1.Research progress on the chemical constituents,pharmacological mechanisms and clinical application of Jiegeng decoction
Yun HUANG ; Shunwang HUANG ; Jinwei QIAO ; Qian XU ; Xiaoming GAO ; Xuemei BAO ; Manqin YANG ; Ruonan XIE ; Ming CAI
China Pharmacy 2025;36(18):2348-2352
Jiegeng decoction is a classic prescription composed of two Chinese medicinal herbs: Platycodon grandiflorum and Glycyrrhiza uralensis. It has the efficacy of diffusing lung qi, resolving phlegm, relieving sore throat and discharging pus, and is commonly used in the treatment of respiratory diseases such as cough and pharyngodynia. This article reviews the chemical components, pharmacological mechanisms and clinical applications of Jiegeng decoction. It was found that Jiegeng decoction contains triterpenoid saponins, flavonoids, glycosides, acids, and other components, with platycodin D, platycodin D2, glycyrrhizic acid, glycyrrhetinic acid, liquiritin, etc., serving as the main active pharmaceutical ingredients. Jiegeng decoction and its chemical constituents exert anti-inflammatory effects by inhibiting signaling pathways such as nuclear factor-κB and mitogen- activated protein kinases, and demonstrate anti-tumor activities through mechanisms like modulating the tumor immune microenvironment and promoting cancer cell apoptosis. Additionally, it exhibits various pharmacological actions including antibacterial, antiviral, and antioxidant effects. Clinically, Jiegeng decoction, its modified prescription and compound combinations are widely used in the treatment of respiratory diseases such as cough, pneumonia, and pharyngitis, as well as digestive system disorders like constipation.
2.Effects of Buyang Huanwu Decoction Regulating Cav-1/Notch1/Hes1 Signaling Pathway on Nerve Regeneration after Cerebral Ischemia in Mice
Fanzuo ZENG ; Xuemei CHEN ; Yin OUYANG ; Bowei CHEN ; Jian YI ; Yaqian XU ; Wanling NING ; Baiyan LIU
Chinese Journal of Information on Traditional Chinese Medicine 2025;32(5):114-120
Objective To investigate the effects of Buyang Huanwu Decoction on nerve regeneration after cerebral ischemia in mice based on Cav-1/Notch1/Hes1 signaling pathway.Methods Wild(WT)and Cav-1-/-(KO)male mice were randomly divided into sham-operation group,model group and Buyang Huanwu Decoction group,with 10 mice in each group.The middle cerebral artery occlusion method was used to construct the mouse cerebral ischemia model.After modeling,5-ethynyl-2'-deoxyuridine(EdU)was injected intraperitoneally every 7 days for 3 times(with an interval of 8 hours).Buyang Huanwu Decoction group was given 18.5 g/kg Buyang Huanwu Decoction by gavage daily,while the sham-operation group and model group were given distilled water of equal volume by gavage for 21 consecutive days.The neurological function of mice was evaluated using modified neurological severity score,brain tissue morphology was observed through HE staining,nerve regeneration in the ischemic side injury area was detected using dual immunofluorescence staining,Western blot was used to detect the expressions of Notch1 and Hes1 protein in ischemic cortical area.Results Compared with the same genotype sham-operation group,the neurological function score of the mice in the WT and KO model groups significantly increased(P<0.01),the arrangement of neurons in the ischemic cortical area was disordered,with nucleoli shrinking,marginalization,and even rupture,widened intercellular spaces,intracellular edema and vacuolar changes,the co-localization of EdU/Nestin,EdU/DCX and EdU/NeuN in the ischemic side injury area significantly increased(P<0.01),and the expression of Notch1 and Hes1 protein in ischemic cortical area significantly increased(P<0.01).Compared with the same genotype model group,the neurological function score of the WT and KO Buyang Huanwu Decoction group significantly decreased(P<0.01),the neurons in the ischemic cortical area were arranged neatly and structurally intact,with reduced intercellular space and clear nucleoli,the co-localization of EdU/Nestin,EdU/DCX and EdU/NeuN in the ischemic side injury area was significantly increased(P<0.01),and the expression of Notch1 and Hes1 protein in ischemic cortical area were significantly decreased(P<0.01).Compared with the corresponding WT group,the neurological function scores of mice in the KO model group and KO Buyang Huanwu Decoction group significantly increased(P<0.01),the neurons in the ischemic cortical area had more severe nucleolar condensation,marginalization and rupture,with more vacuolar changes and ischemic injury,the co-localization of EdU/Nestin and EdU/NeuN in the ischemic side injury area was significantly decreased(P<0.01),and the expression of Notch1 and Hes1 proteins in ischemic cortical area significantly increased(P<0.01).Conclusion Buyang Huanwu Decoction can promote nerve regeneration after cerebral ischemia,which may be related to the regulation of Cav-1 thereby inhibition Notch1//Hes1 signaling pathway activity.
