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Abstract Chronic active Epstein-Barr virus infection (CAEBV) is a progressive and life-threatening disease characterized by persistent or recurrent EBV activation. It has been reported that, γδ T cells, a type of cytotoxic lymphocyte, play a critical role in restricting EBV. However, the functional status of γδ T cells in pediatric CAEBV patients has not yet been described. In this study, flow cytometry analysis was conducted to explore the cytokine production capacity of γδ T cells in CAEBV patients. A diminished frequency of γδ T cells and decreased expression of cytolytic molecule granzyme B were found in CAEBV patients, suggesting a dysfunction in the immune regulatory function of γδ T cells in this disease.

Abstract Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children, and liver involvement in LCH is rare. This retrospective study reported the clinical features and prognosis of patients with hepatic LCH. Liver involvement was defined by histopathological findings, liver dysfunction or abnormalities, or ultrasound imaging. A total of 130 patients (14.5%) with hepatic LCH out of 899 in the LCH population were enrolled. Patients with liver involvement had greater frequencies of skin, lung, hearing system, and haematologic system involvement, and hemophagocytic lymphohistiocytosis (P

Abstract Background The patients with multisystem and risk organ involvement Langerhans cell histiocytosis (MS-RO + LCH) have poor prognosis. The patients with MS-LCH who failed front-line therapy have a high mortality rate and the standard salvage treatment has not been established. The combination of cytarabine (Ara-c), vincristine (VCR) and prednisone might be effective for refractory/relapse MS-RO + LCH, with low toxicity. Methods We retrospectively analyzed pediatric refractory/relapse MS-RO + LCH patients treated with the low-dose Ara-c (100mg/m2/d×5days) or high-dose Ara-c (500mg/m2/d×5days) combined with vindesine (VDS) and prednisone in a single center. The efficacy, outcomes and adverse events were analyzed. Results From January 2013 to December 2016, 13 patients receiving the low-dose Ara-c chemotherapy (LAC) and 7 patients receiving the high-dose Ara-c chemotherapy (HAC) were included in the study. 11 (84.6%) of the 13 patients treated with the LAC regimen and 6 (85.7%) of the 7 patients treated with the HAC regimen had response after four courses of the therapy. All patients in the study were alive during follow-up and the 3-year event-free survival rate (EFS) was 53.7% and 85.7% in the LAC and HAC groups. The most frequent adverse event was Grade 1/2 myelosuppression, which was observed in 38.5% (5/13) and 42.9% (3/7) of the patients receiving the LAC and HAC regimen. Conclusions A combination of Ara-c, VDS and prednisone was effective and safe for some patients with refractory/relapse MS-RO + LCH. The high-dose Ara-c regimen was associated with a numerically higher EFS rate.

Background: Creating a child-friendly social environment is an important component of promoting child-friendly city development. This study aims to explore the key indicators of friendly family, school and community social environments from the perspective of children's conduct problems and prosocial behaviors. Method: The sample included grade 3–5 students from one public elementary school in the urban areas and another public elementary school in the rural areas of a Chinese city pursuing a child-friendly philosophy. A total of 418 participants were included in this study. Data on conduct problems, prosocial behaviors and the social environment were collected. To effectively select important variables and eliminate estimation bias, this study used LASSO regression to identify key indicators predicting children's conduct problems and prosocial behavior, followed by linear regression coefficient estimation and significance testing. Results: Creating a friendly family environment (ensuring family members' assistance with academic problems) and school environment (reducing cheating, fighting, and unfriendly teacher language) was associated with reduced conduct problems in children. Creating a positive family atmosphere (enhancing children's trust in family members), school environment (increasing parents' awareness of school affairs, reinforcing students' prosocial behavior, increasing extracurricular activity programs, and encouraging student engagement in academics) and community environment (respecting all children in the community) was associated with improving children's prosocial behavior. Conclusions: This study transforms the multidimensional, complex child-friendly social environment evaluation indicator system into concise and specific measurement indicators, which can provide theoretical and practical implications for government decision-making in child-friendly city development through empirical research.

