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.

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.

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.

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.

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.

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。

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。

血清骨膜蛋白、多配体蛋白聚糖4与紫癜性肾炎患儿肾脏病理分级的关系

目的探讨紫癜性肾炎(HSPN)患儿血清骨膜蛋白(POSTN)、多配体蛋白聚糖4(SDC4)的水平及临床意义。方法将2019年3月—2021年12月在苏州大学附属儿童医院住院诊治的122例过敏性紫癜(HSP)患儿根据是否并发肾损害分为HSPN组74例和HSP组48例,并选取同期40例健康儿童作为对照组。采用酶联免疫吸附实验(ELISA)检测各组血清POSTN、SDC4水平;分析血清POSTN、SDC4水平与HSPN患儿发病年龄、性别、血脂、凝血功能、肾功能、24 h尿蛋白定量及肾脏病理分级的关系;采用受试者工作特征(ROC)曲线分析血清POSTN、SDC4水平对HSPN的早期诊断价值。结果HSPN组患儿血清POSTN、SDC4、24 h尿蛋白定量与体质量比值、尿微量白蛋白、肌酐、尿素氮水平高于HSP组和对照组,HSP组患儿血清POSTN、SDC4、24 h尿蛋白定量与体质量比值、尿微量白蛋白、肌酐、尿素氮水平高于对照组,差异均有统计学意义(P<0.05)。不同肾脏病理分级的HSPN患儿血清POSTN、SDC4水平比较,差异有统计学意义(P<0.05)。HSPN患儿血清POSTN、SDC4水平与24 h尿蛋白定量与体质量比值呈正相关(P<0.05),血清POSTN与SDC4表达水平呈正相关(P<0.05)。血清POSTN的ROC曲线的曲线下面积为0.788,敏感度为94.1%,特异度为59.2%;血清SDC4的ROC曲线的曲线下面积为0.842,敏感度为84.5%,特异度为69.2%。结论HSPN患儿血清POSTN、SDC4水平升高,二者表达水平与肾组织病理分级及24 h尿蛋白定量与体质量比值有关,检测血清POSTN、SDC4水平有助于HSPN的早期诊断。

349例儿童和成人紫癜性肾炎(血尿和蛋白尿型)临床和病理特点分析

目的:探讨349例儿童和成人HSPN(血尿和蛋白尿)临床和肾脏病理差异及其相关性。方法:回顾分析2017年01月—2019年12月,在河南中医药大学第一附属医院肾内科及儿科住院HSPN(血尿和蛋白尿型)患者的临床、生化及肾脏病理。结果:儿童组与成人组比较,24 h尿蛋白定量和血清胆固醇均显著降低(P<0.01),肾小球和肾小管间质主要病理级别为Ⅲa级和(+)级,成年组为Ⅲb级和(++)级,两组差异具有统计学意义(P<0.001);新月体比例低于成年组(P<0.01)。两组24 h尿蛋白定量与肾小球病理分级呈正相关(P<0.01;P<0.05),成人组血清白蛋白与肾小球病理分级呈负相关(P<0.05)。结论:成人HSPN(血尿和蛋白尿型)患者的临床和新月体比例、病理分级比儿童严重;两组24 h尿蛋白定量与肾脏病理分级呈正相关。

