小儿紫癜性肾炎Pax2与WT1表达关系研究

目的探讨Pax2与WT1在紫癜性肾炎(HSPN)肾组织中的表达及其两者间的关系。方法选取2004年7月至2005年12月在中南大学湘雅二医院住院的HSPN患儿37例为试验组,肾病综合征患儿(微小病变型)10例为对照组。分别对肾小球和肾小管间质的病理改变程度进行半定量评分,免疫组化检测Pax2与WT1的表达,分析肾脏Pax2、WT1表达与肾脏病理积分及其两者之间的关系。结果(1)HSPN患儿肾组织局部Pax2的表达:Ⅱ级1.14±0.38;Ⅲa级1.71±0.47;Ⅲb级2.58±0.51;Ⅳ级3.00±0.00;对照组0.80±0.45。HSPN患儿与对照组之间和HSPN患儿肾脏病理改变各级间,Pax2的表达差异均有统计学意义(P<0.005),随着肾脏病理级别的加重,Pax2的表达增加。(2)HSPN患儿肾组织局部WT1的表达:Ⅱ级2.78±0.38;Ⅲa级1.86±0.53;Ⅲb级1.50±0.52;Ⅳ级1.50±0.58;对照组2.60±0.54。HSPN患儿与对照组之间和HSPN患儿肾脏病理改变多级间,WT1的表达差异有统计学意义(P<0.05),肾脏病理级别的加重,WT1的表达增加。结论HSPN患儿中存在PaX2的重新表达和WT1的表达减少,且随着肾脏病理加重Pax2的表达增加,WT1的表达减少。     

过敏性紫癜患儿尿中肝细胞生长因子检测及临床意义

目的 检测不伴或伴有不同程度白蛋白尿的过敏性紫癜(HSP)患儿尿中肝细胞生长因子(HGF)水平的变化,评价其临床意义.方法 选择山东大学附属省立医院儿科2007年8月至2008年4月收治的急性期HSP患儿78例,按照尿白蛋白排泄率(UAER)分成正常白蛋白尿组(Ⅰ组,38例)、微量白蛋白尿组(Ⅱ组,24例)和大量白蛋白尿组(Ⅲ组,16例),采用双抗体夹心ELISA方法分别检测所有患儿急性期及其中部分患儿恢复期尿HGF,与26例健康对照儿童进行比较.结果 与对照组相比,Ⅰ组和Ⅱ组尿HGF水平均升高,且呈递增趋势,差异有统计学意义(P<0.05);而Ⅲ组尿HGF水平与对照组相比差异无统计学意义(P>0.05);Ⅰ、Ⅱ组患儿恢复期尿HGF水平为1.22±0.67较其急性期1.85±1.15明显降低,差异有统计学意义(P<0.05);Ⅲ组患儿恢复期尿HGF水平为1.43±0.31较其急性期0.30±0.31显著升高,差异有统计学意义(P<0.01).结论 HGF参与紫癜性肾炎(HSPN)患儿肾脏损伤的修复,尿HGF水平的监测有助于评估HSPN的病情及预后,外源性HGF的介入可能成为早期干预、防治HSPN的有效方法.     

过敏性紫癜血清抗内皮细胞抗体检测及其临床意义

目的检测过敏性紫癜患儿血清抗内皮细胞抗体(AECA)水平,探讨其在HSP发病机制中的作用。方法 2010年6月至2011年1月在温州医学院附属育英儿童医院肾脏科住院的过敏性紫癜患儿(HSP)60例,分为紫癜性肾炎(HSPN)组40例和非HSPN组20例,按病程分为急性期和缓解期,另以20名健康儿童作为对照组。应用酶联免疫吸附法(ELISA)检测血清AECA水平。结果对照组血清AECA为(232.31±53.89)ng/mL。HSP患儿急性期为(355.18±58.07)ng/mL,与对照组比较,差异有统计学意义(P<0.01);缓解期为(243.19±58.68)ng/mL,与对照组比较,差异无统计学意义(P>0.05),与急性期比较,差异有统计学意义(P<0.01)。非HSPN组急性期为(328.09±57.97)ng/mL,HSPN组为(369.66±59.20)ng/mL,差异有统计学意义(P<0.05)。结论 AECA可能参与了HSP血管损伤的病理过程,检测AECA可作为判断HSP病情的指标。     

