmental delays in males. Atypical outer ear morphology is characteristic of FXS and may serve as a marker for abnormal auditory function. Despite this abnormality, studies of the hearing of young males with FXS are generally lacking. A few studies…

  8. Fragile X Syndrome: A Guide for Teachers of Young Children.

    Science.gov (United States)

    Mazzocco, Michele M. M.; O'Connor, Rebecca

    1993-01-01

    Fragile X Syndrome is an inherited condition often causing mental retardation in adolescence but difficult to detect in childhood. Early detection allows therapeutic intervention and alerts parents to seek genetic counseling. Symptoms warranting a referral include language delay, autistic behaviors, hand-biting, oversensitivity to touch, large…

  9. Seismic fragility capacity of equipment--horizontal shaft pump test

    International Nuclear Information System (INIS)

    Iijima, T.; Abe, H.; Suzuki, K.

    2005-01-01

    The current seismic fragility capacity of horizontal shaft pump is 1.6 x 9.8 m/s 2 (1.6 g), which was decided from previous vibration tests and we believe that it must have sufficient margin. The purpose of fragility capacity test is to obtain realistic seismic fragility capacity of horizontal shaft pump by vibration tests. Reactor Building Closed Cooling Water (RCW) Pump was tested as a typical horizontal shaft pump, and then bearings and liner rings were tested as important parts to evaluate critical acceleration and dispersion. Regarding RCW pump test, no damage was found, though maximum input acceleration level was 6 x 9.8 m/s 2 (6 g). Some kinds of bearings and liner rings were tested on the element test. Input load was based on seismic motion which was same with the RCW pump test, and maximum load was equivalent to over 20 times of design seismic acceleration. There was not significant damage that caused emergency stop of pump but degradation of surface roughness was found on some kinds of bearings. It would cause reduction of pump life, but such damage on bearings occurred under large seismic load condition that was equivalent to over 10 to 20 g force. Test results show that realistic fragility capacity of horizontal shaft pump would be at least four times as higher as current value which has been used for our seismic PSA. (authors)

  10. State fragility and its regional implications for peace and stability

    DEFF Research Database (Denmark)

    Mandrup, Thomas

    , fragile states and bad governance regional in nature, and cannot be solved by the individual states alone. Regional institutions have been in a weak position dealing with these challenges, and attempts have been to strengthen the capacity of these regional institutions. This paper investigates...

  11. Inter-state Variation in the Burden of Fragility Fractures

    Science.gov (United States)

    Demographic differences may produce inter-state variation in the burden of osteoporosis. The objective of this study was to estimate the burden of fragility fractures by race/ethnicity, age, sex, and service site across 5 diverse and populous states. State inpatient databases for 2000 were used to ...

  12. Seismic fragility of ventilation stack of nuclear power plant

    International Nuclear Information System (INIS)

    Nefedov, S.S.; Yugai, T.Z.; Kalinkin, I.V.; Vizir, P.L.

    2003-01-01

    Fragility study of safety related elements is necessary step in seismic PSA of nuclear power plant (NPP). In present work fragility was analyzed after the example of the ventilation stack of NPP. Ventilation stack, considered in present work, is a separately erected construction with height of 100 m made of cast-in-place reinforced concrete. In accordance with IAEA terminology fragility of element is defined as conditional probability of its failure at given level of seismic loading. Failure of a ventilation stack was considered as development of the plastic hinge in some section of a shaft. Seismic ground acceleration a, which corresponds to failure, could be defined as limit seismic acceleration of ventilation stack [a]. Limit seismic acceleration [a] was considered as random value. Sources of its variation are connected with stochastic nature of factors determining it (properties of construction materials, soils etc.), and also with uncertainties of existing analytical techniques. Random value [a] was assumed to be distributed lognormally. Median m[a] and logarithmically standard deviation β of this distribution were defined by 'scaling method' developed by R.P. Kennedy et al. Using this values fragility curves were plotted for different levels of confidence probability. (author)

  13. Social Approach and Emotion Recognition in Fragile X Syndrome

    Science.gov (United States)

    Williams, Tracey A.; Porter, Melanie A.; Langdon, Robyn

    2014-01-01

    Evidence is emerging that individuals with Fragile X syndrome (FXS) display emotion recognition deficits, which may contribute to their significant social difficulties. The current study investigated the emotion recognition abilities, and social approachability judgments, of FXS individuals when processing emotional stimuli. Relative to…

  14. Distinguishing Features of Autism in Boys with Fragile X Syndrome

    Science.gov (United States)

    Brock, M.; Hatton, D.

    2010-01-01

    Background: Males with fragile X syndrome and autism (FXS/autism) represent a distinct subgroup of males with FXS at risk for markedly poorer outcomes. Early identification and intervention can improve outcomes for males with autism spectrum disorder. Method: To advance the development of a specialised autism screening tool for young males with…

  15. Social Cognition in Adolescent Girls with Fragile X Syndrome

    Science.gov (United States)

    Turkstra, Lyn S.; Abbeduto, Leonard; Meulenbroek, Peter

    2014-01-01

    This study aimed to characterize social cognition, executive functions (EFs), and everyday social functioning in adolescent girls with fragile X syndrome, and identify relationships among these variables. Participants were 20 girls with FXS and 20 age-matched typically developing peers. Results showed significant between-groups differences in…

  16. Infant Development in Fragile X Syndrome: Cross-Syndrome Comparisons

    Science.gov (United States)

    Roberts, Jane E.; McCary, Lindsay M.; Shinkareva, Svetlana V.; Bailey, Donald B., Jr.

    2016-01-01

    This study examined the developmental profile of male infants with fragile X syndrome (FXS) and its divergence from typical development and development of infants at high risk for autism associated with familial recurrence (ASIBs). Participants included 174 boys ranging in age from 5 to 28 months. Cross-sectional profiles on the Mullen Scales of…

  17. Seizures in Fragile X Syndrome: Characteristics and Comorbid Diagnoses

    Science.gov (United States)

    Berry-Kravis, Elizabeth; Raspa, Melissa; Loggin-Hester, Lisa; Bishop, Ellen; Holiday, David; Bailey, Donald B., Jr.

    2010-01-01

    A national survey of caregivers of individuals with fragile X syndrome addressed characteristics of epilepsy and co-occurring conditions. Of the 1,394 individuals (1,090 males and 304 females) with the full mutation, 14% of males and 6% of females reported seizures. Seizures were more often partial, began between ages 4 and 10 years, and were…

  18. Parenting Young Children with and without Fragile X Syndrome

    Science.gov (United States)

    Sterling, Audra; Barnum, Leah; Skinner, Debra; Warren, Steven F.; Fleming, Kandace

    2012-01-01

    The purpose of this study was to examine maternal parenting styles across age-matched siblings using a within-family design, in which one child has Fragile X syndrome. Thirteen families participated; children were aged 16 to 71 months. Mothers completed several videotaped activities with each child separately as well as an interview. Mothers used…

  19. Developmental Trajectories of Young Girls with Fragile X Syndrome

    Science.gov (United States)

    Hatton, Deborah D.; Wheeler, Anne; Sideris, John; Sullivan, Kelly; Reichardt, Alison; Roberts, Jane; Clark, Renee; Bailey, Donald B., Jr.

    2009-01-01

    To describe the early phenotype of girls with full mutation fragile X, we used 54 observations of 15 girls between the ages of 6 months and 9 years to examine developmental trajectories as measured by the Battelle Development Inventory. In this sample, autistic behavior was associated with poorer developmental outcomes, primarily due to…

  20. Reading and Phonological Skills in Boys with Fragile X Syndrome

    Science.gov (United States)

    Klusek, Jessica; Hunt, Anna W.; Mirrett, Penny L.; Hatton, Deborah D.; Hooper, Stephen R.; Roberts, Jane E.; Bailey, Donald B.

    2015-01-01

    Although reading skills are critical for the success of individuals with intellectual disabilities, literacy has received little attention in fragile X syndrome (FXS). This study examined the literacy profile of FXS. Boys with FXS (n = 51; mean age 10.2 years) and mental age-matched boys with typical development (n = 35) participated in…

  1. Brief Report: Autism Symptoms in Infants with Fragile X Syndrome

    Science.gov (United States)

    Roberts, Jane E.; Tonnsen, Bridgette L.; McCary, Lindsay M.; Caravella, Kelly E.; Shinkareva, Svetlana V.

    2016-01-01

    Fragile X syndrome (FXS) is the most common known genetic cause of autism spectrum disorder (ASD). Although 50-75% of children with FXS meet ASD criteria, no studies have compared ASD symptoms in infants with FXS versus other high risk groups, such as siblings of children with ASD (ASIBs). Using the Autism Observation Scale for Infants, our…

  2. Phonological Awareness and Reading in Boys with Fragile X Syndrome

    Science.gov (United States)

    Adlof, Suzanne M.; Klusek, Jessica; Shinkareva, Svetlana V.; Robinson, Marissa L.; Roberts, Jane E.

    2015-01-01

    Background: Reading delays are well documented in children with fragile X syndrome (FXS), but few studies have examined linguistic precursors of reading in this population. This study examined the longitudinal development of phonological awareness and its relationship with basic reading in boys with FXS. Individual differences in genetic,…

  3. Implicit Procedural Learning in Fragile X and Down Syndrome

    Science.gov (United States)

    Bussy, G.; Charrin, E.; Brun, A.; Curie, A.; des Portes, V.

    2011-01-01

    Background: Procedural learning refers to rule-based motor skill learning and storage. It involves the cerebellum, striatum and motor areas of the frontal lobe network. Fragile X syndrome, which has been linked with anatomical abnormalities within the striatum, may result in implicit procedural learning deficit. Methods: To address this issue, a…

  4. The Social Consequences of Raising Medically Fragile and ...

    African Journals Online (AJOL)

    2011-10-02

    Oct 2, 2011 ... comfort could not be achieved, families were likely to limit social activities so that the child who was medically ... The Social Consequences of Raising Medically Fragile and Developmentally Challenged Children in Ghana may prevent ..... Image; “Journal of Nursing Scholarship”,(31): 209-214. DeSantis, L.

  5. Fragile X Syndrome : the quest for targeted treatment

    NARCIS (Netherlands)

    S. Zeidler (Shimriet)

    2017-01-01

    markdownabstractFragile X syndrome is the most common hereditary cause of intellectual disability and autism. Currently, no targeted treatment is available. The identification of its causative gene, followed by the generation of animal models, has boosted research options. The past decades,

  6. Hippocampal dysfunction and cognitive impairment in Fragile-X Syndrome.

    Science.gov (United States)

    Bostrom, Crystal; Yau, Suk-Yu; Majaess, Namat; Vetrici, Mariana; Gil-Mohapel, Joana; Christie, Brian R

    2016-09-01

    Fragile-X Syndrome (FXS) is the most common form of inherited intellectual disability and the leading genetic cause of autism spectrum disorder. FXS is caused by transcriptional silencing of the Fragile X Mental Retardation 1 (Fmr1) gene due to a CGG repeat expansion, resulting in the loss of Fragile X Mental Retardation Protein (FMRP). FMRP is involved in transcriptional regulation and trafficking of mRNA from the nucleus to the cytoplasm and distal sites both in pre- and post-synaptic terminals. Consequently, FXS is a multifaceted disorder associated with impaired synaptic plasticity. One region of the brain that is significantly impacted by the loss of FMRP is the hippocampus, a structure that plays a critical role in the regulation of mood and cognition. This review provides an overview of the neuropathology of Fragile-X Syndrome, highlighting how structural and synaptic deficits in hippocampal subregions, including the CA1 exhibiting exaggerated metabotropic glutamate receptor dependent long-term depression and the dentate gyrus displaying hypofunction of N-methyl-d-aspartate receptors, contribute to cognitive impairments associated with this neurodevelopmental disorder. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Arousal Modulation in Females with Fragile X or Turner Syndrome

    Science.gov (United States)

    Roberts, Jane; Mazzocco, Michele M. M.; Murphy, Melissa M.; Hoehn-Saric, Rudolf

    2008-01-01

    The present study was carried out to examine physiological arousal modulation (heart activity and skin conductance), across baseline and cognitive tasks, in females with fragile X or Turner syndrome and a comparison group of females with neither syndrome. Relative to the comparison group, for whom a greater increase in skin conductance was…

  8. White Religious Educators Resisting White Fragility: Lessons from Mystics

    Science.gov (United States)

    Hess, Mary E.

    2017-01-01

    Decades of work in dismantling racism have not yielded the kind of results for which religious educators have hoped. One primary reason has been what scholars term "white fragility," a symptom of the structural racism which confers systemic privilege upon White people. Lessons learned from Christian mystics point to powerful ways to…

  9. Caracterização e aproveitamento de água cinza para uso não-potável em edificações

    OpenAIRE

    Bazzarella, Bianca Barcellos

    2005-01-01

    Uma das formas de se conservar água nas residências é utilizando fontes alternativas de suprimento. O reúso de águas cinza para fins não-potáveis, tais como lavagem de veículos, rega de jardins e descarga de vasos sanitários, é apenas um exemplo. Este trabalho tem como objetivo pesquisar alternativas para redução do consumo de água potável e da produção de esgotos sanitários em edificações, a partir do aproveitamento do tratamento e reúso de águas cinza em descargas de vasos sanitários. Pa...

  10. Clinical assessment tools identify functional deficits in fragility fracture patients

    Directory of Open Access Journals (Sweden)

    Ames TD

    2016-05-01

    Full Text Available Tyler D Ames,1 Corinne E Wee,1 Khoi M Le,1 Tiffany L Wang,1 Julie Y Bishop,2 Laura S Phieffer,2 Carmen E Quatman2 1The Ohio State University College of Medicine, 2Department of Orthopaedics, The Ohio State University Wexner Medical Center, Columbus, OH, USA Purpose: To identify inexpensive, noninvasive, portable, clinical assessment tools that can be used to assess functional performance measures that may put older patients at risk for falls such as balance, handgrip strength, and lumbopelvic control.Patients and methods: Twenty fragility fracture patients and 21 healthy control subjects were evaluated using clinical assessment tools (Nintendo Wii Balance Board [WBB], a handheld dynamometer, and an application for the Apple iPod Touch, the Level Belt that measure functional performance during activity of daily living tasks. The main outcome measurements were balance (WBB, handgrip strength (handheld dynamometer, and lumbopelvic control (iPod Touch Level Belt, which were compared between fragility fracture patients and healthy controls.Results: Fragility fracture patients had lower scores on the vertical component of the WBB Torso Twist task (P=0.042 and greater medial–lateral lumbopelvic sway during a 40 m walk (P=0.026 when compared to healthy controls. Unexpectedly, the fracture patients had significantly higher scores on the left leg (P=0.020 and total components (P=0.010 of the WBB Single Leg Stand task as well as less faults during the left Single Leg Stand task (P=0.003.Conclusion: The clinical assessment tools utilized in this study are relatively inexpensive and portable tools of performance measures capable of detecting differences in postural sway between fragility fracture patients and controls. Keywords: fall risk, geriatric fracture, Nintendo Wii Balance Board, Level Belt, fragility fracture

  11. Fragility fractures at Auckland City Hospital: we can do better.

    Science.gov (United States)

    Braatvedt, Geoffrey; Wilkinson, Susan; Scott, Marilyn; Mitchell, Paul; Harris, Roger

    2017-12-01

    This study describes in detail the burden of caring for patients aged ≥ 50 years seen in one year with a fragility fracture in a large urban environment and shows that these fractures result in a long length of stay and significant mortality. Intervention to prevent further fracture was poorly done. To examine the epidemiology of fragility fracture in patients over age 50 years and record the number who received appropriate secondary prevention treatment. All patients aged ≥ 50 years presenting with a fracture during the 12 months following July 1 st 2011, to Auckland City Hospital or residing in central Auckland at the time of their fracture, were identified from hospital and Accident Compensation Corporation records. A random sample of 55% of these patient's records were reviewed to establish the type of fracture, prior fracture and falls history, and use of bisphosphonates in the 12 months before presentation. Their length of stay (LOS) by type of fracture was recorded. The use of bisphosphonate drugs in the following 12 months was obtained from centralised national records of prescriptions. 2729 patients aged ≥ 50 years presented with a fragility fracture in the central Auckland region in one year. Fifty-six percent of these patients were seen at Auckland Hospital and of these, 82% patients required admission with a mean LOS of 20 days (SD ± 24 days).The remaining 44% of patients were looked after in the private outpatient sector. Approximately 30% of the admissions were for hip fracture. Sixty-four percent of patients with a fragility fracture did not receive a potent bisphosphonate, 12% were considered not appropriate for treatment, and 24% received a potent bisphosphonate during their admission or in the next 12 months. Approximately 1 in 18 people aged ≥ 50 years presented in one year with a fragility fracture.Secondary prevention strategies were poorly implemented. Additional resources for identifying and initiating secondary fracture prevention

  12. Insetos (Arthropoda, Insecta) em inflorescências de Heliconia bihai (L.) L. (Heliconiaceae)

    OpenAIRE

    THAIS RANIELLE SOUZA DE OLIVEIRA; DANYELLY CRYSTYNE DE ARAÚJO SENA; VIVIAN LOGES; CLAUDIO AUGUSTO GOMES DA CAMARA; ALEUNY COUTINHO REIS

    2010-01-01

    As espécies do gênero Heliconia L. (Heliconiaceae) se destacam por apresentar inflorescências eretas ou pendentes, muito apreciadas por sua beleza. São muito utilizadas no paisagismo, uma vez que exigem pouca manutenção e são adequadas para uso em vasos ou em canteiros. No entanto, vários grupos de insetos (Arthropoda, Insecta) estão associados à Heliconia, tais como besouros (Coleoptera), formigas (Hymenoptera, Formicidae), pulgões (Hemiptera, Aphidoidea) e cochonilhas (Hemiptera, Coccoide...

  13. Equipment fragility data base. Seismic Safety Margins Research Program

    Energy Technology Data Exchange (ETDEWEB)

    Cover, L.E.

    1983-01-10

    Part of the effort of the Seismic Safety Margins Research Program (SSMRP) has been directed at generating a fragility data base for equipment used in control and safety systems in commercial nuclear power plants. Component fragility data have been compiled in various forms, depending on their content, intended use, and level of reduction. The data are stored in a relational data base on the LLNL CEC 7600 computers; this provides easy accessibility for LLNL computer users. This report describes the present structure of the data base and presents its contents through the use of tables. This report is a revision of an earlier one of the same name and numbers (NUREG/CR-2680) and (UCRL-53038). Additional data have been included and the presentation has been revised to enhance its usability.

  14. Representation and propagation of uncertainty in seismic fragilities

    International Nuclear Information System (INIS)

    Phillips, D.W.

    1983-01-01

    Probabilistic seismic risk assessment involves the estimation of site seismic hazard for very low annual exceedance frequencies, and plant failure probabilities for beyond design basis seismic loading. Both of these estimates naturally involve uncertainties, and the way in which the uncertainties are represented can affect significantly the overall assessed seismic risk. To date, the usual representation of uncertainty in seismic fragility has been the log-normal distribution, although other analytic representations are equally consistent with the available seismic fragility information in many instances. The influence of such alternative forms of uncertainty representation is examined and, in addition, the compounding of these influences by propagation of the uncertainties through event trees or fault trees is discussed in the context of general methods of propagation

  15. Finding FMR1 mosaicism in Fragile X syndrome.

    Science.gov (United States)

    Gonçalves, Thaís Fernandez; dos Santos, Jussara Mendonça; Gonçalves, Andressa Pereira; Tassone, Flora; Mendoza-Morales, Guadalupe; Ribeiro, Márcia Gonçalves; Kahn, Evelyn; Boy, Raquel; Pimentel, Márcia Mattos Gonçalves; Santos-Rebouças, Cíntia Barros

    2016-01-01

    Almost all patients with Fragile X Syndrome (FXS) exhibit a CGG repeat expansion (full mutation) in the Fragile Mental Retardation 1 gene (FMR1). Here, the authors report five unrelated males with FXS harboring a somatic full mutation/deletion mosaicism. Mutational profiles were only elucidated by using a combination of molecular approaches (CGG-based PCR, Sanger sequencing, MS-MLPA, Southern blot and mPCR). Four patients exhibited small deletions encompassing the CGG repeats tract and flanking regions, whereas the remaining had a larger deletion comprising at least exon 1 and part of intron 1 of FMR1 gene. The presence of a 2-3 base pairs microhomology in proximal and distal non-recurrent breakpoints without scars supports the involvement of microhomology mediated induced repair (MMBIR) mechanism in three small deletions. The authors data highlights the importance of using different research methods to elucidate atypical FXS mutational profiles, which are clinically undistinguishable and may have been underestimated.

  16. On the importance of uncertain factors in seismic fragility assessment

    International Nuclear Information System (INIS)

    Borgonovo, E.; Zentner, I.; Pellegri, A.; Tarantola, S.; Rocquigny, E. de

    2013-01-01

    This paper addresses the definition of importance measures for helping the modeller to detect the factors on which to focus modelling activity and data collection in seismic fragility analysis. We study sensitivity measures consistent with the decision-support criteria of interest, namely, the (mean) fragility curve and the “High Confidence of Low Probability of Failure” (HCLPF) value. The importance measures are obtained analytically for the EPRI safety factor method, which is nowadays used worldwide for seismic risk assessment of nuclear plants. We illustrate and discuss the use of both variance-based and CDF-based importance measures in the application to two case studies, the first analytical and based on the EPRI method, the second numerical.

  17. Mechanisms of diabetes mellitus-induced bone fragility

    DEFF Research Database (Denmark)

    Napoli, Nicola; Chandran, Manju; Pierroz, Dominique D

    2017-01-01

    The risk of fragility fractures is increased in patients with either type 1 diabetes mellitus (T1DM) or type 2 diabetes mellitus (T2DM). Although BMD is decreased in T1DM, BMD in T2DM is often normal or even slightly elevated compared with an age-matched control population. However, in both T1DM...... and T2DM, bone turnover is decreased and the bone material properties and microstructure of bone are altered; the latter particularly so when microvascular complications are present. The pathophysiological mechanisms underlying bone fragility in diabetes mellitus are complex, and include hyperglycaemia......-induced hypoglycaemia, certain antidiabetic medications with a direct effect on bone and mineral metabolism (such as thiazolidinediones), as well as an increased propensity for falls, all contribute to the increased fracture risk in patients with diabetes mellitus....

  18. Of Men and Mice: Modeling the Fragile X Syndrome

    Science.gov (United States)

    Dahlhaus, Regina

    2018-01-01

    The Fragile X Syndrome (FXS) is one of the most common forms of inherited intellectual disability in all human societies. Caused by the transcriptional silencing of a single gene, the fragile x mental retardation gene FMR1, FXS is characterized by a variety of symptoms, which range from mental disabilities to autism and epilepsy. More than 20 years ago, a first animal model was described, the Fmr1 knock-out mouse. Several other models have been developed since then, including conditional knock-out mice, knock-out rats, a zebrafish and a drosophila model. Using these model systems, various targets for potential pharmaceutical treatments have been identified and many treatments have been shown to be efficient in preclinical studies. However, all attempts to turn these findings into a therapy for patients have failed thus far. In this review, I will discuss underlying difficulties and address potential alternatives for our future research. PMID:29599705

  19. Fragility and structure of Al-Cu alloy melts

    International Nuclear Information System (INIS)

    Lv Xiaoqian; Bian Xiufang; Mao Tan; Li Zhenkuan; Guo Jing; Zhao Yan

    2007-01-01

    The dynamic viscosity measurements are performed for Al-Cu alloy melts with different compositions using an oscillating-cup viscometer. The results show that the viscosities of Al-Cu alloy melts increase with the copper content increasing, and also have a correlation with the correlation radius of clusters, which is measured by the high-temperature X-ray diffractometer. It has also been found that the fragilities of superheated melts (M) of hypereutectic Al-Cu alloys increase with the copper content increasing. There exists a relationship between the fragility and the structure in Al-Cu alloy melts. The value of the M reflects the variation of activation energy for viscous flow

  20. Multifarious Functions of the Fragile X Mental Retardation Protein.

    Science.gov (United States)

    Davis, Jenna K; Broadie, Kendal

    2017-10-01

    Fragile X syndrome (FXS), a heritable intellectual and autism spectrum disorder (ASD), results from the loss of Fragile X mental retardation protein (FMRP). This neurodevelopmental disease state exhibits neural circuit hyperconnectivity and hyperexcitability. Canonically, FMRP functions as an mRNA-binding translation suppressor, but recent findings have enormously expanded its proposed roles. Although connections between burgeoning FMRP functions remain unknown, recent advances have extended understanding of its involvement in RNA, channel, and protein binding that modulate calcium signaling, activity-dependent critical period development, and the excitation-inhibition (E/I) neural circuitry balance. In this review, we contextualize 3 years of FXS model research. Future directions extrapolated from recent advances focus on discovering links between FMRP roles to determine whether FMRP has a multitude of unrelated functions or whether combinatorial mechanisms can explain its multifaceted existence. Copyright © 2017 Elsevier Ltd. All rights reserved.