清营凉血方对过敏性紫癜性肾炎大鼠肾组织超微结构及血栓调节蛋白表达的影响

目的:观察清营凉血方对过敏性紫癜性肾炎(HSPN)大鼠肾组织病理结构、超微结构和血栓调节蛋白(TM)表达的影响,探讨清营凉血方治疗过敏性紫癜性肾炎的作用机制。方法:25只大鼠随机分为5只正常组和20只造模组,造模组采用“牛血清白蛋白+脂多糖+四氯化碳”联合干姜造就HSPN瘀热模型,造模成功后,随机分为模型组、清营凉血方低、高剂量(11.05 g/kg、44.18 g/kg)组及氯沙坦钾片(7.13 mg/kg)组,每组5只。实验造模12周,每组给药5周,给药结束后,分别用光镜及透射电镜观察肾组织病理和超微结构,采用免疫荧光法观察肾组织IgA沉积情况,采用ELISA及WB法分别观察血清及肾组织中TM的表达情况。结果:清营凉血方可以减少肾组织中IgA沉积,所有给药组相较于模型组IgA沉积荧光强度显著降低(P<0.01);清营凉血方低、高剂量可以显著减少血清及肾组织中TM的表达(P<0.01)。结论:清营凉血方可以降低肾组织IgA沉积,修复肾组织病理损伤,改善足细胞足突融合,其机制可能与降低血清及肾组织TM的表达,改善血管内皮结构,减缓足细胞损伤,改善肾小球滤过屏障相关。

基于“咽肾相关”理论研究烙法在治疗紫癜性肾炎中的应用

紫癜性肾炎是过敏性紫癜导致的肾脏损害,临床多见血尿、蛋白尿等表现,常伴有扁桃体反复感染及肿大,现代医学研究认为扁桃体切除术对其治疗有效。中医学“咽肾相关”理论认为,扁桃体病变与紫癜性肾炎病情变化极具相关性。中医烙法广泛应用于慢性扁桃体疾病,能有效缓解患者咽喉症状,消除炎症反应,保留扁桃体的免疫调节与防御功能,故对紫癜性肾炎的治疗有重要意义。

紫癜性肾炎患儿一般临床指标与肾脏病理相关性探讨

目的分析紫癜性肾炎(HSPN)患儿一般临床指标与肾脏病理间的关系,探讨预测肾脏病理分级的临床标志物。方法回顾性分析2017年1月—2021年12月本院收治的经肾活检确诊的69例HSPN患儿的临床和实验室资料,并根据ISKDC分级系统进行肾脏病理分级,分析不同病理分级间各临床指标是否存在差异。结果本组资料中,过敏性紫癜(HSP)患儿肾脏受累常发生在起病后4周内。69例HSPN患儿肾脏活检病理分级示Ⅱ级30例(43.5%),Ⅲa级12例(17.4%),Ⅲb级27例(39.1%),无Ⅰ级、Ⅳ级、Ⅴ级和Ⅵ级。中性粒细胞计数/淋巴细胞计数(NLR)、血小板计数/淋巴细胞计数(PLR)、血免疫球蛋白和补体、凝血和纤溶系统相关指标等在各病理分级间无统计学差异(P>0.05),而血清白蛋白(ALB)、24小时尿蛋白(24 h-UP)、尿白蛋白(UA)、尿IgG(U-IgG)水平在各病理分级间存在统计学差异(P<0.05);进一步两两比较,Ⅱ级与Ⅲa级、Ⅲa级与Ⅲb级间无统计学差异,仅在Ⅱ级与Ⅲb级间存在统计学差异。分析各病理分级的蛋白尿情况,显示Ⅲb级HSPN患儿多表现为肾病范围蛋白尿,且主要为肾小球源性,以UA为主要成分;U-IgG水平亦与肾脏病理分级成正相关,考虑可能系肾损伤较重致其升高。此外,24 h-UP总水平的升高与病理分级成正相关(r=0.308,P=0.011),其预测Ⅲb级病理分级的ROC曲线下面积为0.702,当24 h-UP>24.79 mg kg^(-1)时,预测的灵敏度和特异度分别为61.5%、81.0%。结论HSPN患儿尿蛋白水平的高低与肾脏病理类型密切相关;尤当24 h-UP>24.79 mg kg^(-1)时,提示肾脏病变程度较重,临床可参照此标准初步预测HSPN患儿的肾脏病理分级。

