1.Development and exploration of a closed-loop management model for externally dispensed intravenous prescriptions
Xuhua XIE ; Yun WU ; Songqing HUANG ; Yukun HUANG ; Siyan CHEN ; Zheng ZENG ; Weiyan TANG ; Zuolong HE ; Chunxia ZHOU ; Hongliang ZHANG
China Pharmacy 2026;37(10):1246-1250
OBJECTIVE To construct a closed-loop management model for externally dispensed intravenous prescriptions, and to provide reference for standardized management of externally dispensed intravenous prescriptions. METHODS Based on the Expert Consensus on Closed-loop Management of Externally Dispensed Intravenous Prescriptions in Guangxi Zhuang Autonomous Region previously formulated by our hospital, risk points during the entire process were systematically identified through multidisciplinary team brainstorming and a fishbone diagram. A series of strategies were subsequently formulated and implemented, including qualifying designated external dispensing pharmacies and the drug catalogs, operating and maintaining the hospital information system and the Pharmacy Intravenous Admixture Service (PIVAS) intelligent management platform, and strengthening differentiated training for staff in the whole workflow. A whole-process closed-loop management system was constructed with PIVAS as the co re hub and the daytime chemotherapy center as the safety terminal. RESULTS A total of 3 cooperating pharmacies and an initial drug list comprising 35 product specifications were selected. A closed‑loop management process encompassing hospital outpatient prescribing, patient drug purchase in designated pharmacies, PIVAS drug dispensing, and medication use in daytime chemotherapy center was successfully established. This system enabled the mandatory grouping and association of externally dispensed intravenous prescriptions with in-hospital diluents, full-process verification based on drug traceability codes, intelligent monitoring of infusion parameters, and whole-process data traceability. CONCLUSIONS The constructed model effectively resolves the coordination and safety oversight during the use of externally dispensed intravenous drugs from out-of-hospital circulation to in-hospital use, and has preliminarily enabled procedural standardization, whole-process information traceability, and proactive control of medication risks.
2.Luoshi Neiyi Prescription Treats Endometriosis Through TLR4/NF-κB Signaling Pathway
Yuanyuan RUAN ; Sai XU ; Jiangyue TANG ; Xiang LI ; You ZOU ; Fangli PEI ; Lizheng WU ; Kaidi ZHENG ; Shuhong LIN ; Weilan ZHONG ; Cheng ZENG
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(19):185-196
ObjectiveTo investigate the mechanism by which Luoshi Neiyi prescription treats endometriosis (EMs) through the Toll-like receptor 4 (TLR4)/nuclear factor-κB (NF-κB) signaling pathway. MethodsAnimal experiments were conducted with 50 female SD rats, which were randomized into a sham operation group (10 rats) and a modeling group (40 rats). An autologous endometrial transplantation method was used for the modeling of EMs. The 36 successfully modeled rats were randomly allocated into four groups (n=9 each): The model group, the low-dose (7.87 g·kg-1) Luoshi Neiyi prescription group, the high-dose (15.74 g·kg-1) Luoshi Neiyi prescription group, and the dienogest (0.20 mg·kg-1) group. The physiological status and ectopic lesion volumes of rats in each group were observed. Hematoxylin-eosin (HE) staining was used to observe the morphology of the eutopic endometrial tissue. Enzyme-linked immunosorbent assay (ELISA) was employed to measure the levels of inflammatory factors including interleukin-1β (IL-1β) and prostaglandin E2 (PGE2) in the serum of EMs rats. Immunohistochemistry was used to detect the expression of matrix metalloproteinase-9 (MMP-9) and vascular endothelial growth factor A (VEGFA) in the eutopic endometrial tissue. Western blot was adopted to determine the protein levels of TLR4, myeloid differentiation factor 88 (MyD88), phosphorylated nuclear factor-κB (p-NF-κB)/NF-κB, MMP-9, and VEGFA in the eutopic endometrial tissue. In the cell experiments, the cell-counting kit-8 (CCK-8) assay was employed to screen the optimal concentration of Luoshi Neiyi prescription-containing serum, and a scratch assay was performed to assess the migration ability of iheESCs cells. Interventions with Luoshi Neiyi prescription-containing serum, resatorvid (TAK-242, a TLR4 inhibitor), and lipopolysaccharides (LPS, a TLR4 agonist) were conducted, and Western blot was