肾小球疾病患者尿中Ⅳ型胶原的测定及临床意义

目的探讨尿中Ⅳ型胶原在肾小球疾病诊断中的应用价值。方法应用竞争抑制性酶联免疫吸附实验，检测了２０例健康人及３８例肾小球疾病患者尿中Ⅳ型胶原的水平，并与肾活检的组织病理进行了比较。结果病理改变较为严重的患者以及肾活检为系膜增生性肾小球肾炎并伴有局灶性硬化、粘连和（或）新月体形成的患者，尿中Ⅳ型胶原的含量明显高于正常对照；但肾活检仅显示系膜增生性肾小球肾炎的患者，尿中Ⅳ型胶原水平同正常对照。结论尿中Ⅳ型胶原水平的测定，可以作为一个无创性的临床检测指标，有助于肾小球疾病的病理变化及其程度的诊断。     

儿童膜增生性肾小球肾炎的研究进展

膜增生性肾小球肾炎(membranoproliferative glomerulonephritis,MPGN)为免疫病理诊断,其临床表现多样,预后差且病死率高。根据其发病机制的不同,目前最新的分类方法是将 MPGN 分为免疫复合物介导型和补体介导型。根据分类按照可能潜在的病理生理学过程进行相关的病因评估将有助于为MPGN 患者选择更适当的治疗方法。对儿童特发性 MPGN 长期隔日应用糖皮质激素治疗是有益的,生物制剂用于治疗 MPGN 已取得了很大的进展。     

系膜增生性肾小球肾炎发病机制及治疗进展

肾小球系膜增生是多种肾脏疾病的基本病理过程,系膜增生性肾小球肾炎(MsPGN)是常见的病理类型.目前MsPGN发病机制仍不清楚,近年来以fractalkine、白细胞介素、转化生长因子-β等为代表的细胞因子逐渐成为MsPGN发病机制的研究热点,多种细胞因子在MsPGN发生发展中起重要作用.目前在MsPGN治疗上也取得一定的进展,对吗替麦考酚酯有了更深的认识,其在难治性肾病综合征患儿的治疗中取得了良好效果.     

系膜增生性肾小球肾炎临床与肾小球肾小管间质改变的关系

目的：探讨系膜增生性肾小球肾炎（ＭｓＰＧＮ）的临床与肾小球、肾小管间质改变的关系。方法对４６例ＭｓＰＧＮ临床与肾小球、肾小管间质改变病理特点进行比较分析。结果４６例ＭｓＰＧＮ临床分型为单纯血尿２３例,肾病综合征１８例,肾炎综合征５例。轻度肾小球系膜增生为３２例,中度１３例,重度１例。单纯血尿以轻度为主,占８７％,肾病下轻度为５０％,中度４４％,重度６％;肾炎综合征轻度为６０％中度４０％。肾小管间质     

α平滑肌肌动蛋白在胚胎肾组织和儿童系膜增生性肾小球肾炎中的表达

系膜细胞是肾小球疾病时致病因子作用的主要靶细胞之一 ,并可进一步通过自分泌和旁分泌的机制主动参与疾病进程。系膜增生性肾小球肾炎 (ＭｓＰＧＮ)是儿科临床常见的肾小球病变 ,其临床表现轻重不一 ,预后转归亦各不相同 ,研究表明 ,正常体内成熟的系膜细胞不表达α平滑肌肌动蛋白(α ＳＭＡ) ,但当系膜细胞活跃增殖时 ,细胞内出现α ＳＭＡ的表达 ,这与系膜细胞在机体胚胎发育时期所具有的表型特征相似。因此对α ＳＭＡ在肾脏胚胎发育过程中表达规律的研究对进一步阐述疾病情况下系膜细胞表达α ＳＭＡ与细胞增殖的关系有一定的作用。对…     

IgA肾炎和系膜增生性肾小球肾炎患儿血和尿液转化生长因子-β1的变化

目的检测IgA肾炎(IgAGN)和系膜增生性肾小球肾炎(MsPGN)患儿血浆、血清、尿液转化生长因子-β1(TGF-β1)水平,探讨TGF-β1在两者中的不同作用。方法应用酶联免疫吸附法(ELISA)测定24例IgAGN,30例MsPGN和30例正常儿童血浆、血清、尿液TGF-β1水平,比较各组TGF-β1水平。结果IgAGN组血浆、血清、尿液TGF-β1与MsPGN组及对照组相比较均有显著性差异(P均<0.01);MsPGN组血浆、血清、尿液TGF-β1与对照组相比无显著性差异(P>0.05)。结论TGF-β1在IgAGN和MsPGN中所起的作用不同。     

