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.

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.

Objective:To assess the predictive performance of the risk of cardiovascular diseases (CVD) derived from the China Kadoorie Biobank (CKB-CVD) model, prediction for atherosclerotic cardiovascular disease (ASCVD) risk in China (China-PAR) model, and the risk of fatal and nonfatal ischemic cardiovascular diseases derived from the USA-People′s Republic of China Collaborative Study (USA-PRC) model in Chinese Multi-provincial Cohort Study (CMCS).Methods:In this prospective cohort study, a total of 21 948 individuals aged ≥35 years without CVD were selected from 8 provinces and cities in China during the CMCS survey from 1992 to 2005 for 10-year follow-up. The occurrence of CVD or ASCVD events during the follow-up period was used as the gold standard. The CKB-CVD and China-PAR models were used to calculate the predicted risk of CVD events, while the USA-PRC model was used to calculate the predicted risk of ASCVD events. The discrimination of the models was evaluated using the C-statistic, and the calibration was assessed using the Hosmer-Lemeshow χ2 test and decile plot. Results:During the 10-year follow-up, a total of 955 (4.4%) CVD events, including 791 (3.6%) ASCVD events, were recorded among the study participants. The C-index for the CKB-CVD, China-PAR, and USA-PRC models were 0.775 (95% CI: 0.757-0.793), 0.781 (95% CI: 0.763-0.798), and 0.769 (95% CI: 0.750-0.789) for men, and 0.762 (95% CI: 0.737-0.788), 0.769 (95% CI: 0.745-0.794), and 0.767 (95% CI: 0.741-0.794) for women, respectively. China-PAR model showed good calibration for men ( χ2=2.20), however, both CKB-CVD and USA-PRC models demonstrated poor calibration in both men and women ( χ2>20). The results indicated that the CKB-CVD model overestimated the risk of CVD events in both males and females, while the China-PAR model underestimated the risk in females. Furthermore, the USA-PRC model underestimated the risk of ASCVD in both males and females in most decile groups, but overestimated the risk in the highest decile group. Conclusion:The CKB-CVD, China-PAR, and USA-PRC risk assessment models show some degree of deviation from the actual risk of events in the CMCS cohort, but all exhibit good discrimination.

OBJECTIVE：To preliminarily investigate the improveme nt effects and mechanism of Mongolian medicine Saorilao-4 decoction on specific pulmonary fibrosis model rats. METHODS ：Male SD rats were randomly divided into normal control group ，model group ，positive control group （pirfenidone，0.163 g/kg）and Saorilao- 4 decoction low ，medium and high dose groups （0.899，1.798，3.596 g/kg），8 rats in each group. Except for normal control group ，other groups were given 6 mg/mL bleomycin intratracheally at 5 mg/kg once to induce the specific pulmonary fibrosis model. From the first day after modeling ， normal control group and model group were given normal saline intragastrically ，other groups were given corresponding drugs intragastrically，once a day ，10 mL/kg，for 4 weeks. During the experimental period ，the general condition of the rats in each group was observed and the body mass was weighed. Twenty-four h after last medication ，the appearance morphology of rat l ung in each group were observed. The morphological characteristics of lung tissues were observed by HE and Masson staining. ELISA was adopted to determine the activity of SOD and the content of MDA in serum ，the contents of hydroxyproline （HYP），IL-1β，IL-6，hyaluronidase（HA），laminin（LN）precollagen type Ⅲ（PC-Ⅲ）and collagen type Ⅳ（Col-Ⅳ）in lung tissue. RT-PCR was used to determine the mRNA 发。E-mail：bwf007007@sina.com expression of TGF-β 1，Smad3 and Smad 7 in lung tissue. RESULTS：Compared with model group ，the activity ，hair and diet of the rats in each dose group of Saorilao- 4 decoction and positive control group were significant ly improved ，and the body mass after the last administration was significantly increased ； the pathological change of lung and pulmonary fibrosis were significantly improved ，and the activity of SOD in serum was increased significantly. Serum content of MDA （except for Saorilao- 4 decoction medium dose group ），the contents of HYP （except for Saorilao- 4 decoction high dose group ），IL-1β，IL-6，HA，LN，PC-Ⅲ，Col-Ⅳ（except for Saorilao- 4 decoction high dose group）as well as mRNA expression of TGF-β1 and Smad 3 in lung tissue were significantly decreased ；mRNA expression of Smad 7 was significantly increased （P＜0.05 or P＜0.01）. CONCLUSIONS ：Saorilao-4 decoction can significantly improve the lung pathological changes ，delay and reverse the progression of pulmonary fibrosis in specific pulmonary fibrosis model rats ，the mechanism of which may be related to the inhibition of inflammatory response ， improvement of lipid peroxidation ， down-regulation of TGF-β1 and Smad 3 mRNA expression ，and up-regulation of Smad 7 mRNA expression.

Objective: To discuss the use of butterfly costal cartilage as columella strut graft. Methods: On the costal cartilage that being cut, butterfly graft was designed length of 2.8 cm, upper and lower end thickness of 5 mm, middle part thickness of 1.5 mm. The lower end was designed with a groove about 1 cm in length, the width of the lower end of the butterfly graft was determined according to the degree of retraction at the caudal end of the septum, then insert to the nasal crest. The posterior end of two lamella grafts was sutured and fixed with the middle part of the nasal columnar strut graft. Results: From November 2016 to March 2018, about 150 patients underwent rhinoplasty with this method, follow-up was 3 to 12 months. Two cases had mild nasal tip deviation one month after surgery and were adjusted with auricular cartilage three months later. Three patients had mild postoperative hyporotation and adjustment three months postoperatively, residual had good shape. Conclusions The stability and controllability of the sphenoid columella strut graft are good, out of shape not easily, It is a worthy clinical method.