儿童薄基底膜肾病临床病理分析

目的探讨儿童薄基底膜肾病(TBMN)的临床病理特点。方法选取14例经肾活检病理检查确诊为TBMN的患儿,对其临床及病理资料进行分析。结果 8例临床表现为单纯性血尿,1例血尿合并少量蛋白尿,1例少量血尿合并中等量蛋白尿,4例表现为肾病综合征的症状。肾组织光学显微镜检,7例表现为肾小球系膜轻度增生,3例局灶肾小球系膜增生,2例肾小球毛细血管内增生,1例肾小球轻微病变,1例肾小球局灶硬化。免疫荧光检查,3例免疫荧光阴性,10例IgM±～+,1例IgM++。电子显微镜下肾小球基底膜均弥漫性变薄,厚度均<250 nm。血尿、蛋白尿的严重程度与肾小球基底膜厚度呈负相关(r分别为-0.93、-0.99,P均<0.01)。结论儿童TBMN临床上多表现为单纯性血尿,也可伴蛋白尿,少数表现为肾病综合征。肾活检组织电镜观察见肾小球基底膜变薄。患儿血尿、蛋白尿的严重程度与肾小球基底膜厚度有关。     

急进性肾小球肾炎中抗肾小球基底膜抗体的检测及其临床意义

急进性肾小球肾炎中抗肾小球基底膜抗体的检测及其临床意义刘娜赵明辉章友康郑欣王海燕国外报道抗肾小球基底膜（ＧＢＭ）抗体在包括所有的原发性和继发性急进性肾小球肾炎（ＲＰＧＮ）中阳性率为２０％左右［１］，而国内以往报道的仅为５％～１０％左右［２，３］，这种...     

各类肾小球疾病患者抗肾小球基底膜抗体的检测

本文检测了96例各类肾小球疾病患者血清抗肾小球基底膜(CRM)抗体。ELISA显示19例患者抗GBM抗体用性,占19.7％,其中5例肾组织免疫荧光显示免疫球蛋白沿肾小球基底膜呈颗粒性沉积者中有3例抗GBM抗体阳性,提示部分GBM颗粒性沉积者可能与抗GBM抗体的参与有关。进一步应用免疫印迹法显示7例患者分别存在抗层连蛋白(Laminin)、抗硫酸肝素蛋白多糖(HSPG)、扰Ⅳ受胶原7S抗体。本组病例中无一例临床诊断Goodpasture综合征,也无一例存在抗Ⅳ型胶原NC_1抗体,因而也反证了抗Ⅳ型胶原NC_1抗体对Goodpesture综合征的特异性诊断意义。     

小儿孤立性血尿的病理分析

血尿是肾小球疾病的常见临床特征之一 ,但是当血尿作为唯一的临床表现时 ,其病理意义究竟如何 ?本研究旨在通过对 79例孤立性血尿的病理组织学分析 ,对这一问题作一初步的探讨。1　对象和方法1.1　病例的选择　 1982年 3月～ 1999年 3月间 ,我科共对 6 50例患儿进行了肾活检术 ,其中术前诊断为孤立性血尿的患儿有 79例 ,年龄 2 5～ 12岁 ,男性 51例 ,女性 2 8例。其诊断标准为 :①持续的镜下血尿和 (或 )伴有反复发作的肉眼血尿 ;②不伴有蛋白尿 (2 4ｈ尿蛋白定量 <2 50ｍｇ) ;③肾功能正常 ;④不伴高血压 ;⑤影像学诊断排除泌尿道畸形 ;⑥…     

尿微量蛋白测定法在肾小球性血尿鉴别中的应用

目的 寻找一种新的、简便易行的、非侵入性及无创伤的鉴别肾小球性血尿和非肾小球性血尿的方法。方法 对已知不同原因的镜下血尿及肉眼血尿标本予以离心处理，以排除血红蛋白对尿蛋白测定的影响后，分别以尿微量蛋白测定法测定肾小球性镜下血尿及肉眼血尿的蛋白量下限值，并以此值对已知原因的血尿标本做敏感性、特异性检验。结果 肾小球性镜下血尿蛋白量的下限值为200mg／L，其对诊断肾小球性镜下血尿的敏感性为96％，特异性为91％；肾小球性肉眼血尿蛋白量的下限值为360mg／L，其对诊断肾小球性肉眼血尿的敏感性为93％，特异性为90％。结论 尿微量蛋白测定法可作为鉴别肾小球性血尿及非肾小球性血尿的检查手段。     

