METS-Based Cataloging Toolkit for Digital Library Management System

This toolkit is designed for the Digital Library Management System of Tsinghua University (TH-DLMS). The aim of TH-DLMS is to build up a platform to preserve various kinds of digitalized resources, manage distributed repositories and provide kinds of service for research and education. This toolkit fulfills the cataloging and preservation functions of TH-DLMS. METS (Metadata Encoding and T ransmission Standard) encoded documents are used as the final storage format of metadata, including descriptive metadata, structural metadata and administrative metadata, and submitted to a management system based on Fedora (Flexible Extensible Digital Object and Repository Architecture)

Accuracy of 2D and point shear wave elastography-based measurements for diagnosis of esophageal varices: a systematic review and meta-analysis

PURPOSEThe aim of this meta-analysis is to summarize the diagnostic accuracies of point shear wave elas- tography (pSWE) and two-dimensional (2D) SWE for esophageal varices (EV) and varices needing treatment (VNT).METHODSWe conducted a systematic review and meta-analysis of diagnostic accuracy studies. We searched for studies reporting the EV and VNT diagnostic accuracy of pSWE and 2D SWE using PubMed Cen- tral, SCOPUS, MEDLINE, Embase, and Cochrane databases. STATA software“Midas”package was used for meta-analysis.RESULTSA total of 24 studies with 3867 patients were included in the review. Pooled score sensitivities of pSWE were 91% (95% CI, 80%-96%) for EV, and 94% (95% CI, 86%-97%) for VNT. Pooled score sensi- tivities of 2D SWE were 78% (95% CI, 69%-85%) for EV, and 79% (95% CI, 72%-85%) for VNT. Pooled score specificities of pSWE were 70% (95% CI, 60%-78%) for EV, and 59% (95% CI, 40%-75%) for VNT. Pooled score specificities of 2D SWE for EV were 79% (95% CI, 72%-85%) 72% (95% CI, 66%-77%) for VNT. We found significant heterogeneity for all the elastography-based measurements with the chi- square test results and an I2 statistic >75%.CONCLUSIONBoth pSWE and 2D SWE can diagnose EV and VNT with moderate diagnostic accuracy. Further large- scale setting-specific longitudinal studies are required to establish the best modality

A pathogenic UFSP2 variant in an autosomal recessive form of pediatric neurodevelopmental anomalies and epilepsy

Purpose: Neurodevelopmental disabilities are common and genetically heterogeneous. We identified a homozygous variant in the gene encoding UFM1-specific peptidase 2 (UFSP2), which participates in the UFMylation pathway of protein modification. UFSP2 variants are implicated in autosomal dominant skeletal dysplasias, but not neurodevelopmental disorders. Homozygosity for the variant occurred in eight children from four South Asian families with neurodevelopmental delay and epilepsy. We describe the clinical consequences of this variant and its effect on UFMylation.Methods: Exome sequencing was used to detect potentially pathogenic variants and identify shared regions of homozygosity. Immunoblotting assessed protein expression and post-translational modifications in patient-derived fibroblasts.Results: The variant (c.344T\u3eA; p.V115E) is rare and alters a conserved residue in UFSP2. Immunoblotting in patient-derived fibroblasts revealed reduced UFSP2 abundance and increased abundance of UFMylated targets, indicating the variant may impair de-UFMylation rather than UFMylation. Reconstituting patient-derived fibroblasts with wild-type UFSP2 reduced UFMylation marks. Analysis of UFSP2\u27s structure indicated that variants observed in skeletal disorders localize to the catalytic domain, whereas V115 resides in an N-terminal domain possibly involved in substrate binding.Conclusion: Different UFSP2 variants cause markedly different diseases, with homozygosity for V115E causing a severe syndrome of neurodevelopmental disability and epilepsy

Preparation and Application of Starch/Polyvinyl Alcohol/Citric Acid Ternary Blend Antimicrobial Functional Food Packaging Films

Ternary blend films were prepared with different ratios of starch/polyvinyl alcohol (PVA)/citric acid. The films were characterized by field emission scanning electron microscopy (FE-SEM), thermogravimetric analysis, as well as Fourier transform infrared (FTIR) analysis. The influence of different ratios of starch/polyvinyl alcohol (PVA)/citric acid and different drying times on the performance properties, transparency, tensile strength (TS), water vapor permeability (WVP), water solubility (WS), color difference (ΔE), and antimicrobial activity of the ternary blends films were investigated. The starch/polyvinyl alcohol/citric acid (S/P/C1:1:0, S/P/C3:1:0.08, and S/P/C3:3:0.08) films were all highly transparent. The S/P/C3:3:0.08 had a 54.31 times water-holding capacity of its own weight and its mechanical tensile strength was 46.45 MPa. In addition, its surface had good uniformity and compactness. The S/P/C3:1:0.08 and S/P/C3:3:0.08 showed strong antimicrobial activity to Listeria monocytogenes and Escherichia coli, which were the food-borne pathogenic bacteria used. The freshness test results of fresh figs showed that all of the blends prevented the formation of condensed water on the surface of the film, and the S/P/C3:1:0.08 and S/P/C3:3:0.08 prevented the deterioration of figs during storage. The films can be used as an active food packaging system due to their strong antibacterial effect