紫癜性肾炎患儿单核细胞趋化蛋白-1表达的研究

目的 观察单核细胞趋化蛋白-1(MCP-1)在紫癜性肾炎(Henoch-Schonlein purpura nephritis,HSPN)患儿外周血和肾组织中的表达,探讨其在HSPN发病巾的作用.方法 以实时荧光定量聚合酶链反应方法,检测46例HSPN患儿(活动期28例,恢复期18例)和14例对照组儿童的MCP-1 mRNA定量表达水平(以-△△Ct 值表示).3例反复血尿、蛋白尿的HSPN患儿应用免疫荧光抗体标记、激光扫描共聚焦显微镜技术观察肾组织MCP-1的分布.结果 活动期HSPN患儿外周血MCP-1 mRNA表达为0.685±2.447,明显高于恢复期HSPN患儿(-0.850±1.271)和正常对照组儿童(-0.774±1.118)(P<0.05);恢复期HSPN患儿外周血MCP-1 mRNA的表达与正常儿相比差异无统计学意义.正常肾组织几乎不表达MCP-1,而在活动期HSPN患儿MCP-1蛋白呈节段性分布于肾小球系膜区、还可见于肾小管上皮细胞.结论 HSPN患儿外周血和肾组织MCP-1表达异常,随病情缓解外周血MCP-1 mRNA的水平有明显恢复.对MCP-1等趋化因子及其受体的深入、系统的研究町能为将来特异性治疗药物的开发寻找新的作用靶点.     

过敏性紫癜患儿血清白介素6、白介素8及肿瘤坏死因子α水平和免疫球蛋白的变化

目的探讨血清白介素6、白介素8、肿瘤坏死因子。及免疫球蛋白在过敏性紫癜患儿中的表达及其临床意义。方法采用ELISA法和免疫散射比浊法检测了45例过敏性紫癜患儿(其中20例合并肾脏损害)及43例正常健康儿童的血清白介素6、白介素8、肿瘤坏死因子α及免疫球蛋白水平,分别对过敏性紫癜有无合并肾损害及过敏性紫癜患儿与健康儿童细胞因子和免疫球蛋白水平进行比较;观察是否存在相关关系。结果HSP患儿的血清IL-6、IL-8及TNF-α水平高于正常对照组(P<0·01);无肾损害HSP组与HSPN组的血清IL-6、IL-8水平均无统计学差异,HSPN组TNF-α水平高于无肾损害HSP组;HSP患儿IgA、IgE含量明显高于正常对照组(P<0·01),IgG含量下降(P<0·01),IgM无明显差别(P>0·05);无肾损害HSP组与HSPN组的IgA、IgM、IgG水平无统计学差异(P>0·05),HSPN组的IgE水平高于无肾损害HSP组(P<0·05);HSP患儿的血清TNF-α水平与IL-8存在中等程度正相关关系(r=0·524,P<0·01)。HSP患儿的血清TNF-α水平与IL-6存在中等程度正相关关系(r=0·670,P<0·01)。结论HSP/HSPN的发病过程存在细胞因子和异常水平免疫球蛋白的参与,HSPN的发生与TNF-α异常有相关性。     