抗肾小球基底膜抗体相关疾病的预后研究

目的　探讨决定抗肾小球基底膜 (GBM )抗体相关疾病预后的可能因素。方法　对我科近 6年来确诊的 10 5例抗GBM抗体相关疾病患者中 ,有完整临床和病理资料 ,并接受治疗的69例患者进行回顾性分析。对临床完全缓解 ( 5例 )、部分缓解 ( 10例 )和治疗无效 ( 5 4例 )的患者进行比较 ,确定决定预后的因素。结果　决定肾脏预后的因素为 :1.确诊时Scr >60 0 μmol/L者 ,预后较差 (P <0 .0 1) ;2 .出现少尿或无尿者 ,预后较差 (P <0 .0 1) ;3 .肾小球中新月体所占比例 >85 %者 ,预后较差 (P <0 .0 1) ;4.以肺出血为首发表现者 ,预后较好 ,以肾脏受累为首发表现者 ,预后差(P <0 .0 5 ) ;5 .对于确诊时Scr <60 0 μmol/L或以肺脏受累为首发表现的患者 ,给予血浆置换治疗能够改善预后 (P <0 .0 5 )。结论　及时检测血清抗GBM抗体 ,早诊断 ,早治疗 ,是改善病人预后的关键。治疗首选充分的血浆置换     

抗肾小球基底膜非Goodpasture抗体的致病性

本文观察了肾小球基底膜(GRM)非Goodpasture抗体,即抗层连蛋白(Laminin)、抗硫酸肝素蛋白多糖(HSPG)、抗Ⅳ型胶原7S抗体对大鼠肾脏的致病性,结果示上述抗体对大鼠肾脏均有一定的致病性,如尿蛋白增加,肾小球细胞增生.肾小管上皮细胞空泡变性,间质炎性细胞浸润,部分足突融合等,尤以复合抗体注射组为显著.     

儿童无症状性血尿的病理特征及肾活检指征的探讨

目的 分析儿童无症状性血尿的病理特征,并探讨无症状性血尿患儿的肾活检指征。方法回顾性分析2010-01～2019-01在该院进行肾活检的204例无症状性血尿患儿的病理及临床资料,并根据血尿程度、合并蛋白尿程度及年龄进行分组,比较不同组间的一般临床特征、免疫球蛋白水平及病理类型。结果男性人数比例、年龄、病程、Ig A水平、Ig A肾病(Ig AN)、肾小球轻微病变(GML)人数比例及未见肾小球人数比例在单纯性血尿组、血尿伴微量蛋白尿组和血尿伴中量蛋白尿组间比较差异有统计学意义(P 0. 05)。肉眼血尿组患左肾静脉压迫综合征的人数比例高于镜下血尿组,差异有统计学意义(P 0. 05)。24 h尿蛋白定量水平、GML人数比例在不同年龄组间比较差异有统计学意义(P 0. 05)。结论 不建议将单纯性血尿作为儿童早期肾活检的指征,但对于有肾脏病家族史或伴蛋白尿的无症状血尿患儿可适时行肾活检以早期明确诊断。     

肾小球基底膜中胶原的合成、降解与糖尿病肾病

糖尿病肾病是终末期肾病的主要原因,发病机制复杂.肾小球基底膜增厚及系膜区细胞外基质增生是主要的病理特点,胶原合成增多、降解减少是肾脏病理改变的主要原因.研究肾小球滤过屏障的生理功能与损伤机制,对深入了解糖尿病肾病的发生、发展尤为重要.     

Growth hormone enhances basement membrane thickening in experimental diabetes

Summary Injection of porcine growth hormone (200 μg) or saline 5 days a week for 16 to 20 weeks in streptozotocin-diabetic rats showed that compared to saline growth hormone produced a 2 1/2-fold larger increase in glomerular capillary basement membrane thickness (2p=0.027). The possible significance of this effect of an elevated level of growth hormone for diabetic microangiopathy is discussed.     