  1. Financial structure, financial development and banking fragility: International evidence

    OpenAIRE

    Ruiz-Porras, Antonio

    2008-01-01

    We study the effects of financial structure and financial development on banking fragility. We develop our study by using fixed-effects panel-data regressions and by controlling the effects of certain banking indicators. We use individual and principal-components indicators of the activity, size and efficiency of intermediaries and markets. The indicators include data for 211 countries between 1990 and 2003. Our main findings suggest that banking stability is enhanced in market-based financia...

  2. Neurosteroids Reverse Tonic Inhibition Deficits in Fragile X Syndrome

    Science.gov (United States)

    2017-10-01

    30 min wash induced a >3 fold increase in tonic current in Fmr1 KO animals which was prevented with PKC inhibition. Using a perforated multi...loss of the fragile X mental retardation protein (FMRP). Studies from both FXS patients and animal models have revealed reduced expression levels of...5B&C). Figure 5. NAS-mediated metabotropic enhancement of tonic inhibitory current in DGGC neurons. A. Scheme demonstrating experimental protocol

  3. Treatment of Fragile X Syndrome with a Neuroactive Steroid

    Science.gov (United States)

    2014-08-01

    to a stimulant for their ADHD symptoms ; for those who do not respond to a stimulant, then an alpha agonist such as guanfacine can be very helpful...medications currently available for treating symptoms of FXS, such as stimulants for attention deficit hyperactivity disorder ( ADHD ), selective serotonin...al. ADHD symptoms in children with FXS. Am J Med Genet A 2006;140(21):2275-88 7. Chonchaiya W, Schneider A, Hagerman RJ. Fragile X: a family of

  4. The Evaluation of Corneal Fragility After UVA/Riboflavin Crosslinking.

    Science.gov (United States)

    Li, Zhiwei; Wang, Yumeng; Xu, Yanyun; Jhanji, Vishal; Zhang, Chunxiao; Mu, Guoying

    2017-03-01

    To evaluate the fragility of cornea after UVA/riboflavin crosslinking (CXL). Sixty New Zealand rabbits received UVA/riboflavin crosslinking treatment (wavelength 365 nm, irradiance 3.0 mW/cm, and total dose 5.4 J/cm) on right eyes. Animals were sacrificed before and immediately after treatment (day 0), day 1, 3, 7, and 28 after treatment. A 4×10 mm corneal strip for biomechanical evaluation was harvested after sacrifice. The corneal fragility was evaluated by measurement of elongation rate, whereby the elongation rate equals elongation length/baseline length. The Youngs modulus and maximal stress were 1.41±0.51 MPa and 5.56±1.84 MPa before CXL, and increased to 2.31±0.68 MPa (P=0.008) and 9.25±2.74 MPa (P=0.04), respectively, on day 0, then maintained a stable level within a 28 days follow-up. The elongation rate was 62.04±9.34% before CXL and decreased to 48.95%±8.24% (P=0.02) on day 0, then maintained a stable level within a 28 days follow-up. This study showed an increase in the corneal fragility after UVA/riboflavin crosslinking along with an increase in the corneal stiffness. A long-term follow-up should be taken to evaluate the potential deleterious effect of the increasing corneal fragility after UVA/riboflavin crosslinking.

  5. Fragility of epidermis: acne and post-procedure lesional skin.

    Science.gov (United States)

    Fabbrocini, G; Rossi, A B; Thouvenin, M-D; Peraud, C; Mengeaud, V; Bacquey, A; Saint Aroman, M

    2017-09-01

    'Fragile skin', or skin with lower resistance to aggressors, can be broadly classified into four causal categories: constitutional (age-dependent or associated with specific vulnerable locations on the body, e.g. eyelids), pathological (related to disease), circumstantial (related to environmental or internal factors, e.g. stress) and iatrogenic (caused by medical interventions or treatments). In this supplement, we focus on the fourth category, the iatrogenic origin of fragile skin and the role that dermo-cosmetics can have in restoring the natural protective function of the skin following treatments for skin diseases and medical interventions. We present epidemiological data on the prevalence of fragile skin in three different geographical regions, and the results of two randomized controlled studies investigating the efficacy and tolerability of dermo-cosmetics in combination with topical acne treatment and following physical skin damage. Overall, we found that prevalence across the three regions (23% in Germany, 41% in UAE, 56% in Taiwan) reflected previous global estimates (24-53%) across skin types, with significant associations found with environmental and lifestyle factors, such as stress, humidity and pollution. The iatrogenic effects of topical acne treatments can result in poor compliance or use of over-the-counter moisturizers, which may reduce treatment efficacy. Dermo-cosmetics were found to aid in restoration of fragile skin caused by the acne topical retinoid treatment adapalene 0.1% gel, by reducing transepidermal water loss and improving skin hydration, as well as reducing the side-effects such as skin irritation that are frequently associated with topical retinoids. Additionally, dermo-cosmetic products were found to accelerate wound closure following skin damage in a laser ablation model and reduced the duration of post-procedural side-effects such as itching and burning. © 2017 European Academy of Dermatology and Venereology.

  6. Progress on fragile histidine triad and cell signal transduction

    International Nuclear Information System (INIS)

    Yang Jian; Han Ling

    2007-01-01

    Fragile histidine triad (FHIT) gene is a new tumour surpress gene. During the past decade, evidence has accumulated in surport that plays a roll in many tumors. In this review, describe the recent finding between FHIT and upstream or downsream gene in post-radiation, including ATR/CHK1 gene, bcl-2 geme, caspase family, cyclophilin A gene and nuclear factor-κB. (authors)

  7. The State of Synapses in Fragile X Syndrome

    OpenAIRE

    Pfeiffer, Brad E.; Huber, Kimberly M.

    2009-01-01

    Fragile X Syndrome is the most common inherited form of mental retardation and a leading genetic cause of autism. There is increasing evidence in both FXS and other forms of autism that alterations in synapse number, structure and function are associated and contribute to these prevalent diseases. FXS is caused by loss of function of the Fmr1 gene which encodes the RNA binding protein, FMRP. Therefore, FXS is a tractable model to understand synaptic dysfunction in cognitive disorders. FMRP is...

  8. MANIPULATION OF A FRAGILE OBJECT BY ELDERLY INDIVIDUALS

    Science.gov (United States)

    Gorniak, Stacey L.; Zatsiorsky, Vladimir M.; Latash, Mark L.

    2011-01-01

    We investigated strategies of healthy elderly participants (74–84 years old) during prehension and transport of an object with varying degrees of fragility. Fragility was specified as the maximal normal force that the object could withstand without collapsing. Specifically, kinetic and kinematic variables as well as and force co-variation indices were quantified and compared to those shown by young healthy persons (19–28 years old). We tested three hypotheses related to age-related changes in two safety margins (slip safety margin and crush safety margin) and indices of force co-variation. Compared to young controls, elderly individuals exhibited a decrease in object acceleration and an increase in movement time, an increase in grip force production, a decrease in the correlation between grip and load forces, an overall decrease in indices of multi-digit synergies, and lower safety margin indices computed with respect to both dropping and crushing the object. Elderly participants preferred to be at a relatively lower risk of crushing the object even if this led to a higher risk of dropping it. Both groups showed an increase in the index of synergy stabilizing total normal force produced by the four fingers with increased fragility of the object. Age related changes are viewed as a direct result of physiological changes due to aging, not adaptation to object fragility. Such changes in overall characteristics of prehension likely reflect diminished synergic control by the central nervous system of finger forces with aging. The findings corroborate an earlier hypothesis on an age-related shift from synergic to element-based control. PMID:21667292

  9. Screening and diagnosis for the fragile X syndrome among the mentally retarded: an epidemiological and psychological survey. Collaborative Fragile X Study Group

    NARCIS (Netherlands)

    B.B.A. de Vries (Bert); B.A. Oostra (Ben); M.F. Niermeijer (Martinus); A. Tibben (Arend); A.M.W. van den Ouweland (Ans); S. Mohkamsing; H.J. Duivenvoorden (Hugo); E. Mol; K. Gelsema; M. van Rijn; D.J.J. Halley (Dicky); L.A. Sandkuijl (Lodewijk)

    1997-01-01

    textabstractThe fragile X syndrome is an X-linked mental retardation disorder caused by an expanded CGG repeat in the first exon of the fragile X mental retardation (FMR1) gene. Its frequency, X-linked inheritance, and consequences for relatives all prompt for

  10. Channelopathies and dendritic dysfunction in fragile X syndrome.

    Science.gov (United States)

    Brager, Darrin H; Johnston, Daniel

    2014-04-01

    Dendritic spine abnormalities and the metabotropic glutamate receptor theory put the focus squarely on synapses and protein synthesis as the cellular locus of fragile X syndrome. Synapses however, are only partly responsible for information processing in neuronal networks. Neurotransmitter triggered excitatory postsynaptic potentials (EPSPs) are shaped and integrated by dendritic voltage-gated ion channels. These EPSPs, and in some cases the resultant dendritic spikes, are further modified by dendritic voltage-gated ion channels as they propagate to the soma. If the resultant somatic depolarization is large enough, action potential(s) will be triggered and propagate both orthodromically down the axon, where it may trigger neurotransmitter release, and antidromically back into the dendritic tree, where it can activate and modify dendritic voltage-gated and receptor activated ion channels. Several channelopathies, both soma-dendritic (L-type calcium channels, Slack potassium channels, h-channels, A-type potassium channels) and axo-somatic (BK channels and delayed rectifier potassium channels) were identified in the fmr1-/y mouse model of fragile X syndrome. Pathological function of these channels will strongly influence the excitability of individual neurons as well as overall network function. In this chapter we discuss the role of voltage-gated ion channels in neuronal processing and describe how identified channelopathies in models of fragile X syndrome may play a role in dendritic pathophysiology. Copyright © 2014 Elsevier Inc. All rights reserved.

  11. Robust non-fragile dynamic vibration absorbers with uncertain factors

    Science.gov (United States)

    Zhang, Hui; Shi, Yang; Saadat Mehr, Aryan

    2011-02-01

    In this paper, the design problem for non-fragile dynamic vibration absorbers (DVAs) is investigated. Due to the imprecision of the manufacturing process or the variation during the operation, uncertainty in the parameters of the DVA is unavoidable. The uncertainty may degrade the performance of the designed DVA or even deteriorate the system. Hence, it is practically demanding to propose a design method for a non-fragile DVA, i.e., when the parameters of the DVA vary in an admissible range, an expected vibration suppression level should be guaranteed. The uncertainty of the DVA is feasibly assumed to be norm-bounded. Then, the design problem for the DVA is converted into a static output feedback (SOF) control problem. Sufficient condition for the existence of the non-fragile DVA with a prescribed H∞ level is derived by using a bilinear matrix inequality (BMI). An iterative linear matrix inequality (ILMI) method is employed to solve the BMI condition. Finally, a design example is given to show the effectiveness of the proposed approach.

  12. Beneficial effect of interventional exercise on autistic Fragile X syndrome.

    Science.gov (United States)

    Lee, Seunghoon; Won, Jinyoung; Park, Sookyoung; Lee, Sang-Rae; Chang, Kyu-Tae; Kim, Joo-Heon; Hong, Yonggeun

    2017-04-01

    [Purpose] The purpose of the present review is to discuss recent published articles in the understanding of efficacy of interventional exercise on autistic Fragile X syndrome (FXS) with special emphasis on its significance in clinical application in patients. [Methods] This review article was identified scientifically and/or clinically relevant articles from PubMed that directly/indirectly met the inclusion criteria. [Results] Mutation of fragile X mental retardation 1 ( fmr1 ) gene on the X chromosome is related with loss of fragile X mental retardation protein (FMRP) that affecting physiological and behavioral abnormalities. Autistic FXS individuals exhibit disturbed sleep and altered circadian behavior. Although the underlying molecular mechanisms are not been fully explored, interventional exercise in autistic FXS has been clinically used for the treatment of physiological and behavioral abnormalities as well as psychiatric disorder in autistic FXS. [Conclusion] This review describes beneficial efficacy of interventional exercise and its controversy in patients with autistic FXS. This review also provides interventional strategies for clinicians and scientists that the way of neurophysiological approaches according to the level of physical and behavioral abnormalities.

  13. Component fragility results for reliability and probability studies

    International Nuclear Information System (INIS)

    Bandyopadhyay, K.; Hofmayer, C.

    1991-01-01

    Under a sponsorship of the United States Nuclear Regulatory Commission (NRC), Brookhaven National Laboratory (BNL) has formed a test data base of seismic fragility of nuclear power plant equipment and analyzed the data for use in reliability and probability studies. This paper describes the data base and discusses the analysis methods and results. A large sample of test data has been collected from various sources for the data base. A standardization technique has been applied for conversion of the data to a uniform and consistent data set. The zero period acceleration (ZPA) and the average spectral acceleration (ASA) over the 4-16 Hz frequency range have been selected to represent a test response spectrum and these two parameters have been used in the statistical analysis. A logarithmic distribution of the data has been assumed. Test data of six classes of equipment have been analyzed. For each equipment type, the median value, the coefficients of variance and a high confidence (95%) value corresponding to a low probability (5%) of failure have been computed. These results are obtained for both the ZPA and ASA fragility parameters and for different failure modes. The fragility results are expected to provide a strong basis for future reliability and probability studies. The data base at BNL is being expanded by collection of test information for additional equipment categories. (orig.)

  14. Determination of a modal interaction correction for narrowband fragility data

    International Nuclear Information System (INIS)

    Kana, D.D.; Pomerening, D.J.

    1987-01-01

    Laboratory tests for safety equipment operation under seismic environments in nuclear power plants have typically included various motion simulations based on either successively-applied multiple narrowband waveforms or simultaneous multifrequency broadband random waveforms. However, only broadband excitations are directly applicable when equipment performance is affected by interaction between simultaneously responding modes. Therefore, a modal interaction correction factor is developed so that a narrowed response spectrum can be transformed to an approximately equivalent broadband spectrum which accounts for modal interaction effects. The approach includes study of the fragility response of a simple two-degree-of-freedom oscillator for representative narrowband and broadband excitations, and relating the two resulting fragility response spectra. It is found that multiplication of the narrowband response spectrum by an 0.7 factor produces a conservative equivalent broadband response spectrum. The results are interpreted in terms of a secondary device responding on a primary support structure, or a primary structure having two resonances. The approach is useful for updating existing test results based on narrowband spectra, developing composite spectra for similar equipment, or providing more flexibility in designing new tests, and is applicable to qualification proof test data as well as fragility data. 10 refs., 8 figs

  15. Fragile X syndrome: a review of associated medical problems.

    Science.gov (United States)

    Kidd, Sharon A; Lachiewicz, Ave; Barbouth, Deborah; Blitz, Robin K; Delahunty, Carol; McBrien, Dianne; Visootsak, Jeannie; Berry-Kravis, Elizabeth

    2014-11-01

    Fragile X syndrome (FXS) is the most common known genetic cause of inherited intellectual disability and the most common known single-gene cause of autism spectrum disorder. It has been reported that a spectrum of medical problems are commonly experienced by people with FXS, such as otitis media, seizures, and gastrointestinal problems. Previous studies examining the prevalence of medical problems related to FXS have been challenging to interpret because of their marked differences in population, setting, and sampling. Through this comprehensive review, we update the literature by reviewing studies that have reported on prominent medical problems associated with FXS. We then compare prevalence results from those studies with results from a large cross-sectional database consisting of data collected by fragile X clinics that specialize in the care of children with FXS and are part of the Fragile X Clinical and Research Consortium. It is vital for pediatricians and other clinicians to be familiar with the medical problems related to FXS so that affected patients may receive proper diagnosis and treatment; improved care may lead to better quality of life for these patients and their families. Copyright © 2014 by the American Academy of Pediatrics.

  16. Lithium: a promising treatment for fragile X syndrome.

    Science.gov (United States)

    Liu, Zhonghua; Smith, Carolyn Beebe

    2014-06-18

    Fragile X syndrome (FXS) is an inherited disorder that results in intellectual disability and a characteristic behavioral profile that includes autism spectrum disorder, attention deficit hyperactivity disorder, sensory hypersensitivity, hyperarousal, and anxiety. The epigenetic silencing of FMR1 and the consequent absence of its protein product, FMRP, is the most common cause of fragile X. The development of animal models of fragile X syndrome 20 years ago has produced a considerable increase in our understanding of the consequences of the absence of FMRP on the structure and function of the nervous system. Some of the insights gained have led to proposals of treatment strategies that are based on cellular and molecular changes observed in animals lacking FMRP. One such proposal is treatment with lithium, a drug with a long history of clinical efficacy in psychiatry and a drug with newly described uses in degenerative disorders of the nervous system. Lithium treatment has been studied extensively in both mouse and fruit fly models of FXS, and it has been shown to reverse numerous behavioral, physiological, cellular, and molecular phenotypes. A report of a pilot clinical trial on a limited number of adult FXS patients indicated that measurable improvements in behavior and function were seen after 2 months of lithium treatment. A double-blind clinical trial of lithium treatment in FXS patients is now needed.

  17. Fragility Modeling of Aging Containment Metallic Pressure Boundaries

    International Nuclear Information System (INIS)

    Cherry, J.L.; Ellingwood, B.R.

    1999-01-01

    The containment in a nuclear power plant (NPP) provides a barrier against the release of radioactivity in the event of an accident. Corrosion that has been observed in some steel containments and liners of reinforced concrete containments has raised questions about their ability to perform this function. The performance of corroded containments during events at or beyond the design basis is impacted by numerous sources of uncertainty. A fragility model of the containment provides a relatively simple depiction of the impact of uncertainties on structural performance and a basis for decision-making in the presence of uncertainty. Moreover, it is a necessary ingredient of any time-dependent structural reliability analysis. A nonlinear finite element analysis of containment response furnishes the necessary platform to perform numerical experiments to determine containment fragility. A statistically-based sampling plan minimizes the finite element computations required to develop the fragility curve. The -percentile (or other fractile) then gives a statistically based indication of the lower bound on containment capacity, and can be used as a screening tool to determine whether more refined further analysis or tests to support service life evaluations are warranted

  18. Which side of the balance determines the frequency of vaso-occlusive crises in children with sickle cell anemia: Blood viscosity or microvascular dysfunction?

    Science.gov (United States)

    Charlot, Keyne; Romana, Marc; Moeckesch, Berenike; Jumet, Stéphane; Waltz, Xavier; Divialle-Doumdo, Lydia; Hardy-Dessources, Marie-Dominique; Petras, Marie; Tressières, Benoît; Tarer, Vanessa; Hue, Olivier; Etienne-Julan, Maryse; Antoine-Jonville, Sophie; Connes, Philippe

    2016-01-01

    Vascular resistance and tissue perfusion may be both affected by impaired vascular function and increased blood viscosity. Little is known about the effects of vascular function on the occurrence of painful vaso-occlusive crises (VOC) in children with sickle cell anemia (SCA). The aim of the present study was to determine which side of the balance (blood viscosity or vascular function) is the most deleterious in SCA and increases the risk for frequent hospitalized VOC. Microvascular function, microcirculatory oxygenation and blood viscosity were determined in a group of 22 SCA children/adolescents at steady state and a group of 13 healthy children/adolescents. Univariate analyses demonstrated blunted microvascular reactivity during local thermal heating test and decreased microcirculatory oxygenation in SCA children compared to controls. Multivariate analysis revealed that increased blood viscosity and decreased microcirculatory oxygenation were independent risk factors of frequent VOC in SCA. In contrast, the level of microvascular dysfunction does not predict VOC rate. In conclusion, increased blood viscosity is usually well supported in healthy individuals where vascular function is not impaired. However, in the context of SCA, microvascular function is impaired and any increase of blood viscosity or decrease in microcirculatory oxygenation would increase the risks for frequent VOC. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. Taquicardia por reentrada nodal recurrente en paciente con transposición congénita corregida de grandes vasos. Utilización de mapeo no fluoroscópico

    OpenAIRE

    Aristizábal,Julián; Uribe,William; Duque,Mauricio; Marín,Jorge; Medina,Eduardo; Velásquez,Jorge; Femenía,Francisco; Baranchuk,Adrían

    2013-01-01

    La complejidad anatómica y la variabilidad en el sistema de conducción en la transposición congénita corregida de los grandes vasos plantean intervenciones con el uso de recursos tecnológicos que faciliten un desenlace favorable. Describimos un caso de ablación de taquicardia por reentrada intranodal en donde el mapeo no fluoroscópico facilitó una intervención compleja.

  20. The Seismic Fragility Evaluation of an Offsite Transformer according to Aging Effects

    International Nuclear Information System (INIS)

    Kim, Min Kyu; Choi, In Kil

    2008-01-01

    A seismic fragility analysis was performed, especially for an aged electric power transmission system, in this study. A real electric transformer system for Korean Nuclear Power Plants was selected for the seismic fragility evaluation. In the case of a seismic fragility analysis we should use design material properties and conditions. However material properties and environmental conditions of most structures and equipment are changed according to a lapse of time. Aging conditions greatly affect the integrity of the structures and equipment at NPP sites, but it is very difficult to estimate them qualitatively. Integrity of an anchor bolt system was considered with the aging conditions for an electric transformer system. At first, a seismic fragility analysis was performed for a fine condition for an electric transformer system. After that, a seismic fragility analysis according to the fastener of an anchor bolt system was conducted. This study showed that a looser anchor bolt creates seismic responses and seismic fragility changes of more 10%

  1. Research and development of system/plant level seismic fragility quantization program

    International Nuclear Information System (INIS)

    Fu Zhiwei; Zhang Chunming; Chen Yan; Zuo Jiaxu; Zhang Donghui

    2013-01-01

    The conception and model of seismic fragility were introduced. General quantization process of seismic fragility of system/plant level was studied. The quantization program of seismic fragility of system/plant level based on Monte Carlo simulation was researched and developed. The seismic fragility of China Experimental Fast Reactor (CEFR) accident residual heat removal system was modeled. The system seismic fragility parameters, A m = 1.205 g, β u = 0.42, β r = 0.42, HCLPF = 0.33 g, were obtained. The results show that the CEFR accident residual heat removal system has high seismic capacity, and Monte Carlo simulation is effective method for seismic fragility quantization of system/plant level. (authors)

  2. Nucleosome fragility reveals novel functional states of chromatin and poises genes for activation.

    Science.gov (United States)

    Xi, Yuanxin; Yao, Jianhui; Chen, Rui; Li, Wei; He, Xiangwei

    2011-05-01

    The structural complexity of nucleosomes underlies their functional versatility. Here we report a new type of complexity-nucleosome fragility, manifested as high sensitivity to micrococcal nuclease, in contrast to the common presumption that nucleosomes are similar in resistance to MNase digestion. Using differential MNase digestion of chromatin and high-throughput sequencing, we have identified a special group of nucleosomes termed "fragile nucleosomes" throughout the yeast genome, nearly 1000 of which were at previously determined "nucleosome-free" loci. Nucleosome fragility is broadly implicated in multiple chromatin processes, including transcription, translocation, and replication, in correspondence to specific physiological states of cells. In the environmental-stress-response genes, the presence of fragile nucleosomes prior to the occurrence of environmental changes suggests that nucleosome fragility poises genes for swift up-regulation in response to the environmental changes. We propose that nucleosome fragility underscores distinct functional statuses of the chromatin and provides a new dimension for portraying the landscape of genome organization.

  3. Improved Iris Recognition through Fusion of Hamming Distance and Fragile Bit Distance.

    Science.gov (United States)

    Hollingsworth, Karen P; Bowyer, Kevin W; Flynn, Patrick J

    2011-12-01

    The most common iris biometric algorithm represents the texture of an iris using a binary iris code. Not all bits in an iris code are equally consistent. A bit is deemed fragile if its value changes across iris codes created from different images of the same iris. Previous research has shown that iris recognition performance can be improved by masking these fragile bits. Rather than ignoring fragile bits completely, we consider what beneficial information can be obtained from the fragile bits. We find that the locations of fragile bits tend to be consistent across different iris codes of the same eye. We present a metric, called the fragile bit distance, which quantitatively measures the coincidence of the fragile bit patterns in two iris codes. We find that score fusion of fragile bit distance and Hamming distance works better for recognition than Hamming distance alone. To our knowledge, this is the first and only work to use the coincidence of fragile bit locations to improve the accuracy of matches.

