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Objective: Hypertension caused by vascular Behcet's disease (BD) is an important prognostic factor of paediatric BD. However, much less is known about its clinical features. The objective of this study was to investigate the clinical characteristics of paediatric vascular BD complicated by hypertension. Methods: A retrospective study was carried out in paediatric BD patients complicated by hypertension treated in the Children's Hospital Capital Institute of Paediatrics from Jan 2009 to Dec 2022. Results: Of 65 BD patients, 6 (9.2%) were complicated by hypertension, 5 patients were female, and the median ages of onset and diagnosis were 9.8 years and 11.3 years, respectively. Three patients were found to have cardiac involvement and hypertensive retinopathy secondary to hypertension. Five of the 6 patients with hypertension had right renal artery involvement, and all of them were treated with glucocorticoids and immunosuppressants. Four patients were treated with biological agents. One patient with severe renal artery stenosis underwent unsuccessful vascular interventional therapy. After 3–6 years of follow-up, five patients were found to have renal atrophy, and one patient was at stable condition. Conclusion: Hypertension in paediatric BD is mainly caused by renal artery involvement. Early recognition and treatment of vascular involvement in BD is important to prevent poor prognosis. [ABSTRACT FROM AUTHOR]

Coordination-driven self-assembly of metallacages has garnered significant interest because of their 3D layout and cavity-cored nature. The well-defined, highly tunable metallacage structures render them particularly attractive for investigating the properties of luminophores, as well as for inducing novel photophysical characters that enable widespread applications. In this review, we summarize the recent advances in synthetic methodologies for light-emitting metallacages, and highlight some representative applications of these metallacages. In particular, we focus on the favorable photophysical properties—including high luminescence efficiency in various physical states, good modularity in photophysical properties and stimulus responsiveness—that have resulted from incorporating ligands displaying aggregation-induced emission (AIE) into metallacages. These features show that the synergy between carrying out coordination-driven self-assembly and using luminophores with novel photophysical characteristics like AIE could stimulate the development of supramolecular luminophores for applications in fields as diverse as sensing, biomedicine and catalysis. [ABSTRACT FROM AUTHOR]

Simple Summary: A pig inventory is an important part of realizing accurate and large-scale farming. Distinguishing from the current, mostly overhead scene or static map counting, this study proposes a video-based dynamic counting method through which pigs can be counted automatically and continuously in complex scenes. The improved model effectively reduces the counting error and improves the operation efficiency, which provides data and experimental references for the study of automated pig counting, and it also provides technical support to improve the management efficiency of pig farms, reducing the cost and promoting the intelligent transformation of the animal husbandry industry. A pig inventory is a crucial component of achieving precise and large-scale farming. In complex pigsty environments, due to pigs' stress reactions and frequent obstructions, it is challenging to count them accurately and automatically. This difficulty contrasts with most current deep learning studies, which rely on overhead views or static images for counting. This research proposes a video-based dynamic counting method, combining YOLOv7 with DeepSORT. By utilizing the YOLOv7 network structure and optimizing the second and third 3 × 3 convolution operations in the head network ELAN-W with PConv, the model reduces the computational demand and improves the inference speed without sacrificing accuracy. To ensure that the network acquires accurate position perception information at oblique angles and extracts rich semantic information, we introduce the coordinate attention (CA) mechanism before the three re-referentialization paths (REPConv) in the head network, enhancing robustness in complex scenarios. Experimental results show that, compared to the original model, the improved model increases the mAP by 3.24, 0.05, and 1.00 percentage points for oblique, overhead, and all pig counting datasets, respectively, while reducing the computational cost by 3.6 GFLOPS. The enhanced YOLOv7 outperforms YOLOv5, YOLOv4, YOLOv3, Faster RCNN, and SSD in target detection with mAP improvements of 2.07, 5.20, 2.16, 7.05, and 19.73 percentage points, respectively. In dynamic counting experiments, the improved YOLOv7 combined with DeepSORT was tested on videos with total pig counts of 144, 201, 285, and 295, yielding errors of -3, -3, -4, and -26, respectively, with an average accuracy of 96.58% and an FPS of 22. This demonstrates the model's capability of performing the real-time counting of pigs in various scenes, providing valuable data and references for automated pig counting research. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the clinical and genetic characteristics of 3 children with Haploinsufficiency of A20 (HA20). Methods:The clinical and genetic testing data of 3 children with HA20 treated at Capital Institute of Pediatrics (CIP) between August 2016 and October 2019 were retrospectively analysed. Result: Patient 1 presented with arthritis and inflammatory bowel disease, patient 2 presented with axial spinal arthritis and lupus-like syndrome, and patient 3 presented with recurrent oral ulcers, gastrointestinal ulcers, and perianal abscesses. Regarding laboratory tests, patients were found to have elevated white blood cell (WBC) count, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). The CRP and ESR was reported to be high in all the patients. The WBC was reported to be high in patient 1 and 3. Patient 2 was positive for antinuclear antibodies, anti-Sjögren's syndrome antigen A, dsDNA, rheumatoid factor and Coombs test. Genetic testing showed that all three patients had heterozygous mutation in TNFAIP3 gene. As for the treatment, patient 1 was treated with TNFα antagonist, patient 2 was treated with TNF α antagonist and sulfasalazine, and patient 3 was treated with corticosteroids and thalidomide. Patients 1 and 2 were followed for four and 3 months, respectively. There was an improvement in joint and gastrointestinal symptoms; inflammatory indices and rheumatoid factor (RF) were normal, and dsDNA and Coombs test became negative. Patient 3 was treated at another hospital and showed gradual improvement in oral ulcers and perianal abscesses. Conclusion: HA20 is a single-gene auto-inflammatory disease caused by mutation in tumour necrosis factor (TNF)-α-induced protein 3 (TNFAIP3) gene. It may present as Behçet-like syndrome and resemble various other autoimmune diseases as well. Corticosteroids and immunosuppressive agents are effective treatments, and cytokine antagonists can be used in refractory cases. Whole-exome genetic testing should be proactively performed for children with early-age onset or Behçet-like syndrome to achieve early diagnosis and accurate treatment. [ABSTRACT FROM AUTHOR]

