863 resultados para KNOBLOCH-SYNDROME


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Fenneropenaeus indicus could be protected from white spot disease (WSD) caused by white spot syndrome virus (WSSV) using a formalin-inactivated viral preparation (IVP) derived from WSSV-infected shrimp tissue. The lowest test quantity of lyophilized IVP coated onto feed at 0.025 g–1 (dry weight) and administered at a rate of 0.035 g feed g–1 body weight d–1 for 7 consecutive days was sufficient to provide protection from WSD for a short period (10 d after cessation of IVP administration). Shrimp that survived challenges on the 5th and 10th days after cessation of IVP administration survived repeated challenges although they were sometimes positive for the presence of WSSV by a polymerase chain reaction (PCR) assay specific for WSSV. These results suggest that F. indicus can be protected from WSD by simple oral administration of IVP

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Influence of acute salinity stress on the immunological and physiological response of Penaeus monodon to white spot syndrome virus (WSSV) infection was analysed. P. monodon maintained at 15‰ were subjected to acute salinity changes to 0‰ and 35‰ in 7 h and then challenged orally with WSSV. Immune variables viz., total haemocyte count, phenol oxidase activity (PO), nitroblue tetrazolium salt (NBT) reduction, alkaline phosphatase activity (ALP), acid phosphatase activity (ACP) and metabolic variables viz., total protein, total carbohydrates, total free amino acids (TFAA), total lipids, glucose and cholesterol were determined soon after salinity change and on post challenge days 2 (PCD2) and 5 (PCD5). Acute salinity change induced an increase in metabolic variables in shrimps at 35‰ except TFAA. Immune variables reduced significantly (Pb0.05) in shrimps subjected to salinity stress with the exception of ALP and PO at 35‰ and the reduction was found to be more at 0‰. Better performance of metabolic and immune variables in general could be observed in shrimps maintained at 15‰ that showed significantly higher post challenge survival following infection compared to those under salinity stress. Stress was found to be higher in shrimps subjected to salinity change to lower level (0‰) than to higher level (35‰) as being evidenced by the better immune response and survival at 35‰. THC (Pb0.001), ALP (Pb0.01) and PO (Pb0.05) that together explained a greater percentage of variability in survival rate, could be proposed as the most potential health indicators in shrimp haemolymph. It can be concluded from the study that acute salinity stress induces alterations in the haemolymph metabolic and immune variables of P. monodon affecting the immunocompetence and increasing susceptibility to WSSV, particularly at low salinity stress conditions

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Der eukaryotische Mikroorganismus Dictyostelium discoideum lebt als einzellige Amöbe solange ausreichende Nahrungsressourcen zur Verfügung stehen. Sobald Nahrungsmangel eintritt, entwickeln sich die Zellen von einem einzelligen zu einem mehrzelligen Zustand, der mit einem multizellulären Fruchtkörper abschließt. Dieser Prozess wird durch eine Reihe aufeinanderfolgender Signale organisiert, die eine differentielle Genexpression regulieren. Die Gene der Discoidin I Familie gehören zu den Ersten, die im Laufe des Wachstums-Differenzierungs-Übergangs (engl. GDT) aktiviert werden. Sie eignen sich daher vorzüglich als Marker für den Beginn der Entwicklung. Mit Hilfe einer REMI-Mutagenese und Discoidin I als molekularem Marker sind verschiedene Komponenten des Wachstums-Differenzierungs-Übergangs in unserer Arbeitsgruppe identifiziert worden (Zeng et al., 2000 A und B; Riemann und Nellen, persönliche Mitteilung). Mit demselben Ansatz wurde in der vorliegenden Arbeit eine REMI-Mutante identifiziert, die eine Fehl-Expression von Discoidin zeigte und einen axenischen Wachstumsdefekt bei 15 °C aufwies. Das Gen wurde als Homolog zum humanen Tafazzin-Gen identifiziert. Dieses Gen wurde zur Rekonstruktion des Phänotyps über homologe Rekombination erneut disruptiert, was wie erwartet zu dem zuerst beschriebenen Phänotyp führte. Folgerichtig ergab eine Überexpression des Gens in den Mutanten eine Komplementation des Phänotyps. Immunfluoreszenz-Experimente zeigten eine mitochondriale Lokalisation des Dictyostelium discoideum Taffazzin Proteins. Dass ein mitochondriales Protein in Zusammenhang mit dem Wachstums-Differenzierungs-Übergang steht, ist ein unerwarteter Befund, der aber als Hinweis darauf gewertet werden kann, dass Mitochondrien einen direkten Einfluss auf die entwicklungsspezifische Signaltransduktion ausüben. Die Taffazzin Disruptions-Mutante in Dictyostelium führte zu einem abnormalen Cardiolipin Metabolismus. Dieses Phospholipid ist ein charakteristischer Bestandteil der inneren Mitochondrienmembran und für die Funktion verschiedener Enzyme erforderlich. Unsere vorläufigen Analysen des Phospholipid-Gehalts zeigten Übereinstimmung mit Daten von Patienten mit Barth-Syndrom, einer humanen Erkrankung, bei der das Taffazzin-Gen Mutationen aufweist, und mit Hefe-Mutanten dieses Gens. Dies zeigt den Wert von Dictyostelium discoideum als einen weiteren Modelorganismus zur Untersuchung des Barth-Syndroms und zur Erprobung möglicher Therapieansätze.