  4. Características generales de 29 pacientes con vasculitis de pequeños vasos General characteristics of 29 patients with small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Nicolás Di Benedetto

    2010-04-01

    Full Text Available El objetivo de esta serie fue describir las características generales y manifestaciones clínicas de pacientes con vasculitis de pequeños vasos que concurrieron al servicio de autoinmunidad de un hospital de la comunidad y compararlas con la literatura. Se revisaron de manera retrospectiva las historias clínicas archivadas bajo el rótulo de vasculitis en un período de 16 años. Se seleccionaron aquellos pacientes que cumplieran criterios diagnósticos de vasculitis de pequeños vasos. Se extrajeron los datos de interés y se constató la supervivencia mediante contacto telefónico. Posteriormente se realizó una búsqueda bibliográfica y se compararon los resultados. Se incluyeron 13 pacientes con granulomatosis de Wegener, 6 con síndrome de Churg Strauss y 10 con poliangeítis microscópica. El 55% (16 fueron menores de 55 años al diagnóstico y hubo predominio del sexo femenino (2.6:1. La demora diagnóstica fue mayor a un año en el 46% de los casos. Los sistemas más frecuentemente afectados fueron respiratorio y otorrinolaringológico. El 79% presentó anticuerpos anti-citoplasma de neutrófilos (ANCA positivos. La mortalidad fue del 24% (7/29. Al comparar las características de los pacientes incluidos en esta serie con lo descripto en la literatura, se hallaron diferencias en cuanto a la forma de presentación, sistemas comprometidos y porcentaje de pacientes ANCA positivos; además se observó que el resultado negativo de este marcador parece asociarse con mayor demora diagnóstica y en consecuencia peor pronóstico, lo que remarca su importancia como herramienta adicional no invasiva al momento del diagnóstico.The objective of this series was to describe the general characteristics and clinical manifestations of patients with small vessel vasculitis who were assisted in the autoimmunity department of a community public hospital and to compare the results with the literature. Clinical records under the label of vasculitis in a

  5. La panoplia en los vasos del Tossal de Sant Miquel (Llíria. Ensayo de interpretación iconográfica

    Directory of Open Access Journals (Sweden)

    Pérez Ferrandis, Susana Rosa

    2013-12-01

    Full Text Available The set of more than 225 vases with figurative decoration found in El Tossal de Sant Miquel (TSM, Lliria, with a chronology that it is fitted in the second stage of the Middle Iberian, between IIIrd century B.C. at the beginning of the IInd B. C, and published by first time in the Corpus Vasorum Hispanorum, offers a rich iconography in arms elements. Across his analysis it is tried to determine if a edetana panoply exists and if this one changes according to so called «Styles I and II». (Ballester, 1935, 1954; Bonet, 1995; Aranegui et alii, 1997b; Pérez Ballester, Mata, 1998; Izquierdo, Perez Ballester, 2005.El conjunto de más de 225 vasos con decoración figurada encontrados en El Tossal de Sant Miquel (TSM de Lliria, con una cronología que se encuadra en la segunda etapa del Ibérico Pleno, entre los siglos III a.C. a principios del II a.C, y publicados por vez primera en el Corpus Vasorum Hispanorum, ofrece una iconografía rica en elementos armamentísticos. A través de su análisis se pretende determinar si existe una panoplia edetana y si ésta varía según los llamados «Estilos I y II». (Ballester, 1935, 1954; Bonet, 1995; Aranegui et alii, 1997b; Pérez Ballester, Mata, 1998; Izquierdo, Pérez Ballester, 2005.

  6. Development of fragility functions to estimate homelessness after an earthquake

    Science.gov (United States)

    Brink, Susan A.; Daniell, James; Khazai, Bijan; Wenzel, Friedemann

    2014-05-01

    Immediately after an earthquake, many stakeholders need to make decisions about their response. These decisions often need to be made in a data poor environment as accurate information on the impact can take months or even years to be collected and publicized. Social fragility functions have been developed and applied to provide an estimate of the impact in terms of building damage, deaths and injuries in near real time. These rough estimates can help governments and response agencies determine what aid may be required which can improve their emergency response and facilitate planning for longer term response. Due to building damage, lifeline outages, fear of aftershocks, or other causes, people may become displaced or homeless after an earthquake. Especially in cold and dangerous locations, the rapid provision of safe emergency shelter can be a lifesaving necessity. However, immediately after an event there is little information available about the number of homeless, their locations and whether they require public shelter to aid the response agencies in decision making. In this research, we analyze homelessness after historic earthquakes using the CATDAT Damaging Earthquakes Database. CATDAT includes information on the hazard as well as the physical and social impact of over 7200 damaging earthquakes from 1900-2013 (Daniell et al. 2011). We explore the relationship of both earthquake characteristics and area characteristics with homelessness after the earthquake. We consider modelled variables such as population density, HDI, year, measures of ground motion intensity developed in Daniell (2014) over the time period from 1900-2013 as well as temperature. Using a base methodology based on that used for PAGER fatality fragility curves developed by Jaiswal and Wald (2010), but using regression through time using the socioeconomic parameters developed in Daniell et al. (2012) for "socioeconomic fragility functions", we develop a set of fragility curves that can be

  7. Bone quality: the determinants of bone strength and fragility.

    Science.gov (United States)

    Fonseca, Hélder; Moreira-Gonçalves, Daniel; Coriolano, Hans-Joachim Appell; Duarte, José Alberto

    2014-01-01

    Bone fragility is a major health concern, as the increased risk of bone fractures has devastating outcomes in terms of mortality, decreased autonomy, and healthcare costs. Efforts made to address this problem have considerably increased our knowledge about the mechanisms that regulate bone formation and resorption. In particular, we now have a much better understanding of the cellular events that are triggered when bones are mechanically stimulated and how these events can lead to improvements in bone mass. Despite these findings at the molecular level, most exercise intervention studies reveal either no effects or only minor benefits of exercise programs in improving bone mineral density (BMD) in osteoporotic patients. Nevertheless, and despite that BMD is the gold standard for diagnosing osteoporosis, this measure is only able to provide insights regarding the quantity of bone tissue. In this article, we review the complex structure of bone tissue and highlight the concept that its mechanical strength stems from the interaction of several different features. We revisited the available data showing that bone mineralization degree, hydroxyapatite crystal size and heterogeneity, collagen properties, osteocyte density, trabecular and cortical microarchitecture, as well as whole bone geometry, are determinants of bone strength and that each one of these properties may independently contribute to the increased or decreased risk of fracture, even without meaningful changes in aBMD. Based on these findings, we emphasize that while osteoporosis (almost) always causes bone fragility, bone fragility is not always caused just by osteoporosis, as other important variables also play a major role in this etiology. Furthermore, the results of several studies showing compelling data that physical exercise has the potential to improve bone quality and to decrease fracture risk by influencing each one of these determinants are also reviewed. These findings have meaningful clinical

  8. Seismic Fragility of the LANL Fire Water Distribution System

    Energy Technology Data Exchange (ETDEWEB)

    Greg Mertz

    2007-03-30

    The purpose of this report is to present the results of a site-wide system fragility assessment. This assessment focuses solely on the performance of the water distribution systems that supply Chemical and Metallurgy Research (CMR), Weapons Engineering and Tritium Facility (WETF), Radioactive Liquid Waste Treatment Facility (RLWTF), Waste Characterization, Reduction, Repackaging Facility (WCRRF), and Transuranic Waste Inspectable Storage Project (TWISP). The analysis methodology is based on the American Lifelines Alliance seismic fragility formulations for water systems. System fragilities are convolved with the 1995 LANL seismic hazards to develop failure frequencies. Acceptance is determined by comparing the failure frequencies to the DOE-1020 Performance Goals. This study concludes that: (1) If a significant number of existing isolation valves in the water distribution system are closed to dedicate the entire water system to fighting fires in specific nuclear facilities; (2) Then, the water distribution systems for WETF, RLWTF, WCRRF, and TWISP meet the PC-2 performance goal and the water distribution system for CMR is capable of surviving a 0.06g earthquake. A parametric study of the WETF water distribution system demonstrates that: (1) If a significant number of valves in the water distribution system are NOT closed to dedicate the entire water system to fighting fires in WETF; (2) Then, the water distribution system for WETF has an annual probability of failure on the order of 4 x 10{sup -3} that does not meet the PC-2 performance goal. Similar conclusions are expected for CMR, RLWTF, WCRRF, and TWISP. It is important to note that some of the assumptions made in deriving the results should be verified by personnel in the safety-basis office and may need to be incorporated in technical surveillance requirements in the existing authorization basis documentation if credit for availability of fire protection water is taken at the PC-2 level earthquake levels

  9. Fragility fractures of the pelvis: should they be fixed?

    Science.gov (United States)

    Rommens, P M; Dietz, S-O; Ossendorf, C; Pairon, P; Wagner, D; Hofmann, A

    2015-01-01

    Due to the aging population, there is an increasing number of fragility fractures of the pelvis (FFP). They are the result of low energy trauma. The bone breaks but the ligaments remain intact. Immobilizing pain at the pubic region or at the sacrum is the main symptom. Conventional radiographs reveal pubic rami fractures, but lesions of the dorsal pelvis are hardly visible and easily overlooked. CT of the pelvis with multiplanar reconstructions show the real extension of the lesion. Most patients have a history of osteoporosis or other fragility fractures. The new classification distinguishes between four categories of different and increasing instability. FFP Type I are anterior lesions only, FFP Type II are non-displaced posterior lesions, FFP Type III are displaced unilateral posterior lesions and FFP Type IV are displaced bilateral posterior lesions. Subgroups discriminate between the localization of the dorsal instability. FFP Type I lesions are treated non-operatively. FFP Type II lesions are fixed in a percutaneous procedure when a trial of conservative treatment was not successful. FFP Type III lesions are treated with open reduction and internal fixation (ORIF). FFP Type IV lesions are treated with bilateral ORIF or with a bridging osteosynthesis. Iliosacral screw osteosynthesis is widely used, but has an elevated risk of screw loosening due to diminished bine mineral density. Transsacral bar osteosynthesis enable interfragmentary compression and does not have this danger of loosening. Bridging plate osteosynthesis is used as an additional fixation to iliosacral screw osteosynthesis. Lumbopelvic fixation is restricted to highly unstable lumbopelvic dissociations. More studies are needed to find the optimal treatment for each type of instability. Key words: pelvis, fragility fracture, diagnosis, classification, treatment.

  10. Synaptic vesicle dynamic changes in a model of fragile X.

    Science.gov (United States)

    Broek, Jantine A C; Lin, Zhanmin; de Gruiter, H Martijn; van 't Spijker, Heleen; Haasdijk, Elize D; Cox, David; Ozcan, Sureyya; van Cappellen, Gert W A; Houtsmuller, Adriaan B; Willemsen, Rob; de Zeeuw, Chris I; Bahn, Sabine

    2016-01-01

    Fragile X syndrome (FXS) is a single-gene disorder that is the most common heritable cause of intellectual disability and the most frequent monogenic cause of autism spectrum disorders (ASD). FXS is caused by an expansion of trinucleotide repeats in the promoter region of the fragile X mental retardation gene (Fmr1). This leads to a lack of fragile X mental retardation protein (FMRP), which regulates translation of a wide range of messenger RNAs (mRNAs). The extent of expression level alterations of synaptic proteins affected by FMRP loss and their consequences on synaptic dynamics in FXS has not been fully investigated. Here, we used an Fmr1 knockout (KO) mouse model to investigate the molecular mechanisms underlying FXS by monitoring protein expression changes using shotgun label-free liquid-chromatography mass spectrometry (LC-MS(E)) in brain tissue and synaptosome fractions. FXS-associated candidate proteins were validated using selected reaction monitoring (SRM) in synaptosome fractions for targeted protein quantification. Furthermore, functional alterations in synaptic release and dynamics were evaluated using live-cell imaging, and interpretation of synaptic dynamics differences was investigated using electron microscopy. Key findings relate to altered levels of proteins involved in GABA-signalling, especially in the cerebellum. Further exploration using microscopy studies found reduced synaptic vesicle unloading of hippocampal neurons and increased vesicle unloading in cerebellar neurons, which suggests a general decrease of synaptic transmission. Our findings suggest that FMRP is a regulator of synaptic vesicle dynamics, which supports the role of FMRP in presynaptic functions. Taken together, these studies provide novel insights into the molecular changes associated with FXS.

  11. Seismic Fragility of the LANL Fire Water Distribution System

    International Nuclear Information System (INIS)

    Greg Mertz Jason Cardon Mike Salmon

    2007-01-01

    The purpose of this report is to present the results of a site-wide system fragility assessment. This assessment focuses solely on the performance of the water distribution systems that supply Chemical and Metallurgy Research (CMR), Weapons Engineering and Tritium Facility (WETF), Radioactive Liquid Waste Treatment Facility (RLWTF), Waste Characterization, Reduction, Repackaging Facility (WCRRF), and Transuranic Waste Inspectable Storage Project (TWISP). The analysis methodology is based on the American Lifelines Alliance seismic fragility formulations for water systems. System fragilities are convolved with the 1995 LANL seismic hazards to develop failure frequencies. Acceptance is determined by comparing the failure frequencies to the DOE-1020 Performance Goals. This study concludes that: (1) If a significant number of existing isolation valves in the water distribution system are closed to dedicate the entire water system to fighting fires in specific nuclear facilities; (2) Then, the water distribution systems for WETF, RLWTF, WCRRF, and TWISP meet the PC-2 performance goal and the water distribution system for CMR is capable of surviving a 0.06g earthquake. A parametric study of the WETF water distribution system demonstrates that: (1) If a significant number of valves in the water distribution system are NOT closed to dedicate the entire water system to fighting fires in WETF; (2) Then, the water distribution system for WETF has an annual probability of failure on the order of 4 x 10 -3 that does not meet the PC-2 performance goal. Similar conclusions are expected for CMR, RLWTF, WCRRF, and TWISP. It is important to note that some of the assumptions made in deriving the results should be verified by personnel in the safety-basis office and may need to be incorporated in technical surveillance requirements in the existing authorization basis documentation if credit for availability of fire protection water is taken at the PC-2 level earthquake levels

  12. Another Baby? Father Involvement and Childbearing in Fragile Families

    OpenAIRE

    Kotila, Letitia E.; Dush, Claire M. Kamp

    2012-01-01

    An historic number of women in the US have children outside of marriage, and with more than one father, yet little research has examined the association between family process and women’s childbearing decisions. Using a subsample of unmarried women from the Fragile Families and Child Wellbeing Study (N=2028), a study of primarily low-income unmarried parents, we conducted discrete-time survival analysis models to predict whether women had another child with the focal child’s father (same-fath...

  13. Ageing, fragility and the reversibility window in bulk alloy glasses

    International Nuclear Information System (INIS)

    Chakravarty, S; Georgiev, D G; Boolchand, P; Micoulaut, M

    2005-01-01

    Non-reversing relaxation enthalpies (ΔH nr ) at glass transitions T g (x) in the P x Ge x Se 1-2x ternary display wide, sharp and deep global minima (∼0) in the 0.09 g s become thermally reversing. In this reversibility window, glasses are found not to age, in contrast to ageing observed for fragile glass compositions outside the window. Thermal reversibility and lack of ageing seem to be paradigms of self-organization which molecular glasses share with protein structures which repetitively and reversibly change conformation near T g and the folding temperature respectively. (letter to the editor)

  14. Crescimento e acumulação de nutrientes em plantas de taro sob níveis de sombreamento artificial

    OpenAIRE

    Oliveira,Fabio Luiz de; Araújo,Adelson Paulo; Guerra,Jose Guilherme M

    2011-01-01

    Foi conduzido um experimento em vasos com solo com o objetivo de avaliar o crescimento e acumulação de nutrientes de plantas de taro (Colacasia esculenta) sob níveis de sombreamento artificial. O experimento foi constituído de quatro níveis de sombreamento (0, 25, 50 e 75% de restrição de luz) e nove coletas mensais, no delineamento de blocos casualizados em esquema de parcelas subdivididas, com quatro repetições. Os níveis de sombreamento foram obtidos com uso de armações galvanizadas revest...

  15. HEMANGIOMA HEPÁTICO PRIMÁRIO EM GATA PERSA COM DOENÇA RENAL POLICÍSTICA PRIMARY HEPATIC HEMANGIOMA IN PERSIAN CAT WITH POLYCYSTIC KIDNEY DISEASE

    OpenAIRE

    Valdemiro Amaro da Silva Júnior; Frederico Celso Lyra Maia; Fábio Luiz da Cunha Brito

    2008-01-01

    Hemangiomas primários do fígado são neoplasias benignas que podem comprometer a sobrevivência do animal, em virtude da compressão de vasos, ductos e hepatócitos adjacentes, especialmente pela possibilidade de rupturas e morte por hipovolemia. Por serem raros em felinos, objetiva-se relatar o caso de uma gata Persa, com 10 anos de idade, cujos sinais clínicos eram: aumento de volume abdominal, vômitos intermitentes, apatia e anorexia, além de cios irregular...

  16. The economics of fragility fractures in West Virginia.

    Science.gov (United States)

    Pfister, Alfred K; Sale, William G; Shaukat, Salman

    2009-01-01

    Compared to the United States, West Virginia has a higher proportion of residents aged 60 or older. This situation creates a stronger risk factor for osteoporosis and subsequent fragility fractures. The widespread availability of bone densitometry screening to identify osteoporosis and the marketing of effective agents to reduce fractures in the past decade has resulted in cost savings. We estimated the absolute number of fragility fractures associated with osteoporosis in West Virginia and projected their costs over a 5-year period. Cost savings were based on the variables of the proportion of osteoporotic patients identified and their long-term adherence to prescribed calcium,vitamin D, and bisphosphonate therapy. Our results indicate that only a small amount of potential cost savings (varying between less than 0.5% to 3.0%) is currently realized through diagnosis and adherence to prescribed treatment. We conclude that increased screening and recognition of osteoporosis by the primary care provider is a more feasible strategy to improve cost savings and fracture reduction rather than attempts to improve treatment adherence.

  17. Dementia in Fragile X-associated Tremor/Ataxia Syndrome

    Directory of Open Access Journals (Sweden)

    Ricardo Nitrini

    Full Text Available Abstract Fragile X-associated tremor/ataxia syndrome (FXTAS is a cause of movement disorders and cognitive decline which has probably been underdiagnosed, especially if its prevalence proves similar to those of progressive supranuclear palsy and amyotrophic lateral sclerosis. We report a case of a 74-year-old man who presented with action tremor, gait ataxia and forgetfulness. There was a family history of tremor and dementia, and one of the patient's grandsons was mentally deficient. Neuropsychological evaluation disclosed a frontal network syndrome. MRI showed hyperintensity of both middle cerebellar peduncles, a major diagnostic hallmark of FXTAS. Genetic testing revealed premutation of the FMR1 gene with an expanded (CGG90 repeat. The diagnosis of FXTAS is important for genetic counseling because the daughters of the affected individuals are at high risk of having offspring with fragile X syndrome. Tremors and cognitive decline should raise the diagnostic hypothesis of FXTAS, which MRI may subsequently reinforce, while the detection of the FMR1 premutation can confirm the condition.

  18. Deficient Sleep in Mouse Models of Fragile X Syndrome

    Directory of Open Access Journals (Sweden)

    R. Michelle Saré

    2017-09-01

    Full Text Available In patients with fragile X syndrome (FXS, sleep problems are commonly observed but are not well characterized. In animal models of FXS (dfmr1 and Fmr1 knockout (KO/Fxr2 heterozygote circadian rhythmicity is affected, but sleep per se has not been examined. We used a home-cage monitoring system to assess total sleep time in both light and dark phases in Fmr1 KO mice at different developmental stages. Fmr1 KOs at P21 do not differ from controls, but genotype × phase interactions in both adult (P70 and P180 groups are statistically significant indicating that sleep in Fmr1 KOs is reduced selectively in the light phase compared to controls. Our results show the emergence of abnormal sleep in Fmr1 KOs during the later stages of brain maturation. Treatment of adult Fmr1 KO mice with a GABAB agonist, R-baclofen, did not restore sleep duration in the light phase. In adult (P70 Fmr1 KO/Fxr2 heterozygote animals, total sleep time was further reduced, once again in the light phase. Our data highlight the importance of the fragile X genes (Fmr1 and Fxr2 in sleep physiology and confirm the utility of these mouse models in enhancing our understanding of sleep disorders in FXS.

  19. Fragility assessment method of Concrete Wall Subjected to Impact Loading

    International Nuclear Information System (INIS)

    Hahm, Daegi; Shin, Sang Shup; Choi, In-Kil

    2014-01-01

    These studies have been aimed to verify and ensure the safety of the targeted walls and structures especially in the viewpoint of the deterministic approach. However, recently, the regulation and the assessment of the safety of the nuclear power plants (NPPs) against to an aircraft impact are strongly encouraged to adopt a probabilistic approach, i.e., the probabilistic risk assessment of an aircraft impact. In Korea, research to develop aircraft impact risk quantification technology was initiated in 2012 by Korea Atomic Energy Research Institute (KAERI). In this paper, for the one example of the probabilistic safety assessment approach, a method to estimate the failure probability and fragility of concrete wall subjected to impact loading caused by missiles or engine parts of aircrafts will be introduced. This method and the corresponding results will be used for the total technical roadmap and the procedure to assess the aircraft impact risk (Fig.1). A method and corresponding results of the estimation of the failure probability and fragility for a concrete wall subjected to impact loadings caused by missiles or engine parts of aircrafts was introduced. The detailed information of the target concrete wall in NPP, and the example aircraft engine model is considered safeguard information (SGI), and is not contained in this paper

  20. Why Stay Engaged with a State Deemed Fragile?

    DEFF Research Database (Denmark)

    Andersson, Magnus; Bae, Jinsun

    Based on the constructivist international relations (IR) approach, the authors study Sweden’s engagement with the DPRK as a unique case to understand motivations for engaging in a so-called fragile state. Besides having its embassy in Pyongyang and serving as a protecting power for the U.S., Swed...... approach in accounting for today’s engagement practices involving more stakeholders and less strict cost-benefit calculation.......Based on the constructivist international relations (IR) approach, the authors study Sweden’s engagement with the DPRK as a unique case to understand motivations for engaging in a so-called fragile state. Besides having its embassy in Pyongyang and serving as a protecting power for the U.S., Sweden...... the expectation of gains, the convenience of repeating the work and the logic of appropriateness. This case expands our understanding of engagement that is often understood to a great degree as a rationalist affair between the engaging and target states. It also affirms the usefulness of constructivist IR...

  1. Modeling fragile X syndrome in the Fmr1 knockout mouse

    Science.gov (United States)

    Kazdoba, Tatiana M.; Leach, Prescott T.; Silverman, Jill L.; Crawley, Jacqueline N.

    2014-01-01

    Summary Fragile X Syndrome (FXS) is a commonly inherited form of intellectual disability and one of the leading genetic causes for autism spectrum disorder. Clinical symptoms of FXS can include impaired cognition, anxiety, hyperactivity, social phobia, and repetitive behaviors. FXS is caused by a CGG repeat mutation which expands a region on the X chromosome containing the FMR1 gene. In FXS, a full mutation (> 200 repeats) leads to hypermethylation of FMR1, an epigenetic mechanism that effectively silences FMR1 gene expression and reduces levels of the FMR1 gene product, fragile X mental retardation protein (FMRP). FMRP is an RNA-binding protein that is important for the regulation of protein expression. In an effort to further understand how loss of FMR1 and FMRP contribute to FXS symptomology, several FXS animal models have been created. The most well characterized rodent model is the Fmr1 knockout (KO) mouse, which lacks FMRP protein due to a disruption in its Fmr1 gene. Here, we review the behavioral phenotyping of the Fmr1 KO mouse to date, and discuss the clinical relevance of this mouse model to the human FXS condition. While much remains to be learned about FXS, the Fmr1 KO mouse is a valuable tool for understanding the repercussions of functional loss of FMRP and assessing the efficacy of pharmacological compounds in ameliorating the molecular and behavioral phenotypes relevant to FXS. PMID:25606362

  2. Complex decongestive physiotherapy decreases capillary fragility in lipedema.

    Science.gov (United States)

    Szolnoky, G; Nagy, N; Kovács, R K; Dósa-Rácz, E; Szabó, A; Bársony, K; Balogh, M; Kemény, L

    2008-12-01

    Lipedema is a disproportional obesity featuring frequent hematoma formation due to even minor traumatic injuries. On the basis of clinical observations, complete decongestive physiotherapy diminishes the incidence of hematomas due to minor injuries beyond leg volume reduction. Hematoma development may be caused by altered capillary resistance (CR) or altered capillary fragility (CF). We measured capillary fragility (CF) before and after complex decongestive physiotherapy (CDP) to examine, whether CDP could reduce CF. 38 women with lipedema were included in the study. Twenty-one (21) patients were treated with CDP and 17 using exclusively moisturizers as the control group. CDP comprised once daily manual lymph drainage, intermittent pneumatic compression and multilayered short-stretch bandaging performed throughout a 5-day-course. CF was evaluated with the vacuum suction method (VSM) using Parrot's angiosterrometer in both groups. Decongestive therapy resulted in a significant reduction of the number of petechiae while no change was detected within the control group. Complete decongestive physiotherapy significantly reduced CF in patients with lipedema and this reduction may lead to reduced hematoma formation.

