Henoch-Schnlein purpura complicating adalimumab therapy for Crohn's disease

Anti-tumour necrosis factor-α(TNF) therapy has revolutionised the management of chronic inflammatory conditions.With ever increasing numbers of patients being treated with these agents,uncommon adverse reactions will inevitably occur more frequently.Cutaneous manifestations are associated with many of these chronic conditions and can complicate anti-TNF therapy in about 20% of cases.Vasculitic complications are rarely associated with anti-TNF therapy.Henoch-Schnlein purpura(HSP),a small vessel vasculitis,has been described following infliximab and etanercept therapy but never with adalimumab,a fully humanized TNF antibody.The risk of such immune-mediated reactions is theoretically less with adalimumab compared to infliximab but can still occur.Here we report the f irst case in the literature of HSP that can be attributed to the use of adalimumab in a 19-year-old male with recalcitrant Crohn's disease.

Risk factors for intussusception in children with Henoch-Schönlein purpura:A case-control study

BACKGROUND The etiology of Henoch-Schönlein purpura(HSP)with intussusception remains undefined.AIM To investigate the risk factors for intussusception in children with HSP and gastrointestinal(GI)involvement.METHODS Sixty children with HSP and concomitant intussusception admitted to the Beijing Children’s Hospital of Capital Medical University between January 2006 and December 2018 were enrolled in this study.One hundred pediatric patients with HSP and GI involvement but without intussusception,admitted to the same hospital during the same period,were randomly selected as a control group.The baseline clinical characteristics of all patients,including sex,age of onset,duration of disease,clinical manifestations,laboratory test results,and treatments provided,were assessed.Univariate and multiple logistic regression analyses were performed to identify possible risk factors.RESULTS The 60 children in the intussusception group comprised 27 girls(45%)and 33 boys(55%)and the 100 children in the non-intussusception group comprised 62 girls(62%)and 38 boys(38%).The median age of all patients were 6 years and 5 mo.Univariate and multiple regression analyses revealed age at onset,not receiving glucocorticoid therapy within 72 h of emergence of GI symptoms,hematochezia,and D-dimer levels as independent risk factors for intussusception in children with HSP(P<0.05).CONCLUSION The four independent risk factors for intussusception in pediatric HSP with GI involvement would be a reference for early prevention and treatment of this potentially fatal disease.

Severe Henoch-Schonlein purpura with infliximab forulcerative colitis

Infliximab （IFX） is an anti-tumor necrosis factorchimeric antibody that is effective for treatment ofautoimmune disorders such as Crohn＇s disease andulcerative colitis （UC）. IFX is well tolerated with alow incidence of adverse effects such as infections,skin reactions, autoimmunity, and malignancy.Dermatological manifestations can appear as infusionreaction, vasculitis, cutaneous infections, psoriasis,eczema, and skin cancer. Here, we present anunusual case of extensive and sporadic subcutaneousecchymosis in a 69-year-old woman with severe UC,partial colectomy and cecostomy, following her initialdose of IFX. The reaction occurred during infliximabinfusion, and withdrawal of IFX led to gradual alleviationof her symptoms. We concluded that Henoch-Sch？nleinpurpura, a kind of leukocytoclastic vasculitis, mighthave contributed to the development of the bruising.Although the precise mechanisms of the vasculitis arestill controversial, such a case highlights the importanceof subcutaneous adverse effects in the management ofUC with IFX.

