Fanconi syndrome with lysinuric protein intolerance

We present the case of a 9-year-old child with lysinuric protein intolerance and Fanconi syndrome. She was referred to our hospital with a persistent metabolic acidosis and polyuria. Renal investigations revealed all laboratory signs of Fanconi syndrome, with glucosuria, generalized aminoaciduria, phosphaturia and severe hypercalciuria. The diagnosis of Fanconi syndrome was confirmed by a renal biopsy that showed extensive lesions of proximal tubular epithelial cells with vacuolation of these cells and a sloughing of the brush border

an spm po based polarimetrie two scale model

AbstractA polarimetric two-scale scattering model employed to retrieve the surface parameters of bare soils from polarimetric SAR data is presented. The scattering surface is here considered as composed of randomly tilted rough facets, for which the SPM or the PO hold. The facet random tilt causes a random variation of the local incidence angle, and a random rotation of the local incidence plane around the line-of-sight, which in turn causes a random rotation of the facet scattering matrix. Unlike other similar already existing approaches, our method considers both these effects. The proposed scattering model is then used to retrieve bare soil moisture and large-scale roughness from the co-polarized and cross-polarized ratios

An analysis of feature relevance in the classification of astronomical transients with machine learning methods

The exploitation of present and future synoptic (multi-band and multi-epoch) surveys requires an extensive use of automatic methods for data processing and data interpretation. In this work, using data extracted from the Catalina Real Time Transient Survey (CRTS), we investigate the classification performance of some well tested methods: Random Forest, MLPQNA (Multi Layer Perceptron with Quasi Newton Algorithm) and K-Nearest Neighbors, paying special attention to the feature selection phase. In order to do so, several classification experiments were performed. Namely: identification of cataclysmic variables, separation between galactic and extra-galactic objects and identification of supernovae.Comment: Accepted by MNRAS, 11 figures, 18 page

Randomized Controlled Trials on Renin Angiotensin Aldosterone System Inhibitors in Chronic Kidney Disease Stages 3–5: Are They Robust? A Fragility Index Analysis

Inhibition of the renin-angiotensin-aldosterone system (RAAS) is broadly recommended in many nephrological guidelines to prevent chronic kidney disease (CKD) progression. This work aimed to analyze the robustness of randomized controlled trials (RCTs) investigating the renal and cardiovascular outcomes in CKD stages 3–5 patients treated with RAAS inhibitors (RAASi). We searched for RCTs in MEDLINE (PubMed), EMBASE databases, and the Cochrane register. Fragility indexes (FIs) for every primary and secondary outcome were calculated according to Walsh et al., who first described this novel metric, suggesting 8 as the cut-off to consider a study robust. Spearman coefficient was calculated to correlate FI to p value and sample size of statistically significant primary and secondary outcomes. Twenty-two studies met the inclusion criteria, including 80,455 patients. Sample size considerably varied among the studies (median: 1693.5, range: 73–17,276). The median follow-up was 38 months (range 24–58). The overall median of both primary and secondary outcomes was 0 (range 0–117 and range 0–55, respectively). The median of FI for primary and secondary outcomes with a p value lower than 0.05 was 6 (range: 1–117) and 7.5 (range: 1–55), respectively. The medians of the FI for primary outcomes with a p value lower than 0.05 in CKD and no CKD patients were 5.5 (range 1–117) and 22 (range 1–80), respectively. Only a few RCTs have been shown to be robust. Our analysis underlined the need for further research with appropriate sample sizes and study design to explore the real potentialities of RAASi in the progression of CKD