Background: Elastin-driven genetic diseases are a group of complex diseases driven by elastin protein insufficiency and dominant-negative production of aberrant protein, including supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Here, a Chinese boy with a novel nonsense mutation in the ELN gene is reported.
Case Presentation: We report a 1-year-old boy who presented with exercise intolerance, weight growth restriction with age, a 1-year history of heart murmur, and inguinal hernia.
Objective: To investigate the early predictive factors for Kawasaki disease complicated with macrophage activation syndrome (KD-MAS).
Methods: We performed a retrospective case-control study in children with KD from August 2017 to August 2022, involving 28 cases with KD-MAS and 112 cases not developing KD-MAS. Based on the univariate analysis, binary logistic regression was used to identify the early predictive factors for KD-MAS development, and the receiver operating characteristic curve (ROC) analysis was carried out to obtain the optimal cut-off value.
Excessive discharge of inorganic and organic contaminants in water poses a serious threat to the ecosystems. However, most synthetic adsorbents lack cost-effectiveness in terms of preparation. Interestingly, loofah sponge (LS) was a natural absorbent that could effectively remove pollutions in wastewater, but its adsorption capacity is barely satisfactory.
View Article and Find Full Text PDFTo investigate and analyze the relevant risk factors for hemophagocytic lymphohistiocytosis (HLH) in children with severe adenovirus pneumonia (SAP). A retrospective study of children with SAP was performed in 30 cases developing HLH and 94 cases not developing HLH from December 2018 to August 2019. The binary logistic regression analysis was used to identify risk factors that were significantly associated with the development of HLH after the univariate analysis, and the receiver operating characteristic (ROC) curve was performed to find out the cut-off value for the significant relevant factors.
View Article and Find Full Text PDFObjective: To study the clinical features of children with severe adenovirus pneumonia (SAP) and hemophagocytic syndrome (HPS).
Methods: A retrospective analysis was performed from the chart review data of 30 children with SAP and HPS who were admitted from January 2014 to June 2019. According to the prognosis, the children were divided into a good prognosis group (n=18) and a poor prognosis group (n=12).
This article reports the diagnosis and treatment of two children with coronavirus disease 2019 (COVID-19) and hypertension. Case 1 was a boy aged 13 years and 3 months, with the main manifestations of fever and dry cough; chest CT showed ground-glass opacities, and the nucleic acid test of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) yielded a positive result. Case 2 was a boy aged 13 years and 8 months and had no clinical symptoms; chest CT showed no abnormality, while the nucleic acid test of SARS-CoV-2 yielded a positive result.
View Article and Find Full Text PDFBackground: Xenotropic and polytropic retrovirus receptor 1 (XPR1), a previously identified cellular receptor for several murine leukemia viruses, plays a role in many pathophysiological processes. However, the role of XPR1 in human cancers has not yet been characterized.
Methods: Real-time PCR and western blotting assay were used to measure the expression of XPR1 in tongue squamous cell carcinoma (TSCC) tissues.
Chromobox protein homolog 3 (CBX3), a core component of the heterochromatin proteins 1, is recently proved to be involved in human cancerogenesis and associated with the prognosis of patient. However, the role of CBX3 in Tongue squamous cell carcinoma (TSCC) remains unclear. In the present study we found that CBX3 was upregulated in TSCC tissues when compared to adjacent non-tumor tissues, and multivariable analysis showed that high CBX3 expression was associated with clinical stage and cervical node metastasis, which was an independent prognostic indicator of TSCC.
View Article and Find Full Text PDFTo clarify the relationship between the type of the oxide support and the activity of the gold-doped oxide clusters toward H oxidation, a suitable closed-shell system AuVO is chosen to have a comparative study with AuCeO, the first closed-shell cluster that is reactive toward H oxidation. The reaction of AuVO with H was characterized by mass spectrometry and density functional theory calculations. The AuVO cluster is reactive toward H leading to the major product of VOH (+ Au), whereas the product of AuVO (+ HO) is completely absent in the experiment.
View Article and Find Full Text PDFBACKGROUND This study investigated the mechanism of miR-145 in targeting connective tissue growth factor (CTGF), which affects the proliferation, migration, invasion, and epithelial-mesenchymal transition (EMT) of ESCC cells. MATERIAL AND METHODS A total of 50 ESCC tissues and their corresponding normal adjacent esophageal tissue samples were collected. Then, miR-145 expression in both ESCC clinical specimens and cell lines was detected using quantitative real-time PCR.
View Article and Find Full Text PDFPhys Chem Chem Phys
October 2016
The adsorption behaviour of a single O atom on Au clusters (n = 1-8, q = 0, ±1) was systematically investigated by DFT calculations. Both hybrid and pure GGA functionals (B3LYP and PBE) were used to provide reliable conclusions. The most stable structures of AuO clusters were obtained by using global optimizations with a genetic algorithm.
