Revisiting human IL-12Rβ1 deficiency: A survey of 141 patients from 30 countries

Interleukin-12 receptor β1 (IL-12Rβ1) deficiency is the most common form of Mendelian susceptibility to mycobacterial disease (MSMD). We undertook an international survey of 141 patients from 102 kindreds in 30 countries. Among 102 probands, the first infection occurred at a mean age of 2.4 years. In 78 patients, this infection was caused by Bacille Calmette-Guérin (BCG; n = 65), environmental mycobacteria (EM; also known as atypical or nontuberculous mycobacteria) (n = 9) or Mycobacterium tuberculosis (n = 4). Twenty-two of the remaining 24 probands initially presented with nontyphoidal, extraintestinal salmonellosis. Twenty of the 29 genetically affected sibs displayed clinical signs (69%); however 8 remained asymptomatic (27%). Nine nongenotyped sibs with symptoms died. Recurrent BCG infection was diagnosed in 15 cases, recurrent EM in 3 cases, recurrent salmonellosis in 22 patients. Ninety of the 132 symptomatic patients had infections with a single microorganism. Multiple infections were diagnosed in 40 cases, with combined mycobacteriosis and salmonellosis in 36 individuals. BCG disease strongly protected against subsequent EM disease (p = 0.00008). Various other infectious diseases occurred, albeit each rarely, yet candidiasis was reported in 33 of the patients (23%). Ninety-nine patients (70%) survived, with a mean age at last follow-up visit of 12.7 years ± 9.8 years (range, 0.5-46.4 yr). IL-12Rβ1 deficiency is characterized by childhood-onset mycobacteriosis and salmonellosis, rare recurrences of mycobacterial disease, and more frequent recurrence of salmonellosis. The condition has higher clinical penetrance, broader susceptibility to infections, and less favorable outcome than previously thought. © 2010 Lippincott Williams & Wilkins.

Bases ecofisiológicas de la ganancia genética en el rendimiento del maíz en la Argentina en los últimos 30 años

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The introduction of social adaptation within evacuation modelling

In recent history, a number of tragic events have borne a consistent message; the social structures that existed prior to and during the evacuation significantly affected the decisions made and the actions adopted by the evacuating population in response to the emergency. This type of influence over behaviour has long been neglected in the modelling community. This paper is an attempt to introduce some of these considerations into evacuation models and to demonstrate their impact. To represent this type of behaviour within evacuation models a mechanism to represent the membership and position within social hierarchies is established. In addition, individuals within the social groupings are given the capacity to communicate relevant pieces of data such as the need to evacuate—impacting the response time—and the location of viable exits—impacting route selection. Furthermore, the perception and response to this information is also affected by the social circumstances in which individuals find themselves. Copyright © 2005 John Wiley & Sons, Ltd.

Ambitious mothers – successful daughters: Mothers’ early expectations for children’s education and children’s earnings and sense of control in adult life

Background. Mothers' expectations for their children's educational attainment are related to children's educational and occupational attainment. Studies have yet to establish, however, the long-term links between maternal expectations and offspring earnings, which are not always related to occupational attainment especially in women, or between maternal expectations and offspring sense of control and self-efficacy, which are pivotal factors in career choice and development. Aims. To explore the role of mothers' expectations for their children's educational attainment in children's earnings attainment and sense of control later in life. Method. Data from sweeps of the 1970 British Cohort Study (BCS70) were used. The study sample was those cohort members with complete information on all the variables of interest. The study sample (N = 3,285) was more educated and less disadvantaged than the whole sample. If cohort members of this type are more likely to have a mother who has high expectations, then our results are biased downwards, which suggests that we underestimate the effect of expectations on our two outcome variables. Results. Mothers' expectations at the age of 10 were positively related to daughters' sense of control at the age of 30 even after controlling for ethnicity, educational attainment, and concurrent partner, parent, and labour market participation status, as well as the following confounding variables (measured at the ages of 0–10): general ability and general ability squared, locus of control, emotional and behavioural problems and emotional and behavioural problems squared, socio-economic disadvantage, parental social class, parental family structure, and mothers' education, child-rearing attitudes, and mental health. Mothers' expectations had no effect on sons' adult outcomes. Conclusions. Given that women are particularly at risk for poor psychological and economic outcomes in adulthood, and that this study likely underestimated the effect of expectations on these two outcomes, this is an important conclusion.

Biomonitoring of air pollutants by using lichens (Evernia prunastri) in areas between Kenitra and Mohammedia cities in Morocco.

