Is "milk crust" a transient form of golden retriever ichthyosis?

BACKGROUND A recessive inherited form of lamellar ichthyosis is well recognized in golden retrievers. In this breed, young puppies demonstrate a self-limiting scaling disorder which is commonly recognized by breeders, who use the term "milk crust" to describe this syndrome. HYPOTHESIS/OBJECTIVES To determine whether "milk crust" is a new keratinization disorder or a self-limiting form of golden retriever ichthyosis. ANIMALS A total of 179 golden retriever dogs (21 dams and 158 puppies) were examined. METHODS Dermatological examination and assessment of the patatin-like phospholipase-1 (PNPLA1) genotype by PCR testing of buccal mucosal swabs. Skin biopsies from one affected puppy were evaluated for histopathological abnormalities. RESULTS Forty-five of 158 (28%) puppies exhibited scaling at 8 weeks of age; 113 of 158 (72%) were dermatologically normal. Of 144 analysed samples, 40 of 144 (28%) puppies demonstrated a homozygous mutation of the PNPLA1 genotype [of which, 36 of 40 (90%) had signs of scaling], 77 of 144 (53%) demonstrated a heterozygous mutation and 27 of 144 (19%) were a normal wild-type. In six of 17 (35%) dams, a homozygous mutation of the PNPLA1 genotype was found, eight of 17 (47%) demonstrated a heterozygous mutation and three of 17 (18%) were normal wild-type. Dams with a homozygous mutation were clinically unaffected. A 1 year follow-up revealed that 23 of 28 (82%) puppies affected with this syndrome failed to develop typical signs of ichthyosis. In five of 28 (18%) dogs there was persistence of mild scaling. CONCLUSIONS AND CLINICAL IMPORTANCE We hypothesize that the clinical syndrome termed "milk crust" could represent a transient form of golden retriever ichthyosis. Remission is not fully linked to PNPLA1 genotype, suggesting that unknown factors may contribute to the clinical disease.

Extreme River Floods in Western Switzerland and the Lake of Constance Region in the Period Prior to Instrumental Measurements

The floods that occurred on the Aare and Rhine rivers in May 2015 and the mostly successful handling of this event in terms of flood protection measures are a good reminder of how important it is to comprehend the causes and processes involved in such natural hazards. While the needed data series of gauge measurements and peak discharge calculations reach back to the 19th century, historical records dating further back in time can provide additional and useful information to help understanding extreme flood events and to evaluate prevention measures such as river dams and corrections undertaken prior to instrumental measurements. In my PhD project I will use a wide range of historical sources to assess and quantify past extreme flood events. It is part of the SNF-funded project “Reconstruction of the Genesis, Process and Impact of Major Pre-instrumental Flood Events of Major Swiss Rivers Including a Peak Discharge Quantification” and will cover the research locations Fribourg (Saane R.), Burgdorf (Emme R.), Thun, Bern (both Aare R.), and the Lake of Constance at the locations Lindau, Constance and Rorschach. My main goals are to provide a long time series of quantitative data for extreme flood events, to discuss the occurring changes in these data, and to evaluate the impact of the aforementioned human influences on the drainage system. Extracting information given in account books from the towns of Basel and Solothurn may also enable me to assess the frequency and seasonality of less severe river floods. Finally, historical information will be used for remodeling the historical hydrological regime to homogenize the historical data series to modern day conditions and thus make it comparable to the data provided by instrumental measurements. The method I will apply for processing all information provided by historical sources such as chronicles, newspapers, institutional records, as well as flood marks, paintings and archeological evidence has been developed and successfully applied to the site of Basel by Wetter et al. (2011). They have also shown that data homogenization is possible by reconstructing previous stream flow conditions using historical river profiles and by carefully observing and re-constructing human changes of the river bed and its surroundings. Taken all information into account, peak discharges for past extreme flood events will be calculated with a one-dimensional hydrological model.

Dose-dependent effects of experimental infection with the virulent Neospora caninum Nc-Spain7 isolate in a pregnant mouse model.

