Objective:To explore the diagnostic value of the macrophage activation syndrome/systemic juvenile idiopathic arthritis（MS）score in macrophage activation syndrome（MAS）associated with systemic juvenile idiopathic arthritis（sJIA），and to provide a reference for clinical work.Methods:This study was a retrospective case-control analysis，conducted on the patients initially diagnosed as sJIA-associated with MAS and admitted into the Department of Rheumatology and Immunology of Children's Hospital Affiliated to Xi 'an Jiaotong University from July 1st，2016 to June 30th，2023. All of the patients met the diagnostic criteria for patients with MAS associated with sJIA according to the 2016 European Alliance of Associations for Rheumatology（EULAR）/American College of Rheumatology（ACR）/Pediatric Rheumatology International Trials Organization（PRINTO）standards. The basic information at baseline，clinical manifestations，and auxiliary examination results were collected. The MS score was applied to re-evaluate the children diagnosed as sJIA-associated with MAS. When the MS score ≥-2.1，the possibility of sJIA with MAS was high. Thirty cases of sJIA without MAS were randomly selected as the control group.Results:There were 28 cases in the MAS group，including 13 males（46.43%）and 15 females（53.57%），with an average age of（7.51±4.01）years. Compared with the control group，the MAS group were significantly more likely to have high fever（ χ2=8.539， P=0.003），hepatomegaly（ χ2=11.621， P<0.001），splenomegaly（ χ2=11.710， P<0.001）and neurological involvement（ χ2=27.619， P<0.001），with the differences being statistically significant. Meanwhile，there were statistically significant differences between the two groups in terms of white blood cell count（ Z=-4.001， P<0.001），neutrophil count（ Z=-3.659， P<0.001），platelet count（ Z=-4.687， P<0.001），albumin level（ Z=-4.018， P<0.001），alanine aminotransferase（ Z=-3.846， P<0.001），aspartate aminotransferase（ Z=-5.932， P<0.001），lactate dehydrogenase（ Z=-6.150， P<0.001），triglycerides（ Z=-5.874， P<0.001），fibrinogen（ Z=-5.808， P<0.001），ferritin（ Z=-5.280， P<0.001），erythrocyte sedimentation rate（ Z=-3.971， P<0.001），ferritin/erythrocyte sedimentation rate（ Z=-5.433， P<0.001），reduction of two-line cells in blood（ χ2=11.408， P<0.001）and the presence of hemophagocytosis in bone marrow smears（ χ2=28.260， P<0.001）. Moreover，there was a statistically significant difference in MS scores between the two groups（ Z=-6.148， P<0.001），with higher MS scores in the MAS group. Nevertheless，this study showed the median MS scores of both groups ≥-2.1. Conclusion:The MS score was significant to a certain degree as reference for the diagnosis of MAS，and this study showed that the MS score in the MAS group was significantly higher than the control group. However，the median MS scores in both groups were no less than -2.1. This might be related to the influence of factors during the assessment，which made it necessary to optimize the cutoff values of the MS score. Therefore，prospective studies should be carried out on the role of MS score in early identification of MAS.

Objective:To explore the potential risk factors of recurrence in patients with systemic juvenile idiopathic arthritis (sJIA).Methods:We enrolled patients with sJIA admitted to the Department of Rheumatology and Immunology, Xi′an Children′s Hospital affiliated to Xi′an Jiaotong University from January 2018 to June 2024. Cox proportional hazard models were used to identify clinical features associated with risk of recurrence.Results:This study included a total of 108 children with sJIA, with a median onset age of 79.5 (53.0, 121.5) months. Among them, 52 were male (48.1%) and 56 were female (51.9%). The median time from disease onset to diagnosis was 29.5 (19.0, 57.0) days, and 105 children achieved clinical remission during follow-up. Out of 108 patients with sJIA, 105 patients who achieved clinical remission were enrolled with a median follow-up of 38.0 months. Among them, 28 (26.7%) patients had at least once recurrence. According to the multivariate analysis, macrophage activation syndrome [HR(95% CI)=2.375(1.096, 5.149), P=0.028] and alkaline phosphatase [HR(95% CI)=1.006(1.001, 1.010), P=0.017] were associated with increased risk of recurrence. Conclusion:Our study indicated that recurrence could appear in at least 26.7% patients with sJIA. The high alkaline phosphatase and MAS at initial attack may be independent risk factors of recurrence in patients with systemic Juvenile Idiopathic Arthritis.