In this study, Evernia prunastri, a lichen growing in its natural habitat in Morocco was analysed for the concentration of five heavy metals (Fe, Pb, Zn, Cu and Cr) from eleven sites between Kenitra and Mohammedia cities. The control site was Dar Essalam, an isolated area with low traffic density and dense vegetation. In the investigated areas, the concentration of heavy metals was correlated with vehicular traffic, industrial activity and urbanization. The total metal concentration was highest in Sidi Yahya, followed by Mohammedia and Bouznika. The coefficient of variation was higher for Pb and lower for Cu, Zn and Fe. The concentrations of most heavy metals in the thalli differed significantly between sites (p<0.01). Principal component analysis (PCA) revealed a significant correlation between heavy metal accumulation and atmospheric purity index. This study demonstrated also that the factors most strongly affecting the lichen flora were traffic density, the petroleum industry and paper factories in these areas. Overall, these results suggest that the index of atmospheric purity and assessment of heavy metals in lichen thalli are good indicators of the air quality at the studied sites.

Modelos de desarrollo, unificación y “desterritorialización”.

El presente estudio tiene el objetivo de ofrecer una visión general sobre los Modelos de Desarrollo y Organización Territorial desde la perspectiva de las relaciones entre el medio urbano, “rururbano” y rural, en España. Para ello, tras conocer y valorar los enfoque conceptuales y temáticos, se estudia el crecimiento urbano en nuestro país en las últimas décadas, analizando de manera pormenorizada la importancia que ha cobrado y cobra la aplicación legislativa de leyes, planes y normas, tanto en el propio crecimiento urbano como en la demanda de viviendas en las ciudades españolas y, de igual modo, la vinculación de ambas con el precio de las viviendas, relacionándolo con la problemática de la “rururbanización”, y del medio rural.

Polycyclic aromatic hydrocarbons (PAHS) (I): Measures of prevention and control

Measures of prevention and control against polycyclic aromatic hydrocarbons (PAHs) focus on an official food control, a code of best practice to reduce PAHs levels by controlling industry and in the development of a chemopreventive strategy. Regulation (EU) 835/2011 establishes maximum levels of PAHs for each food group. In addition, Regulations (EU) 333/2007 and 836/2011 set up the methods of sampling and analysis for its official control. Scientific studies prove that the chemopreventive strategy is effective against these genotoxic compounds effects. Most chemopreventive compounds studied with proven protective effects against PAHs are found in fruit and vegetables.

Variaciones termométricas en la planta del pie y piernas valorada en corredores antes y después de correr 30 km

La termometría es una técnica no invasiva que permite cuantificar los cambios en la temperatura cutánea y evaluarla de forma cuantitativa. El aumento significativo de la temperatura puede indicar la existencia de patología. Se ha demostrado que la actividad muscular induce procesos de transferencia de calor entre los músculos y las capas superficiales de tejido. En este estudio queremos cuantificar los cambios de temperatura que se producen en los músculos del pie y miembro inferior tras una carrera de 30 km, para ello hemos utilizado una cámara termográfica de alta resolución. Contamos con la colaboración voluntaria de 32 sujetos sanos a los que procedimos a tomar fotografías de la planta del pie, parte anterior de la pierna, parte posterior de la pierna, parte anterior del muslo y parte posterior del muslo en dos etapas, primero antes de la carrera y segunda toma después de la carrera de 30 km, de esta manera pudimos valorar si había o no variación de temperatura en las zonas seleccionadas. Tras el análisis de los datos obtenidos encontramos significativas variaciones térmicas en Talón, cabeza primer metatarsiano, cabeza segundo metatarsiano, cabeza tercer metatarsiano, cabeza cuarto metatarsiano, cabeza quinto metatarsiano, apófisis estiloides quinto metatarsiano, arco longitudinal interno, maléolo interno, maléolo externo, peroneo lateral largo, vasto interno, vasto externo, recto femoral, tensor de la fascia lata, inserción cuádriceps, gemelo interno, tendón de Aquiles y Biceps femoral.

Communal larval rearing of European lobster (Homarus gammarus): family identification by microsatellite DNA profiling an offspring fitness.

Stock enhancement experiments of European lobster (Homarus gammarus) have been carried out around the Kvitsoy Islands in south-western Norway since 1990. In addition to releases of coded wire tagged lobster juveniles (cultured) and subsequent monitoring of commercial fishery, a lobster hatchery was established in 1997. Several experiments were made on the communal-rearing approach where the performance of mixed larval groups (families) was evaluated under identical conditions. Berried females of wild and cultured origin and their respective fertilised eggs were screened by using microsatellite DNA profiling involving a multiplex set of six lobster specific primers, thereby allowing determination of both parental genotypes. Each female were kept separately during hatching, and the offspring were later mixed and raised in a communal rearing system. The early-larval survival was estimated at stage IV (bottom stage), and the survivors were identified to family and group by microsatellite profiling. Five different communal experiments were conducted, representing offspring from 65 berried females. Of the surviving larvae, 6.3% could not be assigned to family due to degraded DNA and no PCR amplification. Significant differences in early survival between offspring of wild and cultured origin were found in the experiments. No differences between the groups were found in stage IV larval size. Based on the pooled data on survival (as a measure of early larvae fitness) offspring of cultured females displayed a relative fitness of 60% in comparison to offspring from wild females. Large variation in survival was also observed among families within the wild and cultured groups, suggesting a genetic component for these traits and a potential for selective breeding.