used to detect the expression of proteins related to the TLR4/NF-κB signaling pathway. ResultsThis experiment successfully replicated 36 EMs rat models. Compared with the sham operation group, the model group exhibited visible ectopic lesions on the abdominal wall and an increase in the writhing response score (P<0.01). Furthermore, HE staining revealed the model group exhibited a thickened eutopic epithelium, stromal cell disarrangement, and evident infiltration of inflammatory cells. In addition, the model group showed elevated serum levels of IL-1β and PGE2 (P<0.05, P<0.01), increased positive expression of MMP-9 and VEGFA in the ectopic endometrial tissue (P<0.01), and up-regulated protein levels of TLR4, MyD88, p-NF-κB/NF-κB, MMP-9, and VEGFA in the ectopic endometrial tissue (P<0.05, P<0.01). Compared with the model group, all treatment groups exhibited a reduction in the ectopic lesion volume (P<0.01). Furthermore, the writhing response scores were decreased in the high-dose Luoshi Neiyi prescription group and the dienogest group (P<0.05, P<0.01). The pathological state of the ectopic endometrial tissue was alleviated to varying degrees in the treatment groups. Low-dose Luoshi Neiyi prescription reduced serum PGE2 levels, and high-dose Luoshi Neiyi prescription and dienogest decreased serum IL-1β and PGE2 levels in EMs rats (P<0.05, P<0.01). The treatment groups showed decreased positive expression of MMP-9 and VEGFA in the ectopic endometrial tissue (P<0.05, P<0.01) and down-regulated protein levels of TLR4, MyD88, p-NF-κB/NF-κB, MMP-9, and VEGFA in the ectopic endometrial tissue (P<0.05, P<0.01). The cell experiments showed that 5%, 10%, and 20% Luoshi Neiyi prescription-containing sera significantly reduced the viability and inhibited the migration of iheESCs. Compared with the control group, 5%, 10%, and 20% Luoshi Neiyi prescription-containing serum groups and the TAK-242 group showed reduced protein levels of TLR4, MyD88, p-NF-κB/NF-κB, MMP-9, and VEGFA (P<0.01). Compared with the control group, the LPS group showed increased expression of the above proteins (P<0.01). Compared with the LPS group, the LPS+5%, 10%, and 20% Luoshi Neiyi prescription-containing serum groups showed reduced expression of the above proteins (P<0.05, P<0.01). ConclusionLuoshi Neiyi prescription may modulate the TLR4/NF-κB signaling pathway to reduce the inflammatory response and histopathological damage in the eutopic endometrium and suppress the adhesive, invasive, and angiogenic biological processes in the ectopic endometrial tissue, thereby exerting its therapeutic effect on EMs.
3.Analysis of abnormal ALT in blood donors in five Zang autonomous prefectures of Qinghai Province, China: characteristics and screening strategies
Yingnan DANG ; ; Rong TANG ; Liqin HUANG ; Hailin WU ; Tingting CHEN ; Shengju LI ; Yanli SUN ; Xin ZHENG ; Yanxia LI ; Xianlin YE ; Jinfeng ZENG
Chinese Journal of Blood Transfusion 2025;38(4):502-507
[Objective] To investigate the factors associated with alanine aminotransferase (ALT) abnormalities in multi-ethnic blood donors across five Zang autonomous prefectures in the plateau regions of Qinghai Province, and to provide evidence for ensuring blood safety and formulating screening strategies. [Methods] A retrospective analysis was performed on the ALT abnormal test results of blood donors in the Zang autonomous prefectures of Qinghai from 2022 to 2024. The correlations between ALT levels and factors including gender, age, altitude, and infectious markers were investigated. [Results] The overall ALT unqualified rate among blood donors in this region was 9.01%. Significant differences in ALT levels were observed across genders and age groups (P<0.05). Variations in ALT abnormality rates were also noted among different plateau regions (P<0.05). Overall, ALT values exhibited an increasing trend with rising altitude. The average ALT unqualified rates were 11.19% in Zang donors, 7.96% in Han donors, and 4.79% in donors from other ethnic groups (P<0.05). No statistically significant association was observed between ALT abnormality and the presence of HBV/HCV infectious markers (P>0.05). [Conclusion] In the plateau areas of Qinghai, multi-ethnic blood donors have a relatively high ALT levels and ALT unqualified rates, showing distinct regional characteristics. ALT elevation in voluntary blood donors is related to non-pathological factors such as gender, age, and dietary habits, but not to infectious indicators.