  3. Evaluation of Seismic Fragility of Weir Structures in South Korea

    Directory of Open Access Journals (Sweden)

    Bu Seog Ju

    2015-01-01

    Full Text Available In order to reduce earthquake damage of multifunctional weir systems similar to a dam structure, this study focused on probabilistic seismic risk assessment of the weir structure using the fragility methodology based on Monte Carlo simulation (MCS, with emphasis on the uncertainties of the seismic ground motions in terms of near field induced pulse-like motions and far field faults. The 2D simple linear elastic plain strain finite element (FE model including soil structure foundations using tie connection method in ABAQUS was developed to incorporate the uncertainty. In addition, five different limit states as safety criteria were defined for the seismic vulnerability of the weir system. As a consequence, the results obtained from multiple linear time history analyses revealed that the weir structure was more vulnerable to the tensile stress of the mass concrete in both near and far field ground motions specified earthquake hazard levels. In addition, the system subjected to near field motions was primarily more fragile than that under far field ground motions. On the other hand, the probability of failure due to the tensile stress at weir sill and stilling basin showed the similar trend in the overall peak ground acceleration levels.

  4. Fragility of Floating Docks for Small Craft Marinas

    Science.gov (United States)

    Keen, A.; Eskijian, M.; Lynett, P. J.; Ayca, A.

    2015-12-01

    Because of the damage resulting from the 2010 Chile and 2011 Japanese tele-tsunamis, damage to the small craft marinas in California has become an important concern. This paper will explain the methodology and results used to simulate the demand and also the structural capacity of the floating dock system, composed of floating docks, fingers and moored vessels during tsunami events. The intent is to develop a predictive tool to understand the vulnerability of California's small craft harbors to tsunami events. To validate the methodology, the probabilistic model will be applied to Santa Cruz Harbor. Maps of maximum velocity and mean current direction from the 2011 Japan tsunami have been developed using a numerical model. Cleat and pile guide locations will be recorded and georeferenced from aerial images before the event. The fragility curves for each dock/finger system will be compared with damage reports and aerial images from just after the tsunami event. A discussion of how the fragility curves compare with the damage reports will be included. It is anticipated that these curves will be useful to marina operators to use as a tool to determine where rehabilitation might be necessary to mitigate some of the damage from the next event. Conclusions will focus on how results can be used by marina operators to reduce harbor vulnerability to tsunamis.

  5. Neurological and endocrine phenotypes of fragile X carrier women.

    Science.gov (United States)

    Hall, D; Todorova-Koteva, K; Pandya, S; Bernard, B; Ouyang, B; Walsh, M; Pounardjian, T; Deburghraeve, C; Zhou, L; Losh, M; Leehey, M; Berry-Kravis, E

    2016-01-01

    Women who carry fragile X mental retardation 1 (FMR1)gene premutation expansions frequently report neurological or endocrine symptoms and prior studies have predominantly focused on questionnaire report of medical issues. Premutation carrier (PMC) women (n = 33) and non-carrier controls (n = 13) were recruited and evaluated by a neurologist, neuropsychologist, and endocrinologist. Blood and skin biopsies were collected for molecular measures. Scales for movement disorders, neuropathy, cognitive function, psychiatric symptoms, sleep, and quality of life were completed. The average age of the women was 51 years (n = 46) and average CGG repeat size was 91 ± 24.9 in the FMR1 PMC women. Seventy percent of the PMC women had an abnormal neurological examination. PMC women had significantly higher scores on the Fragile X-Associated Tremor Ataxia Syndrome (FXTAS) rating scale, more neuropathy, and difficulty with tandem gait compared to controls. Central sensitivity syndromes, a neuroticism profile on the NEO Personality Profile, and sleep disorders were also prevalent. Discrepancies between subject report and examination findings were also seen. This pilot study suggests that women with the FMR1 premutation may have a phenotype that overlaps with that seen in FXTAS. Additional research with larger sample sizes is warranted to better delineate the clinical features. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Fragility, anharmonicity and anelasticity of silver borate glasses

    International Nuclear Information System (INIS)

    Carini, Giovanni; Carini, Giuseppe; D'Angelo, Giovanna; Tripodo, Gaspare; Bartolotta, Antonio; Marco, Gaetano Di

    2006-01-01

    The fragility and the anharmonicity of (Ag 2 O) x (B 2 O 3 ) 1-x borate glasses have been quantified by measuring the change in the specific heat capacity at the glass transition temperature T g and the room-temperature thermodynamic Grueneisen parameter. Increasing the silver oxide content above X = 0.10 leads to an increase of both the parameters, showing that a growing fragility of a glass-forming liquid is predictive of an increasing overall anharmonicity of its glassy state. The attenuation and velocity of ultrasonic waves of frequencies in the range of 10-70 MHz have also been measured in silver borate glasses as a function of temperature between 1.5 and 300 K. The experimental data reveal anelastic behaviours which are governed by (i) quantum-mechanical tunnelling below 20 K (ii) thermally activated relaxations between 20 and 200 K and (iii) vibrational anharmonicity at even higher temperatures. Evaluation of tunnelling (C) and relaxation (C * ) strengths shows that C is independent of the structural changes affecting the borate network with increasing metal oxide content and is at least one order of magnitude smaller than C * . The latter observation implies that only a small fraction of the locally mobile defects are subjected to tunnelling motions

  7. Drift-free MPEG-4 AVC semi-fragile watermarking

    Science.gov (United States)

    Hasnaoui, M.; Mitrea, M.

    2014-02-01

    While intra frame drifting is a concern for all types of MPEG-4 AVC compressed-domain video processing applications, it has a particular negative impact in watermarking. In order to avoid the drift drawbacks, two classes of solutions are currently considered in the literature. They try either to compensate the drift distortions at the expense of complex decoding/estimation algorithms or to restrict the insertion to the blocks which are not involved in the prediction, thus reducing the data payload. The present study follows a different approach. First, it algebraically models the drift distortion spread problem by considering the analytic expressions of the MPEG-4 AVC encoding operations. Secondly, it solves the underlying algebraic system under drift-free constraints. Finally, the advanced solution is adapted to take into account the watermarking peculiarities. The experiments consider an m-QIM semi-fragile watermarking method and a video surveillance corpus of 80 minutes. For prescribed data payload (100 bit/s), robustness (BER < 0.1 against transcoding at 50% in stream size), fragility (frame modification detection with accuracies of 1/81 from the frame size and 3s) and complexity constraints, the modified insertion results in gains in transparency of 2 dB in PSNR, of 0.4 in AAD, of 0.002 in IF, of 0.03 in SC, of 0.017 NCC and 22 in DVQ.

  8. Delineating the Profile of Autism Spectrum Disorder Characteristics in Cornelia de Lange and Fragile X Syndromes

    Science.gov (United States)

    Moss, Joanna; Oliver, Chris; Nelson, Lisa; Richards, Caroline; Hall, Scott

    2013-01-01

    An atypical presentation of autism spectrum disorder is noted in Cornelia de Lange and Fragile X syndromes, but there are few detailed empirical descriptions. Participants in this study were individuals with Cornelia de Lange syndrome (n = 130, M age = 17.19), Fragile X syndrome (n = 182, M age = 16.94), and autism spectrum disorder (n = 142, M…

  9. The correlation between fragility, density, and atomic interaction in glass-forming liquids.

    Science.gov (United States)

    Wang, Lijin; Guan, Pengfei; Wang, W H

    2016-07-21

    The fragility that controls the temperature-dependent viscous properties of liquids as the glass transition is approached, in various glass-forming liquids with different softness of the repulsive part of atomic interactions at different densities, is investigated by molecular dynamic simulations. We show that the landscape of fragility in purely repulsive systems can be separated into three regions denoted as RI, RII, and RIII, respectively, with qualitatively disparate dynamic behaviors: RI which can be described by "softness makes strong glasses," RII where fragility is independent of softness and can only be tuned by density, and RIII with constant fragility, suggesting that density plays an unexpected role for understanding the repulsive softness dependence of fragility. What is more important is that we unify the long-standing inconsistence with respect to the repulsive softness dependence of fragility by observing that a glass former can be tuned more fragile if nonperturbative attraction is added into it. Moreover, we find that the vastly dissimilar influences of attractive interaction on fragility could be estimated from the structural properties of related zero-temperature glasses.

  10. Cytogenetic Survey for Autistic Fragile X Carriers in a Mental Retardation Center.

    Science.gov (United States)

    Cantu, Eduardo S.; And Others

    1990-01-01

    The cytogenetic survey of 67 individuals previously identified as having mental retardation and autistic behaviors revealed only 1.5 percent with the fragile X chromosome. The finding suggests that most persons with fragile X syndrome do not have autistic behaviors severe enough to be identified as a secondary psychiatric diagnosis. (Author/DB)

  11. Transport of fragile X mental retardation protein via granules in neurites of PC12 cells

    NARCIS (Netherlands)

    Y. de Diego Otero (Yolanda); E.A.W.F.M. Severijnen (Lies-Anne); W.A. van Cappellen (Gert); M. Schrier (Mariëtte); R. Willemsen (Rob); B.A. Oostra (Ben)

    2002-01-01

    textabstractLack of fragile X mental retardation protein (FMRP) causes fragile X syndrome, a common form of inherited mental retardation. FMRP is an RNA binding protein thought to be involved in translation efficiency and/or trafficking of certain mRNAs. Recently, a subset of mRNAs

  12. Savings and extinction of conditioned eyeblink responses in fragile X syndrome

    DEFF Research Database (Denmark)

    Smit, A E; van der Geest, J N; Vellema, M

    2008-01-01

    The fragile X syndrome (FRAXA) is the most widespread heritable form of mental retardation caused by the lack of expression of the fragile X mental retardation protein (FMRP). This lack has been related to deficits in cerebellum-mediated acquisition of conditioned eyelid responses in individuals...

  13. Positron Emission Tomography (PET Quantification of GABAA Receptors in the Brain of Fragile X Patients.

    Directory of Open Access Journals (Sweden)

    Charlotte D'Hulst

    Full Text Available Over the last several years, evidence has accumulated that the GABAA receptor is compromised in animal models for fragile X syndrome (FXS, a common hereditary form of intellectual disability. In mouse and fly models, agonists of the GABAA receptor were able to rescue specific consequences of the fragile X mutation. Here, we imaged and quantified GABAA receptors in vivo in brain of fragile X patients using Positron Emission Topography (PET and [11C]flumazenil, a known high-affinity and specific ligand for the benzodiazepine site of GABAA receptors. We measured regional GABAA receptor availability in 10 fragile X patients and 10 control subjects. We found a significant reduction of on average 10% in GABAA receptor binding potential throughout the brain in fragile X patients. In the thalamus, the brain region showing the largest difference, the GABAA receptor availability was even reduced with 17%. This is one of the first reports of a PET study of human fragile X brain and directly demonstrates that the GABAA receptor availability is reduced in fragile X patients. The study reinforces previous hypotheses that the GABAA receptor is a potential target for rational pharmacological treatment of fragile X syndrome.

  14. Examining the Function of Problem Behavior in Fragile X Syndrome: Preliminary Experimental Analysis

    Science.gov (United States)

    Langthorne, Paul; McGill, Peter; O'Reilly, Mark F.; Lang, Russell; Machalicek, Wendy; Chan, Jeffrey Michael; Rispoli, Mandy

    2011-01-01

    Fragile X syndrome is the most common inherited cause of intellectual and developmental disability. The influence of environmental variables on behaviors associated with the syndrome has received only scant attention. The current study explored the function served by problem behavior in fragile X syndrome by using experimental functional analysis…

  15. Expressive Language during Conversational Speech in Boys with Fragile X Syndrome

    Science.gov (United States)

    Roberts, Joanne E.; Hennon, Elizabeth A.; Price, Johanna R.; Dear, Elizabeth; Anderson, Kathleen; Vandergrift, Nathan A.

    2007-01-01

    We compared the expressive syntax and vocabulary skills of 35 boys with fragile X syndrome and 27 younger typically developing boys who were at similar nonverbal mental levels. During a conversational speech sample, the boys with fragile X syndrome used shorter, less complex utterances and produced fewer different words than did the typically…

  16. Fragile X Syndrome: An Educator's Role in Identification, Prevention, and Intervention.

    Science.gov (United States)

    Santos, Karen E.

    1992-01-01

    This review highlights the physical, intellectual, academic, behavioral, and communication characteristics associated with fragile X syndrome, the most common inherited form of mental retardation. Educationally relevant problems linked to fragile X are noted, including learning disabilities, attention deficit disorders, speech and language…

  17. Discourse Skills of Boys with Fragile X Syndrome in Comparison to Boys with Down Syndrome

    Science.gov (United States)

    Roberts, Joanne; Martin, Gary E.; Moskowitz, Lauren; Harris, Adrianne A.; Foreman, Jamila; Nelson, Lauren

    2007-01-01

    Purpose: This study compared the conversational discourse skills of boys who have fragile X syndrome with and without autism spectrum disorder (ASD) with those of boys with Down syndrome and boys who are typically developing. Method: Participants were boys who have fragile X syndrome with (n = 26) and without (n = 28) ASD, boys with Down syndrome…

  18. A Comparison of Phonological Skill of Boys with Fragile X Syndrome and Down Syndrome

    Science.gov (United States)

    Roberts, Joanne; Long, Steven H.; Malkin, Cheryl; Barnes, Elizabeth; Skinner, Martie; Hennon, Elizabeth A.; Anderson, Kathleen

    2005-01-01

    In this study, the authors compared the phonological accuracy and patterns of sound change of boys with fragile X syndrome, boys with Down syndrome, and typically developing mental-age-matched boys. Participants were 50 boys with fragile X syndrome, ages 3 to 14 years; 32 boys with Down syndrome, ages 4 to 13 years; and 33 typically developing…

  19. Cerebral protein synthesis in a knockin mouse model of the fragile X premutation

    NARCIS (Netherlands)

    M. Qin (Mei); T. Huang (Tianjian); Z. Liu (Zhonghua); M. Kader (Michael); T. Burlin (Thomas); Z. Xia (Zengyan); Z. Zeidler (Zachary); R.K. Hukema (Renate); C.B. Smith (Carolyn B.)

    2014-01-01

    textabstractThe (CGG)n-repeat in the 5’-untransiated region of the fragile X mental retardation gene (FMRi) gene is polymorphic and may become unstable on transmission to the next generation. In fragile X syndrome, CGG repeat lengths exceed 200, resulting in silencing of FMRi and absence of its

  20. Global Identification of Disease-Associated Genes in Fragile X Cells

    Science.gov (United States)

    2017-03-01

    assisted me in writing a book chapter titled “Fragility Extraordinaire: Unsolved mysteries of chromosome fragile sites” in a special edition, “DNA...presented results at three international conferences (detailed in section 6 “Products”) in the form of both oral and poster presentations. o What do you

  1. Effective updating process of seismic fragilities using Bayesian method and information entropy

    International Nuclear Information System (INIS)

    Kato, Masaaki; Takata, Takashi; Yamaguchi, Akira

    2008-01-01

    Seismic probabilistic safety assessment (SPSA) is an effective method for evaluating overall performance of seismic safety of a plant. Seismic fragilities are estimated to quantify the seismically induced accident sequences. It is a great concern that the SPSA results involve uncertainties, a part of which comes from the uncertainty in the seismic fragility of equipment and systems. A straightforward approach to reduce the uncertainty is to perform a seismic qualification test and to reflect the results on the seismic fragility estimate. In this paper, we propose a figure-of-merit to find the most cost-effective condition of the seismic qualification tests about the acceleration level and number of components tested. Then a mathematical method to reflect the test results on the fragility update is developed. A Bayesian method is used for the fragility update procedure. Since a lognormal distribution that is used for the fragility model does not have a Bayes conjugate function, a parameterization method is proposed so that the posterior distribution expresses the characteristics of the fragility. The information entropy is used as the figure-of-merit to express importance of obtained evidence. It is found that the information entropy is strongly associated with the uncertainty of the fragility. (author)

  2. An Investigation of Narrative Ability in Boys with Autism and Fragile X Syndrome

    Science.gov (United States)

    Hogan-Brown, Abigail L.; Losh, Molly; Martin, Gary E.; Mueffelmann, Deborah J.

    2013-01-01

    Whereas pragmatic language difficulties are characteristic of both autism and Fragile X syndrome, it is unclear whether such deficits are qualitatively similar or whether certain skills are differentially affected. This study compared narrative competence in boys with autism, Fragile X syndrome, Down syndrome, and typical development. Results…

  3. Genetics Home Reference: fragile X-associated tremor/ataxia syndrome

    Science.gov (United States)

    ... Share: Email Facebook Twitter Home Health Conditions FXTAS Fragile X-associated tremor/ataxia syndrome Printable PDF Open All ... Javascript to view the expand/collapse boxes. Description Fragile X-associated tremor/ataxia syndrome ( FXTAS ) is characterized by ...

  4. Adaptive Skills, Behavior Problems, and Parenting Stress in Mothers of Boys with Fragile X Syndrome

    Science.gov (United States)

    Sarimski, Klaus

    2010-01-01

    The relationship of temperament, atypical behaviors, and adaptive behavior of young boys with Fragile X syndrome on mothers' parenting stress was analyzed. Twenty-six boys with Fragile X syndrome (30-88 months of age) participated. The overall development of the participants was significantly delayed with a specific profile of adaptive behaviors…

  5. Visual Pathway Deficit in Female Fragile X Premutation Carriers: A Potential Endophenotype

    Science.gov (United States)

    Keri, Szabolcs; Benedek, Gyorgy

    2009-01-01

    Previous studies indicated impaired magnocellular (M) and relatively spared parvocellular (P) visual pathway functioning in patients with fragile X syndrome. In this study, we assessed M and P pathways in 22 female fragile X premutation carriers with normal intelligence and in 20 healthy non-carrier controls. Testing procedure included visual…

  6. Early White-Matter Abnormalities of the Ventral Frontostriatal Pathway in Fragile X Syndrome

    Science.gov (United States)

    Haas, Brian W.; Barnea-Goraly, Naama; Lightbody, Amy A.; Patnaik, Swetapadma S.; Hoeft, Fumiko; Hazlett, Heather; Piven, Joseph; Reiss, Allan L.

    2009-01-01

    Aim: Fragile X syndrome is associated with cognitive deficits in inhibitory control and with abnormal neuronal morphology and development. Method: In this study, we used a diffusion tensor imaging (DTI) tractography approach to reconstruct white-matter fibers in the ventral frontostriatal pathway in young males with fragile X syndrome (n = 17;…

  7. Behavioral Intervention for Problem Behavior in Children with Fragile X Syndrome

    Science.gov (United States)

    Moskowitz, Lauren J.; Carr, Edward G.; Durand, V. Mark

    2011-01-01

    Parents and professionals typically report problem behavior as a significant concern for children with fragile X syndrome. In the present study, the authors explored whether behaviorally based interventions would result in a reduction in problem behavior and an improvement in quality of life for 3 children with fragile X syndrome and their…

  8. Young Adult Female Fragile X Premutation Carriers Show Age- and Genetically-Modulated Cognitive Impairments

    Science.gov (United States)

    Goodrich-Hunsaker, Naomi J.; Wong, Ling M.; McLennan, Yingratana; Srivastava, Siddharth; Tassone, Flora; Harvey, Danielle; Rivera, Susan M.; Simon, Tony J.

    2011-01-01

    The high frequency of the fragile X premutation in the general population and its emerging neurocognitive implications highlight the need to investigate the effects of the premutation on lifespan cognitive development. Until recently, cognitive function in fragile X premutation carriers (fXPCs) was presumed to be unaffected by the mutation. Here…

  9. Resolution of Spatial and Temporal Visual Attention in Infants with Fragile X Syndrome

    Science.gov (United States)

    Farzin, Faraz; Rivera, Susan M.; Whitney, David

    2011-01-01

    Fragile X syndrome is the most common cause of inherited intellectual impairment and the most common single-gene cause of autism. Individuals with fragile X syndrome present with a neurobehavioural phenotype that includes selective deficits in spatiotemporal visual perception associated with neural processing in frontal-parietal networks of the…

  10. The Trajectory of Mathematics Skills and Working Memory Thresholds in Girls with Fragile X Syndrome

    Science.gov (United States)

    Murphy, Melissa M.; Mazzocco, Michele M. M.

    2009-01-01

    Fragile X syndrome is a common genetic disorder associated with executive function deficits and poor mathematics achievement. In the present study, we examined changes in math performance during the elementary and middle school years in girls with fragile X syndrome, changes in the working memory loads under which children could complete a…

  11. Public private cooperation fragile states: Report on field research mission to North Kivu and Ituri

    NARCIS (Netherlands)

    Douma, P.; Bolhuis, E.E.; Klaver, D.C.; Zawadi, Y.

    2009-01-01

    This field study is part of the Schokland project to research among others the possibilities for Public Private Cooperation (PPC) in stimulating Economic Growth in Fragile States. Fragile states are relatively far behind in achieving the Millennium Development Goals (MDGs) and in order to reduce

  12. Structure and management of tuberculosis control programs in fragile states--Afghanistan, DR Congo, Haiti, Somalia

    NARCIS (Netherlands)

    Mauch, Verena; Weil, Diana; Munim, Aayid; Boillot, Francois; Coninx, Rudi; Huseynova, Sevil; Powell, Clydette; Seita, Akihiro; Wembanyama, Henriette; van den Hof, Susan

    2010-01-01

    Health care delivery is particularly problematic in fragile states often connected with increased incidence of communicable diseases, among them tuberculosis. This article draws upon experiences in tuberculosis control in four fragile states from which four lessons learned were derived. A structured

  13. Ocular Motor Indicators of Executive Dysfunction in Fragile X and Turner Syndromes

    Science.gov (United States)

    Lasker, Adrian G.; Mazzocco, Michele M. M.; Zee, David S.

    2007-01-01

    Fragile X and Turner syndromes are two X-chromosome-related disorders associated with executive function and visual spatial deficits. In the present study, we used ocular motor paradigms to examine evidence that disruption to different neurological pathways underlies these deficits. We tested 17 females with fragile X, 19 females with Turner…

  14. Mathematics Learning Disabilities in Girls with Fragile X or Turner Syndrome during Late Elementary School

    Science.gov (United States)

    Murphy, Melissa M.; Mazzocco, Michele M. M.

    2008-01-01

    The present study focuses on math and related skills among 32 girls with fragile X (n = 14) or Turner (n = 18) syndrome during late elementary school. Performance in each syndrome group was assessed relative to Full Scale IQ-matched comparison groups of girls from the general population (n = 32 and n = 89 for fragile X syndrome and Turner…

  15. Behavioral Assessment of Social Anxiety in Females with Turner or Fragile X Syndrome.

    Science.gov (United States)

    Lesniak-Karpiak, Katarzyna; Mazzocco, Michele M. M.; Ross, Judith L.

    2003-01-01

    This study compared 29 females with Turner syndrome and 21 females with fragile X syndrome (ages 6-22) on a videotaped role-play interaction with 34 females in a comparison group. Three of eight behavioral measures of social skills differentiated the participant groups. Fragile-X subjects required more time to initiate interactions and Turner…

  16. Communication for Development Interventions in Fragile States: A Systematic Review

    Science.gov (United States)

    Skuse, Andrew; Rodger, Dianne; Power, Gerry; Mbus, Domenic Friguglietti; Brimacombe, Tait

    2013-01-01

    Executive summary Background A wide range of contextual and programmatic factors frame, affect and constrain communication for development (C4D) interventions undertaken in fragile or conflict affected states. For the purposes of this review, contextual factors include culture, poverty, different stages of conflict (such as latent, open or post-conflict scenarios), policy, legislation and so on, while programmatic factors include the type of intervention, formative and summative evaluation, project design and management, human and financial resources and so on. Understanding the various factors that influence C4D interventions in fragile states is important to improving practice, implementation and evaluation, as well as to the future development of methodologies and frameworks that can be utilised in conflict or crisis situations. Objective The objective of this review is to assess the contextual and programmatic factors that influence communication for development interventions in fragile states. Types of participants Persons regardless of age, gender and ethnicity – living in fragile states. Phenomena of interest The contextual and programmatic factors that influence communication for development (C4D) interventions in fragile states. Types of studies Qualitative peer reviewed studies, expert opinion, discussion papers, project reports, policy papers, position papers and other text. Search strategy Searches were conducted for published and unpublished material (between January 2001 – September 2011), including grey literature, in the English language. Databases searched were: Academic Search Premier; African Women's Bibliographic Database; Anthropology Plus; Bibliography of Asian Studies; Educational Resources Information Centre; Ingenta Connect; JSTOR; Scopus; and Sociological Abstracts; Communication for Social Change Consortium; DevComm (World Bank); Eldis; Search for Common Ground; The Communication Initiative; United Nations Development Programme

  17. Handbook of nuclear power plant seismic fragilities, Seismic Safety Margins Research Program

    Energy Technology Data Exchange (ETDEWEB)

    Cover, L.E.; Bohn, M.P.; Campbell, R.D.; Wesley, D.A.

