儿童免疫球蛋白A肾病临床与病理分析

目的分析儿童免疫球蛋白(Ig)A肾病(IgAN)临床、病理特点及临床和病理的关系。方法经肾活检诊断为原发性IgAN的39例儿童,男32例,女7例,发病年龄2岁11个月~14岁1个月,对其临床表现和肾小球、肾间质及免疫病理进行分析。结果性别和病程不同的儿童IgAN,其病理分级程度无差异性(Uc=0.018 P>0.05;χ2=1.365 P>0.05),临床分型、肾间质病变和免疫沉积种类不同的有差异性(Uc=2.277 P<0.05;χ2=7.66 P<0.01)。结论不能根据病程长短来判断儿童IgAN病理轻重,有蛋白尿者病理分级较重,必须尽早行肾活检明确诊断及病理类型,早期予正确有效治疗,要重视间质病变对预后的影响。     

儿童毛细血管内皮细胞增生性紫癜性肾炎的临床病理及预后分析

Objective To investigate the clinicopathological characteristics and prognosis of Henoch-Schonlein purpura nephritis with diffused endothelial cell proliferation (DEP-HSPN) in children. Methods Data of 8 DEP-HSPN cases in Nanjing Children's Hospital within recent ten years were retrospectively reviewed. The clinicopathological features, efficacy and prognosis were compared between DEP-HSPN cases and 48 cases of non-DEP-HSPN. Non-DEP-HSPN cases were divided into two groups according to the clinical classification or the pathological classification.Results (1) In DEP-HSPN, HSP developed nephritis within 4 to 15 days after the initial onset of purpuric rashes. Hematuria was present in all the 8 patients. The main clinical manifestation of DEP-HSPN was nephritic-nephrotic syndrome (4 cases), nephrotic level proteinuria (3 cases) and acute nephritic syndrome (1 case). Four cases had macrohematuria. Six cases had abdominal symptoms and two cases had arthritis. Pathology of all the cases showed grade Ⅲ-b lesion with diffused endocapillary proliferation and segmental necrotizing lesion of the capillary wall, always accompanied with intraglomerular inflammatory cell infiltration. Crescent was found in 4 cases. (2)Compared to non-DEP-HSPN grades Ⅲ, DEP-HSPN showed a shorter course of disease.Macrohematuria, heavy proteinuria, nephritic-nephrotic syndrome, and segmental necrotizing lesion of capillary wall were more common in DEP-HSPN. Compared to non-DEP-HSPN with nephrotic level proteinuria, DEP-HSPN had a lower rate of crescent. (3) Methylprednisolone pulse therapy in early stage, then prednisone combined with cyclophosphamide were used in the treatment of DEP-HSPN.After an average follow-up period of seven months, one patient showed complete remission, five showed persistent microhematuria, and two showed persistent microhematuria accompanied with minor proteinuria. No significant difference of prognosis was found between DEP-HSPN and nonDEP-HSPN. Conclusions DEP-HSPN has an acute onset. The main clinical manifestation of DEP-HSPN is nephritic-nephrotic syndrome and nephrotic level proteinuria, always accompanied with macrohematuria. Immunosuppressant treatment in the early stage of disease is effective for a short-term outcome.     

姜黄素对大鼠肾毒血清肾炎肾组织细胞外基质积聚的影响

目的:观察姜黄素是否抑制肾炎大鼠肾组织Ⅳ型胶原和纤维连接蛋白(fibronectin, FN)的积聚而对肾脏起保护作用.方法:72只雄性SD大鼠随机分成3组,每组24只.对照组尾静脉及腹腔注射生理盐水作对照;模型组尾静脉注射肾毒血清0.5 ml/d,连用2 d,腹腔注射二甲亚砜0.5 ml*kg-1*d-1;姜黄素组尾静脉注射肾毒血清0.5 ml/d,连用2 d,同时腹腔注射姜黄素50 mg*kg-1*d-1.分别于第3、7、14、28天各处死6只大鼠,部分肾组织福尔马林固定、石蜡包埋后进行免疫组织化学染色.结果:对照组大鼠肾小球基膜Ⅳ型胶原以及FN染色弱阳性.模型组大鼠肾小球Ⅳ型胶原和FN沉积的范围随着病程的进展逐渐增多,与相应时间对照组比较有显著差异(P ＜0.01).姜黄素组肾小球Ⅳ型胶原和FN沉积的范围亦随时间的发展逐渐扩大,然而Ⅳ型胶原较同期模型组比较染色范围却明显缩小 (P ＜0.01) ,而FN于7 d后才比模型组减少(P ＜0.01). 结论:姜黄素可抑制肾炎组织内Ⅳ型胶原和FN的积聚并可能延缓肾小球硬化的发生发展.     

