Von Willebrand disease: diagnosis and management

Von Willebrand Disease is a common cause of excessive bruising and bleeding in children. This short article gives advice on diagnosis and management for paediatricians. Given its prevalence and presenting symptoms, VWD should always be considered in the assessment of children suspected of non-accidental injury. Its diagnosis can be challenging, not only because of the various subtypes of the disorder but because of the considerable overlap between VWD and normal individuals. Laboratory diagnosis requires a range of quantitative and qualitative tests of the VWF protein, with targeted gene analysis increasingly used to confirm the diagnosis of type 2 and type 3 VWD. Bleeding Assessment Tools may be helpful in directed laboratory testing but are often less so in young children who have had limited haemostatic challenges. Treatment for VWD includes the use of antifibrinolytic drugs, vasopressin or VWF-containing clotting factor concentrates. Treatment is often on-demand for individual bleeding episodes but there are specific indications for the use of prophylactic treatment in children.     

The predictive value of crescents in adult Henoch-Sch?lein purpura nephritis

Objective To study the renal prognosis with the type and proportion of crescentic in adult Henoch Schonlein purpura nephritis (HSPN). Methods A total of 275 HSPN cases diagnosed in the First Affiliated Hospital of Zhejiang University were retrospectively analyzed. According to the pathological results, they were divided into four groups: 99 patients in none crescent group (NC), 35 patients in segmental crescents group (SC), 122 patients with circumferential crescent ＜25% (C1), and 19 patients with circumferential crescent≥25% (C2). Renal prognostic events were defined as estimated glomerular filtration rate (eGFR) decreased by 30% over baseline within 2 years, doubling of serum creatinine or end-stage renal disease during follow-up. Kaplan-Meier survival analysis was used to compare the renal survival rate of each group. Univariate and multivariate Cox regression model was used to recognize the risk factor of poor renal outcome. Results There was no significant difference in age, extra renal organ performance and mean arterial pressure among groups. Among NC group, SC group, C1 group and C2 group, difference in serum creatinine (P=0.001), eGFR (P=0.003) and proteinuria levels (P＜0.001) were statistically significant. There was no significant difference in the ratio of global sclerosis, mesangial hypercellularity and interstitial inflammation/fibrosis among the groups. The patients were followed up for 86(58, 116) months. The renal survival rates of NC group, SC group, C1 group and C2 group were 96%, 100%, 83.6% and 68.4% respectively. Kaplan-meier survival analysis showed significant differences (Log Rank=23.24, P＜0.001). Cox multivariate regression analysis indicated that presence of circumferential crescent (HR=3.59, 95%CI 1.34-9.62, P=0.008) and low eGFR (HR=0.979, 95%CI 0.968-0.989, P＜0.001) were independent prognostic factors. Conclusion The presence of circumferential crescent and low eGFR level are independent risk factors for poor renal prognosis in HSPN patients.     

儿童系统性红斑狼疮与肾炎慢病管理及治疗进展

儿童系统性红斑狼疮（childhood-onset systemic lupus erythematosus，cSLE）是特指在18岁之前发病的系统性红斑狼疮。可累积全身多脏器、多系统，有多达60%的患儿会出现狼疮性肾炎（lupus nephritis，LN），其中19%的患儿进展为终末期肾病。感染和终末期肾病是导致发展中国家cSLE死亡的常见原因。由于疾病本身和治疗都会影响患儿的身心和生长发育，做好cSLE与LN的慢病管理对控制病情、提高长期生存率极为重要。本文总结了近年来国内外系统性红斑狼疮慢病管理的经验，并对cSLE与LN慢病管理和治疗进展进行综述，旨在提高治疗达标率，减少疾病复发及并发症的发生，使患儿获益。     