Effect of intestinal flora from children with Henoch-Sch?nlein purpura on visceral sensitivity, gastrointestinal hormones and cytokines secretion in pseudo-sterile rats

Objective:To investigate the effect of intestinal flora from Henoch-Schönlein purpura(HSP)on visceral sensitivity,gastrointestinal hormones and cytokines in pseudo-sterile rats.Methods:The pseudo-sterile rat model was established.The rats were was given fecal microbiota solutions of children with abdominal HSP and healthy children,respectively.The visceral sensitivity was determined by abdominal withdrawal reflex(AWR)which was induced by rectal balloon distention in all the rats.And serum gastrin(Gas),motilin(MTL),cholecystokinin(CCK),substance P(SP),tumor necrosis factor(TNF)-αand interleukin(IL)-6 levels in rats were measured with ELISA method.Results:The volume of rectum water injection under the score 3 of AWR in the rats administrated with fecal microbiota solution from HSP children(HSP group)was significantly decreased compared with that in the rats administrated with fecal microbiota solution from healthy children(HC group),and there was significant difference between these two groups(P<0.05).The serum Gas,MTL,CCK and SP levels were higher in HSP group than those in HC group.And serum MTL,CCK and SP levels in HSP group were significantly different from those in the HC group.The serum TNF-αandIL-6 levels were higher in HSP group than those in HC group,there was significant difference between these two groups(P<0.05).Conclusion:Intestinal flora from HSP can induce the production of visceral sensitivity,inhibit gastrointestinal hormone secretion and prompt cytokine production.

Effect of Activation of the Ca2+-Permeable Acid-Sensing Ion Channel 1a on Acid-Induced Vascular Endothelial Cell Injury of Henoch-Schönlein Purpura Children

Acidosis in local environment plays a critical role in cell injury. One key mediator of acidosis-induced cell injury is the acid-sensing ion channels (ASICs), particularly ASIC1a. Herein, we investigated the role of ASIC1a in acid-induced vascular endothelial cell injury of Henoch-Schonlein purpura (HSP) children. Acid-induced ASIC1a, Calpain and Calcineurin expression in vascular endothelial cells pretreated with IgA1 isolated from HSP were detected by real time quantitative polymerase chain reaction and western blot methods, respectively. Cell cytotoxicity was measured by interleukin-8 and nitric oxide production with ELISA. The results showed acid-induced ASIC1a, Calpain and Calcineurin expression in cells increased, especially at PH6.5. The cytotoxicity of vascular endothelial cells was increased by extracellular acidosis. Moreover non-specific or specific blockers of ASIC1a, Amiloride and PcTX-1 could remarkably decrease these parameters. These findings show that increased [Ca2+]i, mediated via ASIC1a, might contribute to acid-induced vascular endothelial cell injury of HSP.

一项回顾性研究:261例Henoch-Schnlein性紫癜患儿胃肠道表现

目的:小儿Henoch-Schonlein性紫癜(HSP)为儿童的一种IgA介导的自身免疫性血管炎。其常见症状为:紫癜性皮疹、腹痛、肾脏或关节受累等。HSP患儿以腹痛常见,但常被疑为肠套叠或肠穿孔。本文用粪便隐血试验和影像学方法来鉴别腹痛。方法:回顾性研究1991年12月至2001年12月间261例HSP患儿。对腹痛患儿进行影像学检测,包括:腹部超声、腹部CT。