In this paper, a comprehensive study combined multiple characterizations and SPH numerical simulation was conducted to investigate the microstructure evolution, extreme thermomechanical behavior and consequences of the explosive welded CLAM/316L interface. The interfaces of the two welding materials underwent remarkable temperature rise and rapid cooling under high-speed collision conditions, forming a tight bond that presents a wavy structure with vortices. The SPH simulation adequately reproduced the heating and cooling process of the interface during explosive welding, revealing the high heterogeneity of the thermodynamic behavior during the impact process. Furthermore, the EBSD analyses showed the perplexing evolution of crystal structure at the bonding interface with vortex, including ultra-fine grains, columnar grains, equiaxed grains, deformed grains and serious lattice distortion. By correlating the temperature histories and grain characteristics at different positions of the interface, the formation mechanism of diverse grains was revealed, referring to crystallization from liquid, recrystallization from the solid and plastic deformation. The recrystallization at the interface was closely related to the material properties, and the significant variations in dislocation density and plastic strain values at the interface, as well as the thermal softening of the material, could be explained by different degrees of dynamic recovery and recrystallization.

This study proposed a dynamic self-constrained explosive welding method to fabricate the copper-steel bimetallic tube without external mold. The mechanical balance of the self-constrained process was carefully revealed by three-dimensional (3D) numerical simulations. Besides, the microstructure evolution, element distribution, and mechanical properties of the copper-steel interface were systematically investigated. Results showed that the dynamic self-constrained system could effectively strike a balance between the inner and outer tubes in terms of stress, velocity, and displacement. The max hoop tensile stress near the outer surface of the base tube was significantly reduced from 535 MPa (in the unconstrained experiment) to 387 MPa avoiding the appearance of radial cracks. The welding interface varied from a nearly straight one via a large wave to an irregularly small wave. Meanwhile, the microhardness test showed a significant increase in the region near the welding interface.

There is no uniform regimen for refractory Langerhans cell histiocytosis (LCH). We retrospectively described patients with refractory multisystem and risk organ involvement LCH treated with the low-dose (Ara-c, 100 mg/m2/d × 5day; 2-CDA, 5 mg/m2/d × 5day) chemotherapy (LDC) and the intermediate-dose (Ara-c, 500 mg/m2/d × 5day; 2-CDA, 9 mg/m2/d × 5day) chemotherapy (IDC). 26 patients and 10 patients receiving the LDC and IDC regimen from January 2013 to December 2016 were included in the study. The overall response rate exhibited no significant difference between the LDC and IDC groups after four courses (76.9% vs 90%, P = 0.375) and eight courses (80.8% vs 100%, P = 0.135) of treatment. No statistical differences in the overall survival rate were observed between the two groups, but 5-year event-free survival rate of patients in the IDC group was higher than that in the LDC group at the median follow-up of 6.16 and 5.07 years (88.9% vs 52.9%, P = 0.033). The patients in the IDC group had more severe myelosuppression than those in the LDC group (grade 3/4 myelosuppression, 80% vs 19.2%, P = 0.001). The intermediate-dose regimen of 2CDA and Ara-c had a higher event-free survival rate and a similar overall survival rate compared with the low-dose regimen.

A two-layer structure model predictive control (MPC) for the low thermal efficiency of cement raw material pre-decomposition is proposed. In the upper layer, an outlet temperature of the precalciner automatic setting scheme is formulated with craft constraints by taking advantage of the energy flow and thermal energy conversion models. In the lower layer, the stable control of the precalciner is achieved using a model predictive control algorithm. Finally, extensive simulation results verify the proposed two-layer MPC approach with excellent performance in energy saving and consumption reduction of raw meal pre-decomposition link.

Primary hemophagocytic lymphohistiocytosis (pHLH) is a rare immune disorder and hematopoietic stem cell transplan- tation (HSCT) is the only potentially curative treatment. Given the high pre-HSCT mortality of pHLH patients reported in the HLH-2004 study (17%), more regimens to effectively control the disease and form a bridge with HSCT are needed. We conducted a retrospective study of pHLH children treated by ruxolitinib (RUX)-based regimen. Generally, patients received RUX until HSCT or unacceptable toxic side-effect. Methylprednisolone and etoposide were added sequentially when the disease was suboptimally controlled. The primary end point was 1-year overall survival. Twenty-one pHLH patients (12 previously treated and 9 previously untreated) were included with a median follow-up of 1.4 years. At last follow-up, 17 (81.0%) patients were alive with a 1-year overall survival of 90.5% (95% confidence interval: 84.1-96.9). Within the first 8 weeks, all patients had an objective response, of which 19 (90.5%) achieved complete response (CR) and two (9.5%) achieved partial response (PR) as a best response. Seventeen (81.0%) patients received HSCT, of which 13 (76.5%) had CR, three (17.6%) had PR and one (5.9%) had disease reactivation at the time of HSCT. Fifteen (88.2) patients were alive post- HSCT. Notably, eight (38.1%) patients received zero doses of etoposide, suggesting the potential of RUX-based regimen to reduce chemotherapy intensity. Patients tolerated RUX-based regimen well and the most frequently observed adverse events were hematologic adverse events. Overall, RUX-based regimen was effective and safe and could be used as a bridge to HSCT for pHLH children.

