1.Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
Huiyuan YANG ; Wanzhen GUAN ; Ling2 YANG ; Haimei LIU ; Xiaoqing3 LI ; Haiguo YU ; Meiping LU ; Jun YANG ; Xiaohui LIU ; Hongxia ZHANG ; Wei ZHANG ; Jihong XIAO ; Xiaozhong LI ; Guomin LI ; Hong CHANG ; Sheng HAO ; Yue DU ; Daliang XU ; Ling WU ; Wenjie ZHENG ; Li LIU ; Xinhui JIANG ; Shaohui ZHU ; Dongmei ZHAO ; Xuemei TANG ; Li SUN
Chinese Journal of Pediatrics 2025;63(12):1299-1305
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.
2.Numerical simulation technique for predicting effect of self-expanding prosthetic valve stents for treating type 0 bicuspid aortic valve stenosis
Jiachen DU ; Hongtao XU ; Qingqi HAN ; Guomin CUI
Chinese Journal of Medical Imaging Technology 2025;41(1):133-137
Objective To observe the effect of numerical simulation technique for predicting effect of self-expanding prosthetic valve(SEV)stents for treating type 0 bicuspid aortic valve(BAV)stenosis with different implantation strategies.Methods Based on CT angiography data from a patient with type 0 BAV stenosis and asymmetric calcification,three-dimensional models of aortic root wall,aortic valve calcification,native valve and SEV were reconstructed.ABAQUS/Explicit dynamic analysis module was used to observe the interaction between SEV stents and aortic root including root stress and stent deformation under different implantation strategies(implantation depths of high,medium or low,and implantation centers of central or eccentric position).Results When SEV was implanted centrally at low depth and central position,the Von Mises stress on aortic root wall was the highest,with mean and peak value of 2.28 and 45.30 kPa,respectively.When SEV was implanted at medium depth and central position,the average eccentricity of stent across all planes was the highest(0.254),while when implanted at high depth and eccentric position,the average ellipticity in planes S1-S3 was the minimal(0.124).Conclusion Different SEV implantation strategies resulted in varying interactions between stent and aortic root.Numerical simulation technique could be used to determine the implantation center and depth based on the size and location of type 0 BAV calcification,which was helpful to reducing rupture risk of aortic annulus,increasing durability and improving prognosis of SEV stent.
3.Numerical simulation technique for predicting effect of self-expanding prosthetic valve stents for treating type 0 bicuspid aortic valve stenosis
Jiachen DU ; Hongtao XU ; Qingqi HAN ; Guomin CUI
Chinese Journal of Medical Imaging Technology 2025;41(1):133-137
Objective To observe the effect of numerical simulation technique for predicting effect of self-expanding prosthetic valve(SEV)stents for treating type 0 bicuspid aortic valve(BAV)stenosis with different implantation strategies.Methods Based on CT angiography data from a patient with type 0 BAV stenosis and asymmetric calcification,three-dimensional models of aortic root wall,aortic valve calcification,native valve and SEV were reconstructed.ABAQUS/Explicit dynamic analysis module was used to observe the interaction between SEV stents and aortic root including root stress and stent deformation under different implantation strategies(implantation depths of high,medium or low,and implantation centers of central or eccentric position).Results When SEV was implanted centrally at low depth and central position,the Von Mises stress on aortic root wall was the highest,with mean and peak value of 2.28 and 45.30 kPa,respectively.When SEV was implanted at medium depth and central position,the average eccentricity of stent across all planes was the highest(0.254),while when implanted at high depth and eccentric position,the average ellipticity in planes S1-S3 was the minimal(0.124).Conclusion Different SEV implantation strategies resulted in varying interactions between stent and aortic root.Numerical simulation technique could be used to determine the implantation center and depth based on the size and location of type 0 BAV calcification,which was helpful to reducing rupture risk of aortic annulus,increasing durability and improving prognosis of SEV stent.
4.Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
Huiyuan YANG ; Wanzhen GUAN ; Ling2 YANG ; Haimei LIU ; Xiaoqing3 LI ; Haiguo YU ; Meiping LU ; Jun YANG ; Xiaohui LIU ; Hongxia ZHANG ; Wei ZHANG ; Jihong XIAO ; Xiaozhong LI ; Guomin LI ; Hong CHANG ; Sheng HAO ; Yue DU ; Daliang XU ; Ling WU ; Wenjie ZHENG ; Li LIU ; Xinhui JIANG ; Shaohui ZHU ; Dongmei ZHAO ; Xuemei TANG ; Li SUN
Chinese Journal of Pediatrics 2025;63(12):1299-1305
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.