过敏性紫癜性肾炎患儿共刺激分子4-1BB/4-1BBL 变化及其临床意义

目的探讨共刺激分子4-1BB/4-1BBL在过敏性紫癜性肾炎(HSPN)患儿外周血单个核细胞(PBMC)中的表达及其临床意义。方法选取初发过敏性紫癜无肾炎(HSP组)、初发过敏性紫癜性肾炎(HSPN组)患儿各20例(其中15例进行了肾脏病理检查),20例正常儿童为对照组,分离培养PBMC。应用逆转录-聚合酶链式反应(RT-PCR)检测并比较三组PBMC中4-1BB m RNA和4-1BBL m RNA的变化,分析4-1BB/4-1BBL m RNA变化与肾脏病理的关系。结果HSPN、HSP患儿PBMC中4-1 BB m RNA、4-1 BBL m RNA的表达均高于对照组,差异有统计学意义(P均0.01)。HSPN患儿的病理积分与4-1BB/4-1BBL m RNA表达呈正相关关系(r=0.570、0.515,P均0.05)。PBMC中,加入植物血凝素后,HSPN组、HSP组和对照组4-1 BB/4-1 BBL m RNA的表达均高于空白对照组,差异有统计学意义(P均0.01),其中HSP、HSPN组增高幅度较对照组明显,HSPN组增高幅度较HSP组更为显著,差异有统计学意义(P均0.01);加入地塞米松后,HSPN组、HSP组4-1 BB/4-1 BBL m RNA的表达均较空白对照组减少,差异有统计学意义(P均0.01)。结论 HSPN患儿PBMC中共刺激分子4-1BB/4-1BBL m RNA的表达显著增高,植物血凝素能诱导其高表达,而地塞米松能抑制其表达,提示4-1BB/4-1BBL可能参与HSPN的发病。     

过敏性紫癜肾炎患儿白三烯表达水平的临床及病理研究

目的 探讨白三烯(LTs)在过敏性紫癜肾炎(HSPN)发生发展中的作用,为临床使用LTs拮抗剂治疗HSPN提供科学实验依据.方法 收集患儿及健康对照儿童共77例,分成3组,HSPN组34例(18例进行肾穿刺活检术),过敏性紫癜(HSP)组27例,健康对照组16例.分别采集血清和尿液,采用酶联免疫吸附试验法检测各组血清、尿液白三烯B4(LTB4)水平;酶免疫分析法检测各组尿液白三烯E4(LTE4)水平;间接免疫荧光法检测18例进行肾穿刺活检术HSPN患儿肾组织中白三烯C4(LTC4)合酶表达,以3例薄基底膜病、4例临床诊断单纯性血尿(光镜和电镜基本正常)活检标本作对照组;检测HSPN组患儿24 h尿蛋白.结果 (1)HSPN组血清、尿液LTB4及尿液LTE4水平分别为(1164.33 ±300.28)、(841.19 ±115.23)和(1252.31 ±251.62)ng/L,高于HSP组[分别为(559.60 ±180.23)、(574.42±101.17)和(805.93 ±185.52)ng/L]及对照组[分别为(211.95±67.72)、(227.33 ±76.12)和(149.51 ±33.66)ng/L](P均＜0.01);(2)随HSPN病理分级加重,HSPN组血清、尿液LTB4及尿液LTE4水平有升高趋势;(3)随尿蛋白水平的增加,HSPN血清、尿液LTB4及尿液LTE4表达水平逐渐增加(P＜0.01或P＜0.05);(4)与对照组相比,HSPN各组肾活检组织LTC4合酶荧光强度均增强,该荧光表达与其病理分级呈密切正相关.结论 LTs参与并促进HSPN的发生发展,其在肾脏表达水平与HSPN病理分级及尿蛋白排泄密切相关.     

