Current status and prospects of the treatment of juvenile idiopathic arthritis / 中华实用儿科临床杂志

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.

Efficacy of bortezomib regimen and survival analysis in multiple myeloma patients with extramedullary disease / 白血病·淋巴瘤

Objective:To investigate the short-term therapeutic effect and long-term survival of multiple myeloma patients with extramedullary disease (EMD) in the new drug era.Methods:The data of 74 patients with multiple myeloma diagnosed and treated in Anhui Wanbei Coal and Electricity Group General Hospital from January 2015 to January 2020 were retrospectively analyzed, including 17 patients with soft tissue infiltration (EM-S), 9 patients with bone infiltration (EM-B), and 48 patients without EMD (No-EMD). The short-term efficacy, the 4-year progression-free survival (PFS) rate and overall survival (OS) rate, and their influencing factors in three groups of patients after receiving bortezomib regimen were analyzed.Results:After 3-4 courses of early induction therapy of bortezomib regimen, the overall response rate of patients in the EM-S group was lower than that in the No-EMD group and the EM-B group [58.8% (10/17) vs. 85.4% (41/48), 100.0% (9/9)], and the differences were statistically significant ( χ2 = 13.7, P = 0.036; χ2 = 26.5, P = 0.003), while the difference between No-EMD group and EM-B group was not statistically significant ( χ2 = 12.7, P = 0.211). Survival analysis showed that the 4-year PFS rate of No-EMD group was higher than that of the EM-S group and EM-B group (41.0% vs. 7.6%, 0), and the differences were statistically significant ( χ2 = 10.835, P < 0.01; χ2 = 8.276, P = 0.004). Meanwhile, the 4-year OS rate of EM-S group was lower than that of the No-EMD group and EM-B group (16.5% vs. 54.3%, 59.3%), and the differences were statistically significant ( χ2 = 9.146, P = 0.002; χ2 = 4.066, P = 0.044). Conclusion:The early treatment effect of bortezomib regimen, PFS and OS in multiple myeloma patients with EM-S are poor, while the EM-B has no effect on OS.

Analysis of curative effect of methylprednisolone pulse combined with mycophenolate mofetil in treatment of systemic lupus erythematosus in children / 吉林大学学报(医学版)

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.

Current status and prospects of the immune pathogenesis and treatment of IgA vasculitis / 中华实用儿科临床杂志

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.

Blood lipid abnormality and single nucleotide polymorphisms / 国际儿科学杂志

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.

Coronary arterial lesions in Kawasaki disease: a follow-up of 787 cases / 中华风湿病学杂志

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.

Effects of nutritional support on serum amino acid spectrum and neurological function in acute stroke patients / 中华神经科杂志

Objective To investigate serum amino acid spectrum in patients with acute stroke in response to different nutritional support strategies and its effects on neurological function.Methods A total of 60 cases of acute stroke with dysphagia were randomly distributed into two groups:enteral nutrition group (30 cases) and control group (30 cases) using simple randomized design.Serum amino acid spectrum,hemoglobin,total protein,albumin,prealbumin,immunoglobulins,complement,and infection rate were assessed at three time points:within 48 hours,(7 ± 1) days and (14 ± 1) days after admission,and neurological deficit and activities of daily living are scored according to National Institutes of Health Stroke Scale (NIHSS) and Barthel Index (BI).Results (7 ± 1),(14 ± 1) days after admission,serum amino acid spectrum,hemoglobin,total protein,albumin,prealbumin,immunoglobulins,complement,and neurological deficit scores of enteral nutrition group patients were significantly better than those of non-enteral nutrition control group; and infection rate was lower than that of control group.Follow-up for a month,3 months,NIHSS of enteral nutrition group patients (9.0 ± 1.4,7.9 ± 1.3) were significantly better than nonenteral nutrition control group(11.1 ± 1.5,10.6 ± 1.4,F =46.042,P ＜ 0.05).While BI score seemed to be not insignificant different between enteral nutrition group (50.1 ± 1.8,52.0 ± 2.4) and control group (49.0±2.1,51.3 ±2.8,F=2.707,P＞0.05).Conclusion For patients suffering acute stroke with dysphagia,enteral nutrition support could reduce infectious complications,improve short-term neurological function and long-term prognosis by improving serum amino acids level and thus the whole body' s nutritional status.