4.Combined anterior and posterior miniscrews increase apical root resorption of maxillary incisors in protrusion and premolar extraction cases
Zhizun WANG ; Li MEI ; Zhenxing TANG ; Dong WU ; Yue ZHOU ; Ehab A. ABDULGHANI ; Yuan LI ; Wei ZHENG ; Yu LI
The Korean Journal of Orthodontics 2025;55(1):26-36
Objective:
Miniscrews are commonly utilized as temporary anchorage devices (TADs) in cases of maxillary protrusion and premolar extraction. This study aimed to investigate the effects and potential side effects of two conventional miniscrew configurations on the maxillary incisors.
Methods:
Eighty-two adult patients with maxillary dentoalveolar protrusion who had undergone bilateral first premolar extraction were retrospectively divided into three groups: non-TAD, two posterior miniscrews only (P-TADs), and two anterior and two posterior miniscrews combined (AP-TADs). Cone-beam computed tomography was used to evaluate the maxillary central incisors (U1).
Results:
The APTADs group had significantly greater U1 intrusion (1.99 ± 2.37 mm, n = 50) and less retroclination (1.70° ± 8.80°) compared to the P-TADs (–0.07 ± 1.65 mm and 9.45° ± 10.68°, n = 60) and non-TAD group (0.30 ± 1.61 mm and 1.91° ± 9.39°, n = 54).However, the AP-TADs group suffered from significantly greater apical root resorption (ARR) of U1 (2.69 ± 1.38 mm) than the P-TADs (1.63 ± 1.46 mm) and non-TAD group (0.89 ± 0.97 mm). Notably, the incidence of grade IV ARR was 16.6% in the AP-TADs group, significantly higher than the rates observed in the P-TADs (6.7%) and non-TAD (1.9%) groups. Multiple regression analysis revealed that after excluding tooth movement factors, the AP-TADs configuration resulted in an additional 0.5 mm of ARR compared with the P-TADs group.
Conclusions
In cases of maxillary protrusion and premolar extraction, the use of combined anterior and posterior miniscrews enhances incisor intrusion and minimizes torque loss of the maxillary incisors. However, this approach results in more severe ARR, likely due to the increased apical movement and composite force exerted.
5.Multi-omics identification of microvascular endothelium-related genes in patients with diabetic organic erectile dysfunction
Lijuan WU ; Weizhuo WANG ; Kang CHEN ; Zheng TANG ; Kai FU ; Mingyan TANG ; Wanglei YANG
National Journal of Andrology 2025;31(11):972-978
Objective To integrate patient clinical information with single-cell sequencing analysis to identify key genes involved in organic erectile dysfunction(ED)in diabetic patients,and to further validate these findings using genome-wide association study(GWAS)data to pinpoint the genes associated with diabetic organic ED.Methods Single-cell RNA sequen-cing data were downloaded from the GSE206528 dataset,comprising samples from five patients including three individuals with-out ED or diabetes,and two patients diagnosed with diabetic ED.Data preprocessing,cell clustering,and annotation were per-formed using R,followed by extraction of microvascular endothelial cells for differential expression analysis.Enrichment analysis of the identified differentially expressed genes(DEGs)was conducted using the Hiplot platform.Expression quantitative trait loci(eQTL)single nucleotide polymorphisms(SNPs)corresponding to these DEGs were retrieved from the UK Biobank(UKB)da-tabase as exposures.ED(GWAS ID:ebi-a-GCST006956)was defined as the outcome variable.Mendelian randomization(MR)analysis was then performed to identify potential causal genes.Results Using the Seurat package,single-cell RNA se-quencing data from the five patients underwent quality control and integration.After cell type identification,a subset of microvas-cular endothelial cells was selected for differential expression analysis,resulting in the identification of 214 DEGs.Functional enrichment analysis revealed that these genes were significantly enriched in pathways related to diabetic complications,including the AGE-RAGE signaling pathway,TNF signaling pathway,and oxidative phosphorylation.Subsequently,MR analysis was per-formed on the 214 DEGs,using erectile dysfunction(ebi-a-GCST006956)as the outcome.Six genes were identified as potential causal genes including MYL9,NFIB,ENDOD1,DES,NRARP and HSPA1B.Conclusion These findings suggest that MYL9,NFIB,ENDOD1,DES,NRARP and HSPA1B may play a role in the progression of organic ED in diabetic patients.