BackgroundEvidence from previous studies have implicated an important association between gut microbiota (GM) and Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS), but whether there is a definite causal relationship between GM and ME/CFS has not been elucidated.MethodThis study obtained instrumental variables of 211 GM taxa from the Genome Wide Association Study (GWAS), and mendelian randomization (MR) study was carried out to assess the effect of gut microbiota on ME/CFS risk from UK Biobank GWAS (2076 ME/CFS cases and 460,857 controls). Inverse variance weighted (IVW) was the primary method to analyze causality in this study, and a series of sensitivity analyses was performed to validate the robustness of the results.ResultsThe inverse variance weighted (IVW) method indicated that genus Paraprevotella (OR:1.001, 95%CI:1.000–1.003, value of p < 0.05) and Ruminococca- ceae_UCG_014 (OR 1.003, 95% CI 1.000 to 1.005, value of p < 0.05) were positively associated with ME/CFS risk. Results from the weighted median method supported genus Paraprevotella (OR 1.003, 95% CI 1.000 to 1.005, value of p < 0.05) as a risk factor for ME/CFS.ConclusionThis study reveals a causal relationship between genus paraprevotella, genus Ruminococcaceae_UCG_014 and ME/CFS, and our findings provide novel insights for further elucidating the developmental mechanisms mediated by the gut microbiota of ME/CFS.

Background: Positive social relationships are critical for better subjective well-being across ages. Future research will benefit from examining how to improve life satisfaction by utilizing social groups in new, ever-changing social and technological contexts. This study aimed to evaluate the effect of online and offline social network group clusters on life satisfaction across different age groups. Methods: Data were derived from the Chinese Social Survey (CSS) (2019), which is a nationally representative survey. We adopted a K-mode cluster analysis algorithm to categorize participants into four clusters according to their online and offline social network groups. ANOVA and chi-square analysis were used to understand the associations among age groups, social network group clusters, and life satisfaction. Multiple linear regression was applied to identify the association between social network group clusters and life satisfaction across age groups. Results: Younger and older adults had higher life satisfaction than middle-aged adults. Individuals who joined diverse social network groups had the highest life satisfaction, followed by those who joined personal and working social groups, while those who joined restricted social groups had the lowest life satisfaction (F = 81.19, p

Abstract Hemophagocytic lymphohistiocytosis (HLH), particularly primary HLH (pHLH), is a rare, life-threatening disease. Germline genetic deficiency of 12 known HLH genes impairs cytotoxic degranulation in natural killer (NK) cells or cytotoxic T lymphocytes (CTLs) and contributes to pHLH development. However, no pathogenic mutations in these HLH genes are found in nearly 10% of HLH patients, despite a strong suspicion of pHLH, suggesting that the underlying genetic basis of HLH is still unclear. To discover novel susceptibility genes, we first selected 13 children with ppHLH (presumed primary HLH patients in the absence of detectable known HLH gene variants) and their parents for initial screening. Whole-genome sequencing (WGS) in one trio and whole-exome sequencing (WES) in twelve trios revealed that two ppHLH patients carried biallelic NBAS variants, a gene that is involved in Golgi-to-endoplasmic reticulum (ER) retrograde transport upstream of the degranulation pathway. Additionally, two candidate genes, RAB9B and KLC3, showed a direct relationship with known HLH genes in protein-protein interaction (PPI) network analysis. We analyzed NBAS, RAB9B, KLC3 and known HLH genes in an independent validation cohort of 224 pediatric HLH patients. Only biallelic NBAS variants were identified in three patients who harbored no pathogenic variants in any of the known HLH genes. Functionally, impaired NK-cell cytotoxicity and degranulation were revealed in both NBAS biallelic variant patients and in an NBAS-deficient NK-cell line. Knockdown of NBAS in an NK-cell line (IMC-1) using short hairpin RNA (shRNA) resulted in loss of lytic granule polarization and a decreased number of cytotoxic vesicles near the Golgi apparatus. According to our findings, NBAS is the second most frequently mutated gene (2.11%) in our HLH cohort after PRF1. NBAS deficiency may contribute to the development of HLH via a dysregulated lytic vesicle transport pathway.

