抗中性粒细胞胞浆抗体对过敏性紫癜的检测意义

目的：探讨抗中性粒细胞胞浆抗体（ANCA）在过敏性紫癜（HSP）患儿中的检测意义。方法：应用间接免疫荧光法（IIF）和酶联免疫法（ELISA）测定45例过敏性紫癜患儿抗中性粒细胞胞浆抗体的水平和特异性抗原。结果：间接免疫荧光法检测紫癜肾,IgA型ANCA阳性率显著高于过敏性紫癜无肾脏损害者及正常对照组,二者间有显著性差异（P＜0．05）,而过敏性紫癜无肾脏损害者IgA型ANCA阳性率与正常对照组无显著性的差异（P＞0．05）;ELISA法检测过敏性紫癜患儿特异性抗原PR3、MPO,均为阴性。结论：IgA型ANCA定性检测可反映过敏性紫癜肾脏损害的情况,其特异性抗原与其它类型血管炎IgG型ANCA特异性抗原 不同。     

整合素血小板膜糖蛋白IIb、IIIa在系膜增殖性肾炎中的表达

目的 :观察整合素血小板膜糖蛋白Ⅱb、Ⅲa(GPⅡb、GPⅢa)在人类系膜增殖肾炎 (MsPGN)中肾内表达 ,探讨GPⅡb Ⅲa在MsPGN的作用。方法 :18例肾活检标本均行光镜HE、PAS、PASM Masson染色 ,电镜及免疫荧光检查 ,以SABC法测定肾组织中GPⅡb、GPⅢa、P 选择素 (P 140 )、纤维连接蛋白 (FN)、粘连蛋白 (LN)表达与正常对照组分别比较 ,并行计算机图像分析。结果 :PAS阳性物 (PAS+ )、PASM阳性物 (PASM+ )及FN、LN明显增生 ;GPⅡb、GPⅢa、P 140表达上调 ;肾小球细胞数 (n)、平均肾小球体积 (AVG)及肾小球硬化指数显著增加 ;肾小管、间质轻度受损。结论 :GPⅡb、GPⅢa在肾小球及肾小管间质表达上调与系膜细胞基质增生、细胞浸润、肾小球硬化相关 ;可能与肾小球疾病中凝血功能紊乱、血小板在肾脏的致病作用相关     

新型免疫抑制剂霉酚酸酯治疗狼疮性肾炎

目的观察新型免疫抑制剂霉酚酸酯(M.MF)治疗狼疮性肾炎的临床疗效和安全性。方法经肾活检证实的20例狼疮性肾炎,给予糖皮质激素联合MMF治疗。MMF初始剂量1.0～1.5g/d,治疗时间321个月,平均(10.9±6.2)个月。结果MMF治疗后24h尿蛋白定量从治疗前的平均5.55g下降至1.54g;d清白蛋白治疗前平均26.87g升至39g;6/8例肾功能不全患者3个月内Scr恢复正常;d清自身抗体产生明显减少,补体上升,其中ANA转阴率3个月达63％,6个月达81％;抗ds-DNA由治疗前23.77IU／ml下降至9.19IU／ml;2例重复活检,肾小球活动性病变明显减轻。MMF治疗的不良反应较少,偶有白细胞减少、感染、带状疱疹等。结论MMF对于各型狼疮性肾炎,尤其是Ⅳ型重症狼疮,经传统治疗无效者均有较好疗效,且长期应用不良反应少。     

64例狼疮性肾炎大剂量环磷酰胺冲击治疗的研究

目的　观察大剂量环磷酰胺冲击治疗狼疮性肾炎的疗效。方法　对 6 4例狼疮肾炎 ( L N ,其尿红细胞 >10个 /高倍视野 ,2 4小时尿蛋白 >1g,血肌酐 >133μmol/ L )进行大剂量环磷酰胺 ( CTX)冲击治疗 (每月 1次 ,共 6次 ,其后每 3月 1次 ,共 6次 )。结果　 49例 L N患者达到肾脏损害缓解 ( 2 4小时尿蛋白 <1g;血肌酐 <133μmol/ L ;尿沉渣内红细胞 <10个 /高倍视野 ;尿管型消失 )。达到肾脏病变缓解所需冲击次数平均为 3.6次 ( 1～8次 )。每次用 CTX剂量均数 1.1g( 0 .6～ 1.6 g)。副作用有闭经 (发生率 33% ) ;带状疱疹 (发生率 13% ) ;出血性膀胱炎 1例。结论　此治疗方案治疗 L N有效 ,安全 ,副作用小     