6.Research progress on anti-inflammatory effect and molecular mechanism of Artemisia dracunculus
Yifei XU ; Lei ZHAO ; Jie ZHEN ; Guomin ZHAO ; Wei CHEN
Chinese Journal of Integrated Traditional and Western Medicine in Intensive and Critical Care 2024;31(4):508-512
Inflammation is the pathological basis of many diseases,which can infect locally or act on the whole body,and is closely related to the pathogenesis of many diseases.In recent years,in the background of more and more traditional anti-inflammatory drugs showing adverse reactions,scholars have been exploring the use of traditional Chinese medicine in the ascendant.As an herb in the Asteraceae(daisy)family,Artemisia dracunculus possesses low toxicity and a wide range of uses and can be applied to the regulation of metabolism,analgesia,antidepressant,antioxidant,immune regulation,antibacterial,anti-inflammatory,antiparasitic,and other treatment of a variety of diseases.In-depth studies have revealed that the therapeutic effects and potential of Artemisia dracunculus for a variety of diseases are closely related to its anti-inflammatory mechanism.This article will summarize the relevant literature in recent years and review the research progress of the mechanism of Artemisia dracunculus on the regulation of immune cells and inflammatory factors,as well as its mediation of inflammation-related signaling pathways,intending to provide theoretical references for future application of Artemisia dracunculus in clinical trials and treatments.
7.Do HBV DNA-negative HBsAg-positive patients with compensated hepatitis B cirrhosis need antiviral therapy?
Li SU ; Jinghang XU ; Yaomin LIU ; Guomin ZHANG ; Yuting GUO ; Guiqiang WANG
Journal of Clinical Hepatology 2023;39(1):37-42
Hepatitis B virus (HBV) infection is a common cause of liver disease in China, and with the continuous progress in the research on antiviral therapy for chronic hepatitis B, the indications for antiviral therapy are constantly expanding. However, there are still controversies over the indications for antiviral therapy in patients with chronic hepatitis B (CHB), especially those with negative HBV. By analyzing the limitations of HBV DNA detection, the risk of HBV reactivation in HBV-negative CHB patients, the risk of disease progression in the DNA-negative population with compensated hepatitis B cirrhosis, antiviral response, and the economic benefits of antiviral therapy, this article proposes the necessity of antiviral therapy for HBV-negative HBsAg-positive patients with compensated hepatitis B cirrhosis.
8.Clinical treatment guideline for pulmonary blast injury (version 2023)
Zhiming SONG ; Junhua GUO ; Jianming CHEN ; Jing ZHONG ; Yan DOU ; Jiarong MENG ; Guomin ZHANG ; Guodong LIU ; Huaping LIANG ; Hezhong CHEN ; Shuogui XU ; Yufeng ZHANG ; Zhinong WANG ; Daixing ZHONG ; Tao JIANG ; Zhiqiang XUE ; Feihu ZHOU ; Zhixin LIANG ; Yang LIU ; Xu WU ; Kaican CAI ; Yi SHEN ; Yong SONG ; Xiaoli YUAN ; Enwu XU ; Yifeng ZHENG ; Shumin WANG ; Erping XI ; Shengsheng YANG ; Wenke CAI ; Yu CHEN ; Qingxin LI ; Zhiqiang ZOU ; Chang SU ; Hongwei SHANG ; Jiangxing XU ; Yongjing LIU ; Qianjin WANG ; Xiaodong WEI ; Guoan XU ; Gaofeng LIU ; Junhui LUO ; Qinghua LI ; Bin SONG ; Ming GUO ; Chen HUANG ; Xunyu XU ; Yuanrong TU ; Liling ZHENG ; Mingke DUAN ; Renping WAN ; Tengbo YU ; Hai YU ; Yanmei ZHAO ; Yuping WEI ; Jin ZHANG ; Hua GUO ; Jianxin JIANG ; Lianyang ZHANG ; Yunfeng YI
Chinese Journal of Trauma 2023;39(12):1057-1069
Pulmonary blast injury has become the main type of trauma in modern warfare, characterized by externally mild injuries but internally severe injuries, rapid disease progression, and a high rate of early death. The injury is complicated in clinical practice, often with multiple and compound injuries. Currently, there is a lack of effective protective materials, accurate injury detection instrument and portable monitoring and transportation equipment, standardized clinical treatment guidelines in various medical centers, and evidence-based guidelines at home and abroad, resulting in a high mortality in clinlcal practice. Therefore, the Trauma Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Trauma organized military and civilian experts in related fields such as thoracic surgery and traumatic surgery to jointly develop the Clinical treatment guideline for pulmonary blast injury ( version 2023) by combining evidence for effectiveness and clinical first-line treatment experience. This guideline provided 16 recommended opinions surrounding definition, characteristics, pre-hospital diagnosis and treatment, and in-hospital treatment of pulmonary blast injury, hoping to provide a basis for the clinical treatment in hospitals at different levels.
