Clinical Features of Dominant and Recessive Interferon γ Receptor 1 Deficiencies

Background Interferon ? receptor 1 (IFN? R1) deficiency is a primary immunodeficiency with allelic dominant and recessive mutations characterised clinically by severe infections with mycobacteria. We aimed to compare the clinical features of recessive and dominant IFN?R1 deficiencies. Methods We obtained data from a large cohort of patients worldwide. We assessed these people by medical histories, records, and genetic and immunological studies. Data were abstracted onto a standard form. Findings We identified 22 patients with recessive complete IFN?R1 deficiency and 38 with dominant partial deficiency. BCG and environmental mycobacteria were the most frequent pathogens. In recessive patients, 17 (77%) had environmental mycobacterial disease and all nine BCG-vaccinated patients had BCG disease. In dominant patients, 30 (79%) had environmental mycobacterial disease and 11 (73%) of 15 BCG-vaccinated patients had BCG disease. Compared with dominant patients, those with recessive deficiency were younger at onset of first environmental mycobacterial disease (mean 3·1 years [SD 2·5] vs 13·4 years [14·3], p=0·001), had more mycobacterial disease episodes (19 vs 8 per 100 person-years of observation, p=0·0001), had more severe mycobacterial disease (mean number of organs infected by Mycobacterium avium complex 4·1 [SD 0·8] vs 2·0 [1·1], p=0·004), had shorter mean disease-free intervals (1·6 years [SD 1·4] vs 7·2 years [7·6], p

Students perceptions about attention to diversity in Secondary Education Teachers Initial Training

This work aims to analyze the perceptions of students enrolled in the Master's Degree in Secondary Education Teaching, Training and Language Teaching at the University of Jaen, about the initial training received on attention to diversity. A descriptive methodology has been followed using an ad hoc questionnaire as data collection instrument. The results show favorable attitudes of future secondary teachers for diversity, having received an adequate training in curricular and organizational aspects, making it able to fully achieve inclusion of students with special educational needs in the classroom.

¿Barreras invisibles? Actitudes de los estudiantes universitarios ante sus compañeros con discapacidad

El presente trabajo analiza los conocimientos, opiniones y actitudes de los estudiantes universitarios sobre sus compañeros con discapacidad. La integración educativa y social de estos estudiantes es objeto de preocupación y estudio en todo el sistema educativo español, incluida la enseñanza superior en la que 21.942 universitarios con discapacidad se encontraban matriculados en el curso 2013-2014. El estudio se realiza en el marco de dos Proyectos de Innovación Educativa[1] de la Universidad de Málaga en los que se examinaron las actitudes del personal docente e investigador, el alumnado en general y el alumnado con discapacidad de esta institución. En este artículo se recogen las perspectivas de los estudiantes sin discapacidad, necesarias para medir el grado de aceptación dentro de las aulas universitarias. Se empleó una encuesta suministrada mediante correo electrónico a la totalidad de la población de estudiantes de la Universidad de Málaga (N= 32.464). El tamaño de la muestra fue de 620 estudiantes, por lo que trabajamos con un margen de error del 3,9% para la estimación de los porcentajes con un nivel de confianza del 95%. Los datos, analizados mediante análisis estadístico, visibilizan la importancia de un elevado grado de información y conocimiento así como de una experiencia directa con la discapacidad. Ambas circunstancias se traducen en actitudes más positivas y favorables. Tan solo el 20% de los alumnos manifestó haber recibido en alguna ocasión cualquier tipo de información sobre este asunto por parte de la Universidad; no obstante, la mayoría valora positivamente la presencia de alumnos con discapacidad en la Universidad, la posibilidad de contar ellos como compañeros de clase y el enriquecimiento que supone esta convivencia dentro del aula. Se discuten posibles acciones para mejorar las actitudes hacia los estudiantes con discapacidad. Además, se plantea incluir una perspectiva que promueva el empoderamiento de estos estudiantes. [1] PIE 10-135 y PIE 13-103.

Are they adicctied to college students social networks?