祛湿化斑颗粒通过调控IL/STAT信号通路对过敏性紫癜性肾炎大鼠的影响

探讨祛湿化斑颗粒(QSHB Gr)通过调控IL/STAT信号通路对过敏性紫癜性肾炎大鼠的影响及可能机制。将幼龄清洁(SPF)级SD大鼠36只随机分为6组:对照组、模型组、QSHB Gr/0.2剂低剂量组、QSHB Gr/0.4剂中剂量组、QSHB Gr/0.8剂高剂量组及霉酚酸酯(MMF 0.3 g/kg)组。综合模拟IgA肾病与血热证动物模型,复合成为过敏性紫癜肾炎大鼠(HSPN)模型;病理学检测肾脏损伤;测定血清肾脏生化指标总蛋白(TP)、白蛋白(ALB)的质量浓度以及肌酐(Cre)和血尿素氮(BUN)的浓度;收集24 h尿观察尿量并检测尿蛋白排泄情况;免疫组织化检测肾脏免疫复合物IgA、IgG沉积;Elisa法检测IL-17、IL-6、IL-2及TGF-β1的含量;Western blot检测STAT3、STAT5、RORγt、FoxP3蛋白表达。结果显示:与HSPN大鼠相比,QSHB Gr治疗可以通过减轻肾脏组织病理损伤、升高血清TP、ALB浓度,降低Cre、BUN浓度及24 h尿蛋白水平(P<0.01)显著改善HSPN大鼠肾脏损伤,且各个指标随QSHB Gr剂量增加变化越明显。QSHB Gr治疗可以显著抑制肾脏免疫复合物IgA、IgG沉积;显著降低IL-17、IL-6及TGF-β1的含量(P<0.05或P<0.01),升高IL-2含量(P<0.01);显著升高STAT5与FoxP3的表达水平(P<0.05或P<0.01),降低STAT3与RORγt的表达水平(P<0.01),各个指标随QSHB Gr剂量增加而变化越明显。以祛湿化斑颗粒(QSHB Gr)治疗可以改善过敏性紫癜性肾炎,可能是HSPN临床治疗的潜在候选药物。其机制可能与抑制IgA和IgG沉积及IL-6/STAT3与IL-2/STAT5信号通路调控密切相关。

过敏性紫癜患儿预后影响因素及其预测价值研究

目的:探讨过敏性紫癜(HSP)患儿预后的影响因素及其预测价值。方法:选取HSP患儿150例为研究对象。根据患儿治疗后有无复发分为预后不良组(47例)和预后良好组(103例)。比较两组患儿一般资料及生化指标(白细胞计数、血小板计数、血清总蛋白、乳酸脱氢酶、碱性磷酸酶、C-反应蛋白、免疫球蛋白A)。分析HSP患儿预后的影响因素及其对患儿预后不良的预测价值。结果:预后不良组皮疹反复发作、初次发病伴肾损害和呼吸道感染比例高于预后良好组(均P<0.05)。预后不良组血小板计数、C-反应蛋白及免疫球蛋白A水平高于预后良好组(均P<0.05)。皮疹反复发作、初次发病伴肾损害、呼吸道感染、血小板计数、C-反应蛋白及免疫球蛋白A为HSP患儿预后的独立影响因素(均P<0.05)。皮疹反复发作、呼吸道感染、血小板计数、C-反应蛋白及免疫球蛋白A对HSP患儿预后不良具有预测价值(均P<0.05)。结论:皮疹反复发作、初次发病伴肾损害、呼吸道感染、血小板计数、C-反应蛋白及免疫球蛋白A为HSP患儿预后的独立影响因素。除初次发病伴肾损害指标外,其余指标对HSP患儿预后不良具有一定预测价值。

