This cohort study reports the long-term clinical outcomes of the use of intermittent intravenous methylprednisolone pulse therapy combined with mycophenolate mofetil for patients with juvenile dermatomyositis.

Juvenile idiopathic arthritis (JIA) is a genetically heterogeneous group of connective tissue diseases that are commonly characterized by chronic joint synovial inflammation with unknown etiology in childhood.It is cu-rrently incurable and the main therapeutic goal is to achieve clinical remission.The drugs currently used to treat JIA mainly include non-steroid anti-inflammatory drugs, glucocorticoid, disease modifying antirheumatic drugs, and biological agents.In this article, recent advances in the understanding of JIA treatment and related clinical research were reviewed, in an attempt to provide prospects for the future direction of drug development and treatment concepts.

Abstract Background: This study aimed to investigate the clinical effectivity of intravenous methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil in the treatment of newly diagnosed juvenile dermatomyositis and to review the literatures. Methods: Reviewed the clinical data of 12 juvenile dermatomyositis patients, from January 2014 to January 2017, hospitalized and treated with intravenous methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil. Results: A total of 12 patients were enrolled, and their overall treatment response rate was 100%. After treatment, all patients showed significant improvement in cutaneous performance and children myositis assessment scores. The median recovery time of muscle enzyme and children myositis assessment scores were 1.8 months and 19.6 months, respectively. During the intravenous methylprednisolone repeated intermittent pulse treatment, the body weight percentile was higher than that before treatment, blood pressure and intraocular pressure were transiently increased, and the level of serum lipids, glucose and calcium showed no significant changes. During the maintenance treatment period, the height percentile was increased when compared to those before treatment, while the weight percentile, blood pressure and intraocular pressure of the patients showed no significant changes. Two patients with recurrence were followed up for more than 3 years after corticosteroid re-withdrawal, and no relapse was seen till now. Conclusion: Intravenous methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil showed positive outcomes and good safety index in juvenile dermatomyositis patients. It is likely considered to be a promising treatment strategy for juvenile dermatomyositis. Trial registration: ClinicalTnals, ID：NCT05509140. Registered 18 August 2022 - Retrospectively registered, https://register.clinicaltrials.gov/prs/app/action/LogoutUser?uid=U00052MF&ts=12&cx=mcg5gj.

Objective:To investigate the efficacy and side effects of combination of methylprednisolone pulse (MDP)and mycophenolate mofetil(MMF)in the treatment of systemic lupus erythematosus(SLE)in the children.Methods:A total of 16 cases of children with SLE,lupus nephritis(LN)and type Ⅳ diffuse glomerular mesangial proliferative glomerulonephritis diagnosed by pathology were selected.Among them 7 cases were given MDP combined with MMF,and received intermittently oral small dose of corticosteroids(GC),and they were used as pulse therapy group;9 cases were given oral GC and transitional reduction,and they were used as traditional therapy group.SLEDAI was used for the evaluation of the curative effect,and body mass index(BMI),blood pressure(BP),intraocular pressure(IOP),triglycerides(TG),fasting blood glucose(FBG)and serum calcium (Ca)were analyzed during the treatment of 1 year and then the efficacies and side effects of the children in two groups were compared.Results:The SLEDAI scores,levels of complements C3 and C4,24 h urinary protein outcome of the children in pulse therapy group were better than those in traditional therapy group;the differences in SLEDAI scores were statistically significant after treating for 3 and 6 months between two groups(P<0.05);ESR and 24 h urinary protein outcome had significant differences after 6 months of treatment between two groups(P<0.05);the complement C3 difference was statistically significant between two groups(P<0.05)after 12 months of treatment.Compared with traditional therapy group,the BMI,IOP,TG,FBG of the children in pulse therapy group after treatment were decreased(P<0.05);the BMI,IOP and TG had significant differences after treating for 12 months between two groups(P<0.05);the differences in FBG were statistically significant after treating for 6 and 12 months between two groups(P<0.05).The Ca of the patients in pulse therapy group was higher than that in traditional therapy group,but there was no statistically significant difference(P>0.05).The SPB and DBP of the patients in pulse therapy group were higher than those in traditional therapy group,but the differences were not statistically significant(P>0.05).At the same time,gastrointestinal ulcers,bleeding,perforation, pancreatitis,cardiovascular events (such as cardiac arrhythmias)didn't occur in two groups. Conclusion:Compared with traditional therapy,the combined treatment of MDP and MMF can control the symptoms of SLE early and rapidly,and reduce the viscera damage.To choose 1 year after treatment as observation point,its disease activity is lower than the traditional therapy,and the curative effect is better than oral GC transitional reduction with immunosuppressant therapy.The GC-related side effects are lower than traditional therapy.