The development of the Internet and in particular of social networks has supposedly given a new view to the different aspects that surround human behavior. It includes those associated with addictions, but specifically the ones that have to do with technologies. Following a correlational descriptive design we present the results of a study, which involved university students from Social and Legal Sciences as participants, about their addiction to the Internet and in particular to social networks. The sample was conformed of 373 participants from the cities of Granada, Sevilla, Málaga, and Córdoba. To gather the data a questionnaire that was design by Young was translated to Spanish. The main research objective was to determine if university students could be considered social network addicts. The most prominent result was that the participants don’t consider themselves to be addicted to the Internet or to social networks; in particular women reflected a major distance from the social networks. It’s important to know that the results differ from those found in the literature review, which opens the question, are the participants in a phase of denial towards the addiction?

Isotope Shift in the Dielectronic Recombination of Three-Electron ^ANd⁵⁷⁺

<p>Isotope shifts in dielectronic recombination spectra were studied for Li-like <sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);"><em>A</em></sup>Nd<sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);">57+</sup> ions with <em>A</em>=142 and <em>A</em>=150. From the displacement of resonance positions energy shifts <em>d</em><em>E</em><sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);">142?150</sup>(2<em>s</em>-2<em>p</em><sub style="margin: 0px; padding: 0px; border: 0px; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; line-height: 18px; text-align: justify; background-color: rgb(255, 255, 255);">1/2</sub>)=40.2(3)(6)??meV [(stat)(sys)] and <em>d</em><em>E</em><sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);">142?150</sup>(2<em>s</em>-2<em>p</em><sub style="margin: 0px; padding: 0px; border: 0px; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; line-height: 18px; text-align: justify; background-color: rgb(255, 255, 255);">3/2</sub>)=42.3(12)(20)??meV of 2<em>s</em>-2<em>p</em><sub style="margin: 0px; padding: 0px; border: 0px; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; line-height: 18px; text-align: justify; background-color: rgb(255, 255, 255);"><em>j</em></sub>transitions were deduced. An evaluation of these values within a full QED treatment yields a change in the mean-square charge radius of <sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);">142?150</sup><em>d</em>?<em>r</em><sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);">2</sup>?=-1.36(1)(3)??fm<sup style="margin: 0px; padding: 0px; border: 0px; font-size: 0.88em; vertical-align: 0.5em; line-height: 1em; color: rgb(50, 50, 50); font-family: arial, helvetica, sans-serif; text-align: justify; background-color: rgb(255, 255, 255);">2</sup>. The approach is conceptually new and combines the advantage of a simple atomic structure with high sensitivity to nuclear size.</p>

Los grandes sistemas de abastecimiento de agua en el litoral de Alicante

El litoral de Alicante ha sufrido grandes transformaciones territoriales desde las décadas de 1960 y 1970 a raíz de la actividad turística. Uno de los servicios que necesita esta actividad, entre otros, es el abastecimiento de agua potable. En este sentido, por su papel estratégico, el suministro de agua en alta es decisivo para abastecer a los municipios turístico-residenciales. El objetivo de este estudio es conocer y analizar los diferentes sistemas de abastecimiento de agua en alta en la costa de Alicante, sus características, infraestructuras, recursos hídricos disponibles y medidas de gestión llevados a cabo. Algunas de las conclusiones extraídas son el papel estratégico que ejercen en el territorio, especialmente con el agua, un recurso escaso y de vital importancia para la orla costera alicantina, sobre todo en los meses estivales.

Special issue on design and programming of signal processors for multimedia communication:Special issue on design and programming of signal processors for multimedia communication

The technical challenges in the design and programming of signal processors for multimedia communication are discussed. The development of terminal equipment to meet such demand presents a significant technical challenge, considering that it is highly desirable that the equipment be cost effective, power efficient, versatile, and extensible for future upgrades. The main challenges in the design and programming of signal processors for multimedia communication are, general-purpose signal processor design, application-specific signal processor design, operating systems and programming support and application programming. The size of FFT is programmable so that it can be used for various OFDM-based communication systems, such as digital audio broadcasting (DAB), digital video broadcasting-terrestrial (DVB-T) and digital video broadcasting-handheld (DVB-H). The clustered architecture design and distributed ping-pong register files in the PAC DSP raise new challenges of code generation.