基于数据挖掘的紫癜性肾炎动物模型分析

目的 基于文献挖掘探讨紫癜性肾炎动物模型的造模特点,为制备规范化的紫癜性肾炎动物模型提供参考。方法 通过计算机检索中国知网、万方、维普、中国生物医学文献数据库、PubMed中英文数据库中相关研究文献,获取近20年紫癜性肾炎动物实验文献,将实验动物种类、造模方法、给药剂量、给药周期、成模标准及检测指标进行人工筛选,应用Microsoft Excel 2021软件建立数据库并进行统计分析,运用SPSS Modeler 18.0对高频指标进行关联规则分析并运用Cytoscape 3.6.1软件对关联网络图进行可视化升级。结果 归纳总结符合纳入标准的106篇文献,建立紫癜性肾炎动物模型多选用SD大鼠和KM小鼠,造模方式多选用药源性诱导,造模药物以牛血清白蛋白(bovine serum albumin, BSA)+脂多糖(lipopolysaccharide, LPS)+四氯化碳(carbon tetrachloride, CCl_4)+蓖麻油、卵白蛋白(ovalbumin, OVA)+弗氏完全佐剂、麦胶蛋白+印度墨水、牛血清白蛋白+葡萄糖菌肠毒素B(staphylococcus enterotoxin B,SEB)复刻紫癜性肾炎吻合度较高的动物模型,周期一般在5~14周,成模标准多选用皮肤紫癜,尿红细胞数增多,尿蛋白阳性,肾组织可见肾小球系膜增生及以免疫球蛋白A(immunoglobulin A,IgA)为主的免疫复合物沉积于小血管表示造模成功。检测指标共涉及36个医学指标,其中肾及尿液相关的检测指标23个,血液相关检测指标9个,其中使用频率≥10%的有24 h尿蛋白定量、白细胞介素、肾病理、尿红细胞计数、IgA及循环免疫复合物(circulation immune complex, CIC)、肌酐(creatinine, Cr)等10个指标,对高频指标进行聚类分析,结果表明多采用24 h尿蛋白定量-白细胞介素-肾病理-尿红细胞数-IgA综合评价模型。结论 现有的紫癜性肾炎动物实验多选用SD雄性大鼠和KM雌性小鼠,造模方式多采用药源性诱导,其中瘀热证复合IgA肾病法(病证结合法)具有重复性强、成模率高的优点,可为HSPN动物实验模型选择提供参考。

过敏性紫癜患儿血清miRNA-125b、HIF-1α水平与Th1/Th2比值的相关性

目的探讨过敏性紫癜(HSP)患儿血清微RNA(miRNA)-125b、低氧诱导因子-1α(HIF-1α)水平与Th1/Th2比值的相关性。方法前瞻性选取2020年1月至2023年12月商洛市中心医院收治的97例HSP患儿为试验组,97名健康体检儿童为对照组。通过实时荧光定量聚合酶链式反应检测miRNA-125b表达,酶联免疫吸附试验检测HIF-1α、肿瘤坏死因子α(TNF-α)、白细胞介素(IL)-6及IL-1β等水平,流式细胞术检测Th1和Th2细胞数量及Th1/Th2比值。采用Pearson相关分析法分析miRNA-125b和HIF-1α与Th1/Th2比值的关系,并通过受试者操作特征(ROC)曲线评估血清miRNA-125b、HIF-1α及相关细胞因子水平对HSP的诊断价值。结果试验组患儿血清中miRNA-125b、HIF-1α、Th2细胞水平分别为0.82±0.18、125.78±20.54、7.83±1.57,均明显高于对照组(0.37±0.11、56.20±8.99、4.60±0.55),而Th1细胞、Th1/Th2比值水平分别为7.43±1.66、1.02±0.11,均明显低于对照组(11.89±2.80、2.90±0.58),差异均有统计学意义(P<0.05)。试验组患儿血清TNF-α、IL-6、IL-1β和IL-4的水平分别为(32.5±4.8)、(21.3±3.9)、(11.0±1.8)、(5.3±1.1)pg/mL,均明显高于对照组[(14.8±3.7)、(11.7±2.8)、(6.0±1.3)、(2.5±0.7)pg/mL],差异均有统计学意义(P<0.05)。Pearson相关性分析显示,miRNA-125b和HIF-1α与Th1/Th2比值、TNF-α、IL-6、IL-1β水平均呈显著负相关(P<0.05)。血清HIF-1α和miRNA-125b诊断HSP的曲线下面积分别为0.821和0.977。结论HSP患儿血清miRNA-125b和HIF-1α水平升高,Th1/Th2比值及相关免疫因子水平降低,检测血清HIF-1α和miRNA-125b水平对于诊断HSP有较高的临床价值。

