Anti-melanoma differentiation-associated gene 5 and anti-Ro52 antibody-dual positive dermatomyositis accompanied by rapidly lung disease:Three case reports

BACKGROUND Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis(DM)manifestations without muscle weakness.Anti-melanoma differentiation-associated gene 5(anti-MDA5)and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease,and they exhibit an immensely high half-year mortality.CASE SUMMARY We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM.Although we aggressively treated these patients similarly,but they all exhibited different prognoses.We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM.CONCLUSION Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively,thus,the patient’s prognosis can be significantly modified.

Treatment of refractory anti-melanoma differentiation-associated gene 5 anbibody-positive dermatomyositis complicated by rapidly progressing interstitial pulmonary disease:Two case reports

BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlying mechanism with no recommended unified treatment plan.Herein,one of the cases that we report(Case 2)was successfully treated with tocilizumab despite having lung infection.CASE SUMMARY Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo,fever and cough for 1 mo,and chest tightness for 3 d.She was diagnosed with non-myopathic dermatomyositis(anti-MDA5 Ab+)and interstitial pneumonia,and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus.Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo,and chest tightness and dry cough for 4 d.He was diagnosed with dermatomyositis(anti-MDA5 Ab+)and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy(without cyclophosphamide)and the combination of tocilizumab and tacrolimus.The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.CONCLUSION Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+DMRP-ILD who are refractory to intensive immunosuppression.

罕见抗-Di^(b)致严重胎儿新生儿溶血病的实验室检测与相关研究

目的对1例高频抗体导致的胎儿新生儿溶血病(hemolytic disease of the fetus and newborn,HDFN)进行检测、鉴定及配血。方法对患儿进行新生儿溶血试验,对母亲进行血清学意外抗体鉴定,并对母亲红细胞进行常见高频抗原鉴定;对检出抗体进行IgG分型检测,并用流式细胞术进行单核细胞体外吞噬致敏红细胞试验,以检测抗体相关的吞噬率;对患儿母亲、父亲及舅舅进行相关红细胞血型基因测序;利用稀释的母亲血浆和抗人球卡法,在献血者中进行大规模相合血液的筛选。结果产妇鉴定为Di(b-)稀有血型,产生了抗-Di b(效价512)并导致了严重的HDFN;抗-Di b亚型分型为IgG1和IgG2型,单核细胞体外吞噬效率为88.83%(74.7/84.09);产妇亲属中没有相合献血者,后续从5520名献血者中筛选到2例Di(b-)相合血液,患儿接收输血治疗后康复出院。后续在51334名献血者中筛查到17名Di(b-)献血者,该数据表明Di(b-)在广州地区献血者中的分布频率约为三千分之一(0.033%,17/51334)。结论综合利用血型血清学及分子生物学方法诊断了抗-Di b所致的严重HDFN,建立了1种有效大规模筛查Di(b-)稀有血型的方法并找到相合血液,为建立Di(b-)稀有血型库奠定了基础。

4DI110型空压机的技术改造与操作优化

文章通过介绍新引进的4DI110型离心式空压机(以下简称“空压机”)试运行阶段开始以来的运行情况和气温变化对空压机出口流量造成的影响,分析空压机出口流量随气温变化而变化的规律,指出空压机防喘阀V3004阀不能自动调节出口压力,影响空分装置工况的稳定。为实现空压机出口压力的稳定,对其防喘阀V3004阀采取增设自动旁路阀V3008阀的技术改造,同时优化和完善相关逻辑控制并阐述了空压机经技术改造后的操作优化和取得的实际效果。

miR-125b, miR-200c Are Correlated with the Severity of Interstitial Lung Disease in Dermatomyositis/Polymyositis

Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.