Autoimmunity in Acute Poststreptococcal GN: A Neglected Aspect of the Disease

Acute poststreptococcal GN (APSGN) is the prototype of immune complex GN and is associated with manifestations of autoimmune reactivity that have been neglected as epiphenomena. Recently, studies have demonstrated transient antifactor B autoantibodies that activate the alternative complement pathway, bringing self-immunity to a central position in the pathogenesis of APSGN. Therefore, examining other manifestations of autoimmunity that have been reported in association with poststreptococcal GN is of interest. This article reviews the renal and extrarenal manifestations of autoimmune reactivity in APSGN and considers their potential relevance in modifying the usually benign clinical course of the disease. It also discusses related aspects of the nephritogenic antigens, complement activation, and genetic elements associated with immune reactivity and their potential relevance to the familial incidence of the disease.     

Heymann肾炎致病原受体相关蛋白cDNA分子克隆及其mRNA在肾小球上皮细胞的表达

Ｈｅｙｍａｎｎ肾炎是一种用于人类膜性肾病的自身免疫病模型。ｇｐ３３０糖蛋白和４４ｋＤ受体相关蛋白（ＲＡＰ组成Ｈｅｙｍａｎｎ肾炎抗原复合物（ＨＮＡＣ），被证明是ＨＮ的主要致病原。本研究中，我们用ＲＴ－ＰＣＲ技术，克隆了ＲＡＰ基因，用Ｎｅｓｔｅｄ－ＰＣＲ技术克隆了ＲＡＰ羧基端１１８个氨基酸的基因，ＤＮＡ序列分析克隆基因与ＲＡＰ相同，原位杂交发现ＲＡＰｍＲＮＡ在肾小球上皮细胞表达。     

近年血小板减少性紫癜文献分析

本文通过对1995-2003年有关血小板减少性紫癜文献年代分布、期刊分布、著者、文献主题词分布等方面进行统计分析,寻找其研究热点和发展趋势,为临床研究及治疗提供借鉴。     

Progressive tubulointerstitial nephritis and chronic cholestatic liver disease

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenel involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.     

The rheumatic poison: a survey of some published investigations of the immunopathogenesis of Henoch-Schönlein purpura

Laboratory studies of the pathophysiology of Henoch-Schönlein purpura (HSP) have become more numerous in recent years with the recognition of the disease's links with the mucosal immune system in general and IgA nephropathy in particular. There are weak genetic associations with C4 null phenotypes and with HLA B35 and DR4. Studies of plasma proteins in HSP patients show an increased IgA concentration, activation of the alternative pathway of complement and consumption of factor XIII. High molecular weight (polymeric) IgA has been detected in affected individuals, which some investigators have called immune complexes. Many patients synthesise an IgA rheumatoid factor in the acute phase, but other autoantibodies are largely absent. In vitro studies of lymphocytes from HSP patients have demonstrated an increased number of IgA-bearing and secreting B-cells, with altered T-cell regulation of antibody synthesis. While these observations point to immune dysregulation — primarily of IgA production — as a consistent feature of acute HSP, there is as yet insufficient information available to allow a consistent theory of pathogenesis to be formulated.     

Excretion of epidermal growth factor-like material in acute Henoch-Schönlein purpura nephritis

In Henoch-Schönlein purpura nephritis (HSPN), glomeruli may develop cellular crescents composed of infiltrating monocytes and proliferating renal epithelia. In this study, we demonstrate that peripheral human monocytes can release an epidermal growth factor (EGF)-like substance detectable by a radioreceptor assay, which recognizes both EGF and transforming growth factor-alpha (TGF-alpha), but not with a radioimmunoassay, which recognizes only EGF. Furthermore, we report that urine from pediatric patients during the acute phase of HSPN contains a similar EGF-like species in addition to the endogenous EGF which is normally present. The EGF-like material was not present in urine from nine healthy children or from six children with acute post-streptococcal glomerulonephritis. The extent of crescent formation in our patients is uncertain, since renal biopsy was performed in only one case. However, we speculate that the urinary material resembling TGF-alpha which appears during the acute phase of HSPN may derive from monocytes infiltrating the kidney.