粪钙卫蛋白在腹型过敏性紫癜中的相关研究进展

腹型过敏性紫癜(abdominal type of Henoch-Sch nlein purpura,AHSP)是一种由IgA介导的全身性血管炎,常发于儿童。AHSP由于症状非典型,故早期筛查诊断难度较大,常规通过胃肠镜检查确诊。钙卫蛋白由S100A8和S100A9蛋白亚体组成,一般来源于中性粒细胞,具有多种生物学作用。粪钙卫蛋白浓度与肠道炎症相关,用于评估胃肠道炎症。鉴于AHSP常伴胃肠道疾病,粪钙卫蛋白具备作为AHSP早期诊断指标的潜力。本研究综述了粪钙卫蛋白在AHSP早期诊断中的应用价值,旨在为将其作为AHSP的临床诊断指标增加理论依据。

基于384例患者探讨中药治疗重型紫癜性肾炎的用药规律及作用机制

目的:研究中药治疗重型紫癜性肾炎(henoch-schonlein purpura nephritis,HSPN)的用药规律及作用机制。方法:收集2020年1月1日—2023年8月31日期间于河南中医药大学第一附属医院就诊的重型紫癜性肾炎患者的病历数据,汇总其24 h尿蛋白定量最高时的中药处方进行归纳总结,进行用药频次和性味归经统计。然后通过TCMSP数据库获得药物的有效成分及其作用靶点,从疾病数据库获取HSPN的靶点,通过Venny及String数据库进行交集靶点的蛋白相互作用分析,运用Metascape数据库进行KEGG及GO富集分析。最后用分子对接技术验证所得结果。结果:共收集病例384个,进行中西医结合治疗2周后尿蛋白、尿潜血及24 h尿蛋白定量均较之前明显下降(P<0.01),其中药处方中涉及213味中药,性多温或寒,味多甘淡,归肝、脾、肾、肺经,黄芪、茯苓、山茱萸的使用居于前列,有效成分主要为槲皮素、山奈酚、常春藤皂苷元等。经筛选后其与HSPN得到核心靶点21个,主要靶点为TNF、IL-6、IL-β_(1)、AKT1、MMP9。主要作用通路为AGE-RAGE信号通路、NF-κB信号通路等。分子对接结果证明,有效成分与核心靶点的对接性能良好。结论:中药治疗重型紫癜性肾炎的药物多为补脾益肾、滋阴凉血,活血散瘀为多,通过槲皮素、山奈酚、常春藤皂苷元等成分作用于TNF、IL-6、IL-β_(1)、AKT1、MMP9参与了抑制炎症反应,调控细胞凋亡等过程治疗重型紫癜性肾炎。

儿童血管内压力增高性紫癜误诊为过敏性紫癜临床分析

目的探讨儿童血管内压力增高性紫癜误诊为过敏性紫癜的原因、鉴别要点及防范措施。方法回顾性分析2023年3月至2024年3月收治的误诊为过敏性紫癜的血管内压力增高性紫癜患儿38例的临床资料。结果38例患儿初诊时出现不同程度的双侧下肢或双足、踝部针尖大小皮疹,散在或密集分布,不高出皮肤,压之不褪色。8例患儿皮疹亦可见于头面部、双侧上肢及躯干部。既往诊断为过敏性紫癜,予相应治疗未见明显缓解,详细询问患儿病史,观察皮疹形态及分布特点,完善实验室检查排除其他血液系统及免疫系统疾病后诊断为血管内压力增高性紫癜,予停药观察,随访24周。38例患儿中27例皮疹复发,未予药物干预自行消退。误诊时间为2~28周。全部病例均未出现肾脏损害。结论血管内压力增高性紫癜临床表现不典型,无特异性检查指标,易被误诊。临床需加强病史询问、皮疹形态的鉴别诊断,及时完善相关检查,减少误诊。

Henoch-Schönlein purpura nephritis in children:incidence,pathogenesis and management