Objective:To summarize the clinical data of single-center juvenile dermatomyositis（JDM）complicated with interstitial lung disease（ILD），and provide experience for pediatricians.Methods:Data of 61 children with JDM who were admitted to Children's Hospital affiliated to Xi'an Jiaotong University from January 2016 to May 2023 were collected. General data，clinical symptoms，chest high-resolution CT，laboratory examination and myositis antibody spectrum of the children were recorded.Results:Among the 61 children with JDM，there were 30 cases（13 males and 17 females）without ILD. The age of onset was 5.96（3.50，8.92）years and the course of disease was（11.79±20.00）months. There were 31 cases with ILD（14 males and 17 females），the age of onset was 7.42（4.50，10.08）years，and the duration of ILD was（5.47±8.09）months. There was statistical difference in the course of disease between the two groups（ P<0.05），but no statistical difference in gender and age between the two groups（ P>0.05）. Among 61 children with JDM，there were statistical differences in fever between the two groups（ P<0.05），but no statistical differences in heliotrope discoloration，gottron’s papules，calcinosis and myasthenia between the two groups（ P>0.05）. AST and FER showed statistical difference between the two groups（ P<0.05），while CK，LDH，CK-MB，ESR，C3 and C4 showed no statistical difference（ P>0.05）. All 61 cases of children were tested for myositis antibody spectrum，and there was statistical difference in anti-MDA5 antibody between the two groups（ P<0.05），but no statistical difference in the rest（ P>0.05）. There were statistical differences between the two groups in the treatment of methotrexate，hydroxychloroquine and cyclophosphamide（ P<0.05）. A total of 11 cases（36.67%）in the without ILD group were treated with biologics（8 adalimumab，2 infliximab and 1 tofacitinib），and 23 cases（74.19%）in the ILD group were treated with biologics（11 adalimumab，9 tofaciib，2 infliximab and 1 tocilizumab）. All 61 cases with JDM were followed up. Among the 30 children without ILD，1 case was lost to follow-up 2 months after treatment，and the rest were treated effectively without death. Among the 31 children with ILD，3 cases died of severe pulmonary infection with multidrug-resistant bacteria during treatment，of which 1 case was positive for anti-MDA5 antibody and 2 cases were negative for myositis specific antibody. Conclusion:JDM is more likely to be complicated with ILD，fever is more likely to occur in ILD group，and children with positive anti-MDA5 antibody are more likely to occur ILD. Biologic agents such as adalimumab and tofacitinib are effective in combination therapy. In the course of treatment，multi-drug resistant bacteria infection should be guarded against to reduce mortality.

Objective:To summarize the clinical data of single-center juvenile dermatomyositis（JDM）complicated with interstitial lung disease（ILD），and provide experience for pediatricians.Methods:Data of 61 children with JDM who were admitted to Children's Hospital affiliated to Xi'an Jiaotong University from January 2016 to May 2023 were collected. General data，clinical symptoms，chest high-resolution CT，laboratory examination and myositis antibody spectrum of the children were recorded.Results:Among the 61 children with JDM，there were 30 cases（13 males and 17 females）without ILD. The age of onset was 5.96（3.50，8.92）years and the course of disease was（11.79±20.00）months. There were 31 cases with ILD（14 males and 17 females），the age of onset was 7.42（4.50，10.08）years，and the duration of ILD was（5.47±8.09）months. There was statistical difference in the course of disease between the two groups（ P<0.05），but no statistical difference in gender and age between the two groups（ P>0.05）. Among 61 children with JDM，there were statistical differences in fever between the two groups（ P<0.05），but no statistical differences in heliotrope discoloration，gottron’s papules，calcinosis and myasthenia between the two groups（ P>0.05）. AST and FER showed statistical difference between the two groups（ P<0.05），while CK，LDH，CK-MB，ESR，C3 and C4 showed no statistical difference（ P>0.05）. All 61 cases of children were tested for myositis antibody spectrum，and there was statistical difference in anti-MDA5 antibody between the two groups（ P<0.05），but no statistical difference in the rest（ P>0.05）. There were statistical differences between the two groups in the treatment of methotrexate，hydroxychloroquine and cyclophosphamide（ P<0.05）. A total of 11 cases（36.67%）in the without ILD group were treated with biologics（8 adalimumab，2 infliximab and 1 tofacitinib），and 23 cases（74.19%）in the ILD group were treated with biologics（11 adalimumab，9 tofaciib，2 infliximab and 1 tocilizumab）. All 61 cases with JDM were followed up. Among the 30 children without ILD，1 case was lost to follow-up 2 months after treatment，and the rest were treated effectively without death. Among the 31 children with ILD，3 cases died of severe pulmonary infection with multidrug-resistant bacteria during treatment，of which 1 case was positive for anti-MDA5 antibody and 2 cases were negative for myositis specific antibody. Conclusion:JDM is more likely to be complicated with ILD，fever is more likely to occur in ILD group，and children with positive anti-MDA5 antibody are more likely to occur ILD. Biologic agents such as adalimumab and tofacitinib are effective in combination therapy. In the course of treatment，multi-drug resistant bacteria infection should be guarded against to reduce mortality.

