Four-month-old with severe PIK3CA-related overgrowth spectrum disorder successfully treated with alpelisb.

PIK3CA-related overgrowth spectrum (PROS) encompasses different clinical entities caused by somatic activating mutations in PIK3CA. Among PROS, CLOVES syndrome represents a severe phenotype with poor survival rate. We present the case of a 4-month-old girl with CLOVES syndrome successfully treated with alpelisib, a PIKC3A inhibitor.

On the effects of temperature and pH on tropical and temperate holothurians.

Ocean acidification and increased ocean heat content has direct and indirect effects on marine organisms such as holothurians (sea cucumbers) that are vulnerable to changes in pH and temperature. These environmental factors have the potential to influence organismal performance and fitness at different life stages. Tropical and temperate holothurians are more vulnerable to temperature and pH than those from colder water environments. The high level of environmental variation observed in the oceans could influence organismal responses and even produce a wide spectrum of compensatory physiological mechanisms. It is possible that in these areas, larval survival will decline by up to 50% in response to a reduction of 0.5 pH units. Such reduction in pH may trigger low intrinsic growth rates and affect the sustainability of the resource. Here we describe the individual and combined effects that temperature and pH could produce in these organisms. We also describe how these effects can scale from individuals to the population level by using age-structured spatial models in which depensation can be integrated. The approach shows how physiology can improve the conservation of the resource based on the restriction of growth model parameters and by including a density threshold, below which the fitness of the population, specifically intrinsic growth rate, decreases.

Systemic reaction to an extensively hydrolyzed formula in an infant with cow's milk anaphylaxis.

Background: Cow's milk allergy is the most common cause of food allergy in young children. Ingestion of milk products in children with a milk protein allergy can lead to anaphylaxis and must be avoided. Some guidelines suggest the use of an extensively hydrolyzed formula (EHF) in these cases; however, rare allergic reactions can still occur. Here, we presented a 3-month-old boy who developed anaphylaxis to a cow's milk formula. Subsequently, he developed a rare systemic reaction to soy and to an EHF. Case: The patient had an unremarkable medical history and presented with signs and symptoms consistent with anaphylaxis after being fed cow's milk formula for the first time. Symptoms included immediate vomiting, wheezing, stridor, angioedema of eyelids and lips. Although intramuscular epinephrine was given, the patient continued to clinically deteriorate, becoming more lethargic and necessitating admission to the pediatric intensive care unit. Subsequently, a trial of soy formula ingestion reproduced the symptoms and an EHF was given. However, immediately after taking an EHF, he developed facial angioedema and diffuse urticarial lesions. Conclusion: In most patients with a cow's milk allergy, an extensively based formula can be tolerated safely due to a hydrolyzed protein chain. However, medical providers must be vigilant when switching formula because a rare systemic allergic reaction to EHF can still occur.

Application of the Ostrom framework in the analysis of a social-ecological system with multiple resources in a marine protected area.

The framework proposed by Ostrom (2009) has become one of the most utilized tools to address the complexity of social-ecological systems. Most cases use this framework to analyze the systems from the perspective of a single resource unit. However, the livelihoods in several coastal communities are diverse, so that the users interact with multiple common-pool resources, which makes their analysis difficult. In this sense, it is important to identify the key elements of management to achieve the sustainable use of the resources. In this study, we were able to do this in a coastal community where commercial fishing, ecotourism, and recreational fishing coexist. The system of interest, located in the state of Quintana Roo, Mexico, was subdivided by resource type using a multi-method approach to data collection including surveys, interviews, and records review. A conceptual map was developed that shows how the second-tier variables are integrated through the governance and actors with the biophysical system. The actors involved in lobster fishing achieved a more complex governance system, followed by the ecotourism and recreational fishing; the complexity of the governance was related with the equity level of the actors. The analysis revealed the research gaps to develop management strategies and improve the sustainability of the system.

The cost of decompression illness: the case of lobster and sea cucumber fishery in Yucatan, Mexico.