3.Study on relationship between qSOFA score, serum procalcitonin, lactic acid and hypersensitive C-reactive protein levels and with the prognosis of SAP patients
Jun LIU ; Yao CHEN ; Xuemei XU ; Shijie YU
Clinical Medicine of China 2025;41(6):452-458
Objective:To investigate the relationship between the quick sequential organ failure assessment (qSOFA) score, procalcitonin (PCT), blood lactate, hypersensitive C-reactive protein (hs-CRP) levels, and the prognosis of patients with severe acute pancreatitis (SAP).Methods:This retrospective analysis included 225 pancreatitis cases admitted to the Department of Gastroenterology at Ganzi Tibetan Autonomous Prefecture People's Hospital between February 2020 and February 2025. Patients were categorized into three groups based on severity: SAP group ( n=125), moderately severe acute pancreatitis (MSAP) group ( n=50), and mild acute pancreatitis (MAP) group ( n=50). General patient data, qSOFA scores, PCT, lactate, and hs-CRP levels were compared among the groups. Within the SAP group, patients were further divided into a favorable prognosis group ( n=101) and a poor prognosis group ( n=24) based on 28-day survival. The relationship between qSOFA score, PCT, lactate, hs-CRP, and SAP prognosis was analyzed. Normally distributed continuous data were expressed as Mean±SD. Independent samples t-test was used on comparison between two groups, while one-way ANOVA was used on comparison among multiple groups. LSD test was used on post-hoc pairwise comparison. Categorical data were expressed as n(%) and compared by χ2 test. Spearman correlation analysis assessed the correlation between qSOFA score, PCT, lactate, hs-CRP levels, and acute pancreatitis (AP) severity. Multivariable logistic regression modeling was used for joint prediction, incorporating qSOFA score, serum PCT, lactate, and hs-CRP levels to calculate combined predictive efficacy. The predictive performance of each indicator for prognosis was evaluated using receiver operating characteristic (ROC) curves. The areas under the ROC curves (AUC) were compared using DeLong's test. Results:Significant differences were observed among the MAP, MSAP, and SAP groups for qSOFA score [(0.63±0.29), (1.32±0.37), (1.70±0.33) points], serum PCT [(4.06±0.74), (5.35±0.89), (6.12±0.98) μg/L], lactate [(1.74±0.32), (2.77±0.70), (4.29±1.06) mmol/L], and hs-CRP levels [(15.38±3.75), (27.64±5.09), (35.26±6.42) mg/L] ( F values=187.51, 91.83, 169.18, 223.92, respectively, all P<0.001). The SAP group had significantly higher levels of qSOFA score, serum PCT, lactate, and hs-CRP compared to both the MAP and MSAP groups (all P<0.05), and the MSAP group levels were significantly higher than the MAP group (all P<0.05). The poor prognosis group had significantly higher levels of qSOFA score [(1.92±0.19) points], PCT [(6.65±0.47) μg/L], lactate [(5.14±0.62) mmol/L], and hs-CRP [(40.65±4.73) mg/L] compared to the favorable prognosis group [(1.65±0.25) points, (5.99±0.84) μg/L, (4.09±0.94) mmol/L, (34.16±6.09) mg/L] ( t values=4.96, 3.71, 5.20, 4.88, respectively, all P<0.001). Multivariate logistic regression analysis identified qSOFA score ( OR=14.658, 95% CI: 2.612-82.253), PCT ( OR=2.809, 95% CI: 1.288-6.129), lactate ( OR=3.658, 95% CI: 1.635-8.187), and hs-CRP ( OR=1.168, 95% CI: 1.055-1.293) as independent influencing factors for SAP patient prognosis ( P=0.002, 0.010, 0.002, 0.003, respectively). ROC curve analysis showed that the AUCs for qSOFA score, PCT, lactate, and hs-CRP in predicting poor prognosis in SAP patients were 0.754, 0.856, 0.766, and 0.809, with sensitivities of 87.50%, 83.33%, 83.33%, 85.50%, and specificities of 60.40%, 77.20%, 64.40%, 75.20%, respectively. The combined prediction model yielded an AUC of 0.934, with a sensitivity of 95.80% and specificity of 72.30%. Conclusion:The qSOFA score, PCT, blood lactate, and hs-CRP levels are associated with the severity of AP and possess reference value for predicting the prognosis of SAP patients.