Background: Juvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The anti-NXP2 antibody is one of the most common antibodies and muscle ischaemia associated with NXP2 autoantibodies was a severe subtype of JDM. Further information is needed regarding clinical characteristics and factors associated with poor prognosis. But there are no reports about clinical characteristics and high risk factor of poor prognosis. For the first time, we introduced the clinical characteristics and poor predictors of anti-NXP2 antibody-associated juvenile dermatomyositis in Chinese children. Methods: Twenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM Chinese patients were diagnosed from January 2016 to November 2019. Logistic regression was used to analyze the risk factors for refractory cases and mortality. Results: The ratio of male to female was 1:1.9. The median age of onset was 4.5 (1–13) years. Twenty-four cases (92.3%) had rash and muscle weakness. Treatments included glucocorticoids, immunosuppressive agents, biological agents (7 cases), plasma exchange, Janus kinase inhibitor (7 cases) and autologous stem cell transplant (1 case). Refractory JDM patients (11/26, 42.3%) were associated with edema, skin ulcer, muscle strength<=grade 3, CD4/CD8 ratio < 1.4 and ferritin > 200μg/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after autologous stem cell transplant (ASCT). The risk factors for gastrointestinal involvement and mortality were edema, skin ulcer, severe muscle weakness (dysphagia/ hoarseness/ soft voice), BMI < 15 and ANA positive. Conclusions: Edema, skin ulcer and severe muscle weakness predicted refractory disease, GI involvement, and mortality in anti-NXP2 antibody-positive JDM of Chinese children. Decreased CD4/CD8 ratio and high ferritin related with refractory cases, and very low BMI and ANA (+) are probably, associated with gastrointestinal involvement and mortality. Trial registration: http://www.chictr.org.cn/showproj.aspx?proj=49846. [ABSTRACT FROM AUTHOR]

Background: To summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile dermatomyositis (JDM). Methods: Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed. Results: Five patients, including four females and one male, were included in the study. The age of onset of these patients ranged from 3.3 to 9.5 years with the median age of 5.0 years. When these patients were complicated by GI perforation, childhood myositis assessment score (CMAS) ranged from 1 to 5 with the median score of 2. Myositis-specific antibody (MSA) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients. One patient failed to undergo a repair as the perforation was high in position. For the other three patients, perforation repair was successful, of which two patients failed due to recurrent perforation. At 24 months postoperative follow-up, one patient was in complete remission on prednisone (Pred) and methotrexate (MTX) treatment, and her ureteral stricture had disappeared. The other four patients died. Adding these cases with 16 other patients described in the literature, the symptom at onset was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most commonly occurred in the duodenum, although it also occurred at multiple sites or was recurrent. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions: All the five perforation cases in our study subjected to MSA analysis were anti-NXP2 antibody positive. The symptom at onset was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations prevented early diagnosis. GI perforation may be a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM. [ABSTRACT FROM AUTHOR]

Objective To evaluate the use of Janus kinase inhibitor (JAKi) in treating JDM and develop cytokine biomarkers of active disease. Methods This study involved a retrospective cohort study that evaluated JAKi in 101 JDM patients as well as a cross-sectional study of cytokines in 128 JDM patients and 30 controls between November 2017 and December 2021 in the Affiliated Children's Hospital of Capital Institute of Pediatrics (China). Results During the median follow-up period of 19 months, 65.5% of the patients had improved rashes, and CAT-BM scores decreased. Overall, 39.6% of JDM patients eliminated glucocorticoids. Muscle strength was improved in all patients who had abnormal muscle strength before JAKi use. Patients and parents provided positive subjective reviews of JAKi, and no serious adverse events were reported. Potential side effects of JAKi included abnormal leukopoenia (14/95) and cough (16/83), which affected over 10% of the JDM patients. In the cytokine analysis, 12/34 cytokines were significantly elevated in active JDM patients. Compared with active JDM patients with multiple phenotypes, active JDM patients with only rashes demonstrated lower cytokine levels. Anti-NXP2-positive active patients had lower cytokine levels compared with those without positive anti-NXP2 antibodies. Among all increased cytokines, IL-1RA changed most dramatically, reaching over 793 times the mean of normal values. We developed a panel composed of six cytokines to differentiate active or stable status in our cohort (area under the curve = 0.8486, P   <  0.05). Conclusion The preliminary evidence suggested that JAKi is a relatively safe and effective alternative for JDM patients. Cytokine profiles could well reflect the inflammatory status of JDM patients. [ABSTRACT FROM AUTHOR]