Pregnant BALB/c mice have been widely used as an in vivo model to study Neospora caninum infection biology and to provide proof-of-concept for assessments of drugs and vaccines against neosporosis. The fact that this model has been used with different isolates of variable virulence, varying infection routes and differing methods to prepare the parasites for infection, has rendered the comparison of results from different laboratories impossible. In most studies, mice were infected with similar number of parasites (2 × 10(6)) as employed in ruminant models (10(7) for cows and 10(6) for sheep), which seems inappropriate considering the enormous differences in the weight of these species. Thus, for achieving meaningful results in vaccination and drug efficacy experiments, a refinement and standardization of this experimental model is necessary. Thus, 2 × 10(6), 10(5), 10(4), 10(3) and 10(2) tachyzoites of the highly virulent and well-characterised Nc-Spain7 isolate were subcutaneously inoculated into mice at day 7 of pregnancy, and clinical outcome, vertical transmission, parasite burden and antibody responses were compared. Dams from all infected groups presented nervous signs and the percentage of surviving pups at day 30 postpartum was surprisingly low (24%) in mice infected with only 10(2) tachyzoites. Importantly, infection with 10(5) tachyzoites resulted in antibody levels, cerebral parasite burden in dams and 100% mortality rate in pups, which was identical to infection with 2 × 10(6) tachyzoites. Considering these results, it is reasonable to lower the challenge dose to 10(5) tachyzoites in further experiments when assessing drugs or vaccine candidates.

Bioensayo de germinación de Lactuca sativa (L.) : determinación de calidad de agua en represas para riego

En el norte de la provincia de Entre Ríos se ha desarrollado un modelo de producción de arroz basado en el uso de agua superficial mediante la construcción de presas de tierra: existen más de 60 con superficies variables. El uso de agroquímicos en esa zona podría introducir un factor de contaminación en el suelo y en el agua de las represas. El objetivo de este trabajo fue determinar la calidad del agua de 19 represas para riego del centro norte de Entre Ríos, mediante bioensayos de germinación de Lactuca sativa var. mantecosa. Los bioensayos se realizaron en cajas de Petri, con papel de filtro en la base humedecido con 3 ml de agua de la muestra correspondiente. Se sembraron 20 semillas por caja, distribuyéndose los tratamientos en bloques al azar con 4 repeticiones, en cámara de germinación con alternancia de luz y oscuridad. Se registró el porcentaje de germinación y la longitud promedio de la raíz; se calculó un índice de germinación. El porcentaje de germinación promedio de los tratamientos fue de 96,07% y de 97,9% en el testigo. El índice de germinación en todos los casos fue superior al 60% y no se detectó toxicidad en el agua proveniente de las distintas represas.

Para que no pase con Potrerillos lo que pasó con El Carrizal

El embalse Carrizal es una obra realizada sobre el río Tunuyán, que nace a la vida del oasis en el año 1971, acompañada de importantes proyectos de desarrollo económico e integración territorial. La inexistencia de una legislación adecuada a las necesidades generadas por esta infraestructura, la ineficiencia en su administración y la desconexión puesta de manifiesto entre el embalse y su zona de influencia, son las causas que provocan la falta de equipamiento turístico-recreativo adecuado y el aprovechamiento óptimo del recurso agua. En el caso de Potrerillos , se diferencian 3 zonas en donde el impacto de la presa puede producir cambios importantes, potenciando su desarrollo o provocando la aparición de externalidades negativas y mayores gastos de los previstos. Estas son : la márgenes del embalse, en donde se espera, al igual que en El Carrizal, el desarrollo de una serie de proyectos turísticos y recreativos; la zona de influencia de la Villa Potrerillos y la del Alto Potrerillos y la zona de riego del río Mendoza. La inserción de este tipo de obra debe ser planificada, y las políticas y acciones a implementar deben surgir de un análisis profundo de su realidad, de las limitaciones y potencialidades naturales como también de las necesidades y requerimientos de todos los agentes que participan en la construcción del nuevo ecosistema.