    1983-12-01

    The Seismic Safety Margins Research Program (SSMRP) has a gola to develop a complete fully coupled analysis procedure (including methods and computer codes) for estimating the risk of an earthquake-induced radioactive release from a commercial nuclear power plant. As part of this program, calculations of the seismic risk from a typical commercial nuclear reactor were made. These calculations required a knowledge of the probability of failure (fragility) of safety-related components in the reactor system which actively participate in the hypothesized accident scenarios. This report describes the development of the required fragility relations and the data sources and data reduction techniques upon which they are based. Both building and component fragilities are covered. The building fragilities are for the Zion Unit 1 reactor which was the specific plant used for development of methodology in the program. Some of the component fragilities are site-specific also, but most would be usable for other sites as well.

  18. Seismic Safety Margins Research Program Phase I final report: fragilities development (Project VI)

    International Nuclear Information System (INIS)

    Bohn, M.P.; Cover, L.E.; Dong, R.G.; Vagliente, V.N.; Campbell, R.D.; Wesley, D.A.

    1982-12-01

    The Seismic Safety Margins Research Program is an NRC-funded program directed towards estimating the conservatism in the NRC Standard Review Plan seismic safety requirements with the ultimate goal of developing improved requirements. As part of this program, calculations of the seismic risk from a typical commercial nuclear reactor are being made. These calculations require a knowledge of the probability of failure (fragility) of safety-related components in the reactor system which actively participate in the hypothesized accident scenarios. This report describes the development of the required fragility relations and the data sources and data reduction techniques upon which they are based. Both building and component fragilities are covered. The building fragilities are for the Zion Unit 1 reactor which was the specific plant used for development of methodology in the program. Some of the component fragilities are site-specific also, but most have been developed to apply to generic categories of components

  19. Language use in females with fragile X or Turner syndrome during brief initial social interactions.

    Science.gov (United States)

    Mazzocco, Michèle M M; Thompson, Laurie; Sudhalter, Vicki; Belser, Richard C; Lesniak-Karpiak, Katarzyna; Ross, Judith L

    2006-08-01

    Fragile X and Turner syndromes are associated with risk of atypical social function. We examined language use, including normal and atypical speech, during initial social interactions among participants engaged in a brief social role play with an unfamiliar adult. There were 27 participants with Turner syndrome, 20 with fragile X syndrome and 28 in an age-matched comparison group. Females with fragile X did not exhibit more abnormal language, but exhibited less of what is typical during initial interactions. Overall rates of dysfluencies did not differ, although females with fragile X made more phrase repetitions. Females with Turner syndrome had no language use abnormalities. Our findings suggest that language use may influence social function in females with fragile X syndrome and that such language characteristics may be observed in the context of brief encounters with an unfamiliar adult.

  20. Handbook of nuclear power plant seismic fragilities, Seismic Safety Margins Research Program

    International Nuclear Information System (INIS)

    Cover, L.E.; Bohn, M.P.; Campbell, R.D.; Wesley, D.A.

    1983-12-01

    The Seismic Safety Margins Research Program (SSMRP) has a gola to develop a complete fully coupled analysis procedure (including methods and computer codes) for estimating the risk of an earthquake-induced radioactive release from a commercial nuclear power plant. As part of this program, calculations of the seismic risk from a typical commercial nuclear reactor were made. These calculations required a knowledge of the probability of failure (fragility) of safety-related components in the reactor system which actively participate in the hypothesized accident scenarios. This report describes the development of the required fragility relations and the data sources and data reduction techniques upon which they are based. Both building and component fragilities are covered. The building fragilities are for the Zion Unit 1 reactor which was the specific plant used for development of methodology in the program. Some of the component fragilities are site-specific also, but most would be usable for other sites as well

  1. The mGluR theory of fragile X mental retardation.

    Science.gov (United States)

    Bear, Mark F; Huber, Kimberly M; Warren, Stephen T

    2004-07-01

    Many of the diverse functional consequences of activating group 1 metabotropic glutamate receptors require translation of pre-existing mRNA near synapses. One of these consequences is long-term depression (LTD) of transmission at hippocampal synapses. Loss of fragile X mental retardation protein (FMRP), the defect responsible for fragile X syndrome in humans, increases LTD in mouse hippocampus. This finding is consistent with the growing evidence that FMRP normally functions as a repressor of translation of specific mRNAs. Here we present a theory that can account for diverse neurological and psychiatric aspects of fragile X syndrome, based on the assumption that many of the protein-synthesis-dependent functions of metabotropic receptors are exaggerated in fragile X syndrome. The theory suggests new directions for basic research as well as novel therapeutic approaches for the treatment of humans with fragile X, the most frequent inherited cause of mental retardation and an identified cause of autism.

  2. The efficacy of melatonin for sleep problems in children with autism, fragile X syndrome, or autism and fragile X syndrome.

    Science.gov (United States)

    Wirojanan, Juthamas; Jacquemont, Sebastien; Diaz, Rafael; Bacalman, Susan; Anders, Thomas F; Hagerman, Randi J; Goodlin-Jones, Beth L

    2009-04-15

    To determine the efficacy of melatonin on sleep problems in children with autistic spectrum disorder (ASD) and fragile X syndrome (FXS). A 4-week, randomized, double blind, placebo-controlled, crossover design was conducted following a 1-week baseline period. Either melatonin, 3 mg, or placebo was given to participants for 2 weeks and then alternated for another 2 weeks. Sleep variables, including sleep duration, sleep-onset time, sleep-onset latency time, and the number of night awakenings, were recorded using an Actiwatch and from sleep diaries completed by parents. All participants had been thoroughly assessed for ASD and also had DNA testing for the diagnosis of FXS. Data were successfully obtained from the 12 of 18 subjects who completed the study (11 males, age range 2 to 15.25 years, mean 5.47, SD 3.6). Five participants met diagnostic criteria for ASD, 3 for FXS alone, 3 for FXS and ASD, and 1 for fragile X premutation. Eight out of 12 had melatonin first. The conclusions from a nonparametric repeated-measures technique indicate that mean night sleep duration was longer on melatonin than placebo by 21 minutes (p = .02), mean sleep-onset latency was shorter by 28 minutes (p = .0001), and mean sleep-onset time was earlier by 42 minutes (p = .02). The results of this study support the efficacy and tolerability of melatonin treatment for sleep problems in children with ASD and FXS.

  3. Quantitative sensory testing is feasible and is well-tolerated in patients with sickle cell disease following a vaso-occlusive episode

    Directory of Open Access Journals (Sweden)

    Bakshi N

    2018-02-01

    Full Text Available Nitya Bakshi,1 Ines Lukombo,1,2 Inna Belfer,3 Lakshmanan Krishnamurti1 1Division of Pediatric Hematology-Oncology, Department of Pediatrics, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 2University of Pittsburgh, 3Department of Anesthesiology, University of Pittsburgh, Pittsburgh, PA, USA Introduction: Sickle cell disease (SCD is an inherited blood disorder characterized by abnormally shaped sickle cells. The hallmark of this disease is intermittent, painful vaso-occlusive episodes (VOE, but a subset of individuals with SCD experience chronic pain. The mechanism of transition to chronic pain is not well understood in SCD, but there is evidence of altered pain processing in individuals with SCD. The impact of VOE on pain sensitivity is not established. The objective of this study was to determine the feasibility and tolerability of quantitative sensory testing (QST in SCD following a VOE to better understand the contribution of VOE to the development of chronic pain. Methods: As part of a larger pain sensitivity study, pediatric patients with SCD were offered QST following a VOE-related Emergency Room visit or inpatient hospitalization. The feasibility of recruitment and completion of QST was measured, and tolerability of QST was determined using post-QST assessments of pain, and compared with measurements at steady state. Results: Ten participants completed QST following a VOE. The median age was 16.5, and 60% were female. Overall, 10 of 16 (62.5% patients approached for QST following VOE completed QST. This included 8 of 12 patients who had previously completed QST at steady state. There were no statistically significant differences in pain intensity and Gracely Box scores after QST following a VOE, when compared to steady-state QST. Conclusion: QST is feasible and is well-tolerated following a VOE in patients with SCD. Large prospective studies are needed to determine the impact of VOE on experimental pain sensitivity and must

  4. Pain management trend of vaso-occulsive crisis (VOC) at a community hospital emergency department (ED) for patients with sickle cell disease.

    Science.gov (United States)

    Inoue, Susumu; Khan, Isra'a; Mushtaq, Rao; Sanikommu, Srinivasa Reddy; Mbeumo, Carline; LaChance, Jenny; Roebuck, Michael

    2016-01-01

    Pain management at the emergency department (ED) for vaso-occulsive crisis (VOC) for patients with sickle cell disease has not been optimum, with a long delay in giving the initial analgesic. We conducted a retrospective survey over a 7-year period to determine our ED's timing in giving pain medication to patients with VOC as a quality improvement project. We compared different periods, children vs adults, and the influence of gender in the analgesic administration timing. This is a retrospective chart review of three different periods: (1) years 2007-2008, (2) years 2011-2012, and (3) year 2013. We extracted relevant information from ED records. Data were analyzed using Student t test, chi-square analysis, and the Kruskal-Wallis test. There was a progressive improvement in the time interval to the 1st analgesic over these three periods. Children received analgesics more quickly than adults in all periods. Male adult patients received pain medication faster than female adult patients, although initial pain scores were higher in female than in male patients. Progressively fewer pediatric patients utilized ED over these three periods, but no difference for adult patients was observed. The proportion of pediatric patients admitted to the hospital increased with each period. The progressive decrease in both the number of patients and the number of visits to the ED by children suggested that the collective number of VOC in children has decreased, possibly secondary to the dissemination of hydroxyurea use. We failed to observe the same trend in adult patients. The need for IV access, and ordering laboratory tests or imaging studies tends to delay analgesic administration. Delay in administration of the first analgesic was more pronounced for female adult patients than male adult patients in spite of their higher pain score. Health care providers working in ED should make conscious efforts to respect pain in women as well as pain in men. Though not proven from this study

  5. Tracking development assistance for health to fragile states: 2005-2011.

    Science.gov (United States)

    Graves, Casey M; Haakenstad, Annie; Dieleman, Joseph L

    2015-03-19

    Development assistance for health (DAH) has grown substantially, totaling more than $31.3 billion in 2013. However, the degree that countries with high concentrations of armed conflict, ethnic violence, inequality, debt, and corruption have received this health aid and how that assistance might be different from the funding provided to other countries has not been assessed. We combine DAH estimates and a multidimensional fragile states index for 2005 through 2011. We disaggregate and compare total DAH disbursed for fragile states versus stable states. Between 2005 and 2011, DAH per person in fragile countries increased at an annualized rate of 5.4%. In 2011 DAH to fragile countries totaled $6.2 billion, which is $5.05 per person. This is 43% of total DAH that is traced to a country. Comparing low-income countries, funding channeled to fragile countries was $7.22 per person while stable countries received $11.15 per person. Relative to stable countries, donors preferred to provide more funding to low-income fragile countries that have refugees or ongoing external intervention but tended to avoid providing funding to countries with political gridlock, flawed elections, or economic decline. In 2011, Ethiopia received the most health aid of all fragile countries, while the United States provided the most funds to fragile countries. In 2011, 1.2 billion people lived in fragile countries. DAH can bolster health systems and might be especially valuable in providing long-term stability in fragile environments. While external health funding to these countries has increased since 2005, it is, in per person terms, almost half as much as the DAH provided to stable countries of comparable income levels.

  6. Boro em arroz de terras altas cultivado em solução nutritiva

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio Pavinato

    2009-09-01

    Full Text Available O objetivo deste trabalho foi avaliar o efeito de doses de boro na produção de matéria seca e nos parâmetros morfológicos das raízes de duas cultivares de arroz de terras altas. O experimento foi desenvolvido de abril a julho de 2004, em casa de vegetação, em Botucatu (SP, empregando-se duas cultivares de arroz - Caiapó e BRS Talento. As plântulas foram obtidas em condições controladas e após cinco dias, transferidas para vasos de plástico com capacidade para 8 L, utilizando areia lavada como suporte, deixando quatro plantas por vaso. Os tratamentos foram: 0,0; 0,5; 1,0; 2,0; 4,0 e 8,0 mg L-1 de B, aplicados via solução nutritiva. O corte foi realizado aos 70 dias após o transplante, sendo coletadas parte aérea e sistema radicular separadamente, determinado suas matérias secas e concentrações de N e B, bem como o comprimento, área superficial e diâmetro médio radicular. Não foi possível obter efeito positivo da aplicação de boro, evidenciando que para a cultura do arroz a dose ótima é a inferior a 0,5 ml L-1 de B. A aplicação de 3,8 e 3,4 mg L-1 de B causou efeitos tóxicos às cultivares Caiapó e Talento respectivamente. O limite de toxicidade para raízes das duas cultivares é a concentração de 2,7 mg L-1 de B. Sob toxicidade severa de B as plantas de arroz reduzem expressivamente o comprimento e aumentam o diâmetro radicular, resultando em menor área de absorção de nutrientes.

  7. I’m incredible—or am I? : On the socialization of fragile self-views in children

    NARCIS (Netherlands)

    Brummelman, E.

    2015-01-01

    I’m incredible—or am I? This is a recurring and daunting question for children with fragile self-views, whose feelings of self-worth crumble in the face of setbacks. What are the origins of children’s fragile self-views, and how can interventions reduce the fragility of children’s self-views? The

  8. Emerging pharmacologic treatment options for fragile X syndrome

    Science.gov (United States)

    Schaefer, Tori L; Davenport, Matthew H; Erickson, Craig A

    2015-01-01

    Fragile X syndrome (FXS) is the most common single gene cause of intellectual disability and autism spectrum disorder. Caused by a silenced fragile X mental retardation 1 gene and the subsequent deficiency in fragile X mental retardation protein, patients with FXS experience a range of physical, behavioral, and intellectual debilitations. The FXS field, as a whole, has recently met with some challenges, as several targeted clinical trials with high expectations of success have failed to elucidate significant improvements in a variety of symptom domains. As new clinical trials in FXS are planned, there has been much discussion about the use of the commonly used clinical outcome measures, as well as study design considerations, patient stratification, and optimal age range for treatment. The evidence that modification of these drug targets and use of these failed compounds would prove to be efficacious in human clinical study were rooted in years of basic and translational research. There are questions arising as to the use of the mouse models for studying FXS treatment development. This issue is twofold: many of the symptom domains and molecular and biochemical changes assessed and indicative of efficacy in mouse model study are not easily amenable to clinical trials in people with FXS because of the intolerability of the testing paradigm or a lack of noninvasive techniques (prepulse inhibition, sensory hypersensitivity, startle reactivity, or electrophysiologic, biochemical, or structural changes in the brain); and capturing subtle yet meaningful changes in symptom domains such as sociability, anxiety, and hyperactivity in human FXS clinical trials is challenging with the currently used measures (typically parent/caregiver rating scales). Clinicians, researchers, and the pharmaceutical industry have all had to take a step back and critically evaluate the way we think about how to best optimize future investigations into pharmacologic FXS treatments. As new clinical

  9. The burden and undertreatment of fragility fractures among senior women.

    Science.gov (United States)

    Rodrigues, Ana M; Eusébio, Mónica; Santos, Maria José; Gouveia, Nélia; Tavares, Viviana; Coelho, Pedro S; Mendes, Jorge M; Branco, Jaime C; Canhão, Helena

    2018-03-07

    Using a large population database, we showed that fragility fractures were highly prevalent in senior women and were associated with significant physical disability. However, treatment rates were low because osteoporosis treatment was not prescribed or not agreed to by the majority of women with prevalent fragility fractures. The purpose of the study is to estimate prevalence of fragility fractures (FF), risk factors, and treatment rates in senior women and to assess impact of FF on physical function and quality of life. Women aged 65 years and older from the EpiReumaPt study (2011-2013) were evaluated. Rheumatologists collected data regarding FF, clinical risk factors for fractures, and osteoporosis (OP) treatment. Health-related quality of life (EQ5D) and physical function (HAQ) were analyzed. Peripheral dual-energy X-ray absorptiometry was performed. FF was defined as any self-reported low-impact fracture that occurred after 40 years of age. Prevalence estimates of FF were calculated. Among 3877 subjects evaluated in EpiReumaPt, 884 were senior women. The estimated prevalence of FF was 20.7%. Lower leg was the most frequent fracture site reported (37.8%) followed by wrist (18.6%). Only 7.1% of the senior women reporting a prevalent FF were under treatment for OP, and 13.9% never had treatment. OP treatment was not prescribed in 47.7% of FF women, and 23.4% refused treatment. Age (OR = 2.46, 95% CI 1.11-5.47), obesity (OR = 2.05, 95% CI 1.14-3.70), and low wrist BMD (OR = 2.29; 95% CI 1.20, 4.35; p = 0.012) were positively associated with prevalent FF. A significantly higher proportion of women in the lowest quintile of wrist bone mineral density reported FF (OR = 2.29, 95% CI 1.20-4.35). FF were associated with greater physical disability (β = 0.33, 95% CI 0.13-0.51) independent of other comorbidities. FF was frequently reported among senior women as an important cause of physical disability. However, the prevalence of OP treatment was

  10. INVESTIGAÇÃO DO USO DE HEPARINIZAÇÃO REGIONAL DURANTE ISQUEMIA ARTERIAL TEMPORÁRIA EM COELHOS

    Directory of Open Access Journals (Sweden)

    Moreschi Junior Dorival

    1999-01-01

    Full Text Available O objetivo deste trabalho foi avaliar o efeito da heparinização regional durante a isquemia arterial temporária em coelhos. Os parâmetros avaliados foram a medida do tempo de tromboplastina parcial ativada (TTPa e a presença ou ausência de trombos ou edema endotelial nos vasos estudados. Foram utilizados 40 coelhos Nova Zelândia, distribuídos em dois grupos de 20 animais. Após dissecção e reparo da artéria ilíaca esquerda, realizou uma arteriotomia e introduziu-se um cateter de polietileno para injeção de solução de heparina no grupo experimento ou de soro fisiológico no grupo controle. O TTPa foi medido no início do experimento e após 90 minutos de isquemia, não sendo encontrado diferenças significantes entre os valores iniciais e ao final do experimento. Na avaliação microscópica não foi encontrada a presença de trombos nos vasos dos animais de ambos os grupos. Em apenas um animal do grupo controle houve a ocorrência de espessamento intimal e desarranjo da camada média, interpretado como edema endotelial, porém este dado não foi significante. Concluiu-se que a injeção intra-arterial de heparina na dose de 60 UI/kg peso não provocou alterações significantes na coagulação sistêmica do coelho, e que na ausência de lesão endotelial não ocorreu trombose dos vasos estudados, com ou sem a utilização de heparina.

  11. Epidemiology of fragile skin: Internet-based surveys in Mexico and Russia

    Directory of Open Access Journals (Sweden)

    Ocampo-Candiani J

    2017-06-01

    Full Text Available Jorge Ocampo-Candiani,1 Nikolay Murashkin,2 Asmaa Zkik,3 Markéta Saint Aroman,4 Pierre Wolkenstein5 1Dermatology Department, Hospital Universitario Dr Jose Eleuterio Gonzalez, Universidad Autonoma de Nuevo Leon, Nuevo León, Mexico; 2Dermatology Department, Federal State Autonomous Institution, Scientific Center of Children’s Health of the Ministry of Health of the Russian Federation, Moscow, Russia; 3Pierre Fabre S.A., Boulogne Billancourt, 4Laboratoires A-DERMA, Pierre Fabre Dermo-Cosmétique, Lavaur, 5Department of Cancer, Immunity, Transplantation and Infections, Hôpital Henri Mondor (APHP, University Paris Est Créteil, Créteil, France Background: Fragile skin is a poorly understood skin condition, particularly in the general adult population. There are currently limited epidemiological data on the prevalence of fragile skin in adults. The objectives of this study were to assess the prevalence of perceived fragile skin across different skin types in representative samples of the general adult populations in Mexico and Russia, and to identify skin characteristics associated with perceived fragile skin.Methods: Two identical cross-sectional surveys, using a short online self-administered questionnaire, were conducted on samples of recruited individuals that were representative of the general Mexican and Russian populations. Participants responded to questions about fragile skin, with the main question being “In your opinion, do you have fragile skin (ie, skin less resistant and reacting quickly to external aggressions?”. The survey also covered questions relating to skin appearance, skin symptoms, skin disease, dermatological procedures, and living environment and climate.Results: Overall, 1,210 individuals in Mexico (N=606 and Russia (N=604 completed the online survey. Fragile skin was perceived in 50.0% and 45.9% of participants in Mexico and Russia, respectively. The principal skin appearance characteristics reported by

  12. Evaluación de los recursos hídricos en cabecera de las subcuencas de las provincias de Andahuaylas y Chincheros: levantamiento topográfico de vaso de lagunas

    OpenAIRE

    Autoridad Nacional del Agua. Dirección de Conservación y Planeamiento de Recursos Hídricos; Autoridad Nacional del Agua. Administración Local de Agua Bajo Apurímac Pampas

    2013-01-01

    Describe las características principales del levantamiento topográfico de vasos de lagunas en el ámbito de las sub-cuencas de las provincias de Andahuaylas y Chincheros, con el fin de evaluar futuros proyectos de afianzamiento hídrico. Las lagunas descritas en el presente informe corresponde a una selección priorizada en función a la importancia de optimizar el recurso hídrico en el ámbito de estudio.

  13. Mecanismos institucionales de control y transparencia en la rendición de cuentas en la gestión del programa de vaso de leche en el distrito de Carabayllo - Lima, año 2014

    OpenAIRE

    Ángeles Cueto, Víctor Alberto; Caller Loaiza, Alejandrina

    2017-01-01

    El presente trabajo de investigación tuvo como objetivo general, determinar si los mecanismos institucionales de control y transparencia en la rendición de cuentas inciden en la gestión del Programa del Vaso de Leche de la Municipalidad de Carabayllo - Lima, año 2014, contando con el apoyo de la Sub gerencia de dicha corporación edil, ubicada en el Distrito de Carabayllo, Provincia de Lima, Región Lima. Asimismo, se formuló como hipótesis general, los mecanismos institucionales de control...

  14. Expressão de caracteres de tubérculos em função do tamanho de recipiente usado no cultivo de batata na geração de plântulas Expression of tuber traits as a function of pot size used to grow potato seedlings

    Directory of Open Access Journals (Sweden)

    Mario Alvaro Aloisio Verissimo

    2012-12-01

    Full Text Available No melhoramento genético de batata, para alguns caracteres, é possível eliminar genótipos inferiores já nas gerações iniciais, o que permite reduzir o número de genótipos avaliados a campo, proporcionando redução de custos. O objetivo deste trabalho foi verificar a influência do tamanho de vaso na expressão de caracteres de tubérculo, em famílias de batata, na geração de plântula. Em casa de vegetação, dez famílias de batata foram avaliadas em dois tamanhos de recipientes, vaso grande (1 L e vaso pequeno (250 ml. Os caracteres de tubérculos avaliados foram: cor, aspereza, profundidade de olho, sobrancelha, formato, uniformidade de formato, apontamento, curvatura, achatamento, aparência geral, massa por planta, massa média e número de tubérculos. O vaso grande proporciona maior expressão da variação genética para os caracteres profundidade de olho, sobrancelha, curvatura, achatamento, aparência geral e todos os componentes de rendimento. As maiores médias de produção de tubérculos em número, tamanho e massa foram obtidas no vaso grande, enquanto o maior coeficiente de variação genético para os caracteres formato, uniformidade de formato e apontamento de tubérculo foi obtido no vaso pequeno.In potato breeding, the exclusion of inferior genotypes by selection in early generations provides more versatility and cost reduction. The objective of this study was to evaluate the influence of pot size on the expression of tuber traits in potato families grown as seedling generation. Ten potato families were planted in two pot sizes, large (1 L and small (250 ml, in the greenhouse. The following tuber traits were evaluated: color, roughness, depth of eye, eyebrow, shape, uniformity of shape, pointing, curvature, flattening, tuber general appearance, total mass per plant, average tuber weight and number of tubers. The larger pot provided greater expression of genetic variation for the depth of eye, eyebrow, curvature

  15. Seismic fragility test of a 6-inch diameter pipe system

    International Nuclear Information System (INIS)

    Chen, W.P.; Onesto, A.T.; DeVita, V.