9.Treatment of NLRP3 gene mutation associated autoimmune diseases with kanamycin: a report of 4 cases with literature review
Xiaoliang HE ; Yuqing CHEN ; Li SUN ; Guomin LI ; Haimei LIU ; Daliang XU ; Denghuan CHEN ; Yutong GAO ; Yang SHENG ; Shouwei HANG
Chinese Journal of Rheumatology 2023;27(11):740-745
Objective:Four cases with NLRP3-related autoinflammatory diseases were reported to summarize the clinical characteristics, genotype, and treatment responses of the disease, and to improve clinical pediatricians' understanding of the disease.Methods:A retrospective analysis was performed on 4 cases with NLRP3-related autoinflammatory diseases diagnosed in Children's Hospital of Anhui Province in 2016—2021, and the clinical features and treatment progress of NLRP3-related autoinflammatory diseases were retrospectively analyzed based on the clinical features, gene reports, and literature review.Results:① All 4 cases were male. Cases 1, 2, and 3 had the disease onset after birth, and case 4 had the disease onset 6 months after birth. All showed periodic fever, repeated urticaria-like rash, protruding forehead, and saddle nose. White blood cells count, erythrocyte sedimentation rate, and C-reactive protein were increased during the attack period, and those in the interval period were normal, and antibiotic treatment was ineffective. ② The genetic test of all these 4 children showed NLRP3 mutation. Children 1, 2, and 3 were heterozygous mutations, and their parents were wild-type. The mutation was located at chromosome Chr1: 247587658, exon c913 (exon3). G>A, the 305th aspartic acid (Asp) of the protein was changed to asparagine (Asn) in child 1. The mutation was located at the chromosomal Chr1: 247588072, the nucleic acid was changed to c1327(exon3)T>C, and the amino acid was changed to p.Y443H in cases 2 and 3. Somatic heterozygous mutation was found in case 4, and the child's parents were wild-type. In this case, the mutation was located at chromosomal Chr1: 247587658, exon3 G>A, and the 305th Asp of the protein was changed to Asn. ③Children in cases 1, 2, and 3 were treated with glucocorticoids and non-steroidal anti-inflammatory drugs at the initial stage, but the effects were limited. After receiving IL-1 antagonist treatment fever, skin rash, joint swelling and pain disappeared, and the inflammatory indexes were returned to normal. The child 4 received non-steroidal anti-inflammatory drugs and methotrexate, but he failed to respond to the treatment. Treatment with tocilizumab was not effective, however, fever, skin rash, or joint pain disappeared after treated with Khanna.Conclusion:①NLRP3-related autoinflammatory diseases can cause periodic fever, urticaria, joint involvement, and severe involvement of the central nervous system and organ amyloidosis. Which are early misdiagnosis is prone to systemic juvenile idiopathic arthritis. ②The disease was an inflammatory disease mediated by interleukin-1. At present, non-steroidal anti-inflammatory drug, glucocorticoid and chronic anti-rheumatic drugs have limited effects. IL-1 antagonists are effective and safe in the treatment of the disease.
10.Network pharmacology and molecular docking analysis on molecular targets and mechanism prediction of Huanglian Jiedu Decoction in the treatment of COVID-19
XU Xinyi ; LIU Leping ; CAO Xueshuai ; LONG Xi ; PENG Sujuan ; ZHANG Guomin
Digital Chinese Medicine 2022;5(1):18-32
Objective To investigate and predict the molecular targets and mechanism of Huanglian Jiedu Decoction (黄连解毒汤, HLJDD) in the treatment of Corona Virus Disease 2019 (COVID-19) through network pharmacology and molecular docking analysis. Methods The chemical constituents and action targets of HLJDD were retrieved on Traditional Chinese Medicine Systems Pharmacology Database and Analysis Platform (TCMSP), SymMap v2, Encyclopedia of Traditional Chinese Medicine (ETCM), a High-throughput Experiment- and Reference-guided Database of Traditional Chinese Medicine (HERB), and Traditional Chinese Medicine Integrated Database (TCMID). UniProt and GeneCards were used to query the target genes that corresponding to the active compounds, and then a compound-target network was constructed using Cytoscape 3.7.2. Gene Ontology (GO) database was used to annotate GO functions. Kyoto Encyclopedia of Genes and Genomes (KEGG) was used to predict the possible mechanisms of active compounds. The Database for Annotation, Visualization and Integrated Discovery (DAVID) was used to analysis the tissue enrichment. The main active compounds in HLJDD are molecularly docked with their corresponding related targets. Results Seventy-six compounds were screened and 458 corresponding targets in the network were obtained. Gene annotation showed that the targets were involved mainly in 1 953 biological processes. 884 signaling pathways was enriched, involving signaling by interleukins, cytokine signaling in immune system, generic transcription pathway, and RNA polymerase II transcription. The targets mainly distributed in the lung, liver, and placenta, involving a variety of immune cells, such as T cells and B cells. The molecular docking results showed that core compounds such as wogonin, berberine, and baicalein had high affinity with tumor necrosis factor (TNF), insulin (INS), and tumor protein 53 (TP53). Conclusion The active compounds in HLJDD may have a therapeutic effect on COVID-19 through regulating multiple signal pathways by targeting genes such as vascular endothelial growth factor A (VEGFA), INS, interleukin-6 (IL-6), TNF, caspase-3 , TP53, and mitogen-activated protein kinase 3 (MAPK3).

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