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El libro es el resultado de la compilaci??n de las colaboraciones de expertos internacionales reunidos en un Simposio Internacional sobre Psicolog??a y Psicobiolog??a Educativa e Integraci??n Social de las personas con S??ndrome de Down, organizado por Juan Perera (Asociaci??n S??ndrome de Down de Baleares. Universidad de las Islas Baleares) y Jean A. Rondal (Laboratorio de Psicoling????stica de la Universidad de Lieja, B??lgica), bajo los auspicios del Gobierno Balear y otras instituciones y organismos. La colaboraci??n de los autores murcianos (Candel, Carranza y P??rez L??pez) versa sobre el desarrollo socio-afectivo de los ni??os afectados por el S??ndrome y presenta los resultados de una investigaci??n longitudinal sobre el temperamento de los ni??os con s??ndrome Down. Los objetivos de la investigaci??n son: el estudio de los componentes temperamentales en el desarrollo de los ni??os con S??ndrome Down. La evaluaci??n del grado de estabilidad/inestabilidad de las diferencias temperamentales en los ni??os con S??ndrome Down y an lisis del grado de homogeneidad de los componentes temperamentales de estos ni??os como grupo y las diferencias con un grupo de control de ni??os no retrasados. Los resultados confirman la existencia de diferencias individuales en el temperamento de los ni??os con S??ndrome Down.

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Sjögren's syndrome (SS) is a late-onset chronic autoimmune disease (AID) affecting the exocrine glands, mainly the salivary and lachrymal. Genetic studies on twins with primary SS have not been performed, and only a few case reports describing twins have been published. The prevalence of primary SS in siblings has been estimated to be 0.09% while the reported general prevalence of the disease is approximately 0.1%. The observed aggregation of AIDs in families of patients with primary SS is nevertheless supportive for a genetic component in its etiology. In the absence of chromosomal regions identified by linkage studies, research has focused on candidate gene approaches (by biological plausibility) rather than on positional approaches. Ancestral haplotype 8.1 as well as TNF, IL10 and SSA1 loci have been consistently associated with the disease although they are not specific for SS. In this review, the genetic component of SS is discussed on the basis of three known observations: (a) age at onset and sex-dependent presentation, (b) familial clustering of the disease, and (c) dissection of the genetic component. Since there is no strong evidence for a specific genetic component in SS, a large international and collaborative study would be suitable to assess the genetics of this disorder.

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The abdominal compartment syndrome (ACS) is the result of various physiological alterations produced by an abnormal increase of the intra-abdominal pressure. Some of these patients will undergo a surgical procedure for its management. Methods: This is a retrospective case series of 28 patients with ACS who required surgical treatment at the Hospital Occidente de Kennedy between 1999 and 2003. We assessed retrospectively the behavior of McNelis’s equation for prediction of the development of the ACS. Results: The leading cause of ACS in our study was intraabadominal infection (n=6 21,4%). Time elapsed between diagnosis and surgical decompression was less than 4 hours in 75% (n=21) of the cases. The variables that improved significantly after the surgical decompression were CVP (T: 4,0 p: 0,0001), PIM (T: 2,7; p: 0,004), PIA (T1,8; p:0,034) and Urine Output (T:-2,4; p:0,02). The values of BUN, Creatinine and the cardiovascular instability did not show improvement. The ICU and hospital length of stay were 11 days (SD: 9) and 18 days (SD13) respectively. Global mortality was 67,9% (n=19) and mortality directly attributable to the syndrome was 30% (n=8). The behavior of the McNelis’s equation was erratic. Conclusions: The demographic characteristics as well as disease processes associated with ACS are consistent with the literature. The association between physiological variables and ACS is heterogeneous between patients. Mortality rates attributable to ACS in our institution are within the range described world-wide. The behavior of the McNelis’s equation seems to depend greatly upon fluid balance.