Involvement of Nox2 NADPH oxidase in adverse cardiac remodeling after myocardial infarction.

Oxidative stress plays an important role in the development of cardiac remodeling after myocardial infarction (MI), but the sources of oxidative stress remain unclear. We investigated the role of Nox2-containing reduced nicotinamide-adenine dinucleotide phosphate oxidase in the development of cardiac remodeling after MI. Adult Nox2(-/-) and matched wild-type (WT) mice were subjected to coronary artery ligation and studied 4 weeks later. Infarct size after MI was similar in Nox2(-/-) and WT mice. Nox2(-/-) mice exhibited significantly less left ventricular (LV) cavity dilatation and dysfunction after MI than WT mice (eg, echocardiographic LV end-diastolic volume: 75.7+/-5.8 versus 112.4+/-12.3 microL; ejection fraction: 41.6+/-3.7 versus 32.9+/-3.2%; both P

WASP-14b: 7.3 M-J transiting planet in an eccentric orbit

We report the discovery of a 7.3 M-J exoplanet WASP-14b, one of the most massive transiting exoplanets observed to date. The planet orbits the 10th-magnitude F5V star USNO-B1 11118-0262485 with a period of 2.243 752 d and orbital eccentricity e = 0.09. A simultaneous fit of the transit light curve and radial velocity measurements yields a planetary mass of 7.3 +/- 0.5 M-J and a radius of 1.28 +/- 0.08 R-J. This leads to a mean density of about 4.6 g cm(-3) making it the densest transiting exoplanets yet found at an orbital period less than 3 d. We estimate this system to be at a distance of 160 +/- 20 pc. Spectral analysis of the host star reveals a temperature of 6475 +/- 100 K, log g = 4.07 cm s(-2) and v sin i = 4.9 +/- 1.0 km s(-1), and also a high lithium abundance, log N(Li) = 2.84 +/- 0.05. The stellar density, effective temperature and rotation rate suggest an age for the system of about 0.5-1.0 Gyr.

Inward rectifier potassium channels in the HL-1 cardiomyocyte-derived cell line.

HL-1 is a line of immortalized cells of cardiomyocyte origin that are a useful complement to native cardiomyocytes in studies of cardiac gene regulation. Several types of ion channel have been identified in these cells, but not the physiologically important inward rectifier K(+) channels. Our aim was to identify and characterize inward rectifier K(+) channels in HL-1 cells. External Ba(2+) (100?µM) inhibited 44?±?0.05% (mean?±?s.e.m., n?=?11) of inward current in whole-cell patch-clamp recordings. The reversal potential of the Ba(2+)-sensitive current shifted with external [K(+)] as expected for K(+)-selective channels. The slope conductance of the inward Ba(2+)-sensitive current increased with external [K(+)]. The apparent Kd for Ba(2+) was voltage dependent, ranging from 15?µM at -150 ?mV to 148?µM at -75 ?mV in 120 ?mM external K(+). This current was insensitive to 10?µM glybenclamide. A component of whole-cell current was sensitive to 150?µM 4,4'-diisothiocyanatostilbene-2,2'-disulfonic acid (DIDS), although it did not correspond to the Ba(2+)-sensitive component. The effect of external 1 mM Cs(+) was similar to that of Ba(2+). Polymerase chain reaction using HL-1 cDNA as template and primers specific for the cardiac inward rectifier K(ir)2.1 produced a fragment of the expected size that was confirmed to be K(ir)2.1 by DNA sequencing. In conclusion, HL-1 cells express a current that is characteristic of cardiac inward rectifier K(+) channels, and express K(ir)2.1 mRNA. This cell line may have use as a system for studying inward rectifier gene regulation in a cardiomyocyte phenotype.