IGF-1及IGFBP-3在过敏性紫癜及紫癜性肾炎患儿临床检测中的意义

目的：探讨胰岛素样生长因子-1（IGF-1）及胰岛素样生长因子结合蛋白-3（IGFBP-3）在过敏性紫癜（HSP）及紫癜性肾炎（HSPN）患儿临床检测中的意义。方法：选取31例HSP患儿为HSP组,28例HSPN患儿为HSPN组,另选取31例健康儿童为对照组,采用ELISA法测定各组血清标本的IGF-1及IGFBP-3浓度,全自动生化仪检测HSPN组24 h尿蛋白定量;各组儿童均行血清免疫球蛋白（Ig）水平、补体C3、全血细胞计数检测和尿液分析。结果：HSP组血清IGF-1及IGFBP-3水平均高于对照组（均P<0.05）,HSPN组血清IGF-1及IGFBP-3水平均高于HSP组和对照组（均P<0.05）;在12例进行肾穿刺活检的HSPN患儿中,随着HSPN病理分级的加重,血清IGF-1及IGFBP-3水平有增高趋势;有蛋白尿的HSPN患儿血清IGF-1及IGFBP-3水平均高于无蛋白尿的HSPN患儿（均P<0.05）;HSP和HSPN患儿的白细胞、红细胞、血小板计数、补体C3、IgG和IgA水平以及IgA/C3比值均显著高于对照组（均P<0.05）。结论：在HSP急性发作期IGF-1和IGFBP-3水平升高,可能与蛋白尿的程度及肾脏损害程度有关,提示IGF-1和IGFBP-3水平可能是肾脏受累的指示。     

血管紧张素转换酶基因多态性对过敏性紫癜的影响

目的 探讨过敏性紫癜(HSP)与血管紧张素转换酶(ACE)基因多态性间的关系。方法 选择70例HSP患儿,其中无肾脏损害32例,过敏性紫癜性肾炎(HSPN)38例。HSPN中单纯性血尿15例,蛋白尿23例。健康对照儿童100例。采用聚合酶链反应(PCR)检测ACE基因型。结果 1.HSP患儿与健康对照组ACE基因型分布比较无差异(P>0.05);2.非肾脏损害患儿32例与伴单纯性血尿HSPN患儿15例ACE基因型分布比较亦无差异(P>0.05);但与23例伴蛋白尿HSPN患儿ACE基因型分布比较,缺失型(DD),型者发生蛋白尿频率明显升高(P<0.05)。结论 HSPN蛋白尿发生与ACE基因多态性相关,DD型者发生蛋白尿机会明显增多。     

Tenascin在紫癜性肾炎肾组织中的表达及其临床意义

目的　探讨紫癜性肾炎 (HSPN)患儿肾组织中Tenascin(TN)分布、表达及其与病理类型、临床指标间的关系。方法　采用免疫组化法检测 16例HSPN患儿肾组织中TN分布及表达情况。结果　 1.TN在正常肾组织中 ,肾小球及肾小管间质呈局灶性弱阳性表达 ;而在HSPN患儿主要于细胞外基质 (ECM )积聚处表达明显增加 ;且肾小球表达与肾小管间质表达正相关 (r=0 .5 5 9　P =0 .0 2 4) ;TN在肾小球表达与病理类型无关 ,而TN在肾小管间质表达与病理类型正相关 ( r =0 .83 0　P <0 .0 1)。 2 .TN在HSPN患儿肾组织中表达与血肌酐 (Scr)、尿蛋白排泄量正相关 ,而与病程、性别、年龄、总蛋白、白蛋白等无关。结论　 1.TN在HSPN患儿肾组织中ECM积聚处表达明显增加。 2 .与TN在肾小球表达相比 ,肾小管间质的表达更能反映HSPN患儿的病情。TN在肾组织的表达临床上可用来判断患儿病情轻重、预后 ,为治疗提供依据     

Intended management of children with acute idiopathic thrombocytopenic purpura: a national survey