    1987-02-01

    This report contains the test results and assessments of seismic fragility tests performed on a 6-inch diameter piping system. The test was funded by the US Nuclear Regulatory Commission (NRC) and conducted by ETEC. The objective of the test was to investigate the ability of a representative nuclear piping system to withstand high level dynamic seismic and other loadings. Levels of loadings achieved during seismic testing were 20 to 30 times larger than normal elastic design evaluations to ASME Level D limits would permit. Based on failure data obtained during seismic and other dynamic testing, it was concluded that nuclear piping systems are inherently able to withstand much larger dynamic seismic loadings than permitted by current design practice criteria or predicted by the probabilistic risk assessment (PRA) methods and several proposed nonlinear methods of failure analysis

  16. Eastern Seaboard Electric Grid Fragility Maps Supporting Persistent Availability

    Energy Technology Data Exchange (ETDEWEB)

    Walker, Kimberly A [ORNL; Weigand, Gilbert G [ORNL; Fernandez, Steven J [ORNL

    2012-11-01

    Persistently available power transmission can be disrupted by weather causing power outages with economic and social consequences. This research investigated the effects on the national power grid from a specific weather event, Hurricane Irene, that caused approximately 5.7 million customer power outages along the Eastern Seaboard in August of 2011. The objective was to describe the geographic differences in the grid s vulnerability to these events. Individual factors, such as wind speed or precipitation, were correlated with the number of outages to determine the greatest mechanism of power failure in hopes of strengthening the future power grid. The resulting fragility maps not only depicted 18 counties that were less robust than the design-standard robustness model and three counties that were more robust, but also drew new damage contours with correlated wind speeds and county features.

  17. The Fragile X Syndrome: Behavioral Phenotype and Learning Disabilities

    Directory of Open Access Journals (Sweden)

    Claudia GRAU RUBIO

    2016-04-01

    Full Text Available In this article, we describe the behavioral phenotype of individuals with Fragile X Syndrome and its impact in the educational scope. This syndrome is characterized by difficulties in sensory integration, cognitive deficits (verbal reasoning, abstract/ visual and cuantitative skills, short term memory, sequential processing, attention and executive processes, language disorders (phonetic-phonologicals, semanticals, morphosyntacticals and pragmaticals and communication disorders, social anxiety, general hyperarousal, autism, non autistic social difficulties, attention deficit and hyperactivity, and learning disabilities. The behavioral phenotype is highly variable and depends on sex, age, and mutation status (full mutation or premutation. The behavioural phenotype has important repercussions in education, as it enables us to understand the learning disabilities and to develop specific intervention strategies.

  18. Cyclical Cohabitation Among Unmarried Parents in Fragile Families.

    Science.gov (United States)

    Nepomnyaschy, Lenna; Teitler, Julien

    2013-10-01

    Building on past research suggesting that cohabitation is an ambiguous family form, the authors examined an understudied residential pattern among unmarried parents: cyclical cohabitation, in which parents have multiple cohabitation spells with each other. Using 9 years of panel data from the Fragile Families and Child Wellbeing Study ( N = 2,084), they found that 10% of all parents with nonmarital births, and nearly a quarter of those living together when the child is 9 years old, are cyclical cohabitors. Cyclically cohabiting mothers reported more material hardships than mothers in most other relationship patterns but also reported more father involvement with children. On all measures of child well-being, except grade retention, children of cyclically cohabiting parents fared no worse than children of stably cohabiting biological parents and did not differ significantly from any other group.

  19. Fragility of complexity biophysical systems by neutron scattering

    International Nuclear Information System (INIS)

    Magazu, Salvatore; Migliardo, Federica; Bellocco, Ersilia; Lagana, Giuseppina; Mondelli, Claudia

    2006-01-01

    Neutron scattering is an exceptional tool to investigate structural and dynamical properties of systems of biophysical interest, such as proteins, enzymes, lipids and sugars. Moreover, elastic neutron scattering enhances the investigation of atomic motions in hydrated proteins in a wide temperature range and on the picosecond timescale. Homologous disaccharides, such as trehalose, maltose and sucrose, are cryptobiotic substances, since they allow to many organisms to undergo in a 'suspended life' state, known as cryptobiosis in extreme environmental conditions. The present paper is aimed to discuss the fragility degree of disaccharides, as evaluated of the temperature dependence of the mean square displacement by elastic neutron scattering, in order to link this feature with their bioprotective functions

  20. Social cognition in adolescent girls with fragile x syndrome.

    Science.gov (United States)

    Turkstra, Lyn S; Abbeduto, Leonard; Meulenbroek, Peter

    2014-07-01

    This study aimed to characterize social cognition, executive functions (EFs), and everyday social functioning in adolescent girls with fragile X syndrome, and identify relationships among these variables. Participants were 20 girls with FXS and 20 age-matched typically developing peers. Results showed significant between-groups differences in social cognition, accounted for by differences in IQ and language. Within the FXS group, IQ and language were related to social cognition; parent-reported social functioning was related to language and EFs; and self-reported social functioning was generally good and not related to cognitive or social cognition variables. Results suggest that intervention might focus on managing language and cognitive contributions to social functioning, rather than social cognition, and underscore the importance of considering parent and adolescent perspectives.

  1. Mothers’ Partnership Instability and Coparenting among Fragile Families

    Science.gov (United States)

    Cooper, Carey E.; Beck, Audrey N.; Högnäs, Robin S.; Swanson, Jodi

    2015-01-01

    Objectives The rise in nonmarital childbearing has raised concerns about coparenting among unmarried parents with increasingly complicated relationship trajectories. We address this issue by examining associations between mothers’ partnership transitions and coparenting and the moderating role of maternal race/ethnicity and child gender. Methods Data from the Fragile Families Study and ordinary least squares regression techniques are used to examine whether mothers’ partnership transitions are related to coparenting. Lagged and fixed effects models are employed to test the robustness of the findings to selection. Results Coresidential and nonresidential, dating transitions are negatively associated with coparenting, but the association is stronger for coresidential transitions than for dating transitions. Coresidential transitions are stronger predictors of coparenting for White parents than for Black parents and for parents of sons than for parents of daughters. Conclusions Policies aimed at strengthening families should emphasize relationship stability, regardless of the type of union, to promote high quality coparenting among at-risk populations. PMID:26538770

  2. Scaling Up Family Therapy in Fragile, Conflict-Affected States.

    Science.gov (United States)

    Charlés, Laurie L

    2015-09-01

    This article discusses the design and delivery of two international family therapy-focused mental health and psychosocial support training projects, one in a fragile state and one in a post-conflict state. The training projects took place in Southeast Asia and the Middle East/North Africa. Each was funded, supported, and implemented by local, regional, and international stakeholders, and delivered as part of a broader humanitarian agenda to develop human resource capacity to work with families affected by atrocities. The two examples illustrate how task-shifting/task-sharing and transitional justice approaches were used to inform the scaling-up of professionals involved in each project. They also exemplify how state-citizen phenomena in each location affected the project design and delivery. © 2014 Family Process Institute.

  3. Anhydrosaccharides—A new class of the fragile plastic crystals

    Science.gov (United States)

    Kamińska, Ewa; Madejczyk, Olga; Tarnacka, Magdalena; Jurkiewicz, Karolina; Wolnica, Kamila; Śmiszek-Lindert, Wioleta Edyta; Kamiński, Kamil; Paluch, Marian

    2018-02-01

    In this paper, 1,6-anhydro-β-D-glucopyranose (anhGLU), 1,6-anhydro-β-D-mannopyranose (anhMAN), and 1,6-anhydro-β-D-galactopyranose (anhGAL), three new materials that form the Orientationally Disordered Crystal (ODIC) phase, have been thoroughly investigated using various experimental techniques. All measurements clearly indicated that these compounds possess a series of very interesting physical properties that are considerably different than those reported for ordinary plastic crystals. X-Ray diffraction investigations have revealed enormously long-range static correlations between molecules, reaching even 120 Å. Moreover, dielectric studies showed that besides Freon 113, the investigated anhydrosaccharides are the most fragile systems that form the ODIC phase. Further analysis of Fourier transform infrared spectra indicated that such peculiar behavior of anhydrosaccharides might be closely related to multidirectional H-bonds of various strengths that most likely affect the number of available conformations, density states, and the potential barriers in the energy landscape of these compounds. This is consistent with the results from previous reports [L. C. Pardo, J. Chem. Phys. 124, 124911 (2006) and Th. Bauer et al., J Chem. Phys. 133, 144509 (2010)] showing that the higher fragility of Freon 112 as well as a mixture of 60% succinonitrile and 40% glutaronitrile (60SN-40GN) can be closely related to the enhanced conformational ability and additional disorder introduced by various substituents, which further make energy landscape more complex. Finally, by studying the properties of 2,3,4-tri-O-acetyl-1,6-anhydro-β-D-glucopyranose (ac-anhGLU) it was found that besides the shape of the molecules, H-bonds or generally strong intermolecular interactions are extremely important parameters contributing to the ability to form the plastic phase. This is in line with current observations that in most cases the ODIC phase is created in highly interacting compounds.

  4. Genetics of osteoporosis: searching for candidate genes for bone fragility.

    Science.gov (United States)

    Rocha-Braz, Manuela G M; Ferraz-de-Souza, Bruno

    2016-08-01

    The pathogenesis of osteoporosis, a common disease with great morbidity and mortality, comprises environmental and genetic factors. As with other complex disorders, the genetic basis of osteoporosis has been difficult to identify. Nevertheless, several approaches have been undertaken in the past decades in order to identify candidate genes for bone fragility, including the study of rare monogenic syndromes with striking bone phenotypes (e.g. osteogenesis imperfecta and osteopetroses), the analysis of individuals or families with extreme osteoporotic phenotypes (e.g. idiopathic juvenile and pregnancy-related osteoporosis), and, chiefly, genome-wide association studies (GWAS) in large populations. Altogether, these efforts have greatly increased the understanding of molecular mechanisms behind bone remodelling, which has rapidly translated into the development of novel therapeutic strategies, exemplified by the tales of cathepsin K (CTSK) and sclerostin (SOST). Additional biological evidence of involvement in bone physiology still lacks for several candidate genes arisen from GWAS, opening an opportunity for the discovery of new mechanisms regulating bone strength, particularly with the advent of high-throughput genomic technologies. In this review, candidate genes for bone fragility will be presented in comprehensive tables and discussed with regard to how their association with osteoporosis emerged, highlighting key players such as LRP5, WNT1 and PLS3. Current limitations in our understanding of the genetic contribution to osteoporosis, such as yet unidentified genetic modifiers, may be overcome in the near future with better genotypic and phenotypic characterisation of large populations and the detailed study of candidate genes in informative individuals with marked phenotype.

  5. Major osteoporotic fragility fractures: Risk factor updates and societal impact.

    Science.gov (United States)

    Pisani, Paola; Renna, Maria Daniela; Conversano, Francesco; Casciaro, Ernesto; Di Paola, Marco; Quarta, Eugenio; Muratore, Maurizio; Casciaro, Sergio

    2016-03-18

    Osteoporosis is a silent disease without any evidence of disease until a fracture occurs. Approximately 200 million people in the world are affected by osteoporosis and 8.9 million fractures occur each year worldwide. Fractures of the hip are a major public health burden, by means of both social cost and health condition of the elderly because these fractures are one of the main causes of morbidity, impairment, decreased quality of life and mortality in women and men. The aim of this review is to analyze the most important factors related to the enormous impact of osteoporotic fractures on population. Among the most common risk factors, low body mass index; history of fragility fracture, environmental risk, early menopause, smoking, lack of vitamin D, endocrine disorders (for example insulin-dependent diabetes mellitus), use of glucocorticoids, excessive alcohol intake, immobility and others represented the main clinical risk factors associated with augmented risk of fragility fracture. The increasing trend of osteoporosis is accompanied by an underutilization of the available preventive strategies and only a small number of patients at high fracture risk are recognized and successively referred for therapy. This report provides analytic evidences to assess the best practices in osteoporosis management and indications for the adoption of a correct healthcare strategy to significantly reduce the osteoporosis burden. Early diagnosis is the key to resize the impact of osteoporosis on healthcare system. In this context, attention must be focused on the identification of high fracture risk among osteoporotic patients. It is necessary to increase national awareness campaigns across countries in order to reduce the osteoporotic fractures incidence.

  6. Identifying large-scale brain networks in fragile X syndrome.

    Science.gov (United States)

    Hall, Scott S; Jiang, Heidi; Reiss, Allan L; Greicius, Michael D

    2013-11-01

    Fragile X syndrome (FXS) is an X-linked neurogenetic disorder characterized by a cognitive and behavioral phenotype resembling features of autism spectrum disorder. Until now, research has focused largely on identifying regional differences in brain structure and function between individuals with FXS and various control groups. Very little is known about the large-scale brain networks that may underlie the cognitive and behavioral symptoms of FXS. To identify large-scale, resting-state networks in FXS that differ from control individuals matched on age, IQ, and severity of behavioral and cognitive symptoms. Cross-sectional, in vivo neuroimaging study conducted in an academic medical center. Participants (aged 10-23 years) included 17 males and females with FXS and 16 males and females serving as controls. Univariate voxel-based morphometric analyses, fractional amplitude of low-frequency fluctuations (fALFF) analysis, and group-independent component analysis with dual regression. Patients with FXS showed decreased functional connectivity in the salience, precuneus, left executive control, language, and visuospatial networks compared with controls. Decreased fALFF in the bilateral insular, precuneus, and anterior cingulate cortices also was found in patients with FXS compared with control participants. Furthermore, fALFF in the left insular cortex was significantly positively correlated with IQ in patients with FXS. Decreased gray matter density, resting-state connectivity, and fALFF converged in the left insular cortex in patients with FXS. Fragile X syndrome results in widespread reductions in functional connectivity across multiple cognitive and affective brain networks. Converging structural and functional abnormalities in the left insular cortex, a region also implicated in individuals diagnosed with autism spectrum disorder, suggests that insula integrity and connectivity may be compromised in FXS. This method could prove useful in establishing an imaging

  7. New insights of altered lipid profile in Fragile X Syndrome.

    Directory of Open Access Journals (Sweden)

    Artuela Çaku

    Full Text Available Fragile X Syndrome (FXS is the main genetic cause of autism and intellectual deficiency resulting the absence of the Fragile X Mental Retardation Protein (FMRP. Clinical picture is characterized by cognitive impairment associated with a broad spectrum of psychiatric comorbidities including autism spectrum disorders and attention-deficit/hyperactivity disorders. Some of these disorders have been associated with lipid abnormalities and lower cholesterol levels. Since lipids are important for neuronal development, we aim to investigate the lipid profile of French Canadian-FXS individuals and to identify the altered components of cholesterol metabolism as well as their association with clinical profile.Anthropometric data were collected from 25 FXS individuals and 26 controls. Lipid assessment included: total cholesterol (TC, triglycerides, LDL, HDL, ApoB, ApoA1, PCSK9, Lp(a and lipoprotein electrophoresis. Aberrant and adaptive behaviour of affected individuals was respectively assessed by the ABC-C and ABAS questionnaires.FXS participants had a higher body mass index as compared to controls while 38% of them had TC<10th percentile. Lower levels of LDL, HDL and apoA1 were observed in FXS group as compared to controls. However, PCSK9 levels did not differ between the two groups. As expected, PCSK9 levels correlated with total cholesterol (rs = 0.61, p = 0.001 and LDL (rs = 0.46, p = 0.014 in the control group, while no association was present in the FXS group. An inverse relationship was observed between total cholesterol and aberrant behaviour as determined by ABC-C total score.Our results showed the presence of hypocholesterolemia in French Canadian-FXS population, a condition that seems to influence their clinical phenotype. We identified for the first time a potential underlying alteration of PCSK9 function in FXS that could result from the absence of FMRP. Further investigations are warranted to better understand the association between

  8. Assessing the Fragile X Syndrome Newborn Screening Landscape.

    Science.gov (United States)

    Riley, Catharine; Wheeler, Anne

    2017-06-01

    Fragile X syndrome (FXS) is the most common known inherited form of intellectual disability. Early identification is an important step in linking FXS individuals with appropriate and timely medical and social services. Newborn screening (NBS) is 1 approach that has been used for other conditions to facilitate early identification. A literature review was conducted to identify issues, barriers, challenges, and approaches to addressing challenges related to NBS for FXS. Search terms included: fragile X syndrome, FMR1, newborn screening, screening, and genetic testing. To supplement the literature review, 9 key informant interviews were conducted. Information gathered through these interviews supplemented what was identified in the literature. Information from both the literature review and supplemental interviews was reviewed by 3 researchers who discussed and came to consensus on thematic areas and categorization of issues. The barriers and challenges related to NBS for FXS identified in the literature and by experts and stakeholders are categorized into 5 thematic areas: public health burden, treatment, timing, screening/testing methodologies, and translating results. Summaries of these issues and barriers are provided, along with potential approaches to addressing them. The issues and barriers described in this article highlight limited areas of knowledge that need be addressed to improve our understanding of FXS and the potential benefit of NBS. The landscape of NBS for FXS could be influenced by a series of research findings over time or a larger breakthrough that demonstrates an effective targeted treatment that has to be implemented early in life. Copyright © 2017 by the American Academy of Pediatrics.

  9. Fragile X syndrome: Are signaling lipids the missing culprits?

    Science.gov (United States)

    Tabet, Ricardos; Vitale, Nicolas; Moine, Hervé

    2016-11-01

    Fragile X syndrome (FXS) is the most common cause of inherited intellectual disability and autism. FXS results from the absence of FMRP, an RNA binding protein associated to ribosomes that influences the translation of specific mRNAs in post-synaptic compartments of neurons. The main molecular consequence of the absence of FMRP is an excessive translation of neuronal protein in several areas of the brain. This local protein synthesis deregulation is proposed to underlie the defect in synaptic plasticity responsible for FXS. Recent findings in neurons of the fragile X mouse model (Fmr1-KO) uncovered another consequence of the lack of FMRP: a deregulation of the diacylglycerol (DAG)/phosphatidic acid (PA) homeostasis. DAG and PA are two interconvertible lipids that influence membrane architecture and that act as essential signaling molecules that activate various downstream effectors, including master regulators of local protein synthesis and actin polymerization. As a consequence, DAG and PA govern a variety of cellular processes, including cell proliferation, vesicle/membrane trafficking and cytoskeletal organization. At the synapse, the level of these lipids is proposed to influence the synaptic activation status. FMRP appears as a master regulator of this neuronal process by controlling the translation of a diacylglycerol kinase enzyme that converts DAG into PA. The deregulated levels of DAG and PA caused by the absence of FMRP could represent a novel therapeutic target for the treatment of FXS. Copyright © 2016 Elsevier B.V. and Société Française de Biochimie et Biologie Moléculaire (SFBBM). All rights reserved.

  10. Red Blood Cell Mechanical Fragility Test for Clinical Research Applications.

    Science.gov (United States)

    Ziegler, Luke A; Olia, Salim E; Kameneva, Marina V

    2017-07-01

    Red blood cell (RBC) susceptibility to mechanically induced hemolysis, or RBC mechanical fragility (MF), is an important parameter in the characterization of erythrocyte membrane health. The rocker bead test (RBT) and associated calculated mechanical fragility index (MFI) is a simple method for the assessment of RBC MF. Requiring a minimum of 15.5 mL of blood and necessitating adjustment of hematocrit (Ht) to a "standard" value (40%), the current RBT is not suitable for use in most studies involving human subjects. To address these limitations, we propose a 6.5 mL reduced volume RBT and corresponding modified MFI (MMFI) that does not require prior Ht adjustment. This new method was assessed for i) correlation to the existing text, ii) to quantify the effect of Ht on MFI, and iii) validation by reexamining the protective effect of plasma proteins on RBC MF. The reduced volume RBT strongly correlated (r = 0.941) with the established large volume RBT at matched Hts, and an equation was developed to calculate MMFI: a numerical estimation (R 2  = 0.923) of MFI if performed with the reduced volume RBT at "standard" (40%) Ht. An inversely proportional relationship was found between plasma protein concentration and RBC MF using the MMFI-reduced volume method, supporting previous literature findings. The new reduced volume RBT and modified MFI will allow for the measurement of RBC MF in clinical and preclinical studies involving humans or small animals. © 2017 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

  11. Sarcolemmal blebs and osmotic fragility as correlates of irreversible ischemic injury in preconditioned isolated rabbit cardiomyocytes.

    Science.gov (United States)

    Armstrong, S C; Shivell, L C; Ganote, C E

    2001-01-01

    The hypothesis that irreversible ischemic injury is related to sub-sarcolemmal blebbing and an inherent osmotic fragility of the blebs was tested by subjecting isolated control and ischemically preconditioned (IPC) or calyculin A (CalA)-pretreated (protected) rabbit cardiomyocytes to ischemic pelleting followed by resuspension in 340, 170 or 85 mosmol medium containing trypan blue. At time points from 0-240 min, osmotic fragility was assessed by the percentage of trypan blue permeable cells. Membrane blebs were visualized with India ink preparations. Bleb formation, following acute hypo-osmotic swelling, developed by 75 min and increased with longer periods of ischemia. Osmotic fragility developed only after 75 min. Cells resuspended in 340 mosmol media did not form blebs and largely retained the ability to exclude trypan blue, even after 240 min ischemia. Although the latent tendency for osmotic blebbing preceded the development of osmotic fragility, most osmotically fragile cells became permeable without evident sarcolemmal bleb formation. The onset of osmotic fragility was delayed in protected cells, but protection did not reduce the bleb formation. It is concluded that blebbing and osmotic fragility are independent manifestations of ischemic injury. The principal locus of irreversible ischemic injury and the protection provided by IPC may lie within the sarcolemma rather than at sarcolemmal attachments to underlying adherens junctions.

  12. THE IMPORTANCE OF THE ERYTHROCYTES OSMOTIC FRAGILITY TEST PERFORMED IN CHILDREN WITH INDIRECT HYPERBILIRUB1NEMIA

    Directory of Open Access Journals (Sweden)

    Ivana Stojanović

    2005-07-01

    Full Text Available The osmotic fragility test of erythrocytes is useful in the diagnosis of different types of hereditary hemolytic anemias followed with hyperbilirubinemia. Hemolytic anemias, characterized by accelerated destruction of red blood cells, are usually the consequence of many metabolic abnormalities like cellular membrane defect, erythrocyte enzymes defect or hemoglobin abnormalities – hemoglobinopathies. The object of our study was to assess the relationship between osmotic fragility test of erythrocytes and severity of indirect hyperbilirubinemia in some inherited erythrocytes’ disorders. We did the osmotic fragility test of erythrocytes by using Dacie, s method with normal values of erythrocytes hemolysis between 0,48 to 0,34% NaCl (minimal to maximal hemolysis. In hereditary spherocytosis, fragility of erythrocytes was increased (min. at 0,50 % NaCl to max. 0,44 % NaCl . In the child with β- thalassemia and cycle cell anemia erythrocytes fragility was decreased (min . at 0,42 to max. 0,32 % NaCl, that is 0,40% min. of hemolysis and 0,34% max. hemolysis in the second case. In newborn infants with high levels of indirect bilirubin in serum as a cause of physiological jaundice, the osmotic fragility test was within a normal range. Our findings point out the diagnostic value of osmotic fragility test in assessing patients with the indirect hyperbilirubinemia. This simple and important diagnostic test can be performed in small laboratories.

  13. Cytogenetic and Molecular Investigation in Children with Possible Fragile X Syndrome

    Directory of Open Access Journals (Sweden)

    Onur Ozer

    2012-04-01

    Full Text Available Objective: Fragile X syndrome (FXS is the most common cause of inherited mental retardation and is due to a mutation in the X-linked FMR1 gene. Molecular genetic testing and chromosome analysis are indicated for this disorder. In this context, we tried to determine the frequency of the FXS, and other chro¬mosomal abnormalities of Turkish pediatric neurology outpatients. Materials and Methods: Cytogenetic and molecular screenings were performed to esti-mate the prevalence of the fragile X in 107 patients with mental retardation, language disorders, hyperactivity, develop¬mental delay or fragile X syndrome phenotype. Only 26 out of 107 patients were screened, molecularly. Results: Cytogenetically fragile X-positive cells was found in 8 cases (7.5% of 107 patients; in 4.7% of males and in 2.8% of females. The autosomal fragile sites (FS was found in 14 (13.1% cases. One (0.9% patient had pericentric inversion of chromosome 9. Molecular analysis were performed for 26 patients and all patients showed normal CGG expansion. Conclusion: In diagnosis of fragile X syndrome, chromosome analysis must be run in conjunction with the molecular studies. It is recommended that all members of the fragile X family under risk should be screened both by cytogenetic and molecular methods. Genetic counseling can be useful to patients and families considering genetic testing. [Cukurova Med J 2012; 37(2.000: 76-83

  14. Degraded speech sound processing in a rat model of fragile X syndrome.

    Science.gov (United States)

    Engineer, Crystal T; Centanni, Tracy M; Im, Kwok W; Rahebi, Kimiya C; Buell, Elizabeth P; Kilgard, Michael P

    2014-05-20

    Fragile X syndrome is the most common inherited form of intellectual disability and the leading genetic cause of autism. Impaired phonological processing in fragile X syndrome interferes with the development of language skills. Although auditory cortex responses are known to be abnormal in fragile X syndrome, it is not clear how these differences impact speech sound processing. This study provides the first evidence that the cortical representation of speech sounds is impaired in Fmr1 knockout rats, despite normal speech discrimination behavior. Evoked potentials and spiking activity in response to speech sounds, noise burst trains, and tones were significantly degraded in primary auditory cortex, anterior auditory field and the ventral auditory field. Neurometric analysis of speech evoked activity using a pattern classifier confirmed that activity in these fields contains significantly less information about speech sound identity in Fmr1 knockout rats compared to control rats. Responses were normal in the posterior auditory field, which is associated with sound localization. The greatest impairment was observed in the ventral auditory field, which is related to emotional regulation. Dysfunction in the ventral auditory field may contribute to poor emotional regulation in fragile X syndrome and may help explain the observation that later auditory evoked responses are more disturbed in fragile X syndrome compared to earlier responses. Rodent models of fragile X syndrome are likely to prove useful for understanding the biological basis of fragile X syndrome and for testing candidate therapies. Copyright © 2014 Elsevier B.V. All rights reserved.