6.Population Characteristics of Arsenic-containing TCM Compounds in the Treatment of Platelets in Myelodysplastic Syndrome
Jian LIU ; Wenru WANG ; Peizhen JIANG ; Kaizhi LU ; Qinlong ZHENG ; Haixia DI ; Lijuan YAO ; Bing WU ; Jiangwei WAN ; Qifeng LIU ; Ruibai LI ; Xudong TANG
Chinese Journal of Information on Traditional Chinese Medicine 2025;32(9):154-160
Objective To compare the differences of baseline characteristics of patients with myelodysplastic syndrome(MDS)who achieved platelet(PLT)response after arsenic-containing TCM compounds combined with Western medicine treatment.Methods Totally 72 MDS patients were selected from 12 outpatient departments and wards,such as Xiyuan Hospital,China Academy of Chinese Medical Sciences,Dongfang Hospital,Beijing University of Chinese Medicine from October 2021 to October 2024.Among them,45 patients received arsenic-containing TCM compounds combined with Western medicine treatment,27 patients received Western medicine treatment.The blood routine[white blood cell(WBC)count,hemoglobin,PLT,neutrophil count],TCM syndrome scores,safety indicators,and adverse events were observed before and after three courses of treatment.The efficacy of all patients was evaluated,and the baseline characteristics of patients who achieved PLT response in the arsenic-containing TCM compounds group and the Western medicine treatment group were compared.Results Comparing the differences of baseline characteristics of the two groups,it was found that the patients who achieved PLT response in the arsenic-containing TCM compounds group were compared with those in the Western medicine treatment group:Age<60 years old(P=0.038),longer disease duration(P=0.012),lower WBC(P=0.017),lower reticulocyte percentage(P=0.037),lower blood urea nitrogen(P=0.046),lower high-density lipoprotein cholesterol(P=0.014),and lower N-terminal pro-B-type natriuretic peptide(P=0.034),abnormal electrocardiogram(P=0.013),high blasts(P=0.009),grade 0 reticular fiber staining(P<0.01),normal chromosome karyotype(P<0.01),gene mutation(P<0.01)and high TCM syndrome scores(P=0.013)were found.Conclusion Arsenic-containing TCM compounds consisting of Qinghuang Powder and Bushen Jianpi Decoction combined with Western medicine is used to treat MDS.Patients with age<60 years old,long disease duration,low WBC count,low reticulocyte percentage,low blood urea nitrogen,low high-density lipoprotein cholesterol,low N-terminal pro-B-type natriuretic peptide,abnormal electrocardiogram,high blasts,grade 0 reticular fiber staining,normal chromosome karyotype,gene mutation and high TCM syndrome score are more likely to obtain PLT response.
