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Background . Characteristics of X-linked Alport syndrome (XLAS) in a cohort of Chinese children. Methods . This work is a retrospective study covering the clinical information, pathological data, and gene sequencing results of 32 cases with XLAS from 2011 to 2022. Results . Among these 32 patients, the youngest age of onset was 3 months. Renal biopsy was performed on 29 children. The lamellated glomerular basement membrane was observed in 19 children using electron microscopy (65.5%). Of the 26 samples tested, 73.1% were found to be negative for collagen-a5 under immunohistochemical staining, showing clinical significance. Next-generation sequencing (NGS) detected 27 pathogenic gene mutations. A total of 15.4% of patients carried de novo mutations. Conclusions . The boys with XLAS showed more typical pathological performance than the girls. Patients with severe mutation were more likely to have proteinuria and hearing impairment. Renal pathology combined with NSG is an important means of diagnosis of AS.

Objective： Optimize the preparation process of salt-soluble porcine liver protein and improve the utilization rate of porcine liver by-products. Methods： Taking the extraction rate as the index, the effects of NaCl concentration, pH, extraction time and solid-liquid ratio on the extraction rate of SSLP were analyzed, and the optimal preparation process was determined by orthogonal rotation optimization test. Taking water soluble liver proteins （WSLP） as the control, the functional characteristics （solubility, foaming, foam stability, emulsification, emulsification stability, etc.） of SSLP were compared and analyzed, and Fourier transform infrared spectroscopy （FT-IR） was used, the structural changes were analyzed by low field nuclear magnetic resonance （LF-NMR） and differential scanning calorimetry （DSC）. Results： The optimum extraction process of SSLP was NaCl concentration 0.4 mol/L, pH 8, extraction time 12.95 h, solid-liquid ratio 1∶4.6 (g/mL), and the extraction rate was 10.60%. FT-IR analysis showed that the absorption peak of characteristic functional groups of SSLP was obvious and in the secondary structure β-The relative content of corner was 39.91%, α-The relative content of helix was 13.55%, β-The relative content of folding was 21.32%, and the relative content of random curl was 25.23%; LF-NMR analysis showed that SSLP had higher bound water and higher water retention; DSC results showed that the denaturation temperature of SSLP was 78.68 ℃. Conclusion： SSLP prepared under optimal process conditions has better solubility, foaming, emulsification and emulsification stability than WSLP, which is a high-quality source for the development of pig liver protein products.

Inpatient hyperglycemia is common in clinical practice, and is associated with various in-hospital adverse outcomes, increased mortality in critically ill patients, and prolonged hospital stay in non-critically ill patients. Currently, guidelines in endocrinology and metabolism at home and abroad all recommend systematic management of inpatient hyperglycemia, and highlight the principle of individualized management, namely, recommend that glycemic targets should be set individualized based on the patient's individual characteristics. We reviewed the definition, management status, glycemic targets and monitoring methods of inpatient hyperglycemia in worldwide guidelines, and discussed the advances in inpatient hyperglycemia management from a perspective of comparing the similarities and differences in glycemic targets set for different types of inpatient populations and for inpatients with different conditions. This review clearly indicates that the inpatient management of hyperglycemia still needs to adhere to the principle of individualized management, thereby reducing the incidence of complications and all-cause mortality related to hyperglycemia.

C27H26N9O10Pr, monoclinic, P21/c, a = 13.2280(12) Å, b = 5.5628(5) Å, c = 41.140(3) Å, β = 105.109(2)°, V = 2922.6(4) Å3, Z = 4, Rgt (F) = 0.0426, wRref (F 2) = 0.0803, T = 298.15 K.

Context.--Neutralizing antibody detection can assess the incidence of COVID-19 and the effectiveness of vaccines. However, commercial reagents for neutralizing antibodies were developed after the anti-SARS-CoV-2 immunoglobulin (Ig) G and IgM antibodies. Therefore, some laboratories did not perform neutralizing antibody testing services because of multiple factors. Objective.--To find a fast, accurate, and economic alternative for the detection of neutralizing antibodies for the development of COVID-19 screening programs. Design.--The response and correlation of 3 antibodies (anti-spike protein neutralizing antibody, total anti-receptor-binding domain [RBD] antibody, and anti-RBD IgG) were determined by observing the dynamics in 61 participants for 160 days after vaccination. Results.--The levels of neutralizing and anti-RBD IgG antibodies reached their peak values on day 42 after vaccination (120.75 IU/mL and 14.38 signal-to-cutoff ratio [S/CO], respectively). The total antibody levels peaked at 138.47 S/CO on day 35 after vaccination. The strongest correlation was found between neutralizing and anti-RBD IgG antibody levels (r = 0.894, P Conclusions.--Neutralizing and anti-RBD IgG antibody levels were strongly correlated, and thus anti-RBD IgG antibody levels can be used for the accurate assessment of immunity following SARS-CoV-2 infection or vaccination., SARS-CoV-2 is a highly transmissible and pathogenic virus that has caused serious harm to human health and public safety during the ongoing COVID-19 global pandemic. (1,2) Global COVID-19 cases now [...]