  15. Raman and DSC studies of fragility in tellurium-zinc oxide glass formers

    International Nuclear Information System (INIS)

    Stavrou, Elissaios; Kripotou, Sotiria; Raptis, Constantine; Turrell, Sylvia; Syassen, Karl

    2011-01-01

    Raman scattering and differential scanning calorimetry (DSC) measurements have been carried out in four mixed (TeO 2 ) 1-x (ZnO) x (x = 0.1, 0.2, 0.3, 0.4) glasses at high temperatures (Raman and DSC through the glass transition) and high pressures (Raman) with the aim of determining the fragility of these glass forming oxides. Four different criteria, corresponding to four parameters, were applied to assess the fragility of the glasses. From the DSC studies, we have obtained the fragility parameter m which corresponds to the slopes of Arrhenius (lnQ vs. 1/T g , were Q is the heating rate) plots, and the glass transition width ΔT g . Also, from the low-frequency Raman scattering, and in particular the boson peak intensity of the glasses at T g , we have estimated the fragility ratio r R (T g ) = I min /I max whose value serves as another (empirical) fragility criterion. Finally, from high pressure Raman measurements on the glasses, we have estimated the Grueneisen parameter γ T for each glass, which constitutes the fourth fragility parameter adopted in this work. Considering the four parameters ΔT g , m, r (T g ) and γ T and the generally accepted (empirical) fragility criteria, we conclude that the mixed tellurium-zinc oxides constitute strong-to-intermediate glass formers (copyright 2011 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim) (orig.)

  16. Scaling up nutrition in fragile and conflict-affected states: the pivotal role of governance.

    Science.gov (United States)

    Taylor, Sebastian A J; Perez-Ferrer, Carolina; Griffiths, Andrew; Brunner, Eric

    2015-02-01

    Acute and chronic undernutrition undermine conditions for health, stability and socioeconomic development across the developing world. Although fragile and conflict-affected states have some of the highest rates of undernutrition globally, their response to the multilateral 'Scaling Up Nutrition' (SUN) initiative in its first two-year period was ambivalent. The purpose of this research was to investigate factors affecting fragile and conflict-affected states' engagement with SUN, and to examine what differentiated those fragile states that joined SUN in its first phase from those that did not. Drawing on global databases (Unicef, World Bank, UNDP), and qualitative country case studies (Afghanistan, the Democratic Republic of Congo, Sierra Leone, Pakistan and Yemen) we used bivariate logistic regressions and principal component analysis to assess social, economic and political factors across 41 fragile states looking for systematic differences between those that had signed up to SUN before March 2013 (n = 16), and those that had not (n = 25). While prevalence of malnutrition, health system functioning and level of citizen empowerment had little or no impact on a fragile state's likelihood of joining SUN, the quality of governance (QOG) strongly predicted accession. SUN-signatory fragile states scored systematically better on the World Bank's Country Policy and Institutional Assessment (CPIA) and the Worldwide Governance Indicators 'effectiveness of government' indices. We conclude that strengthening governance in fragile states may enhance their engagement with initiatives such as SUN, but also (recognising the potential for endogeneity), that the way aid is structured and delivered in fragile states may be an underlying determinant of whether and how governance in such contexts improves. The research demonstrates that more nuanced analysis of conditions within and among countries classed as 'fragile and conflict-affected' is both possible and necessary if aid

  17. Vascularização arterial dos cornos uterinos em gatas gestantes sem raça definida (Felis catus Linnaeus, 1758

    Directory of Open Access Journals (Sweden)

    Rosana Marques Silva

    2004-02-01

    Full Text Available Utilizaram-se 24 gatas gestantes, sem raça definida, doadas para o estudo da vascularização arterial dos cornos uterinos. Em 20 animais, com o intervalo gestacional entre 7 e 9 semanas, a aorta abdominal foi injetada com Látex-Neoprene 650 corado, associado ao Sulfato de Bário 1004. Os animais foram então radiografados, fixados em solução aquosa de formol 10% e dissecados para estudo da distribuição dos vasos arteriais destinados às regiões paraplacentárias e de cintas placentárias. Em 4 animais realizou-se o exame por Ecografia Power Doppler, onde se observou que nas regiões das cintas placentárias o índice de resistência dos vasos apresenta-se menor do que o encontrado ao longo da artéria uterina. Em todas as observações, a artéria uterina é o principal vaso a irrigar os cornos uterinos, emitindo de 2 a 17 ramos, com maior freqüência de 5 a 12 ramos, os quais se distribuem nas regiões paraplacentárias e/ou das cintas placentárias, formando arcadas anastomósticas. Não houve diferenças estatísticas significativas do número de ramos para cada região individualmente, porém a análise das radiografias contrastadas demonstrou haver uma concentração maior de contraste nas regiões das cintas placentárias. A artéria ovárica participa da vascularização arterial da extremidade cranial dos cornos uterinos, onde se anastomosa com ramos da artéria uterina.

  18. Axonal neuropathy in female carriers of the fragile X premutation with fragile x-associated tremor ataxia syndrome.

    Science.gov (United States)

    Ram, Suresh; Devapriya, Inoka A; Fenton, Grace; Mcvay, Lindsey; Nguyen, Danh V; Tassone, Flora; Maselli, Ricardo A; Hagerman, Randi J

    2015-08-01

    In this study we examined whether females with the fragile X-associated tremor ataxia syndrome (FXTAS) and non-FXTAS premutation carriers have electrophysiological signs of underlying peripheral neuropathy. Nerve conduction studies (NCS) were performed on 19 women with FXTAS, 20 non-FXTAS carriers, and 26 age-matched controls. The results were compared with existing data on corresponding male carriers. Women with FXTAS and non-FXTAS carriers had reduced sensory nerve action potential amplitudes. Also, there was a strong trend for reduced compound muscle action potential amplitudes in women with FXTAS, but not in non-FXTAS carriers. No significant slowing of nerve conduction velocities, prolongation of F-wave latencies, or associations with molecular measures was observed. This study suggests an underlying axonal neuropathy in women with FXTAS. However, in comparison to men with FXTAS, the NCS abnormalities in women were less severe, possibly due to the effect of a normal X chromosome. © 2014 Wiley Periodicals, Inc.

  19. The Search for an Effective Therapy to Treat Fragile X Syndrome: Dream or Reality?

    OpenAIRE

    Castagnola, Sara; Bardoni, Barbara; Maurin, Thomas

    2017-01-01

    Fragile X Syndrome (FXS) is the most common form of intellectual disability and a primary cause of autism. It originates from the lack of the Fragile X Mental Retardation Protein (FMRP), which is an RNA-binding protein encoded by the Fragile X Mental Retardation Gene 1 (FMR1) gene. Multiple roles have been attributed to this protein, ranging from RNA transport (from the nucleus to the cytoplasm, but also along neurites) to translational control of mRNAs. Over the last 20 years many studies ha...

  20. Seismic fragility analysis of the block masonry wall in nuclear power plants

    International Nuclear Information System (INIS)

    Wang, Z-L.; Pandey, M.D.; Xie, X-C.

    2014-01-01

    The evaluation of seismic fragility of a structure is an integral part in the Seismic Probabilistic Risk Analysis (SPRA). The block masonry wall, a commonly used barrier in nuclear power plants, is fairly vulnerable to failure under an earthquake. In practice, the seismic fragility of block walls is commonly evaluated using a simple deterministic approach called Conservative Deterministic Failure Margin (CDFM) method. This paper presents a more formal fragility analysis of a block wall based on rigorous probabilistic methods and the accuracy of the CDFM method is evaluated by comparison to the more rigorous FA method. (author)

  1. Seismic fragility analyses of nuclear power plant structures based on the recorded earthquake data in Korea

    International Nuclear Information System (INIS)

    Joe, Yang Hee; Cho, Sung Gook

    2003-01-01

    This paper briefly introduces an improved method for evaluating seismic fragilities of components of nuclear power plants in Korea. Engineering characteristics of small magnitude earthquake spectra recorded in the Korean peninsula during the last several years are also discussed in this paper. For the purpose of evaluating the effects of the recorded earthquake on the seismic fragilities of Korean nuclear power plant structures, several cases of comparative studies have been performed. The study results show that seismic fragility analysis based on the Newmark's spectra in Korea might over-estimate the seismic capacities of Korean facilities. (author)

  2. Fluorescent in-situ hybridization of cattle and sheep chromosomes with cloned human fragile-X DNA

    DEFF Research Database (Denmark)

    Ali, Ahmd; Thomsen, Preben Dybdahl; Babar, M.E.

    2009-01-01

    An extensive study on spontaneous and 5-Fluorodeoxyuridine induced fragile sites identified Xq31 in cattle (Bos taurus) and (Xq24, Xq26) in sheep (Ovis aries) in addition to several autosomal fragile sites (under publication). A ZOO-FISH study using three cloned human fragile-X probes with CCG....../CGG(n) trinucleotide repeat sequence was carried out to determine homology between human and bovine fragile-X. The hybridisation results showed only a weak signal on a human chromosome that was not an X with all three fragile site probes. No signals were detected in sheep chromosomes. The signal of all three human...... fragile-X probes on cattle chromosomes was however, medium-prominent sub-centromeric signal on two homologues. BrdU administration in 12 h before harvesting identified these homologues to be chromosome number 5. In addition retrospective slides of cattle and sheep chromosomes used for fragile site studies...

  3. Fragility of estimated spatial temperature patterns in climate field reconstructions of the Common Era

    Science.gov (United States)

    Wang, J.; Emile-Geay, J.; Vaccaro, A.; Guillot, D.; Rajaratnam, B.

    2013-12-01

    Climate field reconstructions (CFRs) of the Common Era can provide insight into dynamical causes of low-frequency climate variability. For instance, the Mann et al. [2009] study found that the reconstructed sea-surface temperature difference between the Medieval Climate Anomaly and the Little Ice Age (hereinafter MCA - LIA) is marked by a La-Niña like pattern over the tropical Pacific, and proposed dynamical explanations for this observation. In this talk, we assess the robustness of such spatial patterns. First we examine the impact of the CFR methodology. Starting with the network of Mann et al. [2008] (hereinafter M08), we perform temperature reconstruction using four different CFR techniques: RegEM-TTLS [Schneider, 2001], the Mann et al. [2009] implementation of RegEM-TTLS (hereinafter M09), Canonical Correlation Analysis [Smerdon et al., 2010, CCA] and GraphEM [Guillot et al., in revision]. We find that results are greatly method-dependent even with identical inputs. While the M09 reconstruction displays a La Niña-like pattern over the tropical Pacific for MCA - LIA, CCA gives a neutral pattern, RegEM-TTLS and GraphEM both display El Niño-like pattern but show different amplitudes. Next we assess a given CFR technique's sensitivity to the selection of inputs. Proxies are selected based on the statistical significance of their correlations with HadCRUT3v annual temperature. A multiple hypothesis test [Ventura et al., 2004] is conducted to preclude spurious correlations. This choice has a large impact on resulting CFRs. In particular, whether the correlation is calculated between local or regional temperature-proxy pairs determines the number of significant records included in the proxy network. This in turn greatly affects the reconstructed spatial patterns and the Northern Hemispheric mean temperature time series with all CFR methods investigated. In order to further analyze CFRs' sensitivities to the abovementioned procedural choices, we assemble an

  4. Crescimento e acumulação de nutrientes em plantas de taro sob níveis de sombreamento artificial Growth and nutrient accumulation of taro plants under artificial shading levels

    OpenAIRE

    Fabio Luiz de Oliveira; Adelson Paulo Araújo; Jose Guilherme M Guerra

    2011-01-01

    Foi conduzido um experimento em vasos com solo com o objetivo de avaliar o crescimento e acumulação de nutrientes de plantas de taro (Colacasia esculenta) sob níveis de sombreamento artificial. O experimento foi constituído de quatro níveis de sombreamento (0, 25, 50 e 75% de restrição de luz) e nove coletas mensais, no delineamento de blocos casualizados em esquema de parcelas subdivididas, com quatro repetições. Os níveis de sombreamento foram obtidos com uso de armações galvanizadas revest...

  5. EMQN best practice guidelines for the molecular genetic testing and reporting of fragile X syndrome and other fragile X-associated disorders.

    Science.gov (United States)

    Biancalana, Valérie; Glaeser, Dieter; McQuaid, Shirley; Steinbach, Peter

    2015-04-01

    Different mutations occurring in the unstable CGG repeat in 5' untranslated region of FMR1 gene are responsible for three fragile X-associated disorders. An expansion of over ∼200 CGG repeats when associated with abnormal methylation and inactivation of the promoter is the mutation termed 'full mutation' and is responsible for fragile X syndrome (FXS), a neurodevelopmental disorder described as the most common cause of inherited intellectual impairment. The term 'abnormal methylation' is used here to distinguish the DNA methylation induced by the expanded repeat from the 'normal methylation' occurring on the inactive X chromosomes in females with normal, premutation, and full mutation alleles. All male and roughly half of the female full mutation carriers have FXS. Another anomaly termed 'premutation' is characterized by the presence of 55 to ∼200 CGGs without abnormal methylation, and is the cause of two other diseases with incomplete penetrance. One is fragile X-associated primary ovarian insufficiency (FXPOI), which is characterized by a large spectrum of ovarian dysfunction phenotypes and possible early menopause as the end stage. The other is fragile X-associated tremor/ataxia syndrome (FXTAS), which is a late onset neurodegenerative disorder affecting males and females. Because of the particular pattern and transmission of the CGG repeat, appropriate molecular testing and reporting is very important for the optimal genetic counselling in the three fragile X-associated disorders. Here, we describe best practice guidelines for genetic analysis and reporting in FXS, FXPOI, and FXTAS, including carrier and prenatal testing.

  6. Sistematização da veia cava caudal em búfalos (Bubalus bubalis bubalis Simpson, 1945

    Directory of Open Access Journals (Sweden)

    Adelmar Afonso de Amorim Júnior

    2002-01-01

    Full Text Available Para o presente trabalho utilizou-se 25 animais, fetos de búfalos da Raça Murrah, com idades variando entre 5 e 9 meses, sendo 15 fêmeas e 10 machos, com vistas à dissecação e sistematização dos vasos em estudo. Os animais foram coletados em abatedouro e fixados em solução aquosa de formol 10%. Obtiveramse para a Veia Cava Caudal os seguintes afluentes de origem: as veias ilíacas comuns direita e esquerda, e seus afluentes colaterais; veia sacral mediana; veias frênicas; veias lombares (1-5; veias circunflexas profunda do ílio direita e esquerda; 3-4 veias hepáticas; veia genital direita (testicular ou ovárica; veias adrenais e veias renais direita e esquerda.

  7. Tratamento farmacológico e interações medicamentosas em pacientes com aneurisma da aorta abdominal

    Directory of Open Access Journals (Sweden)

    Heverton Alves Peres

    2015-01-01

    Full Text Available O aneurisma da aorta abdominal (AAA ocorre quando há uma dilatação anormal e irreversível da artéria superior a 50% do calibre esperado para o vaso e associa-se a uma mortalidade de 80 a 90%. O controle dos principais fatores de risco como a hipertensão, dislipidemia, tabagismo e doenças trombóticas em aneurismas pequenos é feito por vários medicamentos que evitam o desenvolvimento e ruptura do aneurisma, no entanto, o uso destes associados a outros medicamentos pode desencadear interações medicamentosas relevantes sendo crucial o conhecimento sobre estas. Atualmente, há poucos dados na literatura sobre o tratamento farmacológico e interações medicamentosas em pacientes com AAA, sendo o objetivo desta revisão, descrever a farmacoterapia e interações medicamentosas em pacientes com AAA.

  8. Proliferação de ramos em plantas comerciais de bico-de-papagaio associada a fitoplasma do grupo 16srIII

    OpenAIRE

    Ribeiro, Luiz Fernando; Bedendo, Ivan Paulo

    2006-01-01

    Bico-de-papagaio é uma espécie ornamental, encontrada tanto na forma de arbustos, presente em jardins residenciais e públicos, como comercializada em vasos para decoração de interiores. Com o objetivo de detectar a presença de fitoplasma em plantas mostrando sintomas de proliferação de ramos, amostras foram obtidas de plantas sintomáticas e assintomáticas cultivadas em jardins e de plantas comerciais sintomáticas envasadas. A técnica de duplo PCR, utilizando os iniciadores R16mF2/mR1 e R16F2n...

  9. The Role of Chain Length in Nonergodicity Factor and Fragility of Polymers

    DEFF Research Database (Denmark)

    Dalle-Ferrie, Cecile; Niss, Kristine; Sokolov, Alexei

    2010-01-01

    The mechanism that leads to different fragility values upon approaching the glass transition remains a topic of active discussion. Many researchers are trying to find an answer in the properties of the frozen glassy state. Following this approach, we focus here on a previously proposed relationship...... between the fragility of glass-formers and their nonergodicity factor, determined by inelastic X-ray scattering (IXS) in the glass. We extend this molecular liquid study to two model polymers— polystyrene (PS) and polyisobutylene (PIB)—for which we change the molecular weight. Polymers offer...... the opportunity to change the fragility without altering the chemical structure, just by changing the chain length. Thus, we specifically chose PS and PIB because they exhibit opposite dependences of fragility with molecular weight. Our analysis for these two polymers reveals no unique correlation between...

  10. Local variation of fragility and glass transition temperature of ultra-thin supported polymer films.

    Science.gov (United States)

    Hanakata, Paul Z; Douglas, Jack F; Starr, Francis W

    2012-12-28

    Despite extensive efforts, a definitive picture of the glass transition of ultra-thin polymer films has yet to emerge. The effect of film thickness h on the glass transition temperature T(g) has been widely examined, but this characterization does not account for the fragility of glass-formation, which quantifies how rapidly relaxation times vary with temperature T. Accordingly, we simulate supported polymer films of a bead-spring model and determine both T(g) and fragility, both as a function of h and film depth. We contrast changes in the relaxation dynamics with density ρ and demonstrate the limitations of the commonly invoked free-volume layer model. As opposed to bulk polymer materials, we find that the fragility and T(g) do not generally vary proportionately. Consequently, the determination of the fragility profile--both locally and for the film as a whole--is essential for the characterization of changes in film dynamics with confinement.

  11. Financial fragility, sovereign default risk and the limits to commercial bank bail-outs

    NARCIS (Netherlands)

    van Wijnbergen, S.; van der Kwaak, C.

    2013-01-01

    We analyse the poisonous interaction between bank rescues, financial fragility and sovereign debt discounts. In our model balance sheet constrained financial intermediaries finance both capital expenditure of intermediate goods producers and government deficits. The financial intermediaries face the

  12. Robustness and fragility in the yeast high osmolarity glycerol (HOG) signal-transduction pathway.

    Science.gov (United States)

    Krantz, Marcus; Ahmadpour, Doryaneh; Ottosson, Lars-Göran; Warringer, Jonas; Waltermann, Christian; Nordlander, Bodil; Klipp, Edda; Blomberg, Anders; Hohmann, Stefan; Kitano, Hiroaki

    2009-01-01

    Cellular signalling networks integrate environmental stimuli with the information on cellular status. These networks must be robust against stochastic fluctuations in stimuli as well as in the amounts of signalling components. Here, we challenge the yeast HOG signal-transduction pathway with systematic perturbations in components' expression levels under various external conditions in search for nodes of fragility. We observe a substantially higher frequency of fragile nodes in this signal-transduction pathway than that has been observed for other cellular processes. These fragilities disperse without any clear pattern over biochemical functions or location in pathway topology and they are largely independent of pathway activation by external stimuli. However, the strongest toxicities are caused by pathway hyperactivation. In silico analysis highlights the impact of model structure on in silico robustness, and suggests complex formation and scaffolding as important contributors to the observed fragility patterns. Thus, in vivo robustness data can be used to discriminate and improve mathematical models.

  13. Side effects of minocycline treatment in patients with fragile X syndrome and exploration of outcome measures.

    Science.gov (United States)

    Utari, Agustini; Chonchaiya, Weerasak; Rivera, Susan M; Schneider, Andrea; Hagerman, Randi J; Faradz, Sultana M H; Ethell, Iryna M; Nguyen, Danh V

    2010-09-01

    Minocycline can rescue the dendritic spine and synaptic structural abnormalities in the fragile X knock-out mouse. This is a review and preliminary survey to document side effects and potential outcome measures for minocycline use in the treatment of individuals with fragile X syndrome. We surveyed 50 patients with fragile X syndrome who received minocycline for at least 2 weeks and found that the most common reported side effect is gastrointestinal difficulty, including loss of appetite. The families reported an improvement in language and behavioral areas. Outcome measures in the design of future randomized clinical trials should include both behavioral and language measures. As with any other treatments, we emphasize that randomized clinical trials are needed to determine the efficacy of minocycline in fragile X syndrome.

  14. Epilepsy in fragile-X-syndrome mimicking panayiotopoulos syndrome: Description of three patients.

    Science.gov (United States)

    Bonanni, Paolo; Casellato, Susanna; Fabbro, Franco; Negrin, Susanna

    2017-10-01

    Fragile-X-syndrome is the most common cause of inherited intellectual disability. Epilepsy is reported to occur in 10-20% of individuals with Fragile-X-syndrome. A frequent seizure/electroencephalogram (EEG) pattern resembles that of benign rolandic epilepsy. We describe the clinical features, EEG findings and evolution in three patients affected by Fragile-X-syndrome and epilepsy mimicking Panayiotopoulos syndrome. Age at seizure onset was between 4 and about 7 years. Seizures pattern comprised a constellation of autonomic symptoms with unilateral deviation of the eyes and ictal syncope. Duration of the seizures could be brief or lengthy. Interictal EEGs revealed functional multifocal abnormalities. The evolution was benign in all patients with seizures remission before the age of 14. This observation expands the spectrum of benign epileptic phenotypes present in Fragile-X-syndrome and may be quite helpful in guiding anticonvulsant management and counseling families as to expectations regarding seizure remission. © 2017 Wiley Periodicals, Inc.

  15. Examining the function of problem behavior in fragile X syndrome: preliminary experimental analysis.

    Science.gov (United States)

    Langthorne, Paul; McGill, Peter; O'Reilly, Mark F; Lang, Russell; Machalicek, Wendy; Chan, Jeffrey Michael; Rispoli, Mandy

    2011-01-01

    Fragile X syndrome is the most common inherited cause of intellectual and developmental disability. The influence of environmental variables on behaviors associated with the syndrome has received only scant attention. The current study explored the function served by problem behavior in fragile X syndrome by using experimental functional analysis methodology with 8 children with fragile X. No child met criteria for attention-maintained problem behavior, 5 children met criteria for escape-maintained problem behavior, and 4 children met criteria for tangible-maintained problem behavior. Results are discussed and compared with previous findings on the function of problem behavior in fragile X syndrome, and implications for intervention are discussed. It is noted that the external validity of these findings is limited by the small sample size.

  16. Earthquake fragility assessment of curved and skewed bridges in Mountain West region : research brief.

    Science.gov (United States)

    2016-09-01

    the ISSUE : the RESEARCH : Earthquake Fragility : Assessment of Curved : and Skewed Bridges in : Mountain West Region : Reinforced concrete bridges with both skew and curvature are common in areas with complex terrains. : These bridges are irregular ...