Diving fisheries are an important source of income and protein for many coastal communities around the world. However, these fisheries are also the cause of both fatal and non-fatal injuries. The aim of this study is to estimate the costs of decompression sickness (DCS) in the diving small-scale fisheries that target benthic resources in the Yucatan, Mexico. The DCS cases that occurred during three fishing seasons for sea cucumber (Isostichopus badionotus) and one for spiny lobster (Panulirus argus) were used to calculate the direct medical costs. The catch data during the same fishing seasons were used to calculate the potential losses caused by disability as indirect costs. In the three years (from 2013 to 2016) the total number of fishermen treated in the region numbered 282; 116 during lobster fishing and 166 during sea cucumber season. The direct medical costs were estimated to be USD $120,269; the temporary loss of income in USD $724,377; and the permanent loss of income was USD $737,053. Considering the direct and indirect costs, the social costs of diving in both small-scale fisheries was USD $1,614,121. This is a first approach to estimate the cost of the use of diving in fisheries for the health services but for the fishing communities as well. Furthermore, this is an important first step on the road to a full economic evaluation of the benthic fisheries in order to improve their management.

Assembling the Dead, Gathering the Living: Radiocarbon Dating and Bayesian Modelling for Copper Age Valencina de la Concepción (Seville, Spain).

The great site of Valencina de la Concepción, near Seville in the lower Guadalquivir valley of southwest Spain, is presented in the context of debate about the nature of Copper Age society in southern Iberia as a whole. Many aspects of the layout, use, character and development of Valencina remain unclear, just as there are major unresolved questions about the kind of society represented there and in southern Iberia, from the late fourth to the late third millennium cal BC. This paper discusses 178 radiocarbon dates, from 17 excavated sectors within the c. 450 ha site, making it the best dated in later Iberian prehistory as a whole. Dates are modelled in a Bayesian statistical framework. The resulting formal date estimates provide the basis for both a new epistemological approach to the site and a much more detailed narrative of its development than previously available. Beginning in the 32nd century cal BC, a long-lasting tradition of simple, mainly collective and often successive burial was established at the site. Mud-vaulted tholoi appear to belong to the 29th or 28th centuries cal BC; large stone-vaulted tholoi such as La Pastora appear to date later in the sequence. There is plenty of evidence for a wide range of other activity, but no clear sign of permanent, large-scale residence or public buildings or spaces. Results in general support a model of increasingly competitive but ultimately unstable social relations, through various phases of emergence, social competition, display and hierarchisation, and eventual decline, over a period of c. 900 years.

Bioeconomic modeling for a small-scale sea cucumber fishery in Yucatan, Mexico.

Due to the heavy exploitation of holothurians over the last few decades, it is necessary to implement fishing regulations aimed at reversing this situation. Holothurians require specific regulations that take into account their biology and ecology. Their behavior to group and form patches as a strategy for feeding, defense and reproduction, makes them vulnerable to overfishing. The higher the population density, the higher the catchability coefficient, and because they are sedentary organisms, the catchability does not change significantly until the density is very low. Hence, the stock assessment of holothurians can be improved by analyzing their spatial distribution. This paper proposes a stock assessment technique that considers the spatial distribution pattern of the sea cucumber Isostichopus badionotus from Yucatan, Mexico. A bioeconomic spatial model was developed to explain the interactions between fishing effort allocation, quasi-profits and the population in the short term. Because of the high price of the species and the low production costs, artisanal fishers preferred to maximize short-term quasi-profits, rather than the long-term benefits they could gain with low fishing mortality rates.

Comparing methods of determining Legionella spp. in complex water matrices.

