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.

Systemic lupus erythematosus（SLE）is an autoimmune disease affecting multiple organs and systems，and acute pancreatitis（AP）is a rare，life-threatening complication of SLE.Early manifestations of pediatric SLE-related acute pancreatitis（SLEAP）lack specificity，which is easy to be misdiagnosed and missed，difficult to treat and poor in prognosis.Understanding the pathogenesis，clinical characteristics，diagnosis and treatment of pediatric SLEAP is of great significance to control the disease and improve the prognosis.This article reviews the latest research progress of pediatric SLEAP，in order to help pediatricians in the diagnosis and treatment of pediatric SLEAP.

Objective To explore the clinical efficacy and medical costs of high ligation and stripping of the great saphenous vein,simple foam sclerotherapy,and high ligation of the great saphenous vein combined with foam sclerotherapy for treating varicose of great saphenous vein.Methods The clinical data of patients with varicose of great saphenous vein undergoing high ligation and stripping of the great saphenous vein,simple foam sclerotherapy,or high ligation of the great saphenous vein combined with foam sclerotherapy were analyzed retrospectively.The evaluation included efficacy assessment,quality of life evaluation,hospitalization days and costs,postoperative complications,and recurrence after surgery.Results There were no statistical differences in gender,age,disease course and clinical etiology anatomy pathophysiology(CEAP)grading system among the three groups(P＞0.05).The clinical efficacy assessments in all patients among the three groups showed significant effects at 3 and 6 months postoperatively.One patient in the group treated with high ligation of the great saphenous vein combined with foam sclerotherapy suffered deep vein thrombosis of lower extremity after operation.In terms of quality of life,there was no statistically significant difference in venous clinical severity score(VCSS)among the three groups at 3 months postoperatively(P＞0.05).The group treated with simple foam sclerotherapy showed significantly shorter hospitalization days compared with the other two groups.Additionally,the group treated with simple foam sclerotherapy showed lower hospitalization expenses,which was not statistically significant when compared to the group treated with high ligation and stripping of the great saphenous vein group(P＞0.05),but there was a statistical difference compared to the group treated with high ligation of the great saphenous vein combined with foam sclerotherapy(P＜0.05).Conclusion There are no significant differences in clinical efficacy,postoperative pain,and quality of life evaluation among the three methods.However,simple foam scle-rotherapy may reduce the length of hospitalization stay and direct medical costs.

Objective To explore the clinical efficacy and medical costs of high ligation and stripping of the great saphenous vein,simple foam sclerotherapy,and high ligation of the great saphenous vein combined with foam sclerotherapy for treating varicose of great saphenous vein.Methods The clinical data of patients with varicose of great saphenous vein undergoing high ligation and stripping of the great saphenous vein,simple foam sclerotherapy,or high ligation of the great saphenous vein combined with foam sclerotherapy were analyzed retrospectively.The evaluation included efficacy assessment,quality of life evaluation,hospitalization days and costs,postoperative complications,and recurrence after surgery.Results There were no statistical differences in gender,age,disease course and clinical etiology anatomy pathophysiology(CEAP)grading system among the three groups(P＞0.05).The clinical efficacy assessments in all patients among the three groups showed significant effects at 3 and 6 months postoperatively.One patient in the group treated with high ligation of the great saphenous vein combined with foam sclerotherapy suffered deep vein thrombosis of lower extremity after operation.In terms of quality of life,there was no statistically significant difference in venous clinical severity score(VCSS)among the three groups at 3 months postoperatively(P＞0.05).The group treated with simple foam sclerotherapy showed significantly shorter hospitalization days compared with the other two groups.Additionally,the group treated with simple foam sclerotherapy showed lower hospitalization expenses,which was not statistically significant when compared to the group treated with high ligation and stripping of the great saphenous vein group(P＞0.05),but there was a statistical difference compared to the group treated with high ligation of the great saphenous vein combined with foam sclerotherapy(P＜0.05).Conclusion There are no significant differences in clinical efficacy,postoperative pain,and quality of life evaluation among the three methods.However,simple foam scle-rotherapy may reduce the length of hospitalization stay and direct medical costs.

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.

Enthesitis related arthritis（ERA）is one of the subtypes of juvenile idiopathic arthritis（JIA）. It is a group of diseases characterized by arthritis，inflammation，axial lesions，and human leucocyte antigen-B27 positive. In the previous classification criteria，ERA does not include all of the subsets of juvenile spondyloarthritis（JSpA）. Based on an evidence-based approach，the Pediatric Rheumatology International trials Organization developed the latest classification criteria in 2019，and proposed the classification criteria of ERA/SpA，to distinguish it from other subtypes of JIA. The new definition added the term spondylitis，included imaging criteria，and adopted a new definition of back pain. The pathogenesis，evolution of classification criteria and prognosis of spondyloarthritis are described in this paper，in order to improve the level of diagnosis and treatment of patients with axial joint involvement，so as to improve the prognosis.