7.Evidence-based guideline for diagnosis and early fixation of severe open tibiofibular fractures (version 2025)
Yongjun RUI ; Yongqing XU ; Qingtang ZHU ; Xin WANG ; Zhao XIE ; Shanlin CHEN ; Jingyi MI ; Xianyou ZHENG ; Juyu TANG ; Xiaoheng DING ; Aixi YU ; Tao SONG ; Jianxi HOU ; Jian QI ; Xinyu FAN ; Jun FEI ; Lin GUO ; Xingwen HAN ; Weixu LI ; Aiguo WANG ; Yun XIE ; Tao XING ; Meng LI ; Baoqing YU ; Yan ZHUANG ; Xiaoqing HE ; Tao SUN ; Pengcheng LI ; Jihui JU ; Hongxiang ZHOU ; Haidong REN ; Guangyue ZHAO ; Gang ZHAO ; Yongwei WU ; Jun LIU ; Yunhong MA ; Yapeng WANG
Chinese Journal of Trauma 2025;41(11):1021-1034
Severe open tibiofibular fractures account for approximately 28.1% of all open fractures. Among them, Gustilo-Anderson type IIIB/C fractures present significant clinical challenges due to associated bone and soft tissue defects, high infection rates, and risk of amputation. Inadequate preoperative assessment may lead to suboptimal emergency surgical planning or intraoperative complications. Historically, external fixation was often preferred, but this approach has been associated with limitations such as restricted joint mobility, delayed bone union, joint stiffness, and disuse osteoporosis, resulting in poor functional recovery. With advancements of debridement techniques, standardization of antibiotic use, and popularization of early soft tissue coverage, early internal fixation has gained broader acceptance. Nevertheless, controversies persist regarding the choice of fixation method, timing of definitive fixation, use of reamed versus unreamed intramedullary nailing, and necessity of fibular fixation. To standardize the diagnosis and early management of severe open tibiofibular fractures, reduce complication rates, and improve functional recovery, the Society of Microsurgery of the Chinese Medical Association organized a panel of domestic experts to develop the Evidence-based guideline for the diagnosis and early fixation of severe open tibiofibular fractures ( version 2025), using evidence-based methodology. The guidelines provided 12 recommendations covering diagnostic and early fixation strategies of severe open tibiofibular fractures, aiming to provide clinicians with scientifically grounded and standardized guidance.
8.Percutaneous coronary intervention vs . medical therapy in patients on dialysis with coronary artery disease in China.
Enmin XIE ; Yaxin WU ; Zixiang YE ; Yong HE ; Hesong ZENG ; Jianfang LUO ; Mulei CHEN ; Wenyue PANG ; Yanmin XU ; Chuanyu GAO ; Xiaogang GUO ; Lin CAI ; Qingwei JI ; Yining YANG ; Di WU ; Yiqiang YUAN ; Jing WAN ; Yuliang MA ; Jun ZHANG ; Zhimin DU ; Qing YANG ; Jinsong CHENG ; Chunhua DING ; Xiang MA ; Chunlin YIN ; Zeyuan FAN ; Qiang TANG ; Yue LI ; Lihua SUN ; Chengzhi LU ; Jufang CHI ; Zhuhua YAO ; Yanxiang GAO ; Changan YU ; Jingyi REN ; Jingang ZHENG
Chinese Medical Journal 2025;138(3):301-310
BACKGROUND:
The available evidence regarding the benefits of percutaneous coronary intervention (PCI) on patients receiving dialysis with coronary artery disease (CAD) is limited and inconsistent. This study aimed to evaluate the association between PCI and clinical outcomes as compared with medical therapy alone in patients undergoing dialysis with CAD in China.
METHODS:
This multicenter, retrospective study was conducted in 30 tertiary medical centers across 12 provinces in China from January 2015 to June 2021 to include patients on dialysis with CAD. The primary outcome was major adverse cardiovascular events (MACE), defined as a composite of cardiovascular death, non-fatal myocardial infarction, and non-fatal stroke. Secondary outcomes included all-cause death, the individual components of MACE, and Bleeding Academic Research Consortium criteria types 2, 3, or 5 bleeding. Multivariable Cox proportional hazard models were used to assess the association between PCI and outcomes. Inverse probability of treatment weighting (IPTW) and propensity score matching (PSM) were performed to account for potential between-group differences.