4.Investigation of Helicobacter pylori infection and analysis of risk factors in permanent residents in a certain area
Xuemei XU ; Jun LIU ; Lamei HAN ; Danni LU ; Ting HE
Journal of Public Health and Preventive Medicine 2025;36(2):78-81
Objective To analyze the status and risk factors of Helicobacter pylori (Hp) infection in permanent residents in a certain area. Methods The clinical data of 6 792 permanent residents surveyed from January 2021 to December 2023 were retrospectively analyzed. All subjects underwent 13C-urea breath test,and relevant general information was collected to analyze Hp infection status. According to whether Hp infection occurred, they were divided into positive group (n=4 283) and negative group (n=2 509). The differences in general information, living habits, and dietary habits between the two groups of subjects were analyzed, and multivariate logistic regression analysis was conducted. Results Among the 6 792 permanent residents surveyed from January 2021 to December 2023, 4283 were positive for 13C-urea breath test, accounting for 63.05% of the total. There were statistically significant differences in age distribution, gender, BMI, tableware cleaning, personal hygiene products use, chopsticks use, and raw food and vegetable cleaning between the positive group and the negative group (P<0.05). The single factors of Hp infection were substituted into multivariate logistic regression analysis equation, and it was found that age ≥45 years old, male, BMI≥24, no use of detergents to clean utensils, sharing personal hygiene products, not using public chopsticks, having a habit of eating raw food, and not cleaning vegetables before eating were independent risk factors for Hp infection. Conclusion The positive rate of Hp infection in this area is relatively high, and the infection factors are related to age, gender, and some lifestyle and dietary habits.
5.Analysis and forecast of the disease burden of schistosomiasis in China from 1992 to 2030
Kai LIN ; Chenhuan ZHANG ; Zhendong XU ; Xuemei LI ; Renzhan HUANG ; Yawen LIU ; Haihang YU ; Lisi GU
Chinese Journal of Schistosomiasis Control 2025;37(1):24-34
Objective To analyze the trends in the disease burden of schistosomiasis in China from 1992 to 2021, and to project the disease burden of schistosomiasis in China from 2022 to 2030, so as to provide insights into the elimination of schistosomiasis in China. Methods The prevalence, age-standardized prevalence, disability-adjusted life year (DALYs) rate and age-standardized DALYs rate of schistosomiasis, as well as the years lost due to disability (YLDs) rate and age-standardized YLDs rate of anemia attributable to Schistosoma infections in China, the world and different socio-demographic index (SDI) regions were captured from the Global Burden of Disease Study 2021 (GBD 2021) data resources, and the trends in the disease burden due to schistosomiasis were evaluated with estimated annual percentage change (EAPC) and its 95% confidence interval (CI). In addition, the age, period and cohort effects on the prevalence of schistosomiasis were examined in China using an age-period-cohort (APC) model, and the disease burden of schistosomiasis was predicted in China from 2022 to 2030 using a Bayesian age-period-cohort (BAPC) model. Results The age-standardized prevalence and DALYs rate of schistosomiasis, and the age-standardized YLDs rate of anemia attributable to Schistosoma infections were 761.32/105, 5.55/105 and 0.38/105 in China in 2021. These rates were all lower than the global levels (1 914.30/105, 21.90/105 and 3.36/105, respectively), as well as those in the medium SDI regions (1 413.61/105, 12.10/105 and 1.93/105, respectively), low-medium SDI regions (2 461.03/105, 26.81/105 and 