Objective:To investigate the safety of tocilizumab (TCZ) in the treatment of children with systemic juvenile idiopathic arthritis (sJIA).Methods:Data of children aged 2 to 18 years with the diagnosis of sJIA and treated with TCZ from June 1, 2017 to June 30, 2022 at our hospital were retrospectively collected. The clinical medication characteristics, incidence, severity and outcome of adverse drug reactions (ADR) were statistically analyzed. Univariate and multivariate analysis were used to analyze the risk factors of TCZ-induced ADR. Univariate comparison between groups were compared to the measured data followed by t test for normal distribution, and the counting data were paired with Chi-square test. Binary logistic regression analysis was used for multivariate analysis. Results:A total of 83 eligible children were enrolled. The age at TCZ initiation was (8.5±3.7) years old. Most of the children received oral glucocorticoid (86.8%) and/or methotrexate (72.3%) prior to TCZ treatment. The mean time of TCZ duration was (1.2±0.9) years, the total TCZ exposure was 92.70 patient years. Fifty-five (66.3%) children reported 123 ADR, with a rate of 132.69/100 patient years. Forty-two (50.6%) children reported 103 general ADR, with a rate of 111.11/100 patient years. Eighteen (21.7%) children reported 20 serious ADR, with a rate of 21.57/100 patient years. The results of univariate analysis showed that the dosage of glucocorticoid in ADR group was higher than that in non-ADR group [(0.76±0.50) mg·kg -1·d -1vs. (0.52±0.41) mg·kg -1·d -1, t=2.27, P=0.026], and the difference was statistically significant. However, there were no significant differences in gender [(male 23, female 32) cases vs. (male 9, female 19) cases, χ2=0.73, P=0.392], age at TCZ initiation [(8.5±3.8) years old vs. (9.0±3.1) years old, t=-0.65, P=0.516], duration of TCZ treatment [(1.24±1.00) years vs. (1.05±0.90) years, t=0.87, P=0.385], methotrexate doses weekly [(8.0±5.2) mg/m 2vs. (7.6±5.1) mg/m 2, t=0.39, P=0.696], and history of drug or food allergy (11 cases vs. 5 cases, χ2=0.06, P=0.815) between the two groups. The results of binary logistic regression analysis showed that the combined use of oral glucocorticoids was an independent risk factor for TCZ-induced ADR [ OR (95% CI) =3.05 (1.11, 8.36), P=0.030]. The risk of ADR was 3.05 times higher in the combined daily dose of glucocorticoids ≥0.76 mg/kg prednisone equivalent than that of < 0.76 mg/kg. Common general ADR to TCZ include infections (38.83/100 patient years) and abnormalities in laboratory parameters (37.76/100 patient years) such as elevated glutamic-pyrupiane transaminase (18.34/100 patient years), dyslipidemia (12.94/100 patient years), and hemocytopenia (5.39/100 patient years). The serious ADR included serious infection (9.71/100 patient years) and serious infusion reaction(7.55/100 patient years). All ADR were improved after drug withdrawal or symptomatic treatment, and no deaths occurred. Conclusion:TCZ has a good safety profile in the treatment of sJIA. Serious infections and severe infusion reactions often lead to discontinuation of the drug. The combination of glucocorticoids≥0.76 mg/kg prednisone equivalent is an independent risk factor for TCZ-induced ADR. Monitoring should be strengthened during the application of TCZ, and ADR should be detected and treated as early as possible to reduce the risk of medication related adverse reactions.