  17. Seismic fragility analyses of nuclear power plant structures based on the recorded earthquake data in Korea

    International Nuclear Information System (INIS)

    Cho, Sung Gook; Joe, Yang Hee

    2005-01-01

    By nature, the seismic fragility analysis results will be considerably affected by the statistical data of design information and site-dependent ground motions. The engineering characteristics of small magnitude earthquake spectra recorded in the Korean peninsula during the last several years are analyzed in this paper. An improved method of seismic fragility analysis is evaluated by comparative analyses to verify its efficiency for practical application to nuclear power plant structures. The effects of the recorded earthquake on the seismic fragilities of Korean nuclear power plant structures are also evaluated from the comparative studies. Observing the obtained results, the proposed method is more efficient for the multi-modes structures. The case study results show that seismic fragility analysis based on the Newmark's spectra in Korea might over-estimate the seismic capacities of Korean facilities

  18. Seismic fragility analyses of nuclear power plant structures based on the recorded earthquake data in Korea

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Sung Gook [Department of Civil and Environmental System Engineering, University of Incheon, 177 Dohwa-dong, Nam-gu, Incheon 402-749 (Korea, Republic of)]. E-mail: sgcho@incheon.ac.kr; Joe, Yang Hee [Department of Civil and Environmental System Engineering, University of Incheon, 177 Dohwa-dong, Nam-gu, Incheon 402-749 (Korea, Republic of)

    2005-08-01

    By nature, the seismic fragility analysis results will be considerably affected by the statistical data of design information and site-dependent ground motions. The engineering characteristics of small magnitude earthquake spectra recorded in the Korean peninsula during the last several years are analyzed in this paper. An improved method of seismic fragility analysis is evaluated by comparative analyses to verify its efficiency for practical application to nuclear power plant structures. The effects of the recorded earthquake on the seismic fragilities of Korean nuclear power plant structures are also evaluated from the comparative studies. Observing the obtained results, the proposed method is more efficient for the multi-modes structures. The case study results show that seismic fragility analysis based on the Newmark's spectra in Korea might over-estimate the seismic capacities of Korean facilities.

  19. A novel approach to simultaneously scan genes at fragile sites

    International Nuclear Information System (INIS)

    Willem, Pascale; Brown, Jacqueline; Schouten, Jan

    2006-01-01

    Fragile sites are regions of the genome sensitive to replication stress and to exposure to environmental carcinogens. The two most commonly expressed fragile sites FRA3B and FRA16D host the histidine triad (FHIT) and WW domain containing oxidoreductase (WWOX) genes respectively. There is growing evidence that both genes contribute to cancer development and they are frequently altered by allelic and homozygous deletions in a variety of tumors. Their status is linked to prognosis in several malignancies and they are thought to be involved in early tumorigenesis. The loci for FHIT and WWOX both span over a megabase but the genes encode for small transcripts. Thus the screening of intragenic deletion can be difficult and has relied on loss of heterozygosity LOH assays, or genomic arrays. Multiplex ligation dependent probe amplification MLPA, allows for the detection of deletions/duplications and relative quantification of up to 40 specific probes in a single assay. A FHIT/WWOX MLPA assay was designed, applied and validated in five esophageal squamous cell carcinoma ESCC, cell lines established in South Africa where this cancer is of high prevalence. Sixteen probes covered all FHIT exons and 7 probes covered WWOX. Both homozygous and hemizygous deletions were detected in FHIT, in four of the cell lines with a preferential deletion of exons 5 and 4. Chromosome 3 short arm was present in normal copy number indicating that deletions were site specific. In contrast WWOX was not altered in any cell lines. RT-PCR expression pattern paralleled the pattern of deletions. Ten primary ESCC tumor specimens were subsequently screened with this assay. FHIT exon deletions were found in four of them. This method offers an alternative to loss of heterozygosity studies. Simultaneous scanning of FHIT and WWOX exons in the context of early tumorigenesis and tumor progression, may help clarify the mechanistic events related to cancer development which are not revealed by imuno

  20. Sobre alguns parasitas em animais domésticos da região do Lubango

    OpenAIRE

    Rosa, Fernanda; Peleteiro, C.; Louzã, A.; Serôdio, J.; Crespo, Maria Virgínia

    2010-01-01

    Apresentação em painel Integrado no Módulo de Diagnóstico Laboratorial do curso de formação sobre Sanidade Animal destinado a Médicos Veterinários Angolanos, realizado na cidade do Lubango em 2008, efectuaram-se necrópsias parasitárias incompletas à pele e aberturas naturais, compartimentos gastrintestinais, vasos mesentéricos, fígado, bexiga, vias respiratórias e pulmões e coração, a diversos animais domésticos com interesse pecuário (bovino, ovino, caprino e ave) e de companhia (cão), nu...

  1. Emergência e crescimento inicial de plantas de Parkinsonia aculeata L. (Fabaceae em substrato salino

    Directory of Open Access Journals (Sweden)

    Francisco Thiago Coelho Bezerra

    2013-08-01

    Full Text Available A espécie Parkinsonia aculeata L. ocorre em zonas áridas, semiáridas e subúmidas, encontrando-se dispersa em várias regiões do planeta, onde é empregada em diferentes usos. Porém, em determinados ambientes, como caatinga e ecossistemas associados do Nordeste brasileiro, a espécie apresenta caráter invasor. Dessa forma, um experimento foi desenvolvido em abrigo telado para avaliar os efeitos da salinidade no comportamento germinativo e vegetativo de plantas de Parkinsonia aculeata. Inicialmente, o material de um Argissolo Vermelho-Amarelo foi irrigado com água salina (0,2; 1,5; 3,0; 4,5; e 6,0 dS m-1. Em seguida, foram acondicionados 16 kg de cada substrato em vasos plásticos com capacidade para 21 L, e os tratamentos foram arranjados em blocos casualizados com nove repetições. Após a emergência, 10 dias após a semeadura, realizou-se o desbaste, deixando quatro plântulas por vaso. As variáveis avaliadas foram: emergência, índice de velocidade de emergência, concentrações das clorofilas a, b e total, diâmetro do caule no nível do solo, altura e número de folhas, 69 dias após a semeadura. O aumento da salinidade no solo de não salino (CEes CEes > 2 dS m-1 e moderadamente salino (8 dS m-1 > CEes > 4 dS m-1 estimulou a emergência, o crescimento inicial e a produção de clorofila nas plantas de Parkinsonia aculeata, ao contrário do que ocorre com a grande maioria das espécies. Em parte, isso explica o sucesso de sua proliferação em determinados sítios da caatinga.

  2. Growth, history, or institutions? What explains state fragility in sub-Saharan Africa

    OpenAIRE

    Bertocchi, Graziella; Guerzoni, Andrea

    2010-01-01

    We explore the determinants of state fragility in sub-Saharan Africa. Controlling for a wide range of economic, demographic, geographic and institutional regressors, we find that institutions, and in particular the civil liberties index and the number of revolutions, are the main determinants of fragility, even taking into account their potential endogeneity. Economic factors such as income growth and investment display a non robust impact after controlling for omitted variables and reverse c...

  3. Nucleosome fragility reveals novel functional states of chromatin and poises genes for activation

    OpenAIRE

    Xi, Yuanxin; Yao, Jianhui; Chen, Rui; Li, Wei; He, Xiangwei

    2011-01-01

    The structural complexity of nucleosomes underlies their functional versatility. Here we report a new type of complexity—nucleosome fragility, manifested as high sensitivity to micrococcal nuclease, in contrast to the common presumption that nucleosomes are similar in resistance to MNase digestion. Using differential MNase digestion of chromatin and high-throughput sequencing, we have identified a special group of nucleosomes termed “fragile nucleosomes” throughout the yeast genome, nearly 10...

  4. Phonological Accuracy and Intelligibility in Connected Speech of Boys with Fragile X Syndrome or Down Syndrome

    Science.gov (United States)

    Barnes, Elizabeth; Roberts, Joanne; Long, Steven H.; Martin, Gary E.; Berni, Mary C.; Mandulak, Kerry C.; Sideris, John

    2009-01-01

    Purpose: To compare the phonological accuracy and speech intelligibility of boys with fragile X syndrome with autism spectrum disorder (FXS-ASD), fragile X syndrome only (FXS-O), Down syndrome (DS), and typically developing (TD) boys. Method: Participants were 32 boys with FXS-O (3-14 years), 31 with FXS-ASD (5-15 years), 34 with DS (4-16 years),…

  5. [Language characterization in the X-fragile syndrome: a review study].

    Science.gov (United States)

    Ferreira, Grace Cristina; Lamônica, Dionísia Aparecida Cusin

    2005-01-01

    X-Fragile Syndrome. To compile information about the language, cognitive and behavior alterations in the X-Fragile Syndrome, using the results of previously published studies and to present the standardized instruments used as testing materials. studies used formal and informal testing to assess language. The results present variability regarding the linguistic deficits, which are influenced by the level of the cognitive deficit and behavior alterations. Alterations of the oral praxes and of speech articulation are also expected.

  6. Developing Xenopus Laevis as a Model to Screen Drugs for Fragile X Syndrome

    Science.gov (United States)

    2014-06-01

    bd). Transcription requires binding of endogenous Sox2/Oct4 transcription factors , and therefore results in protein expression in Sox2/Oct4...differentiation. Notably, Xenopus laevis tadpoles are transparent and develop externally , enabling direct observation of neurogenesis in early...Gordon Research Seminar and Conference: Fragile X and Autism –Related Disorders, May 31-June 6, 2014 Dysregulation of Fragile X Mental Retardation

  7. Banking Fragility in Colombia: An Empirical Analysis Based on Balance Sheets

    OpenAIRE

    Ignacio Lozano; Alexander Guarín

    2014-01-01

    In this paper, we study the empirical relationship between credit funding sources and the financial vulnerability of the Colombian banking system. We propose a statistical model to measure and predict banking-fragility episodes associated with credit funding sources classified into retail deposits and wholesale funds. We compute the probability of financial fragility for both the aggregated banking system and the individual banks. Our approach performs a Bayesian averaging of estimated logit ...

  8. Increasing our Understanding of Human Cognition Through the Study of Fragile X Syndrome

    OpenAIRE

    Denise, Cook; Erin, Nuro; Keith, K. Murai

    2013-01-01

    Fragile X Syndrome (FXS) is considered the most common form of inherited intellectual disability. It is caused by reductions in the expression level or function of a single protein, the Fragile X Mental Retardation Protein (FMRP), a translational regulator which binds to approximately 4% of brain messenger RNAs. Accumulating evidence suggests that FXS is a complex disorder of cognition, involving interactions between genetic and environmental influences, leading to difficulties in acquiring k...

  9. Political economies of corruption in fragile and conflict-affected states: Nuancing the picture

    OpenAIRE

    Dominik, Zaum

    2013-01-01

    Addressing corruption in fragile and conflict-affected environments is constrained by the risk that reforms can spark violent resistance. Two different political economies of corruption in fragile states – distinguished by the character of elite politics – affect the way corruption manifests itself. The differences have important implications for anti-corruption programming. Gradualism, changing elite incentives, and creating political space for reforms are approaches to emphasise. Anti-corru...

  10. Rescue of Synaptic Phenotypes and Spatial Memory in Young Fragile X Mice.

    Science.gov (United States)

    Sun, Miao-Kun; Hongpaisan, Jarin; Alkon, Daniel L

    2016-05-01

    Fragile X syndrome (FXS) is characterized by synaptic immaturity, cognitive impairment, and behavioral changes. The disorder is caused by transcriptional shutdown in neurons of thefragile X mental retardation 1gene product, fragile X mental retardation protein. Fragile X mental retardation protein is a repressor of dendritic mRNA translation and its silencing leads to dysregulation of synaptically driven protein synthesis and impairments of intellect, cognition, and behavior, and FXS is a disorder that currently has no effective therapeutics. Here, young fragile X mice were treated with chronic bryostatin-1, a relatively selective protein kinase Cεactivator, which induces synaptogenesis and synaptic maturation/repair. Chronic treatment with bryostatin-1 rescues young fragile X mice from the disorder phenotypes, including normalization of most FXS abnormalities in 1) hippocampal brain-derived neurotrophic factor expression, 2) postsynaptic density-95 levels, 3) transformation of immature dendritic spines to mature synapses, 4) densities of the presynaptic and postsynaptic membranes, and 5) spatial learning and memory. The therapeutic effects were achieved without downregulation of metabotropic glutamate receptor (mGluR) 5 in the hippocampus and are more dramatic than those of a late-onset treatment in adult fragile X mice. mGluR5 expression was in fact lower in fragile X mice and its expression was restored with the bryostatin-1 treatment. Our results show that synaptic and cognitive function of young FXS mice can be normalized through pharmacological treatment without downregulation of mGluR5 and that bryostatin-1-like agents may represent a novel class of drugs to treat fragile X mental retardation at a young age and in adults. Copyright © 2016 by The American Society for Pharmacology and Experimental Therapeutics.

  11. Therapeutic implications of the mGluR theory of fragile X mental retardation.

    Science.gov (United States)

    Bear, M F

    2005-08-01

    Evidence is reviewed that the consequences of group 1 metabotropic glutamate receptor (Gp1 mGluR) activation are exaggerated in the absence of the fragile X mental retardation protein, likely reflecting altered dendritic protein synthesis. Abnormal mGluR signaling could be responsible for remarkably diverse psychiatric and neurological symptoms in fragile X syndrome, including delayed cognitive development, seizures, anxiety, movement disorders and obesity.

  12. A Male with Cooccurrence of Down Syndrome and Fragile X Syndrome

    OpenAIRE

    Anderson, Tovi; Buterbaugh, Allison; Love, Kaitlin; Visootsak, Jeannie

    2013-01-01

    Down syndrome is the most common identifiable genetic cause of intellectual disability, with a unique physical gestalt that makes diagnosis possible during the newborn period. However, the physical characteristics of Fragile X syndrome are fairly subtle, resulting in the first clinical suspicion often arising from delayed developmental milestones. In addition, maladaptive behavior and autistic-like tendencies, such as hand flapping, poor eye contact, and hand biting, may be noted in Fragile ...

  13. Influência da cultura anterior e da compactação do solo na absorção de macronutrientes em soja

    Directory of Open Access Journals (Sweden)

    Silva Rosemeire Helena da

    2001-01-01

    Full Text Available O objetivo deste trabalho foi avaliar a concentração e a absorção de macronutrientes em soja cultivada em subseqüência a sete espécies vegetais em solo com diferentes níveis de compactação. O experimento foi conduzido em vasos contendo amostras de um Latossolo Vermelho, textura franco-arenosa, com camada de 3,5 cm compactada até as densidades de 1,12, 1,36 e 1,60 Mg m³, em que se cultivou anteriormente aveia-preta, guandu, milheto, mucuna-preta, soja, sorgo granífero, tremoço azul, mantendo-se um tratamento sem planta (pousio. Essas espécies se desenvolveram por 37 a 39 dias, foram cortadas ao nível do solo, picadas em partículas de, aproximadamente, 3 cm, e deixadas sobre a superfície do vaso por 40 dias. As plantas foram colhidas 28 dias após a emergência. Avaliou-se a produção de matéria seca, concentração e acúmulo de macronutrientes na parte aérea das plantas. A compactação do solo em subsuperfície diminui a nutrição da soja. A nutrição da soja é beneficiada quando cultivada em sucessão a plantas de cobertura no solo. O pousio antecedendo a cultura da soja não é recomendado como meio de reduzir os efeitos da compactação do solo.

  14. Manipulations of attention dissociate fragile visual short-term memory from visual working memory.

    Science.gov (United States)

    Vandenbroucke, Annelinde R E; Sligte, Ilja G; Lamme, Victor A F

    2011-05-01

    People often rely on information that is no longer in view, but maintained in visual short-term memory (VSTM). Traditionally, VSTM is thought to operate on either a short time-scale with high capacity - iconic memory - or a long time scale with small capacity - visual working memory. Recent research suggests that in addition, an intermediate stage of memory in between iconic memory and visual working memory exists. This intermediate stage has a large capacity and a lifetime of several seconds, but is easily overwritten by new stimulation. We therefore termed it fragile VSTM. In previous studies, fragile VSTM has been dissociated from iconic memory by the characteristics of the memory trace. In the present study, we dissociated fragile VSTM from visual working memory by showing a differentiation in their dependency on attention. A decrease in attention during presentation of the stimulus array greatly reduced the capacity of visual working memory, while this had only a small effect on the capacity of fragile VSTM. We conclude that fragile VSTM is a separate memory store from visual working memory. Thus, a tripartite division of VSTM appears to be in place, comprising iconic memory, fragile VSTM and visual working memory. Copyright © 2011 Elsevier Ltd. All rights reserved.

  15. Epidemiology of diabetes mellitus in the fragility fracture population of a region of Southern Italy.

    Science.gov (United States)

    Notarnicola, A; Maccagnano, G; Tafuri, S; Moretti, L; Laviola, L; Moretti, B

    2016-01-01

    Increased risk of osteoporosis and its clinical significance in patients with diabetes is controversial. This study aims to increase the data which are available regarding the prevalence of diabetes mellitus in patients affected by fragility fracture in Italy. We retrospectively studied Hospital Discharge Data (HDD) in the Apulian database for the period 2006–2010 to identify a fragility fracture diagnosis in males over 65 years of age and in females over 50. The database was then checked for drug prescriptions to identify those persons who had taken at least one osteoporosis drug. Within this latter group, thanks to hospital admission and prescription records, the subjects affected with diabetes mellitus were identified. Between 2006 and 2010 in Apulia 177,639 patients were hospitalized and diagnosed as having fragility fracture. The greatest number of those fragility fractures were found to be in the 70 to 79 age range (64,917 total; females 56,994, males 7,923). The prevalence of diabetes subjects in Apulia in this period was estimated at 6.5%. In the same region and period 21.1% of subjects affected by diabetes experienced a fragility fracture; in particular, this number was 27% for males and for 20.5% females. This is the first study providing data on the prevalence of fragility fractures and diabetes in the Apulian population. The data confirm that diabetes is a risk factor which influences bone density and risk of fractures and therefore the need of osteoporosis screening and treatment in diabetic patients.

  16. Hair root FMRP expression for screening of fragile X full mutation females

    Directory of Open Access Journals (Sweden)

    Lantip Rujito

    2011-04-01

    Full Text Available The fragile X syndrome is the most common form of inherited mental retardation in humans, caused by an expansion of the cytosine-guanine-guanine (CGG repeat in the fragile X mental retardation 1 (FMR1 gene located on the X chromosome. Antibody tests have been developed to identify fragile X patients, based on the presence or absence of fragile mental retardation protein (FMRP in both lymphocytes and hair roots. The objective of this study was to compare correlations of hair root and lymphocyte FMRP expression with cognitive functioning in female rural area probands carrying the full mutation. Thirty females (normal, premutation, or full mutation were selected from Indonesian fragile X families and were tested for FMRP expression in lymphocytes and hair roots using the FMRP antibody test. Subject genotype was determined by Southern blot analysis, and IQ equivalent by Raven’s Standard Progressive Matrices. Statistical analysis was by Pearson correlation. FMRP expression in blood lymphocytes was relatively higher than that in hair roots, but hair root FMRP expression was strongly correlated with cognitive functioning in female full mutation carriers (r=0.64, p=0.015, whereas no significant correlation between lymphocyte FMRP and cognitive functioning was found (r=0.31, p= 0.281. Around 14% of subjects had a normal and 7% a borderline IQ level, while 79% had mild mental impairment. In conclusion, hair root FMRP expression may be a useful marker for identification of fragile X full mutation females.

  17. Hair root FMRP expression for screening of fragile X full mutation females

    Directory of Open Access Journals (Sweden)

    Lantip Rujito

    2016-02-01

    Full Text Available The fragile X syndrome is the most common form of inherited mental retardation in humans, caused by an expansion of the cytosine-guanine-guanine (CGG repeat in the fragile X mental retardation 1 (FMR1 gene located on the X chromosome. Antibody tests have been developed to identify fragile X patients, based on the presence or absence of fragile mental retardation protein (FMRP in both lymphocytes and hair roots. The objective of this study was to compare correlations of hair root and lymphocyte FMRP expression with cognitive functioning in female rural area probands carrying the full mutation. Thirty females (normal, premutation, or full mutation were selected from Indonesian fragile X families and were tested for FMRP expression in lymphocytes and hair roots using the FMRP antibody test. Subject genotype was determined by Southern blot analysis, and IQ equivalent by Raven’s Standard Progressive Matrices. Statistical analysis was by Pearson correlation. FMRP expression in blood lymphocytes was relatively higher than that in hair roots, but hair root FMRP expression was strongly correlated with cognitive functioning in female full mutation carriers (r=0.64, p=0.015, whereas no significant correlation between lymphocyte FMRP and cognitive functioning was found (r=0.31, p= 0.281. Around 14% of subjects had a normal and 7% a borderline IQ level, while 79% had mild mental impairment. In conclusion, hair root FMRP expression may be a useful marker for identification of fragile X full mutation females.

  18. Baseline red blood cell osmotic fragility does not predict the degree of post-LVAD hemolysis.

    Science.gov (United States)

    Madden, Jesse L; Drakos, Stavros G; Stehlik, Josef; McKellar, Stephen H; Rondina, Matthew T; Weyrich, Andrew S; Selzman, Craig H

    2014-01-01

    Continuous-flow left ventricular assist devices (LVADs) subject elements of the blood to significant stress, resulting in clinically significant and subclinical hemolysis. We sought to prospectively determine whether baseline red-cell osmotic fragility of an advanced heart-failure patient influences the hemolytic response to LVAD support. Osmotic fragility assesses the degree of red-blood-cell hemolysis under varying degrees of osmotic stress. Assays were prospectively obtained on 50 consecutive patients prior to placement of continuous-flow LVADs: HeartMate II (n = 34), Jarvik 2000 (n = 5), HeartWare (n = 6). The mean age of the patients was 60.2 years and 87% were male and 47% were nonischemic. The overall median post-LVAD lactate dehydrogenase (LDH) was 583 (427-965), and there was no difference between devices. Mean hemolysis was 15.68 ± 12.96% at 0.45% NaCl (the inflection point of the osmotic fragility hemolysis curve). A scatter plot did not reveal any relationship between preoperative osmotic fragility and postoperative LDH. Linear regression confirmed no predictive relationship (p = 0.71). In conclusion, preoperative variations in osmotic fragility do not appear to account for differences in hemolysis following ventricular assist device placement. Mechanical forces generated by existing LVADs result in similar levels of biochemical hemolysis, as assessed by LDH, despite baseline differences in a patient's osmotic red-cell fragility.

  19. Component Fragility Research Program: Phase 1, Demonstration tests: Volume 1, Summary report

    International Nuclear Information System (INIS)

    Holman, G.S.; Chou, C.K.; Shipway, G.D.; Glozman, V.

    1987-08-01

    This report describes tests performed in Phase I of the NRC Component Fragility Research Program. The purpose of these tests was to demonstrate procedures for characterizing the seismic fragility of a selected component, investigating how various parameters affect fragility, and finally using test data to develop practical fragility descriptions suitable for application in probabilistic risk assessments. A three-column motor control center housing motor controllers of various types and sizes as well as relays of different types and manufacturers was subjected to seismic input motions up to 2.5g zero period acceleration. To investigate the effect of base flexibility on the structural behavior of the MCC and on the functional behavior of the electrical devices, multiple tests were performed on each of four mounting configurations: four bolts per column with top bracking, four bolts per column with no top brace, four bolts per column with internal diagonal bracking, and two bolts per column with no top or internal bracking. Device fragility was characterized by contact chatter correlated to local in-cabinet response at the device location. Seismic capacities were developed for each device on the basis of local input motion required to cause chatter; these results were then applied to develop probabilistic fragility curves for each type of device, including estimates of the ''high-confidence low probability of failure'' capacity of each

  20. Fragile X founder chromosomes in Italy: A few initial events and possible explanation for their heterogeneity

    Energy Technology Data Exchange (ETDEWEB)

    Chiurazzi, P.; Genuardi, M.; Kozak, L.; Neri, G. [Universita Cattolica and Centro Ricerche per la Disabilita Mentale e Motoria, Roma (Italy)] [and others

    1996-07-12

    A total of 137 fragile X and 235 control chromosomes from various regions of Italy were haplotyped by analyzing two neighbouring marker microsatellites, FRAXAC1 and DXS548. The number of CGG repeats at the 5{prime} end of the FMR1 gene was also assessed in 141 control chromosomes and correlated with their haplotypes. Significant linkage disequilibrium between some {open_quotes}major{close_quotes} haplotypes and fragile X was observed, while other {open_quotes}minor{close_quotes} haplotypes may have originated by subsequent mutation at the marker microsatellite loci and/or recombination between them. Recent evidence suggests that the initial mechanism leading to CGG instability might consist of rare (10{sup -6/-7}) CGG repeat slippage events and/or loss of a stabilizing AGG via A-to-C transversion. Also, the apparently high variety of fragile X chromosomes may be partly due to the relatively high mutation rate (10{sup -4/-5}) of the microsatellite markers used in haplotyping. Our fragile X sample also showed a higher than expected heterozygosity when compared to the control sample and we suggest that this might be explained by the chance occurrence of the few founding events on different chromosomes, irrespective of their actual frequency in the population. Alternatively, a local mechanism could enhance the microsatellite mutation rate only on fragile X chromosomes, or fragile X mutations might occur more frequently on certain background haplotypes. 59 refs., 4 figs.