The WINGS Survey: a progress report

A two-band (B and V) wide-field imaging survey of a complete, all-sky X-ray selected sample of 78 clusters in the redshift range z=0.04-0.07 is presented. The aim of this survey is to provide the astronomical community with a complete set of homogeneous, CCD-based surface photometry and morphological data of nearby cluster galaxies located within 1.5 Mpc from the cluster center. The data collection has been completed in seven observing runs at the INT and ESO-2.2m telescopes. For each cluster, photometric data of about 2500 galaxies (down to V~23) and detailed morphological information of about 600 galaxies (down to V~21) are obtained by using specially designed automatic tools. As a natural follow up of the photometric survey, we also illustrate a long term spectroscopic program we are carrying out with the WHT-WYFFOS and AAT-2dF multifiber spectrographs. Star formation rates and histories, as well as metallicity estimates will be derived for about 350 galaxies per cluster from the line indices and equivalent widths measurements, allowing us to explore the link between the spectral properties and the morphological evolution in high- to low-density environments, and across a wide range in cluster X-ray luminosities and optical properties.Comment: 12 pages, 10 eps figures, Proceedings of the SAIt Conference 200

Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack

Objective: Pheochromocytomas are a hallmark feature of von Hippel–Lindau disease (vHL). To our knowledge, this is the first systematic review with meta-analysis evaluating the frequency of pheochromocytomas and/or paragangliomas (PPGLs) in patients with vHL, as well as among patients with different vHL subtypes. Design: Systematic review with meta-analysis. Methods: We searched on MEDLINE, Scopus, and Web of Science. We included primary studies assessing participants with vHL and reporting on the frequency of PPGL. We performed random-effects meta-analysis to quantitatively assess the frequency of PPGL, followed by meta-regression and subgroup analysis. Risk of bias analysis was performed to assess primary studies’ methodological quality. Results: We included 80 primary studies. In 4263 patients with vHL, the pooled frequency of PPGL was 19.4% (95% CI = 15.9–23.6%, I2 = 86.1%). The frequency increased to 60.0% in patients with vHL type 2 (95% CI = 53.4–66.3%, I2 = 54.6%) and was determined to be of 58.2% in patients with vHL type 2A (95% CI = 49.7–66.3%, I2 = 36.2%), compared to 49.8% in vHL type 2B (95% CI = 39.9–59.7%, I2 = 42.7%), and 84.1% in vHL type 2C (95% CI = 75.1–93.1%, I2 = 0%). In meta-regression analysis, more recent studies were associated with a higher frequency of PPGL. All studies had at least one internal validity item classified as 'high risk of bias,' with 13% studies having low risk of bias in all external validity items. Conclusions: PPGLs are a common manifestation of vHL. Despite methodological limitations and differences across primary studies, our results point to the importance of PPGL screening in patients with vHL

The complexities in genotyping of congenital adrenal hyperplasia: 21-hydroxylase deficiency

The deficiency of 21-hydroxylase due to CYP21A2 pathogenic variants is a rather frequent disease with serious consequences, going from a real mortality risk to infertility and to milder symptoms, nevertheless important for affecting the patients’ self-esteem. In the most severe cases life-threatening adrenal salt wasting crises may occur. Significant morbidity including the possibility of mistaken gender determination, precocious puberty, infertility and growth arrest with consequent short stature may also affect these patients. In the less severe cases milder symptoms like hirsutism will likely affect the image of the self with strong psychological consequences. Its diagnosis is confirmed by 17OH-progesterone dosages exceeding the cut-off value of 10/15 ng/ml but genotyping is progressively assuming an essential role in the study of these patients particularly in confirming difficult cases, determining some aspects of the prognosis and allowing a correct genetic counseling. Genotyping is a difficult process due to the occurrence of both a gene and a highly homologous pseudo gene. However, new tools are opening new possibilities to this analysis and improving the chances of a correct diagnosis and better understanding of the underlying mechanisms of the disease. Beyond the 10 classic pathogenic variants usually searched for in most laboratories, a correct analysis of 21OH-deficiency cases implies completely sequencing of the entire gene and the determination of gene duplications. These are now recognized to occur frequently and can be responsible for some false positive cases. And finally, because gene conversions can include several pathogenic variants one cannot be certain of identifying that both alleles are affected without studying parental DNA samples. A complete genotype characterization should be considered essential in the preparation for pregnancy, even in the case of parents with milder forms of the disease, or even just carriers, since it has been reported that giving birth to progeny with the severe classic forms occurs with a much higher frequency than expected.The work was supported by the Foundation for Science and Technology (FCT) (PTDC/MEC-ONC/31384/2017). SG was funded through FCT grant SFRH/BPD/1117441/2015