Safety of Transcranial Direct Current Electrical Stimulation in Dermatomyositis: A Case Report

Transcranial direct current stimulation (tDCS) has emerged as a nonpharmacological tool in physical rehabilitation. There have currently no studies that evaluated the safety and efficacy of tDCS in patients with dermatomyositis. Case-report: Three adult women with dermatomyositis were allocated randomly to intervention (i-tDCS, one patient) or not (sham-tDCS, two patients) of three consecutive days of tDCS and evaluated in four periods: before-tDCS (PRE), 15 minutes after-tDCS (0th POST), 15 days after-tDCS (15th POST), and 30 days after-tDCS (30th POST). The tDCS was safe throughout the protocol, without disease relapsing or adverse effects related to tDCS. Furthermore, the tDCS increased the muscle torque and total work of dominant and non-dominant elbow flexors in the patient with i-tDCS, when compared to two patients with sham-tDCS. Conclusions: The tDCS was safe and appeared to influence long-term strength in the limb of the patient with stable dermatomyo-sitis.

借助DIS实验突破教学难点——以“磁场对通电导线的作用力”为例

教材中直接得出安培力的公式,该知识点难度较大,传统实验现象不够明显且只能定性探究,学生无法真正理解该物理规律和公式。传统实验加DIS实验可对物理规律进行定性和定量的探究,有利于学生物理学科核心素养的培养。

用DIS电磁定位魔板探究平抛运动的教学设计

随着数字化信息系统在中学物理教学中的逐步融入,我国中学物理实验呈现智能化、现代化的发展趋势.以DIS电磁定位魔板为实验装置,通过科学探究的关键环节,设计探究平抛运动的教学流程.实践发现传感器实验也适合做科学探究,能够充分提升学生科学探究和推理论证的能力.

城市轨道交通减振轨道DI型弹条断裂原因分析及治理效果

[目的]弹条是减振轨道扣件中的关键部件,其力学性能及承受疲劳载荷性能关系到城市轨道交通列车运行安全及轨道使用寿命。针对目前弹条失效破坏频发的情况,有必要对其断裂原因进行分析并制定合理治理措施。[方法]以某城市轨道交通线路某区段出现弹条集中断裂的情况为依托,通过弹条断口分析确定弹条的断裂类型为疲劳断裂,通过分析弹条的化学成分和物理性能未发现异常状态,通过仿真分析和试验分析得到弹条的固有频率。结合弹条断裂区段轨道动态测试得到轨道和弹条的振动特性,由此确定轨道波磨可能是引起弹条出现反复断裂的主要原因。针对该原因制定了合理的治理措施,对治理前后的弹条断裂数量进行了统计。[结果及结论]钢轨打磨措施有效抑制了轨道波磨,大大减少了弹条断裂情况的发生,进一步佐证了轨道波磨是引起弹条断裂的主要原因。

嗜热链球菌c-di-AMP合成酶的结构预测

作为一种新兴第二信使分子,c-di-AMP具有全局调控胞内渗透压平衡、DNA损伤和生物膜形成等生理功能。本实验室在实验前期筛选出了一株高产胞外多糖的嗜热链球菌菌株S-3,但未对其进行c-di-AMP信号通路研究。本研究在嗜热链球菌S-3全基因组中筛选出c-di-AMP合成酶(StDAC),对其二级结构进行预测,结果显示St DAC二级结构包含42.98%α-螺旋、15.32%β-折叠和38.30%无规则卷曲。利用SWISS-MODEL和Modeller对StDAC进行单模板和多模板同源建模,由SWISS-MODEL建立的模型通过了Ramachandran、Errat和Verify-3D评估,表明其结构较为合理;而Modeller建立的模型仅通过了Ramachandran评估,总体结构评分较低。本研究预测的嗜热链球菌StDAC三维模型为解析其结构和功能奠定了基础。

乳酸菌第二信使分子c-di-AMP研究进展

微生物依赖信号识别和信号传递来感知并响应外界环境变化。c-di-AMP作为第二信使分子在信号传导过程中发挥着关键作用,其通过酶、转录调控因子和核糖开关等受体靶标来调控细胞生长、生物膜形成、K^(+)稳态、DNA完整性、细胞壁合成和脂肪酸合成等与生理功能相关的基因和蛋白的表达。尽管对于c-di-AMP代谢调控在枯草芽孢杆菌、单增李斯特氏菌和金黄色葡萄球菌等细菌中的研究较为深入,但对其在乳酸菌中的研究却相对较少。基于此,文章重点关注乳酸菌中cdi-AMP的代谢及其在K^(+)稳态和抑制甘氨酸甜菜碱转运中的信号传导作用;同时,通过总结现有研究中的乳酸菌c-di-AMP代谢调控,深化对乳酸菌第二信使分子信号调控的认知。