患者女 ,13 岁 ,因双下肢疼痛 40 余天 ,双踝关节痛 、 发热 1 个月入院 . 查体 : 脐上方可闻及动脉收缩期杂音 , 双侧桡动脉及足背动脉搏动减弱 ,双下肢肌肉触痛 ,双踝关节触痛伴活动受限 , 肢体无肿胀 . 实验室检查 : 白细胞 9 .95 × 109 /L ,血红蛋白 92 g/L , 血小板 6 .43 × 1011 /L , 血沉 120 mm /1 h ,C 反应蛋白 121 mg/L , 补体 C3 、 C4 升高 , 心肌酶 、 肝功 、 肾功 、 免疫三项 、 抗链球菌溶血素 O (ASO ) 、 抗核抗体 (ANA) 、 类风湿因子未见异常 . 血管超声检查 :双侧颈总动脉 、 双侧锁骨下动脉 、 双侧上肢动脉 、 双侧下肢动脉 、腹主动脉 、 肠系膜上动脉等管壁呈弥漫性相对均匀性增厚 ,管腔狭窄 ; 双侧下肢静脉管壁弥漫增厚 , 以股浅静脉为著 ,管腔充盈良好 ,未见血栓回声 ,符合血管炎声像 (图 1) . 临床诊断 :多发性大动脉炎 (广泛型) . 给予甲泼尼龙琥珀酸钠1000 mg/d × 3 d 冲击治疗 ,冲击治疗当日患儿体温即降至正常 ,肌痛及关节痛随之缓解 ,之后给予糖皮质激素间歇冲击治疗 ,冲击间歇期给予醋酸泼尼松 10 mg /d 口服 . 监测 C 反应蛋白于治疗的第 1 周即降至正常 ,血沉于治疗第 9 周降至正常 ,血小板及血红蛋白于治疗后第 3 周恢复正常 .

IgA vasculitis (IgAV) is a common systemic small-sized vessel vasculitis.It's characterized by non-thrombocytopenic palpable purpura,arthralgia/arthritis,bowel angina,and nephritis,but some details of etiology and pathogenesis is not very clear.The disease course is usually benign and self-limited,however,it is necessary to discuss the therapy of severe or chronic cases,especially there is not enough evidence-based basis for using key drugs to IgAV.Excellent clinical trails is expected to establish scientific system of prediction and evaluation of disease course,which will be helpful to individualize treatment of IgAV.

目的 了解长春地区2012年、2013年儿童疱疹性咽峡炎主要病原类型.方法 采集2012年、2013年儿童疱疹性咽峡炎患者的咽拭子标本,通过半巢式RT-PCR及VP1区基因序列分析进行病原分型鉴定.结果 本研究采集的110份儿童疱疹性咽峡炎标本中,经鉴定肠道病毒(enterovirus,EV)阳性82份,阳性率为74.55％;其中2012年检测前3位的型别分别为CVA10(18/32)、CVA2(7/32)及CVA3、CVA16(3/32);2013年检测前3位的型别分别为CVA6(25/50)、CVA2(13/26)及CVA4、CVA10(3/50).系统进化树分析结果表明,2012年和2013年吉林省肠道病毒的流行均存在3个或3个以上的传播链.结论 2012年、2013年引起长春地区儿童疱疹性咽峡炎的主要病原分别为CVA10及CVA6,且同期存在不同的传播链.

