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.     

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。     

过敏性紫癜性肾炎方药规律及中医证候分析

目的：通过对1993年1月至2019年12月CNKI、VIP、WanFang收录的中医治疗过敏性紫癜性肾炎的文献进行整理分析，探讨中医辨证论治本病的证候和方药规律。方法：采用SPSS 25.0统计软件，对文献中证候、方剂以及药物进行统计归类，总结各证候、方剂、药物出现的频次和所占比例，对药物和临床症状进行聚类分析。结果：共纳入文献93篇，通过整理分析得出39个中医证候，其中脾肾两虚、风盛血热、气不摄血、阴虚火旺、气阴两虚等为中医常见证候；证候要素分析：血热、风热、瘀血、湿热为常见实性病机；肾虚、脾虚、阴虚、气虚为常见虚性病机；病位主要在肾、脾、肺、肝四脏；共得方剂145首，其中自拟方53首，成方92首，二者以补益剂、清热剂、理血剂、祛风剂为主；中药206味，分为13类，以生地黄、牡丹皮、赤芍、当归、茯苓等为常用药。结论：过敏性紫癜性肾炎的病位在肾、脾、肺、肝；风、热、湿、毒、瘀、虚为本病的主要病机。在临床用药中以补虚药、活血药、清热解毒药、利水渗湿药为主，兼以应用祛风药；治疗中应采用中医辨证与辨病相结合的方式。     

滤泡辅助性T细胞及半乳糖缺乏的IgA1在儿童过敏性紫癜中的表达及意义

目的 探讨滤泡辅助性T（Tfh）细胞和半乳糖缺乏的IgA1（Gd-IgA1）在儿童过敏性紫癜（HSP）发病机制中的作用及两者之间的相关性。方法 选取初发HSP患儿36例，根据是否发生紫癜性肾炎（HSPN）分为HSPN组（11例）和非HSPN组（25例）。另选取15例门诊体检儿童作为健康对照组。采用流式细胞术检测外周血中Tfh细胞（CD4⁺CXCR5⁺ICOS⁺）比例。采用ELISA法检测外周血中IL-21、IL-6、血清IgA1、血清Gd-IgA1表达水平。采用Pearson相关分析法分析HSP组患儿血清Gd-IgA1浓度与Tfh细胞比例及其相关因子的相关性。结果 HSPN和非HSPN组患儿外周血Tfh细胞比例及IL-21、IL-6表达水平较健康对照组升高（P < 0.05），HSPN组上述指标较非HSPN组亦明显上升（P < 0.05）。HSPN和非HSPN组患儿血清中IgA1、Gd-IgA1表达水平较健康对照组升高（P < 0.05），HSPN组患儿血清IgA1和Gd-IgA1水平较非HSPN组亦明显升高（P < 0.05）。HSP组患儿血清Gd-IgA1水平与Tfh细胞比例及IL-21、IL-6水平均呈显著正相关关系（P < 0.05）。结论 Tfh细胞及其相关细胞因子和血清Gd-IgA1共同参与HSP/HSPN的发生。Tfh细胞可能介导了Gd-IgA1生成增加。     

胡晓梅论治紫癜风(过敏性紫癜)精要

过敏性紫癜是一种常见的血管变态反应疾病,中医病名为"紫癜风"。胡晓梅教授分期论治紫癜风,并各宗一方,临证强调祛风与化瘀贯穿全程,疏散与酸收并用,止血与散瘀兼顾,疗效显著,阐述精辟。     

自拟紫癜汤治疗过敏性紫癜的临床疗效分析

目的:探究自拟紫癜汤治疗过敏性紫癜的临床疗效。方法:此选取过敏性紫癜患者68例,研究时间为2017年12月-2018年12月,根据治疗方式不同进行分组,分别为对照组与实验组,每组各34例。给予对照组西医治疗,给予实验组自拟紫癜汤治疗。对比治疗效果、症状消失时间、不良反应与免疫学指标,进行统计学分析。结果:观察组有效率优于对照组,P<0.05,差异形成了统计学意义。观察组皮肤紫癜、腹痛、黑便、大便隐血消失时间均优于对照组患者,P<0.05,差异形成了统计学意义。观察组的不良反应优于对照组患者,P<0.05,差异有统计学意义。观察组的治疗后免疫球蛋白A(IgA)、免疫球蛋白M(IgM)水平优于对照组患者,P<0.05,差异形成了统计学意义。结论:自拟紫癜汤治疗过敏性紫癜患者,能有效提高治疗有效率,改善临床症状,缩短治疗时间,安全性高,改善免疫学指标,具有显著的治疗效果。