BACKGROUND: Legionella testing conducted at environmental laboratories plays an essential role in assessing the risk of disease transmission associated with water systems. However, drawbacks of culture-based methodology used for Legionella enumeration can have great impact on the results and interpretation which together can lead to underestimation of the actual risk. Up to 20% of the samples analysed by these laboratories produced inconclusive results, making effective risk management impossible. Overgrowth of competing microbiota was reported as an important factor for culture failure. For quantitative polymerase chain reaction (qPCR), the interpretation of the results from the environmental samples still remains a challenge. Inhibitors may cause up to 10% of inconclusive results. This study compared a quantitative method based on immunomagnetic separation (IMS method) with culture and qPCR, as a new approach to routine monitoring of Legionella. RESULTS: First, pilot studies evaluated the recovery and detectability of Legionella spp using an IMS method, in the presence of microbiota and biocides. The IMS method results were not affected by microbiota while culture counts were significantly reduced (1.4 log) or negative in the same samples. Damage by biocides of viable Legionella was detected by the IMS method. Secondly, a total of 65 water samples were assayed by all three techniques (culture, qPCR and the IMS method). Of these, 27 (41.5%) were recorded as positive by at least one test. Legionella spp was detected by culture in 7 (25.9%) of the 27 samples. Eighteen (66.7%) of the 27 samples were positive by the IMS method, thirteen of them reporting counts below 10(3) colony forming units per liter (CFU l(-1)), six presented interfering microbiota and three presented PCR inhibition. Of the 65 water samples, 24 presented interfering microbiota by culture and 8 presented partial or complete inhibition of the PCR reaction. So the rate of inconclusive results of culture and PCR was 36.9 and 12.3%, respectively, without any inconclusive results reported for the IMS method. CONCLUSION: The IMS method generally improved the recovery and detectability of Legionella in environmental matrices, suggesting the possibility to use IMS method as valuable indicator of risk. Thus, this method may significantly improve our knowledge about the exposure risk to these bacteria, allowing us to implement evidence-based monitoring and disinfection strategies.

Use of the SLW index to calculate growth function in the sea cucumber Isostichopus badionotus.

Age and growth analysis is essential to fisheries management. Indirect methods to calculate growth are widely used; however, length frequency data analysis in sea cucumbers is complicated by high data variability caused by body wall elasticity. Here we calculated Isostichopus badionotus parameters of the von Bertalanffy growth function. In order to address bias produced by body wall elasticity, we compared the performance of four measurements and one compound index that combines different biometric parameters: the square root of the length-width product (SLW). Results showed that variability in length data due to body wall elasticity was controlled by using body length (Le) from the SLW compound index. Growth in I. badionotus follows a negative allometric tendency. Slow or zero growth periods were observed during October and November, when weather conditions were adverse.

Carcinoma lobulillar in situ dentro de un fibroadenoma mamario: reporte de dos casos / Situ lobular carcinoma within a fibroadenoma breast: report of two cases

Un carcinoma originado en un fibroadenoma es poco frecuente. El propósito de este reporte es incrementar el conocimiento de esta entidad y disminuir la práctica de diagnósticos con examinación macroscópica del tumor. Nosotros presentamos dos casos de Carcinoma In Situ Lobular originado en un Fibroadenoma beningno.

A carcinoma arising within a Fibroadenoma in an unusual ocurrence. The purpose of this report is to increase to awareness of this entity and to reduce the practica of rendering a diagnosis on gross examination of the tumor. We present two case of an in Situ Lobular Carcinoma within an benign fibroadenoma.

Biopsia core de la mama con guía ecográfica / Biopsy core of the breast with guide ultrasongraphy

La biopsia percutánea de mama con aguja CORE guiada por ultrasonido es una buena alternativa a la biopsia quirúrgica de lesiones mamarias. Es un procedimiento indicado tanto en lesiones como no palpables, ofrece múltiples ventajas y es de buena eficiencia, sin embargo, este procedimiento aún no se constituye como rutina en nuestro medio. El presente trabajo tiene por finalidad exponer y evaluar la experiencia en nuestra institución con el uso de esta técnica en el diagnóstico del cáncer de mama. Desde enero del 2001 hasta mayo del 2003 se han registrado 103 biopsias percutáneas de la mama bajo guía ecográfica, de las cuales, 95 se realizaron con aguja tipo CORE. De estas 95 pacientes, 35 tuvieron. Posteriormente biopsias y/o tratamiento quirúrgico y 60 tuvieron seguimiento clínico. Del primer grupo de 35 pacientes, la biopsia quirúrgica reportó 18 con cáncer de mama y 17 sin evidencia de cáncer. De los 18 diagnósticos de cáncer por cirugía, 15 coincidieron con los diagnosticados por guía ecográfica, en tanto que 3 fueron subdiagnosticados por este método. Los restantes 17, coincidieron en el diagnóstico de no cáncer, con ambos procedimientos. De estos resultados se infiere que los falsos negativos representa el 16,7 por ciento y no presenta falsos positivos, con lo que la sensibilidad fue de 83.3 por ciento, la especificidad de 100 por ciento y el valor predictivo del resultado positivo cáncer fue de 100 por ciento. No se presentaron ningún tipo de complicaciones. En conclusión, nuestra experiencia avala lo ya publicado por numerosos autores y confirma el buen rendimiento de la biopsia de lesiones mamarias con aguja CORE bajo guía ecográfica en el diagnóstico de cáncer de mama y ratifica las bondades de este procedimiento. Se recomienda su uso en nuestro medio.