RESULTS:
Of the 1146 patients on dialysis with significant CAD, 821 (71.6%) underwent PCI. After a median follow-up of 23.0 months, PCI was associated with a 43.0% significantly lower risk for MACE (33.9% [ n = 278] vs . 43.7% [ n = 142]; adjusted hazards ratio 0.57, 95% confidence interval 0.45-0.71), along with a slightly increased risk for bleeding outcomes that did not reach statistical significance (11.1% vs . 8.3%; adjusted hazards ratio 1.31, 95% confidence interval, 0.82-2.11). Furthermore, PCI was associated with a significant reduction in all-cause and cardiovascular mortalities. Subgroup analysis did not modify the association of PCI with patient outcomes. These primary findings were consistent across IPTW, PSM, and competing risk analyses.
CONCLUSION
This study indicated that PCI in patients on dialysis with CAD was significantly associated with lower MACE and mortality when comparing with those with medical therapy alone, albeit with a slightly increased risk for bleeding events that did not reach statistical significance.
Humans
;
Percutaneous Coronary Intervention/methods*
;
Male
;
Female
;
Coronary Artery Disease/drug therapy*
;
Retrospective Studies
;
Renal Dialysis/methods*
;
Middle Aged
;
Aged
;
China
;
Proportional Hazards Models
;
Treatment Outcome
9.Impact of posterior cruciate ligament resection on the elasticity of the periarticular soft tissue sleeve in the knee joint.
Yun-Feng ZHANG ; De-Jin YANG ; Zhao-Lun WANG ; Yi-Xin ZHOU ; Hao TANG ; Xiang-Dong WU ; Han-Long ZHENG
China Journal of Orthopaedics and Traumatology 2025;38(10):1055-1060
OBJECTIVE:
To evaluate the effects of posterior cruciate ligament(PCL) resection on soft tissue elasticity and knee stability in total knee arthroplasty(TKA).
METHODS:
Six adult cadaveric knee specimens (involving 10 knees) were included in the study. With the assistance of the robotic system(TiRobot Recon, TINAVI, Beijing), total knee arthroplasty (TKA) was performed sequentially using cruciate retaining (CR) prostheses and posterior stabilizing (PS) prostheses. Between the two surgical procedures, the femoral and tibial osteotomy surfaces were not altered;only the posterior cruciate ligament (PCL) was resected and the intercondylar fossa was treated. After installing the femoral trial component, a soft tissue balance solver was used to apply tension ranging from 30 N to 90 N in 5 N increments at 0°, 10°, and 90° of knee flexion. Meanwhile, the medial and lateral joint gaps were measured synchronously. Based on the tension-gap coupling data, the equivalent elastic coefficients of the medial and lateral soft tissue sleeves at different knee flexion angles, as well as the range of the joint line convergence angle (JLCA) under fixed varus-valgus stress, were calculated. Additionally, the gap balance status under 80 N of tension was analyzed. Self-control comparisons of each indicator were conducted before and after PCL resection to analyze the change patterns.
RESULTS:
After PCL resection, in the fully extended position (knee flexion 0°). The medial equivalent elastic coefficient was 32.2 (25.7, 63.3) N·mm-1 for the CR prosthesis and 27.7 (22.0, 51.9) N·mm-1 for the PS prosthesis, and the statistically significant difference (P=0.013). The range of JLCA was 0.41°(0.26, 0.55)° for the CR prosthesis, which was smaller than 0.75° (0.40, 0.98)° for the PS prosthesis, and the difference was statistically significant(P=0.041). At 90° of knee flexion, the medial joint gap was 10.7(10.1, 11.7) mm for the CR prosthesis, which was smaller than 12.1(10.9, 15.1) mm for the PS prosthesis, with a statistically significant difference(P=0.011). No statistically significant differences were observed in other joint gaps.
CONCLUSION
PCL resection reduces the rigidity of the medial soft tissues in the fully extended knee and increases the medial joint gap in the flexed position, thereby affecting knee stability and balance. This finding suggests that PS and CR prostheses may require different morphological designs, and there should be differences in indications and osteotomy strategies between CR-TKA and PS-TKA. CR-TKA is more suitable for patients with preoperative medial soft tissue laxity.
Humans
;
Posterior Cruciate Ligament/physiopathology*
;
Knee Joint/physiopathology*
;
Arthroplasty, Replacement, Knee
;
Elasticity
;
Male
;
Female
;
Middle Aged
;
Aged
;
Biomechanical Phenomena
;
Adult
10.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.

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