Glycosylation of glomerular basement membrane in type 1 (insulin-dependent) diabetic children

Summary Immunoelectrophoresis of glomerular basement membrane antigens in the urine of 20 Type 1 (insulin-dependent) diabetic and 10 healthy children was performed. In 10 of the diabetic children, there was altered -1-mobility, while the other diabetic and normal children showed -2-mobility. After incubation with glucose, glomerular basement membrane antigens in the urine of healthy children showed -1-mobility. Isolated human kidney glomerular basement membrane split products obtained by proteolytic degradation (papain, trypsin, chymotrypsin) were also investigated by immunoelectrophoresis. A difference was observed in the immunoelectrophoretic pattern of native and glycosylated glomerular basement membrane split products. A distinct increase of thiobarbituric acid assay positive glomerular basement membrane structures after incubation with glucose provides suggestive evidence for the occurrence of non-enzymatic glycosylation of glomerular basement membrane proteins. Glycosylated glomerular basement membrane proteins may contribute to both functional and morphological changes in diabetic glomerulosclerosis.     

薄基膜肾 病与小儿单纯性血尿

１９８１￣１９９４年单纯性血尿患儿有肾穿刺活检者５０例，对其中光镜检查病变轻微、免疫荧光阴性的１９例肾组织，参照Ｍｏｒｉｔａ的方法在电镜观察下测量肾小球基膜（ＧＧＢＭ）厚度，发现其中１５产同年龄正常ＧＭＢ厚度的平均值明显变薄，符合薄基膜肾病的诊断，占我院同期单纯性血尿的３０％，略高于ＩｇＡ肾病（２６％）。１例有家族史，５例随访１２￣５８个月（平均２８．３个月），除１例仍未再有肉眼血尿发作外，其余病     

Case of anti-glomerular basement membrane antibody-induced glomerulonephritis with cytomegalovirus-induced thrombotic microangiopathy

Although the involvement of cytomegalovirus (CMV) infections in the development of thrombotic microangiopathy (TMA) in HIV patients and transplant recipients has been reported, it is still controversial whether CMV itself can cause TMA. We report herein a rare case with rapid improvement of TMA by ganciclovir treatment in a patient who is neither HIV-positive nor a transplant recipient, suggesting a pathogenic role for CMV in TMA.     

Thickening of glomerular basement membrane in spontaneously diabetic rats

Summary The glomerular basement membrane of spontaneously diabetic rats was investigated by quantitative analysis using electron microscopy, with special reference to the effect of ageing. Constant agerelated increase in the width of basement membrane was ascertained both in diabetic and control rats, and the mean values of basement membrane thickness were always higher in the spontaneously diabetic rats than in normal control rats. Significant thickening of glomerular basement membrane was found in the diabetic rats at 12 weeks of age, while younger diabetic rats had no definite increase. The difference in basement membrane thickness between diabetic and normal control rats became larger with increasing age.     

Anti-glomerular basement membrane disease in Auckland

Background: Anti‐glomerular basement membrane (GBM) antibody‐mediated disease is rare and classically presents with the syndrome of glomerulonephritis and pulmonary haemorrhage. Aim: This aim of this report was to determine the incidence, clinical features, management and outcomes of anti‐GBM disease in Auckland between 1998 and 2008. Methods: Potential patients were identified by a search for positive anti‐GBM antibody serology, diagnostic renal biopsy, or in‐hospital admissions using International Classification of Diseases 9 and 10 codes between 1998 and 2008. A retrospective case notes review of all potential cases was performed with data censored at 31 December 2010. Results: Twenty‐three cases were identified. The rate of anti‐GBM disease was estimated at 1.79 per million person‐years. There were 12 men and 11 women. The median age was 45 years, range 12–74 years. Sixteen patients were European, three were Pacific peoples, three were NZ Maori and one was Chinese. Eleven were regular smokers and eight ex‐smokers, significantly higher proportions than the population (P≤ 0.001). Smokers were significantly more likely to have respiratory disease (P= 0.03). The mean creatinine at presentation was 474 µmol/L. All patients had a renal biopsy; 20 had crescentic glomerulonephritis. One patient recovered renal function without treatment. Twenty‐two were immunosuppressed with cyclophosphamide and corticosteroids. Seventeen received plasmapheresis. Eighteen were alive, eight with end‐stage renal disease, two with chronic kidney disease and eight with normal renal function. Conclusions: Anti‐GBM disease is a rare condition, which is not overrepresented among indigenous people. With aggressive therapy the prognosis has improved; however, the morbidity and mortality of this condition remain significant.     