过敏性紫癜及过敏性紫癜性肾炎患者中抗β2糖蛋白1抗体水平的研究

目的通过研究比较过敏型紫癜非肾炎患者、过敏性紫癜性肾炎患者与健康人群中抗β2-GP1抗体水平变化,来明确抗β2-GP1抗体在过敏性紫癜及过敏性紫癜性肾炎发病过程中是否有相关性。同时比较抗β2-GP1抗体阳性与阴性患者中,红细胞沉降率(ESR)、C反应蛋白(CRP)水平,来明确抗β2-GP1抗体与炎症指标的相关性。方法将80例过敏性紫癜患者根据是否有肾炎,分为40例过敏性紫癜非肾炎组及40例过敏性紫癜性肾炎组。40例健康者作为健康对照组。采用酶联免疫吸附法检测,比较3组血清中抗β2-GP1抗体阳性率。并比较过敏性紫癜患者中,抗β2-GP1抗体患者与非阳性患者中ESR、CRP水平。结果过敏性紫癜非肾炎组较健康对照组抗β2-GP1抗体阳性率明显增高,差异具有统计学意义(P<0.05);过敏性紫癜性肾炎组较过敏性紫癜非肾炎组抗β2-GP1抗体阳性率明显增高,差异具有统计学意义(P<0.05)。过敏性紫癜非肾炎组及肾炎组中抗β2-GP1抗体阳性患者较抗体阴性患者的ESR、CRP水平明显增高,差异具有统计学意义(P<0.05)。结论抗β2-GP1抗体可能与过敏性紫癜及过敏性紫癜性肾炎有相关性,可能参与炎症反应。

血液红细胞分布宽度、中性粒细胞与淋巴细胞比值对紫癜性肾炎及其新月体形成的诊断价值

目的探讨血红细胞分布宽度(RDW)、中性粒细胞与淋巴细胞比值(NLR)在预测儿童紫癜性肾炎(HSPN)及新月体形成中诊断价值。方法选取确诊为过敏性紫癜(HSP)患者为研究对象,收集患儿血常规、血生化及肾脏病理检查结果,根据有无肾脏损害分为HSPN组和非HSPN组,HSPN组依据肾脏病理检查有无新月体形成分为有新月体组和无新月体组;对各分组临床资料进行非参数检验,采用二元logistic回归分析RDW、NLR是否HSPN及新月体形成的影响因素,受试者特征工作曲线(ROC)下面积(AUC)分析RDW、NLR对HSPN新月体形成的诊断价值,Spearman分析RDW、NLR与C-反应蛋白(CRP)、24 h尿蛋白定量(24-UP)相关性。结果HSPN组患儿的年龄、血红蛋白(HGB)、血小板(PLT)、尿素氮(BUN)、肌酐(SCR)、胱抑素-C(Csy-C)、免疫球蛋白-A(IgA)、24-UP、RDW及NLR的水平高于非HSPN组,CRP、白蛋白(ALB)、免疫球蛋白-G(IgG)水平低于非HSPN组(P<0.05),有新月体组患儿的白细胞(WBC)、PLT、IgA、RDW、NLR高于无新月体组(P<0.05);二元logistic回归分析显示,RDW、NLR是HSP患儿发生HSPN的影响因素,RDW是HSPN新月体形成的影响因素;RDW、NLR诊断HSPN发生的AUC值分别为0.652,0.546,两者联合诊断HSPN发生的AUC值为0.665;RDW、NLR诊断新月体形成的AUC值分别为0.653,0.648,两者联合诊断新月体形成的AUC值为0.654;RDW、NLR与24-UP呈正相关;CRP与RDW呈负相关,与NLR无相关性。结论RDW、NLR是发生HSPN的影响因素,RDW是HSPN新月体形成的影响因素;RDW、NLR联合诊断HSPN的发生及其新月体形成的价值较单一指标意义更大。