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Recurso de bolsillo que ayuda a los profesores y al personal de apoyo a entender las dificultades de los alumnos con síndrome de Asperger y les ofrece estrategias para superar los retos que plantean en el aula. El primer capítulo explica exactamente lo que es, y lo que no es, el citado síndrome, enumera sus características y destaca las complicaciones y los placeres de tener un alumno con estas dificultades en clase. Los capítulos siguientes hacen frente a desafíos específicos: deterioro social; intereses obsesivos; problemas con el cambio; la etapa de la rabia, la etapa de recuperación. Tiene ejemplos ilustrativos y soluciones prácticas. Hace referencia a recursos adicionales, programas de enseñanza, sitios web.

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Recurso que ofrece enfoques y estrategias que los profesores pueden utilizar para ayudar a sus estudiantes con síndrome de Asperger y autismo en el camino hacia el éxito. Analiza los problemas que pueden surgir en el aula de inclusión y cómo los educadores pueden hacer adaptaciones para atender a sus alumnos con autismo sin interferir en las rutinas del aula estándar. Incluye información sobre lo que puede causar ansiedad en el estudiante con estas discapacidades, los posibles incrementos en los problemas de comportamiento, y lo que el profesor puede hacer para ayudar. Cuenta con diez estrategias acompañadas de ejemplos y razones por las que son importantes usarlas.

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A dança é uma actividade de grande exigência atlética, que pode conduzir a um elevado número de lesões, particularmente na região do tornozelo, possivelmente devido à amplitude extrema do movimento articular de flexão plantar do mesmo, que os bailarinos, especialmente do sexo feminino possuem, para realizar a ponta e meia ponta tão características do ballet clássico (Kadel, 2006; Motta-Valencia, 2006; Russel, Kruse, Koutedakis, McEwan, Wyon, 2010). Estas posições de flexão plantar extrema produzem força excessiva na região posterior do tornozelo, o que muitas vezes pode resultar em conflito, dor e incapacidade, representando na maioria das vezes um desafio de diagnóstico. O síndrome do conflito posterior do tornozelo refere-se a um grupo de entidades patológicas que resultam da flexão plantar forçada do tornozelo, de forma repetitiva ou traumática, causando um conflito das estruturas ósseas e/ou de tecidos moles (Hamilton, Geppert, Thompson, 1996; Hamilton, 2008) . Os objectivos deste projecto são compreender os quais os factores de risco, mecânicos e funcionais que contribuem para a mecânica patológica da lesão descritos na literatura, e proceder a uma avaliação biomecânica do movimento de flexão plantar do tornozelo. Método. Realizar uma revisão sistemática de literatura dirigida á mecânica patológica do síndrome do conflito posterior do tornozelo em bailarinas e conduzir um estudo caso-controlo, cujo objectivo é avaliar, comparar e descrever o movimento da flexão plantar do tornozelo realizado ao efectuar os movimentos de ponta e meia-ponta, em bailarinas pré-profissionais com e sem lesão recorrente resultante do conflito posterior do tornozelo. Resultados. Não foram encontrados estudos relacionados especificamente com a mecânica patológica do tornozelo, no entanto vários estudos foram encontrados considerando as características clínicas e anatómicas assim como os procedimentos de tratamento, indicando que os principais factores de risco relacionados com a lesão se dividem em factores mecânicos e funcionais que quando combinados entre si e associados ao sobre-uso podem resultar no conflito posterior do tornozelo. Na avaliação do movimento foram observadas diferenças na actividade muscular entre os sujeitos com lesão e controlos, tendo sido possível a observação de um padrão na sequência de activação para um dos movimentos testados. Na oscilação postural e na rigidez do tornozelo foram também observadas diferenças entre os sujeitos bem como entre as posições realizadas. Conclusão. Concluiu-se que não sendo possível alterar a anatomia do bailarino, por vezes é possível intervir a nível funcional melhorando a capacidade técnica de forma obter um melhor desempenho e a actuar preventivamente em relação às lesões, uma vez que estas podem apresentar padrões cinéticos próprios, relacionados com a função muscular, a estabilidade postural e a rigidez articular.

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This paper reviews a study to determine the maximum rate the acoustic reflex can follow pulsed stimuli in normal hearing subjects and in subjects with Meniere's Syndrome.

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This paper reviews a study to determine the maximum rate the acoustic reflex can follow pulsed stimuli in normal hearing subjects and in subjects with Meniere's Syndrome.