4.48/105, respectively), and low SDI regions (5 832.77/105, 94.48/105 and 10.65/105, respectively), but higher than those in the high SDI regions (59.47/105, 0.49/105 and 0.05/105, respectively) and high-medium SDI regions (123.11/105, 1.20/105 and 0.12/105, respectively). The prevalence and DALYs rate of schistosomiasis were higher among men (820.79/105 and 5.86/105, respectively) than among women (697.96/105 and 5.23/105, respectively) in China in 2021, while the YLDs rate of anemia attributable to Schistosoma infections was higher among women (0.66/105) than among men (0.12/105). The prevalence of schistosomiasis peaked at ages of 30 to 34 years among both men and women, while the DALYs rate of schistosomiasis peaked among men at ages of 15 to 19 years and among women at ages of 20 to 24 years. The age-standardized prevalence of schistosomiasis showed a moderate decline in China from 1992 to 2021 relative to different SDI regions [EAPC = -1.51%, 95% CI: (-1.65%, -1.38%)], while the age-standardized DALYs rate [EAPC = -3.61%, 95% CI: (-3.90%, -3.33%)] and age-standardized YLDs rate of anemia attributable to Schistosoma infections [EAPC = -4.16%, 95% CI: (-4.38%, -3.94%)] appeared the fastest decline in China from1992 to 2021 relative to different SDI regions. APC modeling showed age, period, and cohort effects on the trends in the prevalence of schistosomiasis in China from 1992 to 2021, and the prevalence of schistosomiasis appeared a rise followed by decline with age, and reduced with period and cohort. BAPC modeling revealed that the age-standardized prevalence and age-standardized DALYs rate of schistosomiasis, and age-standardized YLDs rate of anemia attributable to Schistosoma infections all appeared a tendency towards a decline in China from 2022 to 2030, which reduced to 722.72/105 [95% CI: (538.74/105, 906.68/105)], 5.19/105 [95% CI: (3.54/105, 6.84/105)] and 0.30/105 [95% CI: (0.21/105, 0.39/105)] in 2030, respectively. Conclusions The disease burden of schistosomiasis appeared a tendency towards a decline in China from 1992 to 2021, and is projected to appear a tendency towards a decline from 2022 to 2030. There are age, period and cohort effects on the prevalence of schistosomiasis in China. Precision schistosomiasis control is required with adaptations to current prevalence and elimination needs.
6.Novel outpatient infusion model of blinatumomab: case studies of two patients
Guijun LI ; Xuemei JIANG ; Xin WANG ; Qiuxia XU ; Jianhui LI ; Susi DAI ; Ying HE ; Hai YI ; Dan CHEN
Chinese Journal of Blood Transfusion 2025;38(4):557-561
[Objective] To evaluate the feasibility of a novel outpatient infusion model for blinatumomab in two acute lymphoblastic leukemia (ALL) patients, aiming to address challenges of poor treatment tolerance, high healthcare costs, and compromised quality of life, thereby providing clinical insights for broader adoption of this approach. [Methods] Two post-allogeneic hematopoietic stem cell transplantation (allo-HSCT) patients undergoing blinatumomab maintenance therapy were selected to evaluate the efficacy of the outpatient infusion model. Patient selection criteria, nursing protocols, standardized workflows, and advancements in infusion practices were systematically analyzed combined with a review of global developments in this field. [Results] Both patients completed outpatient blinatumomab infusion without severe adverse events, demonstrating preliminary feasibility and safety of this model. The novel approach enhanced treatment convenience, reduced hospitalization costs, and improved quality of life. [Conclusion] Despite the limited sample size, this pilot study highlights the potential of outpatient blinatumomab administration as a viable alternative to traditional inpatient regimens.