OBJECTIVE: In Australia acute idiopathic thrombocytopenic purpura (ITP) is mainly treated by paediatricians (either general paediatricians or paediatric haematologists/oncologists). A survey was conducted to gauge the current practice of treating children with acute ITP in Australia. METHODS: All practising Australian paediatricians registered by the Royal Australasian College of Physicians were surveyed regarding their intended management of children with acute ITP. The questionnaire, adapted from a study of paediatric haematologists/oncologists in North America, presented four clinical scenarios of children with acute ITP with a platelet count of 3000 x 10(9)/L, with and without mucosal bleeding (wet and dry purpura, respectively). Questionnaires were returned by mail or filled in online at a dedicated webpage. RESULTS: Five hundred and sixty-three of 1097 (51%) paediatricians responded to the survey. Data from 140 who had treated at least one child with ITP in the previous 12 months were analysed. Respondents indicated that children with acute ITP are usually or always hospitalised (58-92%) and that 48% would be given active treatment, even with dry purpura. Various regimens of i.v. immunoglobulin or corticosteroids are used when treatment is administered. In comparing Australian and North American management of acute ITP there were many similarities, although Australian paediatricians were less likely to arrange a bone marrow aspirate if corticosteroids were prescribed. CONCLUSIONS: There is great variation in the intended management of children with acute ITP in Australia. Previously published management recommendations regarding investigation and treatment have had little impact on intended practice. Prospective studies are required to evaluate hypotheses so as to produce evidence-based recommendations for treatment of patients with acute ITP.     

High-Dose Intravenous Immunoglobulin as Single Therapy in a Child with Autoimmune Hemolytic Anemia

Between 1975 and 1992 450 children with idiopathic thrombocytopenic purpura (ITP) were diagnosed, and of those 100 (22%) developed the chronic form of the disease. Approximately half the patients with chronic ITP presented with mild to moderate hemorrhagic manifestations at the onset of purpura (30 cases) andlor later during the course of the disease (25 cases). The incidence of intracranial hemorrhage was 1 %, and the mortality rate due to overwhelming septicemia after splenectomy was also 1%. Overall one-third of the patients received no therapy; two-thirds of them went into spontaneous remission within 8 months to 8 years from the onset of ITP. Steroids given in conventional or high doses (51 cases) achieved a transient (if any) rise in platelet count, but in no case were steroids curative. Remission related to intravenous immune globulin (IVIG) therapy was noticed in 38.5% of the children (10 of 26) after variable courses. The response rate to splenectomy was 95.0%. Ultimately the long-term outcome in children with chronic ITP was as follows: remission, 58 cases (spontaneous, 30; after IVIG therapy, 10; after splenectomy, 18); hemostatic platelet values, 22 cases (spontaneous, 16; after IVIG, 5; after splenectomy, I). Thirteen children were lost in follow-up, and 7 remain thrombocytopenic but asymptomatic. These data indicate that chronic ITP in childhood runs a benign course in most cases and may remit with or without therapy euen several years from onset. Therefore, therapeutic intervention has to be individvalized, and splenectomy, which is not always safe, should be reserved for problematic cases that fail to respond to conventional therapeutic modalities.     

�ɳ�����С�����������Ѫ�弰����֯��Ｐ������

??Abstracts?? Objective To investigate the expression of RLX in blood and renal tissue of patients with HSP?? and its correlation with clinical indexes and pathological changes?? and explore its effect on the pathogenesis of HSP. Methods Collect 39 cases of Henoch-Schonlein purpura ??HSP?? from Department of Nephrology in Children’s Hospital of Jiangxi province from November 2011 to June 2012?? with complete follow-up data. According to the clinical urine manifestation?? 39 patients with HSP were divided into non-kidney damage group ??n=20?? and kidney damage group ??n=19??. All 39 patients were collected blood samples in the acute and convalescent phase. Nineteen patients from kidney damage group were divided into mild lesions ??n=5???? moderate lesions ??n=9??and severe lesions ??n=5???? according to the degree of pathological changes. RLX concentration was detected with enzyme-labeled immunosorbent assay ??ELISA?? method.RLX protein expression in renal tissue was detected with immunohistochemistry method. Results In the acute phase?? the serum level of RLX in HSP was significantly higher than normal control?? and its concentration was significantly higher in kidney damage group than in non-kidney damage group ??P??0.05??. Compared with acute phase?? the serum level of RLX in HSP in the convalescent phase was significantly lower?? and the level of decline was slower in kidney damage group than in non-kidney damage ??P??0.05??. Weak expression of RLX in renal tissue was found in normal control??while the expression of RLX in renal tissue significantly increased in HSPN ??P??0.05???? with the pathological changes of renal tissue aggravating. The expression of RLX in renal tissue in HSPN was significantly correlated with the serum concentration of RLX ??P??0.05?? and the total urine protein for 24 hours ??P??0.05??. Conclusion There is a modest up-regulation in serum and renal RLX protein expression in HSP?? and the severity of the disease is closely related with RLX expression?? which suggests that RLX may be involved in the pathogenesis of HSP/HSPN?? and it may be an important mechanism that upregulation of RLX may play a defensive role in delaying disease progression of HSP/HSPN.     