The genus Xylaria comprises a diverse group of fungi with a global distribution and significant ecological importance, known for being a source of bioactive secondary metabolites with antibacterial, antioxidative, anticarcinogenic, and additional properties. In this study, we present a comprehensive taxonomic revision of the species of Xylaria found in some parts of southern China, characterized by an extensive multilocus phylogeny analysis based on internal transcribed spacer (ITS), TUB2 (β‐tubulin), and DNA‐directed RNA polymerase II subunit 2 (rpb2) gene regions. Morphological examination and detailed comparative analyses of the collected specimens were conducted to determine the distinctiveness of each species. The multilocus phylogeny approach allowed us to infer evolutionary relationships and assess species boundaries accurately, leading to the identification of 40 novel Xylaria species hitherto unknown to science. The newly described species are: X. baoshanensis, X. bawanglingensis, X. botryoidalis, X. dadugangensis, X. doupengshanensis, X. fanglanii, X. glaucae, X. guizhouensis, X. japonica, X. jinghongensis, X. jinshanensis, X. kuankuoshuiensis, X. liboensis, X. negundinis, X. orbiculati, X. ovata, X. pseudoanisopleura, X. pseudocubensis, X. pseudobambusicola, X. pseudoglobosa, X. pseudohemisphaerica, X. pseudohypoxylon, X. puerensis, X. qianensis, X. qiongzhouensis, X. rhombostroma, X. serratifoliae, X. shishangensis, X. shuqunii, X. shuangjiangensis, X. sinensis, X. tongrenensis, X. umbellata, X. xishuiensis, X. yaorenshanensis, X. yinggelingensis, X. yumingii, X. yunnanensis, X. zangmui, and X. zonghuangii. The study's findings shed light on the distinctiveness of the newly described species, supported by both morphological distinctions and phylogenetic relationships with their close relatives. This taxonomic revision significantly contributes to our understanding the diversity of Xylaria in China and enriches the knowledge of fungal biodiversity worldwide. [ABSTRACT FROM AUTHOR]

Endocervical adenocarcinoma (ECA) is reported increasingly often in young women, and this aggressive disease lacks effective methods of targeted therapy. Since mismatch repair deficiency (dMMR) is an important biomarker for predicting response to immune checkpoint inhibitors, it is important to investigate the clinicopathological features and immune microenvironment of dMMR ECAs. We assessed 617 ECAs from representative tissue microarray sections, gathered clinicopathologic information, reviewed histological characteristics, and performed immunohistochemical staining for MMR, programmed cell death 1 (PD-L1), and other immune markers. Of 617 ECA samples, 20 (3.2%) cases had dMMR. Among them, loss of MMR-related proteins expression was observed in 17/562 (3.0%) human papilloma virus-associated (HPVA) adenocarcinoma and 3/55 (5.5%) non-HPV-associated (NHPVA) adenocarcinoma. In NHPVA cohort, dMMR status was observed in 3 (3/14, 15.0%) patients with clear cells. dMMR ECAs had a higher tendency to have a family history of cancer, larger tumor size, p16 negative, HPV E6/E7 mRNA in situ hybridization (HPV E6/E7 RNAscope) negative, and lower ki-67 index. Among the morphological variables evaluated, poor differentiation, necrosis, stromal tumor-infiltrating lymphocytes, peritumoral lymphocytes, and lymphoid follicles were easily recognized in the dMMR ECAs. In addition, dMMR ECAs had higher CD3+, CD8+, CD38+, CD68+ and PD-1+ immune cells. A relatively high prevalence of PD-L1 expression was observed in dMMR ECAs. dMMR ECAs were significantly more likely to present with a tumor-infiltrating lymphocytes -high/PD-L1-positive status. In conclusion, dMMR ECAs have some specific morphological features and a critical impact on the immune microenvironment, which may provide insights into improving responses to immunotherapy-included comprehensive treatment for ECAs in the future. [ABSTRACT FROM AUTHOR]