狼疮肾炎合并抗磷脂抗体综合征的中西医结合诊疗

抗磷脂抗体综合征(APS)是系统性红斑狼疮(SLE)常见继发病症。以循环中出现抗磷脂抗体、血栓形成和病态妊娠为特征性表现。合并APS的狼疮肾炎(LN)患者易出现更多并发症，预后较差，对治疗的敏感性降低。抗凝和(或)抗血小板是治疗LN合并APS的关键，血浆置换则对重症APS患者有一定疗效。近年来，新型药物与治疗方法如利伐沙班、美罗华及干细胞移植展现出了巨大的治疗潜力。中西医结合治疗是另一种极有希望的治疗方法，多位中医大家均认为活血化瘀法可有效治疗LN合并APS患者。现代临床与药理研究也发现了中药治疗LN合并APS的价值。     

滋阴益肾法治疗特发性血小板减少性紫癜疗效及对患者血清IL-4、IL-6和免疫功能的影响

目的: 探讨滋阴益肾法治疗特发性血小板减少性紫癜(ITP)的疗效及对血清IL-4、IL-6、TNF-α及免疫功能的影响。方法:选取ITP患者84例,随机数字表法分为对照组和观察组,各42例。对照组采用口服泼尼松片治疗,观察组在对照组用药基础上给予滋阴益肾法治疗。比较两组临床疗效,治疗前后IL-4、IL-6、TNF-α、外周血T淋巴细胞亚群(CD₃⁺、CD₄⁺、CD₈⁺、CD₄⁺ /CD₈⁺)及免疫球蛋白水平变化情况。结果:两组治疗后中医症候积分明显降低,观察组优于对照组(P<0.05)。观察组总有效率95.24%高于对照组78.57%(P<0.05); 两组治疗后PLT计数均明显升高、PAIgG表达水平降低,观察组更明显(P<0.05); 两组治疗前IL-4、IL-6、TNF-α及外周血T淋巴细胞亚群水平比较差异无统计学意义(P>0.05); 治疗后CD₃⁺、CD₄⁺、CD₄⁺ /CD₈⁺均升高,IL-4、IL-6、TNF-α、CD₈⁺均降低(P<0.05); 观察组治疗后CD₃⁺、CD₄⁺、CD₄⁺ /CD₈⁺均高于对照组,IL-4、IL-6、TNF-α、CD₈⁺均低于对照组(P<0.05)。治疗后观察组的血清IgA和IgG水平明显高于对照组(P<0.05),而血清IgM水平差异无统计学意义(P>0.05)。两组不良反应情况比较无统计学差异(P>0.05)。结论: 滋阴益肾法可显著降低ITP患者血清IL-4、IL-6等因子水平,提高患者免疫能力。     

基于Citespace的中医药治疗过敏性紫癜可视化分析

目的:挖掘中医药治疗过敏性紫癜(HSP)的主要研究内容、研究前沿、作者合作网络、机构合作网络,为中医药治疗HSP的研究和发展提供参考。方法:利用Citespace("引文空间")软件系统对从中国知网数据库检索到的2878篇中医药治疗HSP相关文献进行可视化分析,对文献关键词进行聚类分析、突现分析,对作者、机构内容进行共现分析。结果:自1995年开始相关文献数量增长速度逐渐加快,2005年以后每年发表文献数量均稳定在100篇以上;聚类分析得到32个聚类,由396个节点,638条线组成,主要聚类结果包括小儿过敏性紫癜、活血化瘀药、紫癜性肾炎、凉血药、临床观察等;突现分析得出52个突现词,可看出中医药治疗HSP在治法上以凉血为主,其次是活血祛瘀和清热,常用药为牡丹皮、生地黄、赤芍等,病证表现上肾脏损伤的相关研究较多,临床上较重视名医经验、小儿过敏性紫癜的研究等;作者合作网络得到以丁樱、孙轶秋、何平为核心的3个主要合作团队图谱;机构合作网络分布图谱密度Density=0.0071。结论:中医药治疗HSP主要研究内容包括小儿过敏性紫癜、活血化瘀药、紫癜性肾炎、凉血药、临床观察等,当前的研究热点以小儿过敏性紫癜、名医经验、紫癜性肾炎、临床疗效为主,已形成相对较稳定的若干研究团队,但在机构合作上联系较为分散。