Manifestaciones cardiacas en pacientes con la infección por el virus de la inmunodeficiencia humana / Cardiac manifestations in patients infected with the human immunodeficiency virus

Introducción. La afección cardíaca por el virus de la inmunodeficiencia humana ha sido poco descrita. Por lo general es producida por agentes oportunistas. El objetivo de este trabajo fue determinar la prevalencia de afección cardíaca en pacientes con la infección por el VIH.Material y Métodos. Pacientes con la infección por el VIH de Mérida, Yucatán, México, a los que se les efectuó un exámen clínico, se les completó un cuestionario con datos clínico-epidemiológico y se les realizó electrocardiograma en reposo, teleradiografía de torax y ecocardiograma. Se determinó la prevalencia de afección cardíaca y se compararon las variables categóricas a través de la X2 y/o la prueba exacta de Fisher. Resultados. De junio de 1996 a mayo de 1999, se estudiaron a 37 sujetos, 3(8 por ciento) mujeres y 34 (92 por ciento) hombres. Veintisiete (73 por ciento) se encontraban en etapa Sida y las alteraciones cardiológicas estuvieron presentes en 13 (48 por ciento) pacientes en etapa Sida y en ninguno en otras etapas de la enfermedad (p<0.001). Las alteraciones más comúnes fueron la miopericarditis 7/13 (54 por ciento) sujetos y la miocarditis sola 3/13 (23 por ciento) pacientes. Discusión. Se confirma la participación cardíaca en la infección por el VIH, observándose una prevalencia global del 48 por ciento. Todos los pacientes que presentaron alteraciones se encontraban en etapa Sida de la enfermedad, lo que confirma que estas alteraciones se presentan en estadios avanzados de la infección.

Marcapasos de doble camara: implantación en un escolar por lesión traumática del tejido de conducción / Doble chamber pace markers: its implantation in a schollboy who suffered a traumatic lesion of the conduction tissue

El avance tecnológico de la estimulación cardíaca artificial en los últimos 15 años ha sido espectacular. El aumento del tiempo de vida de los generadores con la manufactura de las baterías de litio, así como los nuevos electrodo con platino anodizado que reduce su tensión de polarización, aumentando al máximo posible la densidad de la corriente y reduciendo en esta forma los umbrales crónicos de estimulación, marcan una nueva etapa en la estimulación cardíaca artificial. Durante la década actual tres han sido los mayores avances. La estimulación con marcapasos de doble cámara tipo de operación DDD; generadores activados por movimiento muscular, y los prototipos de marcapasos automáticos cardiverter-desfibrilador en fase de prueba. Se presenta el caso de un paciente escolar de 11 años de edad que sufrió lesión traumática del tejido de conducción por disparo accidental con arama de fuego, (perdigón) el cual se alojó en las proximidades de la unión auriculoventricular, presentando cuadros sincopales por la presencia de bloqueo AV completo, a quien se le implantó por vía endocárdica un marcapaso Symbios de doble cámara con el cual se le restituyó a una vida normal, inclusive en cuanto a actividad deportiva se refiere

Prefabricación

Contenido:1.- Introduccion y diseño, Industrializacion, Prefabricacion, Prefabismo, Criterios de clasificacion de los diferentes sistemas de prefabricacion, Grado de prefabricacion, Condiciones previas, tipificacion y normalizacion, Tolerancias dimensionales y de juntas. Capitulo 2.- Diseño basico de una planta de prefabricacion, Tecnologia: moldeo, vibracion, curado, Vapor atmosferico, Almacenaje y transporte, Desmoldeo, Almacenado, Transporte, Montaje Conclusiones.