Background:Henoch-Schönlein purpura(HSP)is one of the most common vasculitides in children.It is manifested by skin purpura,arthritis,abdominal pain,renal involvement,etc.Typically,HSP is considered to be self-limiting,although renal involvement(HSP purpura nephritis,HSPN)is the principal cause of morbidity from this disease.For this reason,it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for HSPN.In this article,we review the updated pathophysiology and treatment strategies for HSPN.Data sources:We searched databases including PubMed,Elsevier and Wanfang for the folowing key words:Henoch-Schönlein purpura,nephritis,mechanism and treatment,and we selected those publications written in English that we judged to be relevant to the topic of this review.Results:Based on the data present in the literature,we reviewed the following topics:1)the possible pathogenesis of HSPN:several studies suggest that immunoglobulin A immune complexes deposit in the mesangium and induce renal injury;2)multiple-drug treatment for HSPN:although there have been few evidence-based treatment strategies for HSPN,several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological severity.Conclusions:HSPN is a severe disease of childhood.To better understand this disease,detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.

可溶性血管细胞黏附分子-1、中性粒细胞与淋巴细胞比值在过敏性紫癜患儿血清中的变化及检测意义

目的:探讨可溶性血管细胞黏附分子-1(sVCAM-1)、中性粒细胞与淋巴细胞比值(NLR)在过敏性紫癜(HSP)患儿血清中的变化及检测意义。方法:选取接受诊治的HSP患儿86例(HSP组),另选取同时期体检健康儿童101例(对照组)。检测中性粒细胞、淋巴细胞及血清sVCAM-1,计算NLR。比较对照组和HSP组NLR、sVCAM-1水平变化。比较HSP组不同疾病分期患儿NLR、sVCAM-1水平变化。随访6个月,根据肾脏病变或复发情况将患儿分为预后不良组(14例)和预后良好组(72例)。比较HSP组不同预后患儿NLR与sVCAM-1水平变化,分析NLR与sVCAM-1水平对HSP患儿预后不良的预测价值。采用Pearson法分析HSP组NLR与sVCAM-1水平的相关性。结果:与对照组比较,HSP患儿NLR、sVCAM-1水平更高(均P<0.05)。在HSP组中,急性期患儿NLR、sVCAM-1水平高于恢复期患儿(均P<0.05)。预后不良组NLR、sVCAM-1水平高于预后良好组(均P<0.05)。NLR联合sVCAM-1预测患儿预后不良的AUC为0.712,高于NLR与sVCAM-1单独预测的AUC(均P<0.05)。NLR与sVCAM-1水平呈正相关(P<0.05)。结论:HSP患儿sVCAM-1和NLR水平升高,与病情活动性和预后有关,两者联合对患儿预后预测具有一定价值。

Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children

Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.

中医外治法治疗小儿过敏性紫癜的研究进展

过敏性紫癜(HSP)是一种以免疫球蛋白A介导的血管炎为主要特征的系统性血管炎症疾病,临床常表现为皮肤紫癜、关节炎、腹痛、肾病伴蛋白尿。目前HSP的发病机制尚不完全清楚,因此治疗的重点是对症支持治疗,常采用抗组胺药和血管保护药物等进行治疗。而中医外治法(如中药熏洗、灌肠、针灸)可直接作用于病变部位,治疗方式多样且疗效显著,可减少西药引起的胃肠反应及肝肾功能损伤,且患儿接受度高,有望在临床大量应用,以促进HSP患儿更快、更好恢复。

儿童过敏性紫癜临床特征分析(附184例报道)

目的 了解过敏性紫癜儿童的临床特征。方法 回顾性分析过敏性紫癜患儿 184例 ,对其临床表现及特征进行分析。结果 ①发病年龄均 >2岁 ,平均发病年龄 (7.8± 2 .6 )岁 ;6 7.4 %患儿于秋冬季节发病 ;6 2 .5 %患儿有明确诱因 ,以上呼吸道感染为主。②所有病人均有典型皮肤紫癜 ,分布于下肢 (10 0 % )、臀部(47.3% )、上肢 (17.4 % )等。 89.1%患儿以皮肤紫癜首发 ,少数以胃肠道及关节症状为首发症状后 2周内出疹 ,先出现肾脏损害而后出疹者罕见。胃肠道及关节症状发生率分别为 6 0 .9%和 4 4 .6 %。紫癜性肾炎发生率为 5 2 .2 % ,临床上主要表现为血尿和 (或 )蛋白尿 (6 8.8% ) ,病理改变主要为Ⅰ～Ⅲ级 (85 .2 % )。③ 4 4 .0 %病人血沉增快 ,2 4 .1%ASO增高 ;6 3.7%病人IgA增高 ,IgG ,IgM及补体绝大多数正常。 结论 过敏性紫癜为儿童常见病 。

