82 research outputs found

    Treatment experience for different risk groups of Kaposiform hemangioendothelioma

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    BackgroundKaposiform hemangioendothelioma (KHE) is a rare vascular tumor with a high risk of mortality. Few studies with large samples of KHE have been reported. KHE may develop into the Kasabach–Merritt phenomenon (KMP), which is characterized by thrombocytopenia and consumptive coagulopathy. The features of severe symptomatic anemia and life-threatening low platelets make the management of KHE associated with KMP challenging.ObjectiveThe aim of this study was to examine the clinical characteristics of patients with KHE and discuss the treatment experience for different risk groups of KHE.MethodsThrough a retrospective review of 70 patients diagnosed with KHE between 2017 and 2022 in our center, we classify lesions into three clinicopathological stages based on the tumor involving depth, and divided the severity of KHE into three levels by estimating clinicopathological stages and severity of thrombocytopenia. Treatments of different severity groups were estimated with sufficient data.ResultsIn our cohort, 27% were neonates, and KHE lesion occurred at birth in 84% of patients. There was a slight male predominance (32 girls and 38 boys). Common clinical characteristics included associated coagulation disorder (100%), locally aggressive cutaneous blue–purple mass (89%), thrombocytopenia (78%), and local pain or joint dysfunction (20%). The lower extremities were the dominant location (35%), followed by the trunk (29%), the maxillofacial region and neck (24%), and the upper extremities (10%). Of the total cohort, 78% developed KMP; the median age at which thrombocytopenia occurred was 27.8 days. The median platelet count of patients who were associated with KMP was 24,000/µL in our cohort. Ninety-two percent of patients were given surgery treatment and 89% of these patients were given high-dose methylprednisolone (5-6 mg/kg daily) before surgery. In 55 patients with KMP, 36% were sensitive to high-dose corticosteroid therapy. Patients from the low-risk group (eight cases) underwent operation, all of whom recovered without recurrence after a maximum follow-up of 5 years. Out of 26 patients from the high-risk group, 25 underwent surgery treatment, with 1 case undergoing secondary surgery after recurrence and 1 case taking sirolimus. Out of 36 cases from the extremely high-risk group, 32 underwent surgery (including 2 cases who underwent external carotid artery ligation and catheterization), 3 of whom underwent secondary operation after recurrence, and the remaining 4 cases took medicine. The mean length of having sirolimus was 21 months; two cases stopped taking sirolimus due to severe pneumonia. Two cases died at 1 and 3 months after discharge.ConclusionsOur study describes the largest assessment of high-risk patients with KHE who have undergone an operation to date, with 5 years of follow-up to track recovery, which provides invaluable knowledge for the future treatment of patients with KHE and KMP from different risk groups: Early surgical intervention may be the most definitive treatment option for most patients with KHE; multimodality treatment is the best choice for the extremely high-risk group

    Real-time Monitoring for the Next Core-Collapse Supernova in JUNO

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    Core-collapse supernova (CCSN) is one of the most energetic astrophysical events in the Universe. The early and prompt detection of neutrinos before (pre-SN) and during the SN burst is a unique opportunity to realize the multi-messenger observation of the CCSN events. In this work, we describe the monitoring concept and present the sensitivity of the system to the pre-SN and SN neutrinos at the Jiangmen Underground Neutrino Observatory (JUNO), which is a 20 kton liquid scintillator detector under construction in South China. The real-time monitoring system is designed with both the prompt monitors on the electronic board and online monitors at the data acquisition stage, in order to ensure both the alert speed and alert coverage of progenitor stars. By assuming a false alert rate of 1 per year, this monitoring system can be sensitive to the pre-SN neutrinos up to the distance of about 1.6 (0.9) kpc and SN neutrinos up to about 370 (360) kpc for a progenitor mass of 30MM_{\odot} for the case of normal (inverted) mass ordering. The pointing ability of the CCSN is evaluated by using the accumulated event anisotropy of the inverse beta decay interactions from pre-SN or SN neutrinos, which, along with the early alert, can play important roles for the followup multi-messenger observations of the next Galactic or nearby extragalactic CCSN.Comment: 24 pages, 9 figure

    Potential of Core-Collapse Supernova Neutrino Detection at JUNO

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    JUNO is an underground neutrino observatory under construction in Jiangmen, China. It uses 20kton liquid scintillator as target, which enables it to detect supernova burst neutrinos of a large statistics for the next galactic core-collapse supernova (CCSN) and also pre-supernova neutrinos from the nearby CCSN progenitors. All flavors of supernova burst neutrinos can be detected by JUNO via several interaction channels, including inverse beta decay, elastic scattering on electron and proton, interactions on C12 nuclei, etc. This retains the possibility for JUNO to reconstruct the energy spectra of supernova burst neutrinos of all flavors. The real time monitoring systems based on FPGA and DAQ are under development in JUNO, which allow prompt alert and trigger-less data acquisition of CCSN events. The alert performances of both monitoring systems have been thoroughly studied using simulations. Moreover, once a CCSN is tagged, the system can give fast characterizations, such as directionality and light curve