系膜增生性肾小球肾炎红细胞和白细胞免疫粘附功能及其相关性的研究

为从红细胞免疫角度探讨系膜增生性肾小球肾炎（ＭｓＰＧＮ）患儿的免疫发病机制,用郭峰法检测了３１例ＭｓＰＧＮ的红细胞和白细胞免疫粘附功能,并进行比较研究。结果提示：（１）作为检测红细胞免疫粘附功能的红细胞Ｃ３ｂ受体花环经（ＲＣＲ）和肿瘤红细胞花环率（ＴＲＲ）均较对照组明显降低,红细胞免疫复合物花环率（ＲＩＣＲ）差异不显著。（２）作为检测白细胞免疫功能的肿瘤料花环率（ＴＮＲ）和肿瘤淋巴细胞花环率（ＴＬ     

红细胞免疫黏附功能在系膜增生性肾小球肾炎发病机制中的作用

目的从红细胞免疫角度探讨系膜增生性肾小球肾炎(MsPGN)患儿的免疫发病机制。方法检测31例非IgA肾病,24例IgA肾病(IgAN)的红细胞和白细胞免疫黏附功能,并进行比较研究。结果1.作为检测红细胞免疫黏附功能的红细胞C3b受体花环率(RCR)、肿瘤红细胞花环率(TRR)均较对照组明显降低,红细胞免疫复合物花环率(RICR)差异不显著;2.作为检测白细胞免疫黏附功能的肿瘤粒细胞花环率(TNR)、肿瘤淋巴细胞花环率(TLR)均较对照组降低;3红细胞和白细胞免疫黏附功能的下降程度呈显著正相关。结论红细胞和白细胞免疫黏附功能紊乱可能是MsPGN的发病机制之一,提高红细胞和白细胞免疫黏附功能,可能为临床治疗MsPGN提供新思路。RCR、RICR、TRR、TNR、TLR均可作为判断病情、疗效、预后的指标应用于临床。     

川崎病合并膜增生性肾小球肾炎一例

患儿男 ,8岁半。因发热 ,伴皮疹 1周入院。体检 :Ｔ39 7℃ ,Ｐ 10 0次 /ｍｉｎ ,Ｒ 2 2次 /ｍｉｎ ,ＢＰ 90 / 6 0ｍｍＨｇ(1ｍｍＨｇ =0 133ｋＰａ)。发热面容 ,全身皮肤弥漫性潮红 ,密布红色粟粒疹 (猩红热样皮疹 )。颈浅部可扪及数枚黄豆至蚕豆大小淋巴结 ,双眼结合膜充血 ,口唇粘膜干燥、潮红、皲裂 ,杨梅舌 ;咽部充血 ,双侧扁桃体Ⅰ度肿大 ,颈软 ;双肺可闻少许干音 ;ＨＲ10 0次 /ｍｉｎ ,心、腹未见异常 ;指趾端硬肿。实验室检查 :入院时ＥＳＲ 4 5～ 5 2ｍｍ/ 1ｈ ,Ｈｂ 10 8ｇ/Ｌ ,ＲＢＣ 3 87× 10 12 /Ｌ ,ＷＢＣ 34 3× 10 9/…     

毛细血管内增生性肾炎转变为膜增生性肾炎1例报告#br#

目的：探讨由毛细血管内增生性肾小球肾炎(EnPGN)转为膜增生性肾小球肾炎(MPGN)的发病机制、诊断要点及治疗。方法回顾性分析1例肾脏病理由EnPGN转为MPGN患儿的临床资料及肾活检病理结果。结果患儿临床表现为蛋白尿及镜下血尿,持续性低补体C3血症,病理类型由EnPGN转为MPGN,经口服激素联合他克莫司治疗后,尿蛋白转阴,补体C3仍低。结论持续补体C3降低的患儿,病理类型可转变,需要早期诊断及时治疗。     

增生性肾炎患者系膜细胞增生与细胞周期调控蛋白的相关研究

目的 观察增生性肾炎患者肾小球系膜细胞（MC）在不同增生程度时细胞周期调控蛋白表达水平的变化。方法 采用免疫组织化学技术,检测34例肾不小球肾炎患者肾刺标本中细胞正性调控蛋白周期素D1（cyclinD1)、负性调控蛋白P21^CIP1(P21)、P27^KIP1(P27)和增殖细胞核抗原（PCNA）表达。结果 在正常对照及非增生性肾炎组中,cyclin D1、P21不表达,P27呈高表达。在增生性肾炎组中,cyclin D1、P21和P27阳性表达主要在系膜区,随着系膜增生程度的加重,肾小球cyclinD1、P21表达增加,并与PCNA呈正相关（P均＜0．01）;P27则表达减少并与PCNA呈负相关（P＜0．05）;肾小球P27表达还与血肌酐之间呈显著性负相关（P＜0．01）。结论 在人类增生性肾炎中,cyclin D1、P21和P27参与MC的增生;P27在肾组织的表达量可能与疾病进展密切相关。     