Thermodynamic pathways and reaction initiation mechanisms of shocked TNT (2, 4, 6-trinitrotoluene, formula C6H2(NO2)3CH3) with shock velocities in the range of 6 -10 km⋅s-1 using the first-principles-based ReaxFF reactive force field molecular dynamics and the multiscale shock technique (MSST) are reported in this paper. The decomposition reactions occur at a shock velocity of 7 km⋅s-1 or higher. The shock initiation pressure, 25.1 GPa, is obtained from Rankine−Hugoniot relation. According to the link between macroscopic shock initiation and microscopic chemical reaction events, the formation of TNT-dimer and decomposition to C7H5O5N3 are the dominant initial route for shock induced reaction initiation. At shock speeds equal to or higher than 8km⋅s-1, TNT-dimer is formed and subsequently decomposed to C7H5O5N3, NO2 and NO. The quantity of NO2 molecules reaches maximum when TNT molecules decompose completely. Furthermore, when NO2 molecules are consumed fully, the volume of reaction system begins to expand. TNT molecules are dimerized at each shock condition, and the quantity of dimers is the largest at a shock initiation velocity of 7 km⋅s-1. Finally, the formation and evolution of carbon-containing clusters in shocked TNT are analyzed. [ABSTRACT FROM AUTHOR]

Background: The genetic etiology of nonsyndromic cleft lip with or without cleft palate (NSCL/P) has not been fully clarified to date. Epidermal growth factor receptor (EGFR) was reportedly involved in its biological establishment and regulation of cell migration during the embryonic stage. Methods: We selected a super pathway of endocytic trafficking of EGFR and investigated the associations of single‐nucleotide polymorphisms (SNPs) in the super pathway with the risk of NSCL/P by analyzing our published genome‐wide association study (GWAS) data from 504 NSCL/P individuals and 455 controls. After the false discovery rate (FDR) control, we conducted linkage disequilibrium (LD) analyses and conditional regression analyses to obtain independent lead SNPs. We performed LD analyses between the lead SNPs and the reported SNPs to find novel ones from our study. We annotated the lead SNPs and investigated their mapped genes in silico. Results: A total of 82 SNPs showed a statistical association with the risk of NSCL/P after FDR control. They contained three reported SNPs which were g.117068049G>A (rs7078160), g.117086783C>G (rs10886040), and g.117101266G>T (rs17095681). Four independent lead SNPs were obtained, including g.116979803 T>C (rs1905539) and g.117037960A>G (rs7902502) at 10q25.3, g.35720163G>C (rs75656820) at 17q12, and g.156864512G>A (rs1800877) at 1q23.1. Three of them were in low LD (r2 < 0.5) with the reported SNPs except g.117037960A>G (rs7902502), so these three were newly identified. Lead SNPs were mapped to three genes: SHTN1, AP2B1, and NTRK1. The three genes were relatively more highly expressed in the human craniofacial region and in the proximal maxillary location during the craniofacial development stage of the embryonic mouse. Conclusion: Our results suggested that SHTN1, AP2B1, and NTRK1 might be associated with the development of NSCL/P. We selected a super pathway of endocytic trafficking of EGFR and investigated the associations of SNPs of genes in the super pathway with the risk of NSCL/P by analyzing our GWAS data. We found three newly identified susceptible SNPs (rs1905539, rs75656820, and rs1800877) and obtained three risk genes: SHTN1, AP2B1, and NTRK1. [ABSTRACT FROM AUTHOR]

Objective: To follow up the refractory juvenile dermatomyositis (JDM) with autologous hematopoietic stem cell transplantation (AHSCT) in a long time and to investigate whether AHSCT is effective and safe to treat refractory JDM. Methods: We collected the AHSCT and follow-up data of three patients with refractory JDM who received autologous peripheral blood CD34+ cell transplantation in our hospital between June 2004 and July 2015. Those data include: hight, weight, routine blood and urine tests, ESR, CK, ALT, AST, LDH, renal functional tests, lymphocyte subpopulations, HRCT and muscle MRI. The last follow-up was done in June 2017. Results: All three patients had complete remission and could stop prednisone after 3–12 months. None of them relapsed at 144, 113 and 23 months follow-up. Twelve months after their AHSCT, all of their monitoring indexes have returned to normal and they have stopped all medications. Until the date of this article, none of them relapsed or need medicine. Conclusion: Our study suggests that AHSCT is safe and effective in treating refractory JDM, and it can provides long term drug-free survival. However, more cases are needed for further confirmation. [ABSTRACT FROM AUTHOR]