小儿紫癜性肾炎肾脏病理定量分析

目的 :探讨小儿紫癜性肾炎 (APN)病理定量与临床的关系 ,为APN治疗与预后判断提供理论依据。方法 :对 30例APN患儿进行肾小球、肾小管病理评分。结果 :肾小球病理评分 ,0级 0例 ,1级 6例 (2 0 % ) ,2级 17例 (5 6 7% ) ,3级 7例 (2 3 3% ) ;肾小管病理评分 ,0级 2例 (6 7% ) ,1级 6例 (2 0 % ) ,2级 14例 (46 7% ) ,3级 8例 (2 6 7% )。肾小球与肾小管病变程度呈等级正相关 (r =0 .783,P <0 .0 1)。临床表现为肾病综合征组肾小球及肾小管病理评分高于非肾病综合征组 (P <0 .0 1) ;单纯性蛋白尿、急性肾炎组肾小球病理评分差异无显著性 (P >0 .0 5 ) ;而单纯性蛋白尿、肾小管病理评分高于单纯性血尿及急性肾炎组 (P <0 .0 1和P <0 .0 5 ) ;肾小球、肾小管病理评分与病程长短呈正相关 (r =0 .5 6 3,P <0 .0 1) ;与血清尿素氮、血肌酐水平无相关性。结论 :APN患儿同时存在肾小球与肾小管病变 ,两者呈平行关系 ;长期蛋白尿、病程长是APN肾小球、肾小管病理进展的危险因素。血清尿素氮、血肌酐正常不能反映肾脏病理改变程度。     

Pax2在肾病综合征糖皮质激素耐药中的作用

目的：通过观察原发性肾病综合征（PNS）激素敏感与激素耐药组病例中Pax2和P53表达的差异,探讨Pax2在肾脏糖皮质激素耐药中的作用。方法：采用免疫组织化学方法观察40例PNS患儿肾活检组织中Pax2和P53表达差异,分别对肾小球与肾小管间质的病理改变进行半定量评分,对Pax2,P53及病理积分进行相关分析。结果：正常对照组肾组织未见Pax2及P53阳性表达。所有PNS患儿肾活检组织可见不同程度肾小管上皮细胞Pax2表达,足细胞微弱表达或未表达Pax2,其它肾固有细胞未见Pax2表达。激素耐药组近端小管及远端小管上皮细胞Pax2表达较激素敏感组明显增多（P<0.01）,表达密集部位的肾小管多呈现扩张、萎缩或结构不完整,小管绒毛明显脱失。肾小管上皮细胞Pax2阳性表达与肾小管病理积分呈正相关（P<0.01）。激素敏感组未见P53表达,激素耐药组中部分病例检测到P53表达,主要分布于肾小管上皮细胞。相关性分析发现P53表达与Pax2表达、肾小管病理积分均呈正相关（均P<0.01）。结论：肾小管上皮细胞Pax2过度表达,在一定的应激状态上调P53蛋白表达,使肾小管病理改变进一步加重,这可能是导致PNS患儿对激素耐药的机制之一。     

尿RBP与成人原发性肾病综合征的激素敏感性

目的：通过测定成人原发生肾病综合征患者尿RBP和NAG,了解患者肾小管功能与肾病综合征激素治疗效果之间的关系。方法：测定成人原发性肾病综合征患者治疗前和激素治疗8周后尿RBP和NAG含量,根据治疗后尿蛋白水平将患者分为未缓解、部分缓解和完全缓解3组并与正常人作为对照,比较各组间治疗前后肾小管功能指标变化。结果：①各病例组间治疗前尿RBP,NAG和蛋白质水平差异无显著（P＞0.05);②激素治疗有效者,尿中RBP和NAG明显降低,与治疗前比较,差异有显著性（P＜0.01);③三组肾病综合征患者之间治疗后尿蛋白、RBP和NAG比较,差异有显著性（P＜0.01)。结论：尿RBP可预测成人原发性肾病综合征患者激素敏感性,且效果优于NAG。     