7.Effect of hand transmitted vibration on fingertip thermotactile perception threshold in grinding workers
Danying ZHANG ; Maosheng YAN ; Hansheng LIN ; Sizhi CHEN ; Xuemei SONG ; Bin XIAO ; Hua YAN ; Guoyong XU ; Zhipeng HE
China Occupational Medicine 2025;52(5):540-544
Objective To analyze the effect of hand transmitted vibration on fingertip thermotactile perception threshold (TPT) among grinding workers in a sports equipment manufacturing enterprise. Methods A total of 151 male grinding workers from a sports equipment manufacturing enterprise in Guangdong Province were enrolled in the vibration exposure group, and 51 male workers without hand-transmitted vibration exposure were enrolled in the control group, using the judgment sampling method. Basic health conditions of the workers were surveyed in both groups. The TPTs of the distal phalanges of the index and little fingers on both hands were tested using an HVLab thermal perception tester. Results The detection rates of finger numbness and tingling among workers in the vibration exposure group were higher than those in the control group (92.1% vs 7.8% and 59.6% vs 0.0%, respectively, both P<0.01). The detection rates of numbness or tingling of different fingers in the vibration exposure group descended in the following order: index finger, middle finger, ring finger, thumb and little finger. The hot threshold of the index finger and little finger increased (all P<0.01), while the cold threshold decreased (all P<0.01) in the vibration exposure group, compared with the control group. The results of generalized linear regression analysis showed that with the increase of the duration of hand-transmitted vibration, the hot threshold of the index finger and the little finger of both hands increased (all P<0.01), and the cold threshold decreased (all P<0.01). The hot thresholds of workers with numbness of both the index fingers and left little finger were higher than those of workers without numbness of the same finger (all P<0.05). The hot threshold of workers with tingling of the left index finger was higher than those without tingling (P<0.05). The cold thresholds of workers with tingling of the both index fingers and left little finger were lower than those without tingling of the same finger (all P<0.05). The hot threshold of the right little finger increased with age (P<0.01), while the cold thresholds of both little fingers decreased with age (all P<0.01). Conclusion Hand-transmitted vibration exposure increases fingertip hot threshold and decreases fingertip cold threshold of workers exposed to hand-transmitted vibration. Years of service in hand-transmitted vibration work, finger numbness or tingling, and age were influencing factors of TPT. Fingertip TPT examination can be used to assist in the early detection of vibration-induced nerve injury in workers exposed to hand-transmitted vibration.
8.Comparison of efficacy and safety of crisaborole ointment 2% versus pimecrolimus cream 1% in the treatment of mild to moderate atopic dermatitis in children: a multicenter, randomized, controlled clinical trial
Xing XIAO ; Shan WANG ; Huan YANG ; Hong SHU ; Yanping GUO ; Jinping CHEN ; Yao LU ; Qinfeng LI ; Yuan LIANG ; Mutong ZHAO ; Xiaoyan LUO ; Limin MIAO ; Rui XU ; Xuemei LI ; Sha LAI ; Jianhong LI ; Zhen LUO ; Lu YU ; Lu XING ; Meitan WANG ; Xiaoli LI ; Haitao XU ; Ping LI ; Hua WANG ; Lin MA
Chinese Journal of Dermatology 2025;58(5):425-430
Objective:To compare the efficacy and safety of crisaborole ointment 2% versus pimecrolimus cream 1% in the treatment of mild to moderate atopic dermatitis in children aged 2 years or older.Methods:A multicenter, randomized, open-label, controlled clinical trial was conducted. A total of 120 pediatric patients aged 2 - 17 years with mild to moderate atopic dermatitis were enrolled from departments of dermatology of 8 hospitals in China between March 2022 and February 2023. The participants were randomly assigned in a 1∶1 ratio to the crisaborole group and the pimecrolimus group, and received the treatment with crisaborole ointment 2% and pimecrolimus cream 1% respectively, twice a day for 4 weeks. Visits were scheduled at baseline/on day 1, as well as on days 8, 15, and 29. The primary efficacy outcome was the percentage of patients achieving the Investigator's Static Global Assessment (ISGA) success (defined as clear [0] or almost