地塞米松对肾小球系膜细胞肿瘤坏死因子α产生和基因表达的影响

为观察地塞米松对内毒素诱导的肾小球系膜细胞肿瘤坏死因子α（ＴＮＦα）的产生及其ｍＲＮＡ表达的影响，采用免疫学和分子生物学方法，对正常、内毒素诱导后及加入地塞米松的内毒素诱导后大鼠系膜细胞的ＴＮＦα水平及ＴＮＦαｍＲＮＡ表达进行了检测。结果：（１）ＴＮＦα在正常系膜细胞中分泌量极微，ＴＮＦαｍＲＮＡ的表达处于低水平；（２）内毒素诱导系膜细胞ＴＮＦα的产生及ｍＲＮＡ表达增加；（３）地塞米松对内毒素诱导的系膜细胞ＴＮＦα的产生及其ｍＲＮＡ表达有明显的抑制作用，呈剂量依赖关系。结论：地塞米松是系膜细胞产生细胞因子ＴＮＦα的较强抑制剂。     

多中心多学科团队疑难病例讨论(第7例)

肾小球系膜增生是多种肾脏疾病的基本病理过程,是导致肾小球硬化的主要原因之一。多种因素作用于系膜细胞,使之产生复杂的细胞生物学变化,导致系膜细胞增生和基质沉积。1系膜增生性肾炎Anti-thy肾炎特征是初期系膜细胞溶解、继发系膜细胞增生和系膜基质沉积,最后基质吸收、组织学形态恢复正常。1·1Anti-thy1·1大鼠肾炎模型的实验原理[1]Thy-1(CD90)抗原是一种镶嵌在胸腺细胞和系膜细胞膜蛋白上的糖基化磷脂酰肌醇,针对该抗原的多克隆或单克隆抗体的IgG抗体能够吸附补体,复制出以蛋白尿为主要表现的动物模型。如果不吸附或激活补体则以…     

Pulse methylprednisolone therapy in children with membranoproliferative glomerulonephritis

The effects of pulse methylprednisolone (PM) therapy were studied in 15 patients (aged 3–14 years) with biopsy proven membranoproliferative glomerulonephritis (MPGN). Patients were treated with intravenous PM 30 mg/kg (max 1 g) given over 30 min every other day for a mean of 9.8 days (3–15 days). Oral prednisolone therapy was continued at a dose of 1 mg/kg/24 h for 1 month and subsequently tapered off the following month. Eight patients had hematuria and six had medically controlled hypertension. Serum C₃ levels were low in 11 patients and all of the patients had proteinuria. Following PM therapy proteinuria was significantly reduced from 2602.9 ± 1852.5 mg/24 h to 1871.2 ± 2090.8 mg/24 h (P < 0.05) and at final evaluation, proteinuria was 774.33 ± 1225.67 mg/24 h which was significantly lower than pre- and post-PM therapy values (P < 0.05). Serum creatinine levels were high in five patients before PM therapy and remained high in one of the patients who progressed to end-stage renal failure. After PM therapy, high serum creatinine levels normalized in three patients and was reduced, but still above normal, in one. One patient, with initially normal serum creatinine, had elevated levels afterwards. Nine of the patients were considered responsive and six non-responsive according to our tentatively defined criteria. Mean follow-up period was 27.4 ± 24.1 months (6–84 months). Three patients were lost for follow-up, and 12 were re-evaluated. At final evaluation, all of the patients except one with end-stage renal failure had normal creatinine levels. There was no correlation between the clinical and laboratory features at onset and the outcome of the disease in the responder or non-responder patients. Results of our study show that PM therapy reduced proteinuria and may affect renal function positively in patients with MPGN. However, a prospective controlled study with repeat biopsies is required to draw a conclusion.     

Outcome of idiopathic membranoproliferative glomerulonephritis in children

The aim of this multicentre study was to analyse the long-term outcome of idiopathic membranoproliferative glomerulonephritis (MPGN) according to histological type and to the presence of C3 nephritic factor. Fifty patients aged 2 14 years at the onset of the study were followed over 2 20 years; 26 patients had MPGN type I, 17 had type II and 7 had type III. Treatment was variable. At the last observation, 30 patients had reached terminal and four pre-terminal renal failure. The median survival probability until renal death was 15.3, 8.7 and 15.9 years for disease types I, II and III respectively (difference between MPGN types I + III versus type II: p = 0.013). The presence of an initial nephrotic syndrome was associated with a more rapid progression ( p = 0.018). C3 nephritic factor was of no prognostic value. We conclude that the outcome of MPGN mainly depends on the histological type observed.     