外卖包装材料对我国青年群体暴露DBP和DiBP的影响

青年群体是外卖食品最大的消费群体,外卖包装材料中的污染物对青年群体的危害不容忽视。文章分析了外卖包装材料对我国青年群体暴露邻苯二甲酸二正丁酯(DBP)和邻苯二甲酸二异丁酯(DiBP)的影响,结果表明,发泡聚苯乙烯(PS)餐盒中DBP和DiBP的浓度(2986.27 ng/g和1345.20 ng/g)显著高于聚丙烯(PP)餐盒、纸质餐盒和锡纸餐盒,青年人群使用PS餐盒暴露DBP和DiBP的暴露量约为使用纸质餐盒和锡纸餐盒的2~5倍,青年人群经外卖餐盒暴露DBP和DiBP的暴露量占经饮食途径暴露DBP和DiBP暴露总量的1.72%~9.49%,占体内DBP和DiBP暴露总量的0.09%~0.47%。外卖包装材料是青年人群暴露DBP和DiBP的重要来源之一。

DIS实验助力高中物理教学探究——以图象问题为例

实验探究在高中物理教学中扮演着十分重要的角色。在数字化信息系统(DIS)实验的帮助下,弥补了一些传统实验在定量探究上的局限,极大地提升了实验教学效率。这种创新的教学方式有助于培养学生的实验探究精神,让他们养成严谨对待实验结果并深入剖析数据的科学习惯。本文立足于教学实践,详细探讨了如何有效利用DIS实验助力高中物理教学探究。以"用DIS实验探索力的相互作用、探究向心力实验、验证动量定理的实验案例"为例,展示了DIS实验在课堂中的精准、直观以及实际操作价值。

Transcranial Direct Current Stimulation Is Safe and Relieves Post-Herpetic Neuralgia in Patient with Dermatomyositis: A Case Report

To the best of our knowledge, this is the first report in the literature showing the efficacy of transcranial direct current stimulation (tDCS) in treating refractory post-herpetic neuralgia in a patient with dermatomyositis. In addition, our results showed that tDCS sessions are safe and do not lead to disease relapse. Therefore, despite the limitations of being a case report, this study provides encouraging results that need to be widely explored in studies with a representative number of patients and with an appropriate design for patients with this systemic rheumatic autoimmune disease.

基于核心素养的高中物理DIS实验 校本课程开发的实践研究 ——以“探究平抛运动的特点”为例

运用传感器和计算机技术的DIS实验,兼具精确测量数据和快速直观展示结果的特点,在教育信息化的大背景下,逐渐在高中物理教学中普及。文章以探究平抛运动的特点为例,借助DIS实验的优点进行校本课程的开发,实现对学生核心素养的补充培养。通过教学过程中的实践和反思提出了基于学科核心素养应用DIS实验开发校本课程应注意的一些问题。

城轨交通DI型弹条接触疲劳断裂机理研究

以DI型弹条为研究对象,基于实验得到正常行车状态下弹条的频域特性,采用有限元仿真对弹条进行失效特性分析。研究表明,弹条的断裂为疲劳断裂,表面缺陷产生的应力集中是弹条发生疲劳断裂的主要原因;在正常行车条件下,弹条和钢轨均在360 Hz和700 Hz附近产生峰值,其中钢轨的垂向及横向振动频谱变化趋势基本一致;弹条安装深度过大将导致弹条与铁垫板接触点产生一定应力集中,在长期强迫振动激励作用下弹条容易发生疲劳折断。

Radiofrequency catheter ablation of ventricular tachycardia in a patient with dermatomyositis

A 51-year old male who presented at our hospital for recurrent palpitation for several months was diagnosed dermatomyositis ten years ago and had interstitial lung disease since two years ago. Recently, he was admitted for atypical hepatitis, and received maintenance treatment of oral corticosteroids.