Objective:To investigate the efficacy of a proximal femoral locking plate (LPFP) versus a proximal femoral anti-rotation intramedullary nail (PFNA) in the treatment of femoral intertrochanteric fractures in older adult patients. Methods:A total of 130 older adult patients with femoral intertrochanteric fractures who received treatment in Linghu People's Hospital of Huzhou from May 2017 to June 2020 were included in this study. They were randomly assigned to undergo treatment with either a PFNA (observation group, n = 65) or an LPFP (control group, n = 65). Intraoperative blood loss, incision length, operative time, and time to fracture healing were determined in each group. At 1, 3, and 6 months after surgery, the Harris hip score was used to evaluate hip joint recovery. Coxa vara, incision infection, and internal fixation loosening were compared between the two groups. Results:Intraoperative blood loss in the observation group was less than that in the control group [(189.26 ± 48.15) mL vs. (96.47 ± 40.21) mL, t = -11.93, P < 0.001]. Incision length, operative time, and time to fracture healing in the observation group were significantly shorter than those in the control group [(4.03 ± 1.48) cm vs. (12.16 ± 1.55) cm, (72.13 ± 28.75) minutes vs. (120.34 ± 29.01) minutes, (9.89 ± 1.52) weeks vs. (13.63 ± 1.74) weeks, t = -30.59, -9.52, -13.05, all P < 0.001]. At 1 month after surgery, there was no significant difference in Harris hip score between the two groups ( t = 1.28, P > 0.05). At 3 and 6 months after surgery, the Harris hip score gradually increased in the control and observation groups ( F = 13.44, 8.26, both P < 0.001). At 3 and 6 months after surgery, Harris hip scores in the observation group were significantly higher than those in the control group [(85.17 ± 4.29) points vs. (79.50 ± 4.12) points, (95.30 ± 1.04) points vs. (87.69 ± 1.25) points, t = 7.69, 37.73, both P < 0.001]. The incidence of complications in the observation group was significantly lower than that in the control group [1.54% (1/65) vs. 10.77% (7/65), χ2 = 4.80, P = 0.029). Conclusion:Compared with LPFP, PFNA can effectively reduce intraoperative blood loss in older adult patients with femoral intertrochanteric fractures, accelerate the progress of fracture healing, promote the recovery of the hip joint, and has fewer complications. Therefore, PFNA is worthy of popularization.

Objective:To analyze the in-hospital mortality and influential factors of total hip arthroplasty (THR) in older adult patients.Methods:A total of 130 older adult patients subjected to THR in Department of Orthopedics, Huzhou Linghu People's Hospital between August 2019 and August 2021 were included in this study. In-hospital mortality was calculated. These patients were divided into death and survival groups according to whether they were dead or alive. Sex, age, smoking, drinking, disease type, complications, cardiovascular disease history, operation severity score, operative time, amount of intraoperative blood loss, postoperative osteoporosis treatment and postoperative rehabilitation training were compared between the two groups. The risk factors that influence in-hospital mortality were analyzed using logistic analysis method.Results:Among 130 patients, 9 patients died in Huzhou Linghu People's Hospital, with the mortality of 6.92%. Thus, there were 9 patients in the death group and 121 patients in the survival group. There were significant differences in age, smoking, complications, operation severity score and postoperative osteoporosis treatment between the two groups ( t = 1.70, χ2 = 5.48, χ2 = 4.09, t = 2.86, χ2 = 4.03, all P < 0.05). Multivariate logistic analysis showed that the age (≥ 85 years old), smoking (yes), complications (≥ 3), operation severity score (≥ 15 points) were the risk factors that influence in-hospital mortality. Postoperative osteoporosis treatment was the protective factor of THR. Conclusion:The in-hospital mortality of older adult patients after THR is high. Age ≥ 85 years old, smoking, complications ≥ 3, and operation severity score ≥ 15 are the risk factors of THR and may greatly affect the prognosis. More attention should be paid to older adult patients who have these risk factors.

Objective:To analyze the clinical characteristics and risk factors of juvenile dermatomyositis (JDM) with relapses by comparing clinical features, treatment and disease course among JDM patients with and without relapses.Methods:A retrospective analysis of 102 JDM patients from Children's Hospital of Nanjing Medical University between March 2017 and March 2021 was carried out. Patients were divided into two groups based on whether a JDM relapse had occurred or not. Initial clinical features, laboratory tests and treatment were compared between the two groups. T-test or Mann-Whitney U test was used for measurement data, chi-square test or fisher exact probability was used for count data. The features associated with risk of relapses were analyzed by multivariate logistic regression. Results:Among 102 children with JDM, twenty patients (19.6%) relapsed during drug reduction or after drug withdrawal. The mean duration to the first relapse was 3.24 years (range: 9 months to 7 years). Myositis specific antibodies (MSA) were positive for 8 (40.0%) patients with relapses. With 5 cases were anti-nuclear matrix protein 2 positive, 2 cases were anti-transcription interme-diary factor 1 gamma positive, 1 case was anti-signal recognition particle (SRP) positive, the other 12 cases were MSA negative. By binary logistic regression analysis, we found that peripheral calcinosis [ OR(95% CI)=17.54(1.55, 198.64), P=0.021], and interstitial lung disease [ OR(95% CI)=3.83(1.27, 11.59), P=0.017] were independently related to JDM with relapses. Fifty-three patients (51.9%) received methylpre-dnisolone pulse therapy for initial treatment and 13 (65.0%) patients with relapses received methylprednisolone pulse for initial treatment. There was no significant difference between the two groups ( χ2=1.70 , P=0.193). Tumor necrosis factor alpha antagonist combined with methotrexate (MTX) had achieved good results in clinical treatment in children with relapses. Conclusion:The risk of relapses is high in children with JDM. Calcinosis and interstitial lung disease at disease onset can predict a relapsing disease course. Aggressive treatment is urgently demanded for patients with JDM, especially those with relapses.