去甲肾上腺素所致心肌损害及其发病机理

<正>心肌病是由一系列已知的(继发性)或未知的(原发性)因素所引起的一种心肌疾病,原发性心肌病的病因及发病机理未明,可能与病毒感染、自身免疫反应、遗传因素、营养障碍、内分泌代谢障碍等因素有关.早在1943年Reeb及1960年Maling就曾经报道静脉持续滴注一定剂量、一定时间儿茶酚胺就可以造成心肌组织的病理损害.1966年Shenk发现给兔静脉滴注去甲肾上腺素(NE)可引起心肌细胞的变性、坏死,并伴有强烈的炎症反应,发病机理不清楚.1978年Dowing和Lee观察到短时间内(90分钟)给予适当剂量NE(2~3μg/min/kg)就能引起免心肌的持续性广泛损害,并通过光学显微镜     

无主机床边连续静静脉血液透析滤过在肾衰竭中的应用

目的　探讨无主机床边连续静静脉血液透析滤过 (CVVHDF)在肾衰竭中的应用。方法　采用无主机床边CVVHDF治疗 6例肾衰竭并其他脏器损伤危重患儿。结果　CVVHDF治疗后全身水肿、肺水肿消失 ,重症高血压得以纠正。与治疗前比较血BUN、Cr显著降低 ,高血钾、低钠、低氯纠正 (P <0 .0 1)。高AG(阴离子间隙 )代谢性酸中毒纠正 ;血 pH显著升高 (P <0 .0 5 ) ;HCO-3 、BE显著升高、AG显著降低 (P均 <0 .0 1)。结论　CVVHDF在并肾衰竭不宜搬动危重儿是行之有效的治疗方法之一。其血管通路建立可采用单针双腔中心静脉插管留置术 ,穿刺部位以颈内静脉穿刺为佳     

基质金属蛋白酶及其抑制剂与肾小球硬化

基质金属蛋白酶(MMPs)是一组结构与功能同源的锌离子依赖性蛋白酶超基因家族。在生理条件下,MMPs的合成、分泌及激活过程都受到严密的调控。大量实验资料证实MMPs及其特定的组织金属蛋白酶抑制剂在细胞外基质降解过程中起着重要作用,一些细胞因子、生长因子及激素等能调节MMPs的合成,而其特异性组织抑制剂是其主要的调节因素。MMPs活性下降是导致细胞外基质成分在肾小球内不断积聚,从而导致肾小球硬化的重要机制。对于MMPs及其抑制剂的研究将阐明肾脏病慢性进展机制,为防治各种肾脏疾病提供理论依据。     

小儿血液透析并发症防范的探讨

血液透析(HD)在小儿肾衰竭抢救中占有重要地位。由于与成人相比,小儿对血液动力学改变较敏感且耐受性差,易出现体外循环容量变化所引起的一系列并发症。近3年来,我们对行HD的24例(共166次)肾衰竭患儿就透析中并发症的防范方法,进行了临床观察和比较,现报道如下。1资料与方法1·     