IntroductionChina has the largest youth population in the world. To better implement the Smoke-free School Initiative, this study aims to examine the protective and risk factors for different smoking behaviors (never smoked, experimental smoking, and current smoking) among school adolescents based on social cognitive theory.MethodsThis research was a secondary analysis of a cross-sectional survey of middle schools in Huli District of Xiamen, China. The final sample consisted of 1937 participants with an average age of 15.41 (SD = 1.64). Descriptive statistics were used to summarize the sociodemographic characteristics of the sample. Multivariate multinomial logistic regression analysis was performed using four models.ResultsOf the respondents, 1685 (86.99%) were never smokers, 210 (10.84%) were experimental smokers, and 42 (2.17%) were current smokers. Social norms, positive outcome expectations, anti-smoking self-efficacy, and attitudes toward control tobacco policies were associated with adolescents' smoking behaviors. The number of smoking family members, classmates smoking, the perception that smoking is cool and attractive, and attitudes toward control tobacco policies were the predictors of current smoking behavior (p < 0.05). In contrast, friends smoking and individual and social relationship motivation were associated with only experimental smoking (p < 0.05).DiscussionThe relationship of social norms, positive outcome expectations, anti-smoking self-efficacy, and attitudes toward control tobacco policies varied across smoking behaviors. Family, school, society and the government need to cooperate in prevention and intervention programs for adolescent smoking. The relationships between these factors and adolescents' different smoking behaviors needs to be further verified.

This work presents a systematic study of microstructure and mechanical property of TiNi Alloy/Q235 steel explosive-welded interface. The structure evolution as well as the thermodynamic state during the welding process was simulated using Smoothed Particle Hydrodynamic (SPH) numerical method. The interface is featured by regular wave structure with a period of ∼400 μm and an amplitude of ∼120 μm, resulting from the periodic interaction process of jets and colliding plates. Furthermore, it was found that the waves do not form in the initial collision zone, but undergo an evolutionary process of straight-irregular small wave-steady wave transformation. Intermetallic compounds like Fe2Ti and Ni3Ti were found in melting zones, which are the source of the microscopic cracks. Even so, the interface exhibits good bonding strength of 291 MPa that is higher than the Q235 steel of 180 MPa. The nanoindentation results show the hardness values decrease at first and then increase to the initial value with increasing distance from the bonding interface. The melting zone exhibits an ultrahigh hardness of 11.06 GPa, which confirms the formation of brittle intermetallic in the melting zone.

Adolescence is a time of dramatic physical and mental change when adolescents are extremely vulnerable to various mental health problems. Depression and poor sleep duration are increasingly common among adolescents. This study is mainly aimed to verify the important mediating role of collective integration on sleep duration and depression and examine the interrelationship between sleep duration and depression in adolescents longitudinally. The data were obtained from the Wave 1 (in 2013–2014) and Wave 2 (in 2014–2015) longitudinal surveys of China Education Panel Survey (CEPS). The analytic sample in the present study included 8,829 seventh-grade students aged about 14 years (51.50% boys and 48.50% girls). A structural equation modeling (SEM) was used to investigate parent–child/teacher factors affecting adolescent sleep duration and depression, and Monte Carlo resampling with R was employed to confirm the significance of the mediation effects of collective integration. An autoregressive cross-lagged model was employed to analyze the interrelationship between adolescent sleep duration and depression. The findings were as follows. Firstly, collective integration strongly mediated the relationships among academic self-efficacy, parental involvement, teacher praise/criticism, sleep duration, and depression. Secondly, sleep duration and depression were found to have enduring effects and have effects on each other. Thirdly, parental involvement and teacher praise were positively associated with sleep quality and negatively associated with depression. Teacher criticism was negatively associated with sleep quality and positively associated with depression. Compared with teacher praise, teacher criticism has stronger effects on youth sleep duration and depression. In conclusion, improving sleep problems and depression in adolescents as early as possible can stop the persistent and long-term consequences of these problems. Increasing teacher praise, decreasing teacher criticism, and increasing adolescents’ collective integration were effective ways to improve adolescents’ sleep duration and mediate depression.