clear [1] on the ISGA scale, combined with ≥ 2‐grade improvement from baseline) on day 29. The secondary efficacy outcomes included changes in the Eczema Area and Severity Index (EASI) total scores from baseline to day 29, percentages of patients achieving ISGA improvement (defined as clear [0] or almost clear [1] on the ISGA scale), as well as changes in the Peak Pruritus Numerical Rating Scale (NRS) scores, Dermatology Life Quality Index (DLQI) /Infants' Dermatology Life Quality Index (IDLQI) /Children's Dermatology Life Quality Index (CDLQI) scores, and in the Dermatitis Family Impact (DFI) scores. Drug safety was evaluated according to the incidence of adverse events. Categorical data were compared using the chi-square test. Since measurement data did not follow a normal distribution, the rank sum test was used for comparisons of measurement data between groups.Results:A total of 106 children with mild to moderate atopic dermatitis were included in the per-protocol analysis set, with 52 in the crisaborole group (26 males and 26 females) and 54 in the pimecrolimus group (27 males and 27 females). There were no significant differences in age, disease duration, ISGA and EASI scores at baseline between the two groups (all P > 0.05). On day 29, 22 patients (42.31%) in the crisaborole group and 25 (46.30%) in the pimecrolimus group achieved ISGA success, with no significant difference between the two groups ( χ2 = 0.17, P = 0.68) ; 35 patients (67.31%) in the crisaborole group and 45 (83.33%) in the pimecrolimus group achieved ISGA improvement, also with no significant difference between the two groups ( χ2 = 3.68, P = 0.06) ; additionally, there were no significant differences in the EASI, pruritus NRS, DLQI/IDLQI/CDLQI, or DFI scores between the two groups (all P > 0.05). Adverse reactions to the two topical agents were mainly local reactions such as mild to moderate pain, itching, or worsening of itching, and no obvious systemic adverse reactions occurred. The incidence of drug-related adverse reactions was 46.15% (24 cases) in the crisaborole group and 37.04% (20 cases) in the pimecrolimus group, with no significant difference between the two groups ( χ2 = 0.91, P = 0.34) . Conclusion:The efficacy of crisaborole ointment 2% was comparable to that of pimecrolimus cream 1% in the treatment of mild to moderate atopic dermatitis in children aged ≥ 2 years, and it yielded early and rapid improvement in the quality of life of patients and their families, with good safety and tolerability profiles.
9.Role of cancer-associated fibroblasts autophagy in papillary thyroid cancer
Xuemei ZHANG ; Danyang SUN ; Ning LI ; Qicheng ZHANG ; Ke XU ; Wei ZHENG ; Qiang JIA ; Jian TAN ; Zhaowei MENG
Chinese Journal of Endocrinology and Metabolism 2025;41(2):135-144
Objective:To investigate the inpact of thyroid cancer-derived cancer-associated fibroblasts(CAF) autophagy on papillary thyroid cancer(PTC).Methods:CAF and normal fibroblasts were isolated from cancerous and adjacent normal thyroid tissues from four PTC patients. Expressions of fibroblast activation protein(FAP) and α-smooth muscle actin in cells were assessed. Conditioned medium of CAF and normal fibroblasts were prepared and used to culture PTC cells. The effects of CAF and normal fibroblasts on survival, proliferation, migration, invasion and iodine uptake of PTC cells were evaluated through cell proliferation assay, cell scratch assay, cell invasion assay, and cell iodine uptake assay. The autophagy level of CAF was also evaluated. Autophagy inhibition and activation were used to regulate the autophagy of CAF, and then their effects on PTC cell proliferation, migration and invasion were further evaluated. The in vivo effect of CAF autophagy on PTC xenograft tumor growth was evaluated.Results:CAF exhibited higher FAP expression and basal autophagy levels. PTC cells co-cultured with CAF-conditioned media showed enhanced proliferation, migration, invasion, and reduced iodine uptake. Autophagy inhibition reduced these effects, while autophagy activation further promoted them. In vivo, inhibiting CAF autophagy suppressed tumor growth.Conclusions:CAF promotes PTC cell malignancy through autophagy activation, enhancing proliferation, migration, and invasion while reducing iodine uptake.
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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