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。

过敏紫癜性肾炎肾损害发生时间与临床病理的联系

目的 :回顾性分析过敏紫癜性肾炎 (HSPN)患者肾脏损害发生时间不同与临床病理及预后的联系。 方法 :1983年 1月至 2 0 0 1年 12月在我院肾活检确诊HSPN、明确记载皮肤紫癜与肾损害发生时间 ,并定期随访的患者 14 5例。分为三组 :组 1(肾损害早发组 ,n =84 ) ,肾炎在皮肤紫癜出现后 1个月内起病 ,病程中皮肤紫癜仅出现 1次 ;组 2 (反复紫癜 /肾损害迟发组 ,n =4 8) ,皮肤紫癜发作至少 2次或皮损发生 1个月后出现肾损害 ;组 3(肾损害首发组 ,n =13) ,肾损害出现在皮肤紫癜前。预后判断 ,正常 :无任何症状 ,尿检正常 ,肾功能正常 ;轻度尿检异常 :蛋白尿 (<1g/2 4h)或镜下血尿 ,肾功能正常 ,无高血压 ;恶化 :蛋白尿 (>1g/2 4h) ,肉眼血尿 ,高血压和(或 )肾功能不全。 结果 :三组间患者起病年龄、关节、胃肠受累率无显著性差异。组 1患者肾损害表现为尿检异常 /孤立性肉眼血尿 ,高血压和肾功能不全发生率低 ,肾活检病理以系膜增生为主 ,可伴少量细胞性新月体形成。组 2患者以中等量、大量蛋白尿多见 ,低白蛋白血症 2 2 9% ,病理上细胞性、细胞纤维性新月体、球囊粘连比例高。组 3患者病程最长 ,肾损害重 ,高血压发生率 15 4 % ,肉眼血尿发生率 38 5 % ,大量蛋白尿占 2 3 1% ,3例以急进性肾炎起病 。

蛋白尿在成人紫癜性肾炎中的临床意义

目的探讨蛋白尿在成人紫癜性肾炎中的临床意义。方法收集103例成人紫癜性肾炎患者的临床资料,对患者的临床表现和肾脏病理结果进行分析。结果少量、中等量蛋白尿患者分别占40.8%和37.9%,大量蛋白尿患者占21.4%。蛋白尿程度越重的患者,血压、血胆固醇水平越高,血浆白蛋白水平越低,组间比较差异有显著性(P<0.01)。随着蛋白尿程度的加重,肾小球损害、肾小管间质、血管损害加重(P<0.01)。结论蛋白尿能够间接反映紫癜性肾炎肾脏病变的轻重,随着蛋白尿程度的加重;肾脏的各项病理改变加重、肾小球损害与肾小管间质、血管的病变程度平行。

过敏性紫癜血清中某些细胞因子和IgA1含量变化的临床意义

目的观察普通过敏性紫癜(GHSP)及紫殿性肾炎(HSPN)患儿血清白细胞介素21(IL-21)、转化生长因子β(TGF-β)、肿瘤坏死因子α(TNF-α)和免疫球蛋白A1(IgA1)含量,进一步探讨过敏性紫癜(HSP)及HSPN的发病机制,为临床诊断和治疗HSP提供新思路。方法抽取2014年1月~2016年1月在河北北方学院附属第一医院儿科住院治疗的65例HSP(38例GHSP,27例HSPN)患儿和30例健康儿童(对照组)外周血,离心后分离血清,采用双抗夹心酶联免疫吸附法(ELISA)检测血清IL-21、TGF-β、TNF-α、IgA1含量,对结果进行统计学分析。结果 HSP患儿IL-21、TGF-β、TNF-α、IgA1水平均高于对照组(P<0.05),且HSPN患儿TGF-β、TNF-α、IgA1水平均明显高于GHSP患儿(P<0.05),而IL-21水平在两组患儿之间差异无统计学意义(P>0.05)。HSPN患儿血清TGF-β与IgA1呈正相关(r=0.346,P<0.05),TNF-α与IgA1呈正相关(r=0.582,P<0.05)。结论 IL-21、TGF-β、TNF-α和IgA1参与GHSP及HSPN的发病及病情进展,血清IgA1含量可作为判断HSPN患儿预后的重要指标之一。