Testing the three-dimensional IRI-SIRMUP-P mapping of the ionosphere for disturbed periods

This paper describes the three-dimensional (3-D) electron density mapping of the ionosphere given as output by the assimilative IRI-SIRMUP-P (ISP) model for three different geomagnetic storms. Results of the 3-D model are shown by comparing the electron density profiles given by the model with the ones measured at two testing ionospheric stations: Roquetes (40.8 °N,0.5 °E), Spain, and San Vito (40.6°N,17.8 °E), Italy. The reference ionospheric stations from which the autoscaled foF2 and M(3000)F2 data as well as the real-time vertical electron density profiles are assimilated by the ISP model are those of El Arenosillo (37.1 °N,353.3° E), Spain, Rome (41.8 °N,12.5 °E), and Gibilmanna (37.9° N,14.0 °E), Italy. Overall, the representation of the ionosphere made by the ISP model is better than the climatological representation made by only the IRI-URSI and the IRI-CCIR models. However, there are few cases for which the assimilation of the autoscaled data from the reference stations causes either a strong underestimation or a strong overestimation of the real conditions of the ionosphere, which is in these cases better represented by only the IRI-URSI model. This ISP misrepresentation is mainly due to the fact that the reference ionospheric stations covering the region mapped by the model turn out to be few, especially for disturbed periods when the ionosphere is very variable both in time and in space and hence a larger number of stations would be required. The inclusion of new additional reference ionospheric stations could surely smooth out this concern

Importance of a real-time monitoring of the Earth's ionosphere

The ionosphere affects the electromagnetic wave propagation and then its study is important for Earth-Earth, satellite-Earth, and satellite-satellite communication purposes. Diffractive and refractive processes due to irregular electron density structures cause signal fluctuations that can disrupt satellite-ground communications and represent a hazard for navigation systems. The study and the real-time monitoring of the ionosphere are important for Space Weather purposes. The ionospheric vertical sounding is described, together with the automatic scaling of the ionograms.UnpublishedRome2A. Fisica dell'alta atmosferaope

Nutrition rehabilitation of undernourished children utilizing Spiruline and Misola

BACKGROUND: Malnutrition constitutes a public health problem throughout the world and particularly in developing countries. AIMS: The objective of the study is to assess the impact of an elementary integrator composed of Spiruline (Spirulina platensis) and Misola (millet, soja, peanut) produced at the Centre Medical St Camille (CMSC) of Ouagadougou, Burkina Faso, on the nutritional status of undernourished children. MATERIALS AND METHODS: 550 undernourished children of less than 5 years old were enrolled in this study, 455 showed severe marasma, 57 marasma of medium severity and 38 kwashiorkor plus marasma. We divided the children randomly into four groups: 170 were given Misola (731 ± 7 kcal/day), 170 were given Spiruline plus traditional meals (748 ± 6 kcal/day), 170 were given Spiruline plus Misola (767 ± 5 kcal/day). Forty children received only traditional meals (722 ± 8 kcal/day) and functioned as the control group. The duration of this study was eight weeks. RESULTS AND DISCUSSION: Anthropometrics and haematological parameters allowed us to appreciate both the nutritional and biological evolution of these children. The rehabilitation with Spiruline plus Misola (this association gave an energy intake of 767 ± 5 kcal/day with a protein assumption of 33.3 ± 1.2 g a day), both greater than Misola or Spiruline alone, seems to correct weight loss more quickly. CONCLUSION: Our results indicate that Misola, Spiruline plus traditional meals or Spiruline plus Misola are all a good food supplement for undernourished children, but the rehabilitation by Spiruline plus Misola seems synergically favour the nutrition rehabilitation better than the simple addition of protein and energy intake