原发肾病综合征合并急性肾损伤患者尿液及肾组织中肾损伤因子1的表达及意义

目的分析原发肾病综合征合并急性肾损伤患者尿液及肾组织中肾损伤因子1(KIM-1)的表达及其意义。方法选取2010年1月至2012年6月大连大学附属中山医院诊治的52例原发肾病综合征患者(试验组)和同期50例健康体检者(对照组),检测两组受试者的KIM-1水平,探讨其与患者病理类型及尿毒氮等临床相关指标的关系。结果试验组患者的KIM-1水平显著高于对照组(P<0.05),且试验组患者尿液KIM-1水平与肾组织KIM-1、血β2微球蛋白、24 h尿蛋白定量、血肌酐和尿素氮呈显著正相关(r=0.281,P<0.05))。结论原发肾病综合征合并急性肾损伤患者尿液及肾组织中的KIM-1表达显著增高,可作为反映患者急性肾小管坏死的一个较为敏感的临床指标。     

Dysregulation of apoptosis: a possible mechanism leading to chronic progressive renal histological changes in lupus nephritis

Objective To evaluate apoptosis in lupus nephritis and the relationship between the existe nce of apoptotic cells in renal tissue and histopathological or clinical changes .Methods Apoptosis was detected by in situ nick-end labeling techniques (TUNEL) in renal biopsies from 25 patients with type Ⅳ lupus nephritis (LN), 12 patients with I gA nephropathy IgAN, 4 patients with idiopathic mesangioproliferative glomerulon ephritis (MsPGN) and 3 patients with acute poststreptococcal glomerulonephritis (APGN).Normal renal tissue obtained at nephrectomy for hypernephroma in 4 adul ts was used as control.Proliferating cells were identified by proliferating ce ll nuclear antigen (PCNA) in these patients. Results Compared to other proliferative glomerulo-nephritis and controls, the patients w ith lupus nephritis had less apoptotic cells, a higher ratio of PCNA+ cells/TdT+ cells (P/T) in renal tissues; and their P/T ratio in glomeruli and tubuloin terstitium correlated with the chronicity index, r=0.4983 (P=0.0132), r=0.8399 (P&lt;0.001), r=0.6614 (P=0.0033), respectively.P/T ratios in the glomerulus and tubule had a positive correlation with 24-hour uri nary protein, r=0.8554 (P&lt;0.001) and r=0.7134 (P=0.001); and a negative correlation with creatinine clearance (Ccr), r=-0.4880 (P=0 .0133) and r=-0.7229 (P=0.001), which in tubules positively corre lated with serum creatinine (Scr), r=0.4107 (P=0.0414).Conclusions Apoptosis is reduced in proliferative lupus nephritis.Intense proliferation wi thout a commensurate increase in apoptosis is a possible mechanism that leads to chronic progressive renal histopathological changes.     

MCP-1在肾小球肾炎中的表达及其意义

目的：探讨肾小球肾炎患者肾组织单核细胞趋化蛋白－１（ＭＣＰ－１）的表达及其与肾间质中浸润的单核细胞（ＭＣ）数、肾小管病理损伤、尿蛋白量之间的关系。方法：用免疫组化技术检测５例正常及３１例原发性肾炎肾组织中ＭＣＰ－１、ＣＤ６８的表达。结果：正常肾组织有极少量ＭＣＰ－１表达及ＭＣ浸润;膜性肾小球肾炎（ＭＮ）组肾小球内未见ＭＣＰ－１、ＣＤ６８表达;系膜增生性肾小球肾炎（ＭｓＰＧＮ）、膜性增生性肾小球肾炎     

Relationship between clinical predictors and tubulointerstitial damage in adult-onset primary nephrotic syndrome

BACKGROUND: Tubulointerstitial damage (TID) is an important mediator in the progression of chronic proteinuric nephropathies. Our aim in this study was to evaluate the relationship between several clinical predictors and TID in adult-onset primary nephrotic syndrome in China. METHODS: One hundred ninety-five adult inpatients who were diagnosed with primary nephrotic syndrome based on clinical presentation and biopsy results were enrolled in this study from March 2003 to September 2005. The degree of TID was graded by a semiquantitative method including <2 score and >or=2 score. RESULTS: In all patients, the rate of glomerulosclerosis was correlated with the severity of TID. Serum creatinine and uric acid (r = 0.183, p = 0.012 and r = 0.377, p = 0.00001, respectively) but not serum lipid or total 24-h urinary protein were related with TID. In 64 patients, urinary excretion of IgG (r = 0.443, p = 0.00001) but not of albumin, transferrin, retinal-binding protein, or alpha1-microglobulin were significantly associated with the extent of TID. Proteinuria selectivity index based upon IgG also correlated significantly with the extent of TID (p = 0.0001) (score 0-1 vs. score >or=2). CONCLUSIONS: These results showed that serum creatinine and uric acid, the excretion of urinary IgG and proteinuria selectivity index based upon IgG, were highly correlated with the severity of TID in adult-onset primary nephrotic syndrome. These clinical parameters might be useful for predicting the development and progression of proteinuric nephropathy as independent risk factors.     