View Article and Find Full Text PDFPhys Chem Chem Phys
April 2016
The bonding properties between a single atom and its support have a close relationship with the stability and reactivity of single-atom catalysts. As a model system, the structural and electronic properties of bimetallic oxide clusters MV3Oy(q) (M = Au or Ag, q = 0, ±1, and y = 6-8) are systematically studied using density functional theory. The single noble metal atom Au or Ag tends to be adsorbed on the periphery of the V oxide clusters.
View Article and Find Full Text PDFThe ~80 amino acid A box DNA-binding domain of high mobility group box 1 (HMGB1) protein antagonizes proinflammatory responses during myocardial ischemia reperfusion (I/R) injury. The exact role of microRNA-21 (miR-21) is unknown, but its altered levels are evident in I/R injury. This study examined the roles of HMGB1 A-box and miR-21 in rat myocardial I/R injury model.
View Article and Find Full Text PDFInt J Syst Evol Microbiol
August 2014
A Gram-staining-positive, endospore-forming, moderately alkaliphilic bacterium, strain NPK15(T), was isolated from a typical sandy loam soil under long-term NPK fertilization in northern China and was subjected to a polyphasic taxonomic study. The diamino acid of the cell-wall peptidoglycan of strain NPK15(T) was found to be meso-diaminopimelic acid and the cell-wall sugars were xylose, glucose and traces of mannose. The only respiratory quinone found in strain NPK15(T) was menaquinone 7 (MK-7).
View Article and Find Full Text PDFVegetation is a component of the natural river. To understand the interaction between vegetation and water flow is of scientific and practical significance for the protection of wildlife habitats, the control of water body eutrophication, the ecological restoration of rivers and lakes, and the management of riverways. This paper reviewed the researches about the interaction between vegetation and water flow in riverways, summarized the research progress in the effects of the vegetation on the resistance coefficient and water flow structure, and introduced the applications of numerical simulation in this research field.
View Article and Find Full Text PDFYing Yong Sheng Tai Xue Bao
February 2013
By using Biolog technique, this paper studied the diversity of carbon source metabolism of microbial community in three farmland soils with different levels of arsenic (As) in an As mining area, and the relationships between the diversity of the carbon source metabolism and the main soil chemical properties. The total N (TN), total P (TP), total K (TK), organic matter (OM), total Cu, and total Zn contents in the three soils were in the order of medium level As > high level As > low level As. The average well color development (AWCD), Shannon index (H'), Simpson index (D), and McIntosh index (U) of soil microbes were significantly higher in the soils with medium and high levels As than in the soil with low level As.
View Article and Find Full Text PDFThe microbial number, microbial biomass, and enzymatic activities in five upland soils under agricultural utilization for 50-700 years were determined, with the correlations between soil microbiological characteristics and agricultural utilization duration analyzed. In the meantime, the functional diversity of microbes in soils having been utilized for 50, 100, and 700 years were investigated. The results showed that at the early stage (< 100 years) of agricultural utilization, the number of soil fungi (F) had a slight increase, while the bacterial number (B), B/F ratio, microbial biomass C (C(mic)), microbial biomass N (N(mic)), and the activities of catalase, invertase and urease all decreased markedly.
View Article and Find Full Text PDFA pot experiment was conducted to examine the roles of earthworm in As uptake from original As-polluted soil by maize (Zea mays L.), and their effects on As, P fractions in the rhizosphere. The As-polluted soils with three As levels were collected from the arable soil near As mine.
View Article and Find Full Text PDFBatch experiments were conducted to study the biosorption characteristics of a cationic dye, Malachite Green (MG), onto aerobic granules. Effects of pH, aerobic granule dosage, contact time and solution temperature on MG biosorption by aerobic granules were evaluated. Simultaneity the thermodynamic analysis was also performed.
View Article and Find Full Text PDFDevelopment of aerobic granules for the biological degradation of 2,4-dichlorophenol (2,4-DCP) in a sequencing batch reactor was reported. A key strategy was involving the addition of glucose as a co-substrate and step increase in influent 2,4-DCP concentration. After operation of 39d, stable granules with a diameter range of 1-2mm and a clearly defined shape and appearance were obtained.
View Article and Find Full Text PDFDefects of hematopoietic stem cells (HSCs) have been suggested to contribute to the development of systemic lupus erythematosus (SLE). The aim of this study was to investigate the phenotypic characteristics of bone marrow (BM) CD34(+) cells in patients with SLE and its relationship with SLE disease activity. Ten SLE patients and 10 healthy subjects were recruited and their BM CD34(+) cells were analyzed by flow cytometric analysis with CD45/SSC gating for the expression of CD90, CD95, CD117, CD123, CD164, CD166, FAS-L, and HLA-DR.
View Article and Find Full Text PDF