Studies have indicated approximate 50% cases of coronary heart disease are closely associat-ed with genetic factors and it. It is well evidenced that lipid abnormality is the important risk factors of coronary heart disease. Many single nucleotide polymorphisms that influence levels of blood lipids were found and con-firmed in recent years. The article summarized the single nucleotide polymorphisms associated with the level of lipid.

Objective To investigate the coronary arterial lesions (CAL) in Kawasaki disease (KD) in Jilin Province and Inner Mongolia Autonomous Region from 1999 through 2012 and evaluate the influence of glucocorticoids (GCs) on prognosis.Methods We performed a retrospective study among patients of KD in Jilin Province and Inner Mongolia Autonomous Region from 1999 to 2012,in order to observe CAL in KD,the incidence of sequelae and the influence of CGs on disease outcome.Comparison between groups was carried out by Chi-square test.Results Eight hundred and ten KD cases were reported from 32 hospitals and 787 patients accepted an Echo examination,as the main objective of this study.Fivehundred and forty were boys and 247 were girls,with the male to female ratio of 2.19:1,of which 498 cases (63.3％) had CAL.Most CAL occurred in patients at 58 day to 14 year old (2.7±2.4).The most common abnormality was CAL including dilatation (49.5％) and stenosis (13.4％).Compared incomplete to complete KD,the result showed that the occurrence of CAL was similar between these two groups,no significant differences could be found between different gender.Nineteen (2.4％) of them had cardiac sequelae and the male/female ratio was 3.67:1.The younger the age,the higher the incidence of cardiac sequelae.The most frequent cardiac sequelae was coronary artery dilatation.GCs therapy could increase the occurrence of CAL [80.2％(97/121) vs 62.6％ (419/666),x2=8.71,P＜0.05].Conclusion CAL is not different between patients with complete or incomplete KD and the occurrence of CAL is not associated with gender,but with age.The younger the age,the higher the incidence of CAL and the higher the incidence of cardiac sequelae.It is possible that patients treated with GCs are prone to CAL.

BACKGROUND & OBJECTIVEWhen leukemia patients achieve complete remission after chemotherapy, a few tumor cells still exist in other tissues outside bone marrow, which is called minimal residual disease (MRD), and it is the base of relapse. To cure leukemia, we should not only find MRD in time, but also quantitate MRD for instructing treatment and predicting prognosis. This study was to establish a real-time reverse transcription-polymerase chain reaction (RT-PCR) system to quantitate bcr-abl mRNA.METHODSbcr-abl mRNA in leukemia cell line K562 was amplified by RT-PCR. T-A clone was used to construct the combined plasmid to be standard template; the standard curve of bcr-abl oncogene was drawn. bcr-abl mRNA in bone marrow samples of 16 chronic myelocytic leukemia (CML) patients was quantitated by this method. The sensitivity, stability, and repetition of this method were evaluated.RESULTSThe sensitivity is 10 copies of the recombined plasmid. The coefficient variations (CV) of repetition and stability were 2.19% and 3.21%. The correlation (R) of the standard curve was 0.984. The median bcr-abl level of the 16 CML patients was 4.58x10(4) kb/microg RNA.CONCLUSIONSReal-time PCR has high sensitivity, repetition, and specificity. It can quantitate the copy number of bcr-abl oncogene.

我国报道的戊型肝炎病毒(Hepatitis E virus HEV)基因组序列,大多数为散发型HEV部分基因序列的测定,仅少数为全基因序列的分析结果,而且,各实验室所分析的HEV片段不同,因此,很难确定新型变异株,远远不能满足中国基因分型的研究和临床诊断的需要.为此,我们对长春地区一株散发性HEV进行了全基因cDNA序列测定.