罗格列酮对大鼠系膜增生性肾炎的治疗作用

目的 研究罗格列酮对大鼠系膜增生性肾炎(MsPGN)的治疗作用并探讨其可能的作用机制,寻找治疗系膜增生性肾炎的新途径.方法 大鼠随机分为3组:李白对照组(A组)、模型对照组(B组)、罗格列酮治疗组(C组).B、C组以四氯化碳及牛血清白蛋白灌胃制备系膜增生性肾炎模型,制模成功后.A、B组予生理盐水,治疗组予罗格列酮分别灌胃均8周,每周测各组大鼠24 h尿蛋白排泄量及尿红细胞计数.实验结束时,测血清白蛋白、血糖、肝肾功能、肾脏系膜细胞及系膜基质增生情况(光镜、PAS染色)及各组大鼠肾脏转化生长因子-β1(TGF-β1)、α-平滑肌肌动蛋白(α-SMA)表达水平.结果 治疗组大鼠24 h尿蛋白排泄量、尿红细胞汁数、TGF-β1及α-SMA表达水平、肾小球PAS染色灰度值较模型组均明显减少,血清白蛋白明显升高,差异均有统计学意义.3组之间血精及肝肾功能比较差异无统计学意义.结论 罗格列酮对大鼠系膜增生性肾炎具有治疗作用,其作用机制之一可能是通过抑制TGF-β1及其下游因子表达.     

Extracapillary proliferative glomerulonephritis in children.

The clinical course and renal pathology of 17 children with acute extracapillary proliferative glomerulonephritis is reported. Patients with systemic diseases associated with renal involvement were excluded. The onset followed streptococcal infection in ten; of these, four have died, one has been transplanted, and the remaining five have completely healed. The mean follow-up of the latter five patients was 32 months (range, 18 to 57 months). Of the other seven patients without evidence of precedeing streptococcal infection, two have died, two have been transplanted, and the remaining three all have hypertension, proteinuria, and reduced creatinine clearance. The mean follow-up of the latter three patients was 29 months (range, 14 to 38 months). The initial renal histopathologic changes and their progress in later renal biopsies is described. The role of various therapeutic agents is discussed. The prognosis in acute extracapillary proliferative glomerulonephritis following streptococcal infection appears to be better than in ones without preceding streptococcal infection.     

Long-term prognosis and prognostic factors of Japanese children with mesangial proliferative glomerulonephritis without IgA deposition

Mesangial proliferative glomerulonephritis without IgA deposition (non-IgA MesPGN) is commonly detected in biopsy specimens, but the clinicopathological correlation with the long-term prognosis still remains obscure. The aim of our study is to elucidate the long-term prognosis and the clinicopathological prognostic factors in patients with non-IgA MesPGN. We mailed questionnaires to 122 patients with primary glomerulonephritis who were biopsied between 1963 and 1975. Information was obtained from 109 of these 122 patients and 55 were histologically rediagnosed as having non-IgA MesPGN. The histological alterations of glomeruli and tubulointerstitium were classified into five grades. The mean period between the biopsy and the questionnaires was 20.5 years. Six of the 55 patients with non-IgA MesPGN developed end-stage renal failure and histopathological alterations of renal biopsies from these six patients were classified into grade IV or V. The presence of hypertension, heavy proteinuria of over 2 + or renal insufficiency at the biopsy was related to the severe histological changes, a grade of IV or V and to a poor prognosis. The renal survival rate of all the 55 patients was 88.3% at 20 years after the biopsy, while that of the 12 patients with severe histological changes was 48.6%. Although non-IgA MesPGN is considered to be a heterogeneous disease, we cannot ignore the incidence of this disease and thus consider it to be one of the important primary glomerulonephritides that occur in childhood.     

细胞外基质在胚胎和成熟肾组织中的表达

目的观察肾小球细胞外基质成分在胚胎和成熟肾组织中表达的时空顺序、细胞来源，为阐明它们在肾组织中的生物学功能提供理论依据。方法选用针对板层蛋白的α１、α２、β１、β２、γ１链、Ⅳ型胶原α１、α３、α５链和纤维连接蛋白的单抗隆抗体，采用间接免疫碱性磷酸酶技术观察其在１１例胎儿肾中的表达和分布，并以正常成人肾组织作为对照。结果板层蛋白的α１、β２、γ１链在肾组织中的表达随着胎龄增加和肾脏成熟而增加，但各自分布不同；α２链在肾组织未见表达；β１链在幼稚肾小球为阳性，成熟肾小球中则为阴性。纤维连接蛋白和Ⅳ型胶原α３、α５链在肾单位有血管侵入时才出现表达，Ⅳ型胶原α１链则在肾发育早期即有表达。它们各自分布并不一致。结论在肾小球发生和成熟过程中存在着细胞外基质成分分布的时空顺序及个别链抗原的隐蔽或丢失，推测它们可能对肾小球的发生、成熟起重要作用