Transformación de las políticas de gestión de riesgos en Quebec

En 1996, las lluvias excepcionalmente abundantes que cayeron en la región de Saguenay (Quebec - Canadá) provocaron la ruptura o el desbordamiento de presas, inundaciones, deslizamientos de tierra (más de 1000 en 36 horas) y numerosos cortes de electricidad. Estos eventos repentinos causaron dos muertes, forzaron la evacuación de más de 16.000 personas y generaron perdidas económicas estimadas en aproximadamente un millar de dólares canadienses. Cerca de quince años más tarde se hace un balance de algunas de las principales repercusiones que dichos eventos tuvieron en la manera como el gobierno de Quebec enfrenta estas situaciones. Dos leyes (ley sobre la seguridad de las presas (2000) y la ley sobre la seguridad publica 2001)) y una política (la política nacional del agua, 2002) se inspiraron directamente en las lecciones dejadas por el desastre de Saguenay y son brevemente presentadas en este artículo. Estas lecciones, así como aquellas obtenidas de la tormenta de hielo que vivió Quebec en 1998, abrieron la vía a un enfoque mas global y estructurado de la seguridad civil: la gestión de riesgos.

Ingeniería de presas escolleras

La construcción de la Presa Potrerillos en el río Mendoza, durante una época muy especial de la ingeniería de grandes presas (1998-2005), caracterizada por la diversidad de las corrientes de opinión acerca del desarrollo del aprovechamiento de los recursos hidráulicos de regiones y países, la elaboración y difusión de criterios del informe final de la Comisión Mundial de Presas (World Commission on Dams) junto con la publicación del mismo y la vigencia en la Provincia de Mendoza de la Ley provincial nº 5961 de preservación del Medio Ambiente, ha sido acompañada por los sectores técnicos de la sociedad según modos muy diversos, entroncados con el nivel de las culturas particulares de sus integrantes y del estado de la práctica local. Hubo voces favorables y otras desalentadoras que, como en el caso de la presa de Aswan, jamás callarán, a pesar del balance de beneficios y perjuicios, el cual demuestra lo acertado en la decisión de llevarla a cabo, a pesar de los inconvenientes que debieron ser superados.

(Table 1) Summary of accelerator mass spectrometer (AMS) radiocarbon ages in ODP Sites 169-1033 and 169-1034

Continuous coring in Saanich Inlet (Ocean Drilling Program, ODP Leg 169S), British Columbia, Canada, yielded a detailed record of Late Quaternary climate, oceanography, marine productivity, and terrestrial vegetation. Two sites (1033 and 1034) were drilled to maximum depths of 105 and 118 m, recovering sediments ranging in age from 13,300 to less than 300 14C yr. Earliest sediments consist of dense, largely massive, gray glaciomarine muds with dropstones and sand and silt laminae deposited during the waning stages of glaciation. Deposition of organic-rich olive gray sediments began in the fjord about 12,000 14C yr ago, under well-oxygenated conditions as reflected by the presence of bioturbation and a diverse infaunal bivalve community. At about 10,500 14C yr, a massive, gray unit, 40-50 cm thick, was emplaced in a very short span of time. The unit is marked by a sharp lower contact, a gradational upper contact and an abundance of reworked Tertiary microfossils. It has been interpreted as resulting from massive flood events caused by the collapse of glacial dams in the Fraser Valley of mainland British Columbia. Progressively greater anoxia in bottom waters of Saanich Inlet began about 7000 14C yr ago. This is reflected in the preservation of varved sediments consisting of diatomaceous spring-summer laminae and terrigenous winter laminae. Correlation of the sediments was based on: marked lithologic changes, the presence of massive intervals (reflecting localized sediment gravity flow events), the Mazama Ash, occasional thin gray laminae (indicative of abnormal flood events in nearby watersheds), varve counts between marker horizons, and 71 accelerator mass spectrometry (AMS) radiocarbon dates.