    Detection of the Diffuse Supernova Neutrino Background with JUNO

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    As an underground multi-purpose neutrino detector with 20 kton liquid scintillator, Jiangmen Underground Neutrino Observatory (JUNO) is competitive with and complementary to the water-Cherenkov detectors on the search for the diffuse supernova neutrino background (DSNB). Typical supernova models predict 2-4 events per year within the optimal observation window in the JUNO detector. The dominant background is from the neutral-current (NC) interaction of atmospheric neutrinos with 12C nuclei, which surpasses the DSNB by more than one order of magnitude. We evaluated the systematic uncertainty of NC background from the spread of a variety of data-driven models and further developed a method to determine NC background within 15\% with {\it{in}} {\it{situ}} measurements after ten years of running. Besides, the NC-like backgrounds can be effectively suppressed by the intrinsic pulse-shape discrimination (PSD) capabilities of liquid scintillators. In this talk, I will present in detail the improvements on NC background uncertainty evaluation, PSD discriminator development, and finally, the potential of DSNB sensitivity in JUNO

    Analysis of Aerosol Properties in Beijing Based on Ground-Based Sun Photometer and Air Quality Monitoring Observations from 2005 to 2014

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    Aerosol particles are the major contributor to the deterioration of air quality in China’s capital, Beijing. Using ground-based sun photometer observations from 2005 to 2014, the long-term variations in optical properties and microphysical properties of aerosol in and around Beijing were investigated in this study. The results indicated little inter-annual variations in aerosol optic depth (AOD) but an increase in the fine mode AODs both in and outside Beijing. Furthermore, the single scattering albedo in urban Beijing is larger, while observations at the site that is southeast of Beijing suggested that the aerosol there has become more absorbing. The intra-annual aspects were as follow: The largest AOD and high amount of fine mode aerosols are observed in the summer. However, the result of air pollution index (API) that mainly affected by the dry density of near-surface aerosol indicated that the air quality has been improving since 2006. Winter and spring were the most polluted seasons considering only the API values. The inconsistency between AOD and API suggested that fine aerosol particles may have a more important role in the deterioration of air quality and that neglecting particulate matter with aerodynamic diameter less than 2.5 μm (PM2.5) in the calculation of API might not be appropriate in air quality evaluation. Through analysis of the aerosol properties in high API days, the results suggested that the fine mode aerosol, especially PM2.5 has become a major contributor to the aerosol pollution in Beijing

    Identification of Igf2bp1 gene family and effect on chicken myoblast proliferation

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    The function of Igf2bp1 regulated muscle development in Lueyang black-bone chickens is to be investigated. The insulin-like growth factor 2 mRNA-binding protein 1 (IGF2BP1) belongs to the family of single-stranded RNA-binding proteins (IGF2BP1-3). IGF2BP1 is abundantly expressed in a broad range of foetal tissues, but only in a limited number of normal adult tissues, and is involved in biological regulation processes, including cell proliferation, differentiation, metabolism and migration, especially in tumour cells. Recent study has reported Igf2bp1 was a key gene that regulated the body size of Pekin ducks. We found that the expression level of Igf2bp1 gradually decreased with age in the leg muscle of Lueyang black-bone chicken, but its function in chicken muscle development is unknown. Firstly, the evolutionary relationship of Igf2bp1 gene family was analysed in chicken. Additionally, Igf2bp1-overexpressed myoblasts of Lueyang black-bone chicken were constructed. The results showed that Igf2bp1 plays a negative role in cell proliferation by the CCK8 and 5-ethynyl-2′-deoxyuridine assay. Moreover, Igf2bp1 inhibited myogenesis by detecting the myoblast proliferation markers of pax7, the expression of myogenic differentiation markers, MyoD, MyoG and Myf5 in myoblasts. Thus, Igf2bp1 can inhibit chicken myoblast proliferation and differentiation by regulating key gene expression of myogenesis, which is not conducive to the growth and development of skeletal muscle in Lueyang black chickens

    Genomic analyses reveal distinct genetic architectures and selective pressures in buffaloes

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    Background: The domestic buffalo (Bubalus bubalis) is an essential farm animal in tropical and subtropical regions, whose genomic diversity is yet to be fully discovered. Results: In this study, we describe the demographic events and selective pressures of buffalo by analyzing 121 whole genomes (98 newly reported) from 25 swamp and river buffalo breeds. Both uniparental and biparental markers were investigated to provide the final scenario. The ancestors of swamp and river buffalo diverged ∼0.23 million years ago and then experienced independent demographic histories. They were domesticated in different regions, the swamp buffalo at the border between southwest China and southeast Asia, while the river buffalo in south Asia. The domestic stocks migrated to other regions and further differentiated, as testified by (at least) 2 ancestral components identified in each subspecies. Different signals of selective pressures were also detected in these 2 types of buffalo. The swamp buffalo, historically used as a draft animal, shows selection signatures in genes associated with the nervous system, while in river dairy breeds, genes under selection are related to heat stress and immunity. Conclusions: Our findings substantially expand the catalogue of genetic variants in buffalo and reveal new insights into the evolutionary history and distinct selective pressures in river and swamp buffalo
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