Effectiveness of early prednisone treatment in preventing the development of nephropathy in anaphylactoid purpura

A prospective study was performed to verify whether early administration of prednisone could be useful in preventing the development of nephropathy in anaphylactoid purpura. Only patients without signs of nephropathy upon initial presentation entered into the study. A total of 84 patients received delta-prednisone (1 mg/kg per day per os for 2 weeks), and 84 patients did not receive steroids. The patients were followed for 24–36 months. None of the 84 patients treated with steroids and 10 (11.9%) of the 84 control patients developed nephropathy 2–6 weeks after the acute episode. In 2 other patients of the untreated group, signs of renal involvement appeared 2 and 6 years after the acute episode respectively. The difference in the prevalence of nephropathy between the two groups is highly significant (P<0.001).     

儿童过敏性紫癜不同时期凝血功能状态检测及分析

目的　研究儿童过敏性紫癜 (HSP)发作期及缓解期凝血状态的改变情况及其机制。方法　 2 0 0 1～2 0 0 3年深圳市儿童医院收治的HSP患儿共 6 0例 ,根据发病期和缓解期的不同 ,相应分为HSP发作组 (30例 )和HSP缓解组 (30例 ) ,检测凝血酶原时间比 (PTR)、血浆D 二聚体 (D dimer)及组织因子途径抑制物 (TFPI)水平 ,并与对照组 35例健康体检儿童比较。结果　两组HSP患儿的PTR与对照组比较差异无显著性 (均P >0 0 5 ) ;HSP发作组血浆D dimer明显升高 (P <0 0 5 ) ,而HSP缓解组血浆D dimer浓度下降 ,与对照组差异无显著性(P >0 0 5 ) ;HSP发作组血浆TFPI含量高于对照组 (P <0 0 5 ) ,HSP缓解组降至正常水平 ,和对照组比较差异无显著性 (P >0 0 5 )。结论　HSP患儿发作期凝血解溶状态处于高活动状态 ,随着病情进入缓解期高凝状态可恢复正常 ;TFPI在HSP患儿发作期可能具有防止血管内凝血活动过度和扩散的重要作用。     

血管紧张素原基因多态性与儿童过敏性紫癜及紫癜性肾炎的关系

目的 探讨血管紧张素原(AGT)基因多态性与儿童过敏性紫癜(HSP)及紫癜性肾炎(HSPN)的相关性.方法 使用病例对照研究,经PCR和RFLP方法检测AGT基因型.结果 AGT基因型构成比在患病组与对照组、HSPN组与对照组之间差异有统计学意义(x2=17.92、17.08,P均<0.01),但在HSP和HSPN之间差异无统计学意义(x2=1.78,P=0.41).AGT-TT基因型在患病组和HSPN组均明显高于对照组(P<0.01).结论 用标记物对过敏性紫癜以及紫瘢性肾炎易感性的追踪提示,AGT M235T摹因型与中国儿童HSP、HSPN之间有关联.