Contribution to Theme A of the Benchmark Workshop: Effect of Concrete Swelling on the Equilibrium and Displacements of an Arch Dam

The Kariba dam is undergoing concrete expansion as a result of an alkali-aggregate reaction. The model adopted to simulate the process is explained in the paper; it is based on the model first proposed by Ulm et al, as later modified by Saouma and Perotti. It has been implemented in the commercial finite element code Abaqus and applied to solve the benchmark problem. The parameters of the model were calibrated using the data recorded up to 1995. The calibrated model was then used for predicting the evolution of the dam up to the present date. Apart from this prediction the paper offers a number of conclusions, such as the fact that the stress level appears to have a major influence on the expansion process; and it presents some suggestions to improve the formulation of the benchmark, such as providing temperature data and widening the locations and conditions of the data employed in the calibration

Effect of Concrete Swelling on the Equilibrium and Displacements of an Arch Dam

The Kariba dam is undergoing concrete expansion as a result of an alkali-aggregate reaction. The model adopted to simulate the process is explained in the paper; it is based on the model first proposed by Ulm et al, as later modified by Saouma and Perotti. It has been implemented in the commercial finite element code Abaqus and applied to solve the benchmark problem. The parameters of the model were calibrated using the data recorded up to 1995. The calibrated model was then used for predicting the evolution of the dam up to the present date. Apart from this prediction the paper offers a number of conclusions, such as the fact that the stress level appears to have a major influence on the expansion process; and it presents some suggestions to improve the formulation of the benchmark, such as providing temperature data and widening the locations and conditions of the data employed in the calibration

Sistemas de control de roturas en presas y caracterización de sistemas hidráulicos

Este proyecto nace de la necesidad de automatizar el estudio de sistemas hidráulicos y de control de roturas en presas. Para realizar el estudio de sistemas hidráulicos se usarán un número indeterminado de sensores de nivel, presión y caudal. El número de sensores que se pueden utilizar viene determinado por el material disponible. Estos sensores se conectarán a unas tarjetas de National Instruments modelo NI 9208 y éstas a su vez a un chasis modelo CompactDAQ NI-9174 con cuatro ranuras. Conectando este chasis al ordenador podremos obtener los datos provenientes de los sensores. También se podrá controlar una válvula para determinar la cantidad de agua que fluye en nuestro experimento. Está válvula está conectada a una tarjeta NI-9264 que se conectará al chasis en su última posición Para detectar y estudiar posibles roturas en presas se dispone de un motor y un láser con los cuales se puede barrer la superficie de una presa y obtener una imagen en tres dimensiones de la misma procesando los datos provenientes del laser. Para recoger los datos de los sensores y controlar una válvula se ha desarrollado una aplicación utilizando LabVIEW, un programa creado por National Instruments. Para poder controlar el motor y el láser se parte de una aplicación que ya estaba realizada en LabVIEW. El objetivo ha sido detectar y corregir una serie de errores en la misma. Dentro del proyecto, además de la explicación detallada de la aplicación para los sensores y la válvula, y las pruebas realizadas para detectar y corregir los errores de la aplicación del láser y el motor, existe: una breve introducción a la programación en LabVIEW, la descripción de los pasos realizados para el conexionado de los sensores con las tarjetas, los manuales de usuario de las aplicaciones y la descripción de los equipos utilizados. This project stars from the need to automate the study of hydraulic systems and control dam breaks. For the study of hydraulic systems it will be used an unspecified number of level, pressure and flow sensors. The number of sensors that can be used is determined by the available material. These sensors are connected to a NI 9208 National Instruments target and these cards to a NI-9174 CompactDAQ chassis with four slots. Connecting the chassis to a computer we will obtain data from the sensors. We also can control a valve to determine the amount of water flowing in our experiment. This valve is connected to a NI-9264 card and this card to the last position of the chassis. To detect and study dams breakage it used a motor and a laser. With these two devices we can scan the surface of a dam and obtain a three-dimensional image processing data from the laser. To collect data from the sensors and control the valve it has developed an application using LabVIEW, a program created by National Instruments. To control the motor and the laser it is used an application that was already created using LabVIEW. The aim of this part has been detect and correct a number of errors in this application. Within the project, in addition to the detailed explanation of the application for sensors and valve, and tests to detect and correct errors in the application of lasers and the motor, there is: a brief introduction to programming in LabVIEW, the description of the steps taken for connecting the sensors with cards, user manuals and application description of the equipment used.