Clinical Phenotype of Japanese Patients with Dermatomyositis—Classification Based on Dermatomyositis-Specific Autoantibodies

Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 27 with clinically amyopathic dermatomyositis, CADM) were screened for autoantibodies using immunoprecipitation assays. Patients with antibodies against aminoacyl transfer RNA synthetase (ARS), Mi-2 or who had other autoantibodies were assessed for clinical symptoms and laboratory findings. Results: Sera from 27 of 94 DM patients (29%) were found to have anti-ARS antibodies. Nineteen (20%) had anti-CADM-140/MDA5, 5 (5%) had anti-Mi-2, and 8 (6%) had anti-p155/TIF1-γ. Anti-MJ/NXP-2 was not found in our series of adult DM. Seventeen patients with anti-ARS had fever and 22 had arthritis and interstitial lung disease (ILD), compatible with a diagnosis of anti-ARS syndrome. Seventeen of 19 (89%) with anti-CADM-140/MDA5 had ILD, 16 (84%) of whom developed rapidly progressive ILD (RP-ILD). Four of 5 (80%) with anti-Mi-2 had heliotrope rash and/or Gottron’s sign/papules, and 2 (40%) had V-sign and/or shawl-sign rash, whereas no ILD or malignancy was detected. As seen with anti-Mi-2-positive patients, a low frequency of ILD (13%) was found in patients with anti-p155/TIF1-γ but 6 of 8 (75%) had malignancy during their course. The frequency of ILD was significantly higher in patients with anti-ARS or anti-CADM-140/MDA5 compared with anti-Mi-2 or anti-p155/TIF1-γ (81% and 89%, respectively). It should be noted that anti-CADM-140/MDA5-positive patients suffered significantly more RP-ILD compared to patients with anti-ARS (84% vs. 7%, P < 0.0001). On the other hand, anti-p155/TIF1-γ positive patients had a significantly higher rate of malignancy compared with anti-ARS-, anti-CADM-140/MDA5-and anti-Mi-2-positive patients (75% vs. 7%: P = 0.0004, 5%: P = 0.0006, 0%: P = 0.02, respectively). Conclusions: These results indicate that in addition to antibodies previously identified as specific for DM, autoantibodies newly found in these patients are useful for stratifying them into clinical subgroups.

First results of CO_(2) dispersion interferometer on EAST tokamak

A dispersion interferometer(DI)has been installed and operates on the Experimental Advanced Superconducting Tokamak(EAST).This DI system utilizes a continuous-wave 9.3μm CO_(2)laser source to measure line-averaged electron densities accurately.In contrast to conventional interferometers,the DI does not require substantial vibration isolations or compensating systems to reduce the impact of vibrations in the optical path.It also employs a ratio of modulation amplitudes,ensuring it remains immune to the variations in detected intensities.Without a variation compensation system,the DI system on EAST reaches a density resolution of less than1.8×10^(-2)πrad and a temporal resolution of 20μs.The measurements made by the POlarimeterINTerferometer(POINT)system and the far-infrared hydrogen cyanide(HCN)interferometer are remarkably consistent with the DI’s results.The possibility of fringe jumps and the impact of refraction in high-density discharge can be significantly decreased using a shorter wavelength laser source.A rapid density change of 3×10^(19)m^(-3)during 0.15 s has been measured accurately in shot No.114755 of EAST.Additionally,the DI system demonstrates dependability and stability under 305 s long-pulse discharges in shot No.122054.

基于学程包和DIS实验提升教学有效性——以“再探NaOH溶液与CO_(2)的反应”为例

随着时代发展,信息技术与教学的深度融合趋势越来越明显。以“再探NaOH溶液与CO_(2)反应”为例,利用学程包软件模拟装置搭建,推送习题并反馈答题数据,利用DIS实验精准测出压强的变化数据,帮助学生更好地理解实验原理,从反应物消耗和生成物增加两个角度证明发生了反应。