An innovative explosive welding method for the fabrication of multiple plates (EWMP) was proposed to further improve the energy efficiency of explosive welding explosive charges and reduce the impact on the surrounding environment. In this method, six metal plates were placed in parallel and three composite plates were obtained through one explosion in this paper. The microstructure of the joint was analyzed by optical microscopy (OM), scanning electron microscopy (SEM), energy dispersive spectroscopy (EDS), and electron backscattering diffraction (EBSD). Compared to traditional explosive welding (TEW), the EWMP dramatically reduced the noise and vibration and increased the energy efficiency of explosives. The results showed the vibration data of EWMP were reduced by 16.7, 16.4, 2.8, and 1.5%, respectively, and at places of 3, 4, 5, and 6 m away from the explosion center, and explosive consumption of EWMP was reduced by at least half, compared to the traditional method when obtaining the same amount of welded plates. Through numerical simulation, the collision speeds of the composite plate are 610, 516, and 471 m/s, respectively, and the waveform of the composite plate is basically in coincidence with the result of the experiment.

Abstract Purpose We intended to investigate the clinical features of paediatric patients with chronic active Epstein–Barr virus infection (CAEBV) and to examine the effectiveness of the L-DEP regimen before haematopoietic stem cell transplantation (HSCT). Methods A retrospective analysis was performed on 35 patients with CAEBV at Beijing Children’s Hospital from January 2016 to January 2020. The efficacy and adverse events of the L-DEP regimen were evaluated. Results The median age of the 35 patients was 7.0 years old (range 2.5–17.5 years). Twenty-eight patients achieved a clinical response (80.0%, 22 in clinical CR, 6 in clinical PR) after L-DEP. In terms of virological response, 7 patients (20%) were assessed as having virological CR, and 23 patients (65.7%) had virological PR. Finally, 29 patients underwent allo-HSCT. The median survival time was 18 months (2–50 months). The 3-year overall survival rates in patients treated with chemotherapy only (n = 6) and chemotherapy followed by HSCT (n = 25) were 33.3% and 75.4%, respectively. After L-DEP 1st treatment and L-DEP 2nd treatment, the EBV-DNA loads in blood and plasma were significantly reduced compared with those before chemotherapy (median: 4.29 × 105 copies/ml vs. 1.84 × 106 copies/ml, Mann–Whitney U: P = 0.0004; 5.00 × 102 copies/ml vs. 3.17 × 103 copies/ml, Mann–Whitney U; P = 0.003; 2.27 × 105 copies/ml vs. 1.84 × 106 copies/ml, P = 0.0001; 5.00 × 102 copies/ml vs. 3.17 × 103 copies/ml, P = 0.003). Compared with the liver and spleen size before chemotherapy, the size of the liver and spleen shrank significantly after L-DEP 2nd (median 3.8 cm vs. 1.9 cm, P = 0.003; 3.8 cm vs. 0 cm, P

The microstructural characteristics of the explosive welded niobium-steel interface were systematically investigated by various characterizations and a two-step numerical simulation. A coupled Lagrange-Eulerian simulation was firstly developed to compute the impact velocity, this model allows to establish an accurate impact condition. Microstructure observations show that the niobium-steel interface is featured by a wave structure with two vortices located at the wave front and back, and micro defects like voids and cracks were detected inside the vortex. Grains of the niobium and steel matrix near the interface were characterized by elongated and fine equiaxed structures, respectively, while that in vortex area was fine columnar grains. Then, the interfacial thermokinetics was investigated by a smoothed particles hydrodynamics simulation to reveal the formation of the observed defects and diverse grains. The ultimate strength of the composite exceeds 350 MPa with disparate heat-treated temperatures obtained by the tensile tests. Meanwhile, tensile-shear test shows a robust bonding strength of 220 MPa. The strong mechanical properties of the composite guarantee a great prospect of its application in the nuclear industry.