Estudio de la reactividad álcali-sílice originada por componentes reactivos minoritarios

El objetivo de la presente tesis se enmarca dentro del estudio del estado de hormigones de presas, desarrollado en los últimos años en el Laboratorio Central del CEDEX, en el que se ratifica que una de las causas más importantes del deterioro de obras hidráulicas en España es la reacción álcali-sílice. La tesis que se presenta pretende contribuir al mejor conocimiento de la reacción álcali sílice con fines normativos preventivos, abordando los aspectos relativos a la identificación de áridos reactivos en el hormigón. El conocimiento de los áridos reactivos en España (origen de la reactividad, tipos de reacción y su comportamiento, así como las herramientas disponibles para su detección) es imprescindible para evitar la futura aparición de esta patología en nuevas estructuras, ya sea evitando el uso de áridos reactivos o tomando las medidas preventivas necesarias si su utilización es inevitable. A partir del Estudio Bibliográfico realizado se han detectado diversas lagunas en la identificación y caracterización de áridos de reacción rápida, cuya característica principal es que son reactivos con concentraciones muy bajas de diferentes componentes reactivos. Para resolver las lagunas identificadas se ha planeado un estudio experimental, consistente en el análisis de áridos cuya reactividad es conocida porque han sido empleados en obras afectadas por la reacción álcali sílice. Sobre el árido grueso extraído de estas estructuras se han realizado una serie de ensayos normalizados (estudio petrográfico, ensayo acelerado de probetas de mortero, ensayo Gel Pat y ensayos químicos). El análisis de los resultados experimentales ha permitido conocer las limitaciones reales en áridos reactivos españoles de las diferentes técnicas existentes, tratando de minimizarlas para áridos cuya reactividad es debida a componentes minoritarios (áridos de reacción rápida). Además, se ha evaluado la utilización de la difracción de rayos X (no normalizada) y la creación de un nuevo ensayo (Gel Pat Modificado). Finalmente, el estudio experimental ha permitido fijar una metodología de ensayo para el estudio de áridos reactivos por su contenido en componentes minoritarios (áridos de reacción rápida). The objective of this Thesis fits into the research program developed in CEDEX the last years and focused on the durability of concrete in Dams. This research work confirms that one of the main problems related to the deterioration of hydraulic structures is the alkali silica reaction. This Thesis aims to contribute to a better understanding of alkali-silica reaction, for preventive regulation purposes, considering the aspects related to the identification of reactive aggregates. The knowledge of Spanish reactive aggregates (origin of the reactivity, types of reaction and their behavior, and the tools available to detect and describe them) is essential to avoid the appearance of this pathology in new structures, either not using the reactive aggregate or taking the necessary preventive measures available in bibliography if the use of the reactive aggregate is inevitable. From the State-of –the-Art developed, several gaps have been detected in the detection and description of rapid reactive aggregates, which main characteristic if that they are reactive with low content of some reactive components. An experimental programme has been designed to solve these gaps, consisting on studying the reactivity of aggregates used in Spanish structures affected by the alkali silica reaction. Several Standard Tests have been carried out on coarse aggregates removed from the affected structures (Petrographic description, Accelerated Mortar Bar Test, Gel Pat Test and Chemical Tests). The analysis of the results obtained in Spanish reactive aggregates allows to know the advantages and limitations of each test, trying to minimize the disadvantages to detect Spanish reactive aggregates because of the minority content of rapid reactive components (rapid reactive aggregates). Moreover, X ray diffraction (not standardized) has been tested to detect rapid reactive aggregates and also a new test has been developed for the same purpose (Optimized Gel Pat Test). Finally, the experimental programme has made possible to define a methodology for detection of Spanish rapid reactive aggregates.