临床表现为肾病综合征的特发性膜性肾病的临床和病理特点以及预后不良的因素分析

目的 通过比较有无临床表现为肾病综合征的特发性膜性肾病（IMN）患者肾穿刺基线时的临床病理资料,探讨预后相关的因素。方法 回顾性分析2006年9月至2019年11月浙江省温州市中医院经肾活检确诊为IMN并有完整随访资料的患者共345例,根据患者有无临床表现为肾病综合征分为有肾病综合征组202例和无肾病综合征组143例,比较两组间肾穿刺基线时的临床、病理资料,并对两组的预后进行分析。主要结局：血肌酐翻倍或全因死亡或进展至终末期肾病（ESRD）;次要结局：完全缓解（CR）和部分缓解（PR）。结果 有肾病综合征组男性居多,更多合并浮肿（91.1%）和血尿（45.0%）,收缩压和舒张压偏高,病程更短,白蛋白、血IgG更低,尿蛋白更多,纤维蛋白原、血肌酐、LDL更高,病理程度更重,系膜基质增生、间质炎性细胞浸润、肾小管萎缩、肾脏C3沉积都更严重,与无肾病综合征组相比有统计学差异（P<0.05）。Cox多因素回归分析发现年龄、血补体C3、BUN、Scr、UA、TG、eGFR是肾脏预后不良的危险因素,而高血压、尿蛋白、血沉、肾脏IgA沉积等是临床缓解的预后因素。采用 Kaplan-Meier 法绘制肾脏生存曲线,比较有无肾病综合征两组间主要终点事件和次要终点事件的差异。发现有肾病综合征组较无肾脏综合征组累积肾脏存活率更低,更容易发生不良结局（P=0.0018）,而次要终点事件的发生无统计学差异（P=0.9104）。结论 肾穿刺基线时临床表现为肾病综合征的IMN患者临床病情和病理程度均较无肾病综合征组更重,年龄、血尿素氮、血肌酐、血尿酸、eGFR、甘油三酯是肾脏不良结局的独立危险因素,临床表现为肾病综合征的IMN患者预后更差,更容易进展至ESRD。     

Experimental study of proteinuria caused by chronic exposure to mercury

Proteinuria induced by chronic exposure to mercury and the relationship between urinary mercury and kidney damage were explored in rats using ultrafiltration concentration, gel chromatography, and transmission electron microscopy. The results showed that the primary site of damage was the proximal renal tubule and that the glomerulus was eventually involved. Of the tubular cell ultrastructures, the lysosome was the most sensitive to mercury, and there was a close relation between the excretion of urinary mercury and the mercury detoxication mechanism of the kidney. Many deposits were found in the endothelia and mesangia of the glomeruli. The results of the study showed that urinary mercury consisted of three components, that filtration through the glomeruli was an important source of urinary mercury, and that the mercury excreted from the renal tubules reflected the mercury-loading status of the kidney.     

尿RBP与成人原发性肾病综合征的激素敏感性

目的 :通过测定成人原发性肾病综合征患者尿RBP和NAG ,了解患者肾小管功能与肾病综合征激素治疗效果之间的关系。方法 :测定成人原发性肾病综合征患者治疗前和激素治疗 8周后尿RBP和NAG含量 ,根据治疗后尿蛋白水平将患者分为未缓解、部分缓解和完全缓解 3组并与正常人作为对照 ,比较各组间治疗前后肾小管功能指标变化。结果 :①各病例组间治疗前尿RBP ,NAG和蛋白质水平差异无显著 (P >0 .0 5 ) ;②激素治疗有效者 ,尿中RBP和NAG明显降低 ,与治疗前比较 ,差异有显著性 (P <0 .0 1) ;③三组肾病综合征患者之间治疗后尿蛋白、RBP和NAG比较 ,差异有显著性 (P <0 .0 1)。结论 :尿RBP可预测成人原发性肾病综合征患者激素敏感性 ,且效果优于NAG。