Abstract Objective To investigate the clinical characteristics, treatment, prognosis and risk factors for chronic active Epstein–Barr Virus infection (CAEBV) associated with coronary artery dilatation (CAD) in children. Methods Children with CAEBV associated with CAD hospitalized at Beijing Children’s Hospital, Capital Medical University from March 2016 to December 2019 were analyzed. Children with CAEBV without CAD were selected as the control group and matched by sex, age, treatment and admission time. The clinical manifestations, laboratory and ultrasound examinations, treatment and prognosis of the children were collected in both groups. Results There were 10 children with CAEBV combined with CAD, including 6 males and 4 females, accounting for 8.9% (10/112) of CAEBV patients in the same period, with an onset age of 6.05 (2.8–14.3) years. The median follow-up time was 20 (6–48) months. All the patients had high copies of EBV-DNA in whole blood [1.18 × 107 (1.90 × 105–3.96 × 107) copies/mL] and plasma [1.81 × 104 (1.54 × 103–1.76 × 106) copies/mL], and all biopsy samples (bone marrow, lymph nodes or liver) were all positive for Epstein–Barr virus-encoded small RNA. Among the 10 children, 8 had bilateral CAD, and 2 patients had unilateral CAD. After diagnosis, 7 children were treated with L-DEP chemotherapy in our hospital. After chemotherapy, four patients underwent allogeneic hematopoietic stem cell transplantation (HSCT). The others were waiting for HSCT. At the time of the last patients follow up record, the CAD had returned to normal in 3 patients, and the time from the diagnosis of CAD to recovery was 21 (18–68) days. LDH, serum ferritin, TNF-α and IL-10 levels were statistically significantly different between the two groups (P = 0.009, 0.008, 0.026 and 0.030). There were no significant differences in survival rate between the two groups (P = 0.416). Conclusion The incidence of CAEBV with CAD was low. CAEBV with CAD did not influence the prognosis. Patients who had high LDH, serum ferritin, TNF-α, and IL-10 levels early in their illness were more likely to develop CAD.

In order to obtain high-quality CuCrZr/316L clad plates prepared by explosive welding (EW), theoretical analysis and numerical simulation were introduced to optimize welding parameters. The bonding properties of the achieved joints were systematically studied through multiscale interface analyses, mechanical performance tests, and evaluation of sealing properties. It was concluded that the theoretical analysis in collaboration with numerical calculations is an effective way to design parameters, because its predicted result was in good agreement with the ultrasonic test (UT) one. In both microstructure observations and mechanical tests, an excellent bonding quality of the CuCrZr/316L bimetallic plate was confirmed, where a uniform wavy morphology without defects such as voids and cracks was found at the bonding interface. The bonding strength was higher than the CuCrZr alloy. Moreover, a self-designed hydraulically pressed mold method was developed to manufacture a U-shaped CuCrZr/316L component, and the resulting sample matches well with the designed dimensions. Finally, the helium-leak test results demonstrate that the sealing property of the CuCrZr/316L bimetallic plate meets the practical requirements, indicating that the welded specimens were sound.

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially fatal illness characterized by impaired natural killer and cytotoxic T cell function. Chronic granulomatous disease (CGD) is an inherited immune deficiency caused by a defect in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. CGD patients display an increased susceptibility to infection with bacteria and fungi. Repeated infections lead to an increased risk for developing HLH. The case of CGD with repeated Salmonella septicemia complicated with HLH is very rare, and the CGD mutation identified has not been reported. Case presentation A 3-year-old boy was admitted to our hospital for fever, hepatosplenomegaly and pancytopenia. According to the clinical manifestations and laboratory results, hemophagocytic lymphohistiocytosis (HLH) was diagnosed. Blood and bone marrow culture confirmed septicemia due to Salmonella Typhimurium. On the basis of antiinfection treatment, methylprednisolone was used to control HLH. After treatment, the clinical symptoms and laboratory results improved. Gene analysis showed a novel hemizygous CYBB gene mutation: c.302A > G (p.H101P). Combined with a past history of recurrent infection, the child was diagnosed with HLH secondary to CGD triggered by septicemia. Conclusions In case of a known (or highly suspected) CGD with a documented infection, clinical or biological features of HLH should encourage the physician to make possible to confirm or not the HLH. Therefore, to initiate the adequate treatment in association with anti-infective therapy.

The first-wall materials and components are the most thermally loaded in-vessel components of a fusion machine, which are required to survive at high heat fluxes induced by the intense plasma bombardment. As a candidate material, the W/Cu composite was investigated from the aspects of choosing a feasible and preferable welding method for high performance and high strength. For this, a strong confinement thermal explosive welding technology was proposed to solve the key problems in producing high-quality W/Cu composite material without damage and shock-induced defects. The microstructural evolution of the welding interface was observed and analyzed in the paper. Combining with the numerical simulation, the evolution mechanism of the bonding interface and the changing rule of the grain structure of the material were studied. This comprehensive research allows to provide a new perspective for preparing high-quality W/Cu composite.