41例抗肾小球基底膜抗体相关疾病的临床和病理分析

目的了解抗肾小球基底膜（GBM）抗体相关疾病的不同临床类型及其临床病理特点。方法对我科近6年来检测出的41例抗GBM抗体相关疾病的临床病理资料进行回顾性分析。结果22/41例为Goodpasture病（GP）,其中2例肾功能正常。32/41例患者为单纯抗GBM抗体阳性,其中31/32例男性,平均年龄（26.8±9.7）岁。9/41例抗GBM抗体伴抗中性粒细胞胞浆抗体（ANCA）阳性,其中7/9例为女性,平均年龄（44.5±19.6）岁。两组在性别和发病年龄上差异有显著性（P＜0.05）。32/41例作了肾活检,平均发病至肾活检时间为（62.7±43.5）d。2/32例肾功能正常的GP患者为轻度系膜增生性肾炎。30/32例为新月体肾炎,其中24/30例（80%）患者的肾小球100%受累,多伴有毛细血管襻和球囊严重破坏。免疫荧光检查仅16/23例呈典型的IgG、C3沿肾小球毛细血管襻（GCW）呈线样沉积;7/23例表现为IgG和（或）C3等沿GCW呈细颗粒状沉积,少数伴有系膜区沉积。典型和不典型的免疫荧光改变与光镜病理的严重程度不相关（P＞0.05）。所有患者均有贫血、血尿和蛋白尿,其中7/41例表现为肾病综合征。经强化免疫抑制治疗,4例患者临床完全缓解或好转,包括2例轻度系膜增生性肾炎的GP患者。其余患者均依赖肾脏替代疗法或死亡。结论中国人抗GBM抗体相关疾病并不少见。单纯抗GBM抗体组多发于青年男性,双抗体阳性组多发于中老年女性。抗GBM抗体相关疾病多预后差,肾脏病理多为新月体性肾炎,但免疫病理并非均表现为典型的IgG、C3沿GCW呈线条样沉积。仅少数无少尿的轻型患者或肾功能损伤轻者可临床完全缓解或好转。     

Studies on glomerular basement membrane in experimental diabetes using lectin histochemistry in Wistar rats

Summary This study was designed to establish whether specific early changes in carbohydrate content of proteins in the glomerulus of the diabetic rat could be detected. Lectin staining of kidney sections from streptozotocin-induced diabetic rats were compared with similar sections from healthy and diabetic rats that were treated with insulin. Animal groups were killed 1 month, 3 months and 6 months after induction of diabetes. There were no differences in the staining of the glomerular basement membrane between control, insulin-treated and diabetic rats for the lectins concanavalin A, lotus tetragonolobus, soybean and kidney bean, with and without trypsinisation. Staining of the glomerulur basement membrane with wheat germ agglutinin after trypsinisation was significantly increased in the diabetic group when compared to both healthy and insulin-treated groups (p < 0.01). It was concluded that, in experimental diabetes mellitus in the rat, there is an accumulation of substances in the glomerular basement membrane and mesangium with an affinity for wheat germ agglutinin, most probably N-acetyl glucosamine, and this is partially prevented by insulin treatment.     

Morphometric studies of the peripheral glomerular basement membrane in early juvenile diabetes I. Development of initial basement membrane thickening

Summary An extension of a previously reported quantitative electronmicroscopic study of the glomerular basement membrane in juvenile diabetes is presented. The initial phase in the development of basement membrane thickening in diabetic glomeruli has also been studied. — Measurements of the basement membrane were obtained from photomontages of glomerular cross sections produced from electron micrographs. A total of 16 glomeruli from five non-diabetics and 83 glomeruli from diabetics were measured. — The results showed that the peripheral glomerular basement membrane is normal at the onset of acute, juvenile diabetes, but a thickening is demonstrable in patients with a duration of the disease of about 2 years. — These findings support the hypothesis that diabetic angiopathy is a consequence of the metabolic derangement in diabetics.Supported by grants from: Statens lægevidenskabelige forskningsråd, Michaelsen fonden, Novo fonden, Rask Ørsted fonden.