甘露糖结合凝集素基因多态性与中国汉族儿童过敏性紫癜的相关性研究

目的 近来有研究认为某些自身免疫性疾病可能与甘露糖结合凝集素 (MBL)缺陷有关。MBL基因多态性影响MBL水平。过敏性紫癜 (Henoch Sch nleinpurpura ,HSP)是一种全身性血管炎综合征 ,其病因被认为可能与免疫损伤有关。该研究探讨MBL基因第 5 4号密码子多态性与中国汉族儿童过敏性紫癜 (HSP)的关系。方法 应用聚合酶链反应 -限制性内切酶片段长度多态性分析 ,对 1 6 0例健康中国汉族儿童及 1 0 4例过敏性紫癜(HSP)患儿的MBL基因多态性进行检测。结果 ①HSP患儿MBL基因GGC/GAC基因型频率明显高于健康对照组 (5 1 .9%vs 2 5 .0 % ) (P <0 .0 5 ) ,而GGC/GGC基因型频率显著低于健康对照组 (4 6 .2 %vs 73.8% ) (P <0 .0 5 )。HSP患儿GAC等位基因频率明显高于健康对照组 (0 .2 79vs 0 .1 38) (P <0 .0 5 ) ,而GGC等位基因频率明显低于健康对照组 (0 .72 1vs 0 .86 2 ) (P <0 .0 5 ) ;GAC等位基因与HSP的发病明显相关 (OR =2 .4 6 ,95 %CI =1 .32 - 4 .4 8,P <0 .0 5 )。②在HSP患儿中 ,GAC型等位基因携带者中有前驱感染史者明显多于GGC纯合子 (P<0 .0 5 )。结论 HSP发病受遗传背景影响 ,MBL第 5

吗替麦考酚酯治疗以蛋白尿为主的儿童紫癜性肾炎疗效观察

目的评价吗替麦考酚酯(MMF)分散片联合小剂量激素治疗以蛋白尿为主的儿童难治性紫癜性肾炎的临床疗效及安全性。方法选取19例以蛋白尿为主的难治性紫癜性肾炎儿童作为研究对象,其中5例在MMF治疗之前接受肾活检(轻度系膜增殖性肾炎2例,中度系膜增殖性肾炎伴新月体形成1例,重度系膜增殖性肾炎1例,局灶节段性肾小球硬化1例)。泼尼松0.5～1.0 mg/(kg.d),平均0.74 mg/(kg.d),尿蛋白转阴2周后逐渐减量,半月减5～10 mg至5～10 mg/d维持;MMF分散片20～25 mg/(kg.d),2次/d。随访1～1.7年。治疗期间定期复查血常规、尿常规、肾功能、肝功能以及24 h尿蛋白定量等,治疗3个月后进行疗效及安全性评价。结果 MMF分散片联合小剂量激素治疗前、后24 h尿蛋白定量和血清白蛋白差异有统计学意义(P<0.01)。治疗3个月后19例中完全有效17例,部分有效2例。1例应用MMF后2个月合并水痘,1例出现了胃肠道症状,2例一过性白细胞减少。结论吗替麦考酚酯分散片联合小剂量泼尼松能有效缓解以蛋白尿为主的儿童紫癜性肾炎尿蛋白,改善病情,且近期不良反应少,安全性高,依从性好。