Objective: To investigate the clinical characteristics, treatment, and prognosis of children with systemic juvenile xanthogranuloma (JXG).Methods: Clinical data of children with JXG who were hospitalized in Beijing Children's Hospital, Capital Medical University, from January 2012 to December 2019 were retrospectively analyzed, including clinical manifestations, laboratory determinations, treatment, and prognosis of the children. Patients were treated with vindesine + prednisone as the first-line treatment and cytarabine + vindesine + dexamethasone ± cladribine as the second-line treatment.Results: Ten patients, including 8 males and 2 females, with a median of onset age of 1.95 (0.80–7.30) years, exhibited multi-system dysfunction. The median age of diagnosis was 2.45 (1.30–12.10) years. The most common location of extracutaneous lesions was the central nervous system (6 cases), followed by the lung (5 cases) and bone (4 cases). Nine patients underwent first-line chemotherapy, and 6 patients underwent second-line chemotherapy, including 5 patients with poorly controlled disease after first-line treatment. The median observation time was 29 (3–115) months. Nine patients survived, whereas one patient died of respiratory failure caused by pulmonary infection. At the end of follow-up, 7 patients were in active disease (AD)/regression state (AD-better), and 2 patients were in an AD/stable state (AD-stable). Three patients had permanent sequelae, mainly central diabetes insipidus. The rates of response to the first-line treatment and the second-line treatment were 40.0 and 66.7% respectively.Conclusion: The chemotherapy protocol for Langerhans cell histiocytosis (LCH) may be effective for patients with systemic JXG. Central nervous system involvement may not impact overall survival, but serious permanent sequelae may occur.

Considering the unsafety of the present blasting network used in the blasting mining of coalfield fires, a unidirectional explosive element (named explosive diode) is proposed according to explosive logic element principles. Through theoretical and experimental analysis, the internal structure and mechanism of the unidirectional transmission of the detonation signal were studied. For an explosive diode, the length of the quenching channel was defined to be the key parameter. The explosive diode was implemented in the traditional blasting network, obtaining an explosive logic network. To evaluate the safety and reliability of the explosive diode and explosive logic network, detonation propagation and explosion-proof experiments were conducted in the lab. The optimum length of the quenching channel to obtain unidirectional detonation transmission was established. The results showed that the explosive diode could reliably control the propagation direction of the detonation signal when the length of the quenching channel was between 15 mm and 25 mm. The explosive logic network achieved a reliable detonation propagation and was explosion-proof. In comparison with traditional networks, the explosive logic network showed increased safety and reliability as the number of subnets increased. This is a significant improvement to mining safety and demonstrates great promise for engineering applications.

As the only solid detonation product of CHNO negative oxygen balance explosive, the condensed carbon plays an important role in the energy-releasing and working capability by coagulation and phase transition. But the process of condensed carbon transformation has always been controversial because the detonation is so fast and the carbon phase is so complex (amorphous phase, graphite phase, diamond phase, liquid phase, etc.), that spatial and temporal resolution of existing test techniques is difficult to meet the requirements of quantitative analysis. If we can accurately understand the phase transition and coagulation process of carbon condensation during detonation, we can provide effective reference data for the accurate evaluation of explosive energy release process and damage effect, rational design of formula, and accurate correction of non-ideal explosive detonation model.

Achieving high quality tantalum coatings on common metals is still a challenging task, due to potential technical problems such as high differences in melting points and formation of intermetallic compounds. In this work, an improved explosive welding technique was successfully employed to prepare tantalum coatings on a steel substrate, where a special charging structure and a double-layer buffer structure were used to obtain an ideal welding condition. The microstructure analyses of the achieved joints were conducted using electron backscatter diffraction (EBSD), and scanning electron microscope (SEM) cooperated with energy dispersive X-ray spectroscopy (EDS) system. Tensile, three-point bending, and nanoindentation tests were carried out to investigate the mechanical properties. It was concluded that the improved explosive welding technique was a suitable method to produce Ta coatings on steel substrate. In both microstructure observation and mechanical tests, an excellent bonding quality between Ta foil and Q235 plate was confirmed. In addition, a novel vortex structure, good for preventing crack propagation from the brittle melted area, was observed at the Ta/Fe interface. Finally, the corrosion test indicated that the corrosion resistance of Q235 plate was remarkably improved after being coated by Ta foils using explosive welding. Keywords: Explosive welding, Tantalum foil, Steel plate, Corrosion resistance