Objective:To explore the potential risk factors of recurrence in patients with systemic juvenile idiopathic arthritis (sJIA).Methods:We enrolled patients with sJIA admitted to the Department of Rheumatology and Immunology, Xi′an Children′s Hospital affiliated to Xi′an Jiaotong University from January 2018 to June 2024. Cox proportional hazard models were used to identify clinical features associated with risk of recurrence.Results:This study included a total of 108 children with sJIA, with a median onset age of 79.5 (53.0, 121.5) months. Among them, 52 were male (48.1%) and 56 were female (51.9%). The median time from disease onset to diagnosis was 29.5 (19.0, 57.0) days, and 105 children achieved clinical remission during follow-up. Out of 108 patients with sJIA, 105 patients who achieved clinical remission were enrolled with a median follow-up of 38.0 months. Among them, 28 (26.7%) patients had at least once recurrence. According to the multivariate analysis, macrophage activation syndrome [HR(95% CI)=2.375(1.096, 5.149), P=0.028] and alkaline phosphatase [HR(95% CI)=1.006(1.001, 1.010), P=0.017] were associated with increased risk of recurrence. Conclusion:Our study indicated that recurrence could appear in at least 26.7% patients with sJIA. The high alkaline phosphatase and MAS at initial attack may be independent risk factors of recurrence in patients with systemic Juvenile Idiopathic Arthritis.

BACKGROUND: The presence of fibrosis is a criterion for subtype classification in the newly updated hypersensitivity pneumonitis (HP) guidelines. The present study aimed to summarize differences in clinical characteristics and prognosis of non-fibrotic hypersensitivity pneumonitis (NFHP) and fibrotic hypersensitivity pneumonitis (FHP) and explore factors associated with the presence of fibrosis. METHODS: In this prospective cohort study, patients diagnosed with HP through a multidisciplinary discussion were enrolled. Collected data included demographic and clinical characteristics, laboratory findings, and radiologic and histopathological features. Logistic regression analyses were performed to explore factors related to the presence of fibrosis. RESULTS: A total of 202 patients with HP were enrolled, including 87 (43.1%) NFHP patients and 115 (56.9%) FHP patients. Patients with FHP were older and more frequently presented with dyspnea, crackles, and digital clubbing than patients with NFHP. Serum levels of carcinoembryonic antigen, carbohydrate antigen 125, carbohydrate antigen 153, gastrin-releasing peptide precursor, squamous cell carcinoma antigen, and antigen cytokeratin 21-1, and count of bronchoalveolar lavage (BAL) eosinophils were higher in the FHP group than in the NFHP group. BAL lymphocytosis was present in both groups, but less pronounced in the FHP group. Multivariable regression analyses revealed that older age, <20% of lymphocyte in BAL, and ≥1.75% of eosinophil in BAL were risk factors for the development of FHP. Twelve patients developed adverse outcomes, with a median survival time of 12.5 months, all of whom had FHP. CONCLUSIONS Older age, <20% of lymphocyte in BAL, and ≥1.75% of eosinophil in BAL were risk factors associated with the development of FHP. Prognosis of patients with NFHP was better than that of patients with FHP. These results may provide insights into the mechanisms of fibrosis in HP.
Humans ; Bronchoalveolar Lavage Fluid ; Prospective Studies ; Alveolitis, Extrinsic Allergic/diagnosis* ; Fibrosis ; Carbohydrates

The advantages of saliva-based biomarkers detection in cancer were accurate,simple and noninvasive.Currently,biomarkers validated for saliva detection include protein,mRNA,miRNA and DNA,using PAGE,microarray and sequencing,respectively.Analysis of literatures shows;that saliva biopsy plays an important role in cancer diagnosis,clinical treatment and prognosis.Due to the design of experiments reported werediversification,a large number of validations are necessary to standardize saliva collecting,processing,testing methods and quality assurance.

Objective To explore the application effect between ultrasound ( US)-guided central line insertion combined with modified guide wire insertion technique and the conventional US-guided peripherally inserted central catheter ( PICC) placement. Methods Totally 120 cases with PICC from January 2014 to December 2015 in Departwent of General surgery were randomly divided into the control group and the observation group. Patients in the control group received conventional US-guided PICC line placement, while patients in the observation group received US-guided central line insertion combined with modified guide wire insertion technique. The success rate and the incidence of complications related to catheter were compared in two groups.Results A statistically difference suggested that the success rate in observation group was significantly higher than the control group (χ2 =11. 582, P<0. 05 ) . Differences of the incidence of phlebophlogosis and thrombus in two groups weren′t significant(P>0.05).Conclusions US-guided central line insertion combined with modified guide wire insertion technique has a higher successful rate compared to the conventional US-guided PICC line placement.

Wearing transfemoral prosthesis is the only way to complete daily physical activity for amputees. Motion pattern recognition is important for the control of prosthesis, especially in the recognizing swing phase and stance phase. In this paper, it is reported that surface electromyography (sEMG) signal is used in swing and stance phase recognition. sEMG signal of related muscles was sampled by Infiniti of a Canadian company. The sEMG signal was then filtered by weighted filtering window and analyzed by height permitted window. The starting time of stance phase and swing phase is determined through analyzing special muscles. The sEMG signal of rectus femoris was used in stance phase recognition and sEMG signal of tibialis anterior is used in swing phase recognition. In a certain tolerating range, the double windows theory, including weighted filtering window and height permitted window, can reach a high accuracy rate. Through experiments, the real walking consciousness of the people was reflected by sEMG signal of related muscles. Using related muscles to recognize swing and stance phase is reachable. The theory used in this paper is useful for analyzing sEMG signal and actual prosthesis control.
Artificial Limbs ; Electromyography ; Humans ; Leg ; Muscle, Skeletal ; physiology ; Walking ; physiology