基质金属蛋白酶及其抑制剂与肾小球硬化

基质金属蛋白酶（MMPs)是一组结构与功能同源的锌离子依赖性蛋白酶超基因家族。在生理条件下，MMPs的合成、分泌及激活过程都受到严密的调控。大量实验资料证实MMPs及其特定的组织金属蛋白酶凶制剂在细胞外基质降解过程中起着重要作用，一些细胞因子、生长因子及激素等能调节MMPs的合成，而其特异性组织抑制剂是其主要的调节因素。MMPs活性下降是导致细胞外基质成分在肾小球内不断积聚，从而导致肾小球硬化的重要机制。对于MMPs及其抑制剂的研究将阐明肾脏病慢性进展机制，为防治各种肾脏疾病提供理论依据。     

NADPH oxidase-derived reactive oxygen species involved in angiotensin Ⅱ-induced monocyte chemoattractant protein-1 expression in mesangial cells

Objective To investigate the origin of oxidative stress induced by angiotensin H (Ang Ⅱ ) in human mesangial cells and the role of reactive oxygen species ( ROS) in Ang Ⅱ -induced monocyte chemoattractant protein-1 (MCP-1) expression.Methods MCP-1 expression was determined by real time RT-PCR.ROS production was measured by DCFDA fluorescence.Nicotinamide adenine dinucleotide phosphate (NADPH) oxidase activity was examined by lucigenin chemiluminescence.p47phox and p67phox translocation was assayed by Western blot.Twenty-four male mice were randomly divided into three groups; the control,the Ang Ⅱ infusion [ Ang Ⅱ 400 ng/(kg±min) ],and the apocynin treatment.Ang Ⅱ was infused by subcutaneously osmotic minipump for 14 days.Urinary albumin and 8-isoprostane excretion were measured by ELISA.Results In cultured human mesangial cells,Ang Ⅱ induced the MCP-1 expression in a dose-dependent manner with 3.56 fold increase as compared with the control.Ang Ⅱ increased intracellular ROS production as early as 3 min with the peak at 60 min and was in a time and dose-dependent.Incubation with different dosages of Ang Ⅱ ( 1μmol/L,10μmol/L,and 100μmol/L Ang Ⅱ ) for 60 min,ROS production increased at 1.82,2.92,and 4.08 folds respectively.Ang Ⅱ-induced ROS generation was sensitive to diphenyleneiodonium sulfate (DPI,10 μmol/L) and apocynin (500μmol/L) ,two structurally distinct NADPH oxidase inhibitors.In contrast,inhibitors of other oxidant- producing enzymes,including the mitochondrial complex I inhibitor rotenone,the xanthine oxidase inhibitor allopurinol,the cyclooxygenase inhibitor indomethacin,the lipoxygenase inhibitor nordihydroguiaretic acid,the cytochrome P450 oxygenase inhibitor ketoconazole and the nitric oxide synthase inhibitor G-nitro-L- arginine methyl ester were without an effect Ang Ⅱ -induced ROS generation was inhibited by the ATI antagonist losartan (10μmol/L) but not the AT2 antagonist PD123319 (10μmol/L).Ang Ⅱ treatment induced translocation of cytosolic of p47 and p67 to the membrane.The antioxidants almost abolished Ang Ⅱ -induced MCP-1 expression.Ang Ⅱ infusion increased urinary and p67 translocation by 2.69-,2.97-,and 2.67-fold,respectively.Conclusions NADPH oxidase-derived ROS is involved in Ang Ⅱ-induced MCP-1 expression.Inhibition of NADPH oxidase alleviates Ang Ⅱ -induced renal injury.     

尿微量蛋白测定与小儿急性肾炎

本文通过对急性肾炎患儿尿β2－M、ALb、IgG、SIgA、THP含量的测定，观察其与肾小球基底膜通透性的关系，及与肾功能的关系，现将结果报告如下。对象和方法对象本组22例，均为本院住院患儿，男13例，女9例，平均年龄8．5岁（3～14岁）。急性肾炎诊断标准符合1981年全国儿科协作组制定标准，并排除含合并症的（高血压、循环充血、急性肾功能衰竭）急性肾炎。另设年龄相仿正常对照组16例，男10例，女6例。方法采用放射免疫法测定尿微量蛋白含量。尿ALb‘民一M测定盒系中国同位素公司北方免疫试剂研究所提供，SIgA、IgG、THP测定盒系…