IgA肾病系膜区IgA沉积的机制

IgA肾病(IgAN)是最常见的原发性肾小球肾炎。在我国，IgAN占原发性肾炎的39．5％。IgAN患者预后悬殊，20％～40％患者20～25年后进展至终末期肾功能衰竭。IgAN的始发因素是系膜区IgA沉积，其中主要是多聚IgA1(pIgA1)沉积，伴或不伴IgG、C3沉积。IgA沉积于系膜区后将引起肾小球炎症和损伤，病变可完全吸收消散或遗留硬化性病变，也可出现肾小管萎缩、间质纤维化，导致进行性肾功能减退。其中，     

原发性系膜性IgA肾病临床分型的意义

作者分析了463例IgA肾病病人,根据临床、组织学、免疫病理特点及治疗反应等,将其分为反复肉眼血尿、孤立性肉眼血尿、尿检异常、肾病综合征、非肾病性大量蛋白尿及高血压等六型。此分型有助于提高本病的治疗效果。     

IgA肾病患者血清低糖基化IgA1水平的变化及其临床意义

目的　探讨IgA肾病患者血清低糖基化IgA1的程度与临床表现、肾脏病理及近期疗效间的关系。方法　用ELISA法对 68例IgA肾病及 2 0例肾病综合征肾小球微小病变 (MCD )患者血清IgA1糖基化程度进行检测 ,并经治疗后随访。 结果　IgA肾病患者蚕豆凝集素与血清IgA1的结合力明显高于对照组 (P <0 .0 2 5 )。IgA肾病患者中 2 0例低糖基化组和 48例正常糖基化组比较 ,前者发病年龄轻、血IgA浓度及肌酐清除率低于后者 (P值均 <0 .0 5 ) ,而病程、尿蛋白排泄量、总胆固醇水平、血压、肾小球病理分级和近期疗效比较差异无显著性 (P值均 >0 .0 5 )。结论　IgA肾病患者血清IgA1呈低糖基化 ,血清低糖基化程度重者发病年龄较轻 ,但肾功能损伤较重 ,血IgA1低糖基化程度不能推测肾脏病理轻重或近期疗效     

糖尿病肾病合并IgA肾病

病史摘要 病史患者男性,40岁,因“口干、多饮、多尿半年,尿检异常2月余”入院。患者入院前半年自觉口干、多饮、多尿,夜尿2～3次／夜,无视物模糊、手足麻木等不适,未治。     

一种特殊类型的IgA肾病

报告６例ＩｇＡ肾病，有以下特点：青年男性，病程短，在短期内迅速进展至肾功能衰竭（肾衰），甚至终末期肾衰；大量蛋白尿伴血浆白蛋白下降，呈肾病综合征或肾病综合征倾向；本组４例以急进性或恶性高血压为首发表现。肾活检１例为新月体性肾炎，５例有肾小球硬化伴新月体形成，小管间质病变严重，免疫荧光示ＩｇＡ在系膜区弥漫性沉积，ＩｇＭ在毛细血管壁沉积。肾脏病理为判断此类ＩｇＡ肾病的预后提供了依据。治疗上，应严格控制血压，并作好透析或肾移植的准备。     

肾移植活体供肾的现状

自1954年Merrill等实施人类首例同卵双生兄弟间活体亲属供肾移植以来，肾移植技术，现已成为救治终末期肾脏病（ESRD）的最有效手段。特别在西方国家，活体供肾已被广泛应用。美国从2001年以来，活体供肾年移植量显然超过尸肾移植。在伊朗，自从1984年以来已做肾脏移植16000例，约95％以上的肾脏移植来源于活体供肾。与尸肾移植比较，活体供肾移植可获得更好的人／肾存活率，特别在一开始透析时就做活体肾移植的效果更好。透析时间0～6个月移植肾5年存活率78％，透析时间〉24个月者为58％；10年存活率分别为63％和29％。本文主要综述活体供肾移植的现状。     

结缔组织病合并IgA肾病临床分析

目的分析结缔组织病合并IgA肾病的临床特点。方法回顾性分析了本院近6年来16例结缔组织病合并IgA肾病患者的临床表现、肾脏病理和治疗、随诊旨况。结果16例结缔组织病合并IgA肾病患者中男性3例，女性13例，年龄17-58岁，病程10d-10年，其中肾脏症状出现于其他症状之前的4例,肾脏症状于结缔组织病起病同时发现或起病后发现的12例；16例患者中包括5例未分化结缔组织病、2例原发性干燥综合征、6例系统性血管炎、3例类风湿关节炎。结论多种结缔组织病的肾脏受累可以表现为IgA肾病，病理为系膜增生性肾小球肾炎，Lee分级Ⅱ-Ⅳ级；有些结缔组织病肾脏受累不同于该疾病的典型肾脏病理表现。需要在临床工作中引起重视。     

IgA肾病合并线粒体病

16岁女性,典型肾病综合征起病,且足量激素治疗有效.肾活检病理组织学见肾小球节段轻度系膜增生性病变,免疫荧光IgG、IgA及Clq系膜区沉积,电镜下系膜区增宽,并见系膜区电子致密物沉积,肾小球足细胞足突广泛融合,病理诊断为IgA肾病(微小病变型).2年后患者肾病综合征复发,并出现多系统损害,血白细胞基因检测示8969G＞A基因突变,符合线粒体病.尽管重切电镜标本观察到肾小管上皮细胞胞浆中异常线粒体蓄积,但不能证实肾脏疾病与线粒体相关,最终诊断为IgA肾病合并线粒体病.     

中老年IgA肾病的临床病理特征和转归

目的　探讨中老年IgA肾病临床和病理特性及其与预后的关系。方法　分析 65例 45岁以上IgA肾病临床和病理特征 ,并与 659例年轻患者 (≤ 45岁 )比较。分析肾存活率并与年轻组比较。结果　中老年组血胆固醇、甘油三酯以及高血压或肾功能不全发生率显著高于年轻组 (P<0 0 5)。≥ 60岁亚组收缩压和尿蛋白显著高于 45～ 59岁亚组 (P <0 0 5 )。两组Lee氏病理分级分布无显著性差别 (P >0 0 5)。中老年组肾小管 间质指数和血管指数显著高于年轻组 (P <0 0 5) ,但肾小球指数和新月体指数无显著性差异 (P >0 0 5)。两组 5年肾存活率均为 85 6 % ,无显著性差别(P >0 0 5)。结论　中老年IgA肾病高血压、高脂血症和肾功能不全发生率较高 ,肾血管和肾间质损害比较突出 ,故应注意积极控制血压和高脂血症。     

Recurrence of IgA Nephropathy after Kidney Transplantation in Adults

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Metabolic control and progression of complications in insulin-dependent diabetic patients after kidney transplantation.

Patient survival and progression of complications were monitored for 3 years after kidney transplantation in 29 type-1 diabetic patients. Ten age-matched, non-diabetic kidney-transplanted patients served as controls. Five diabetic patients died during follow-up (three cardiovascular events, two infections), three diabetic patients had a non-fatal myocardial infarction and four developed cerebrovascular complications after transplantation. Of the diabetic patients, 69% suffered from proliferative retinopathy before transplantation; 20% of them improved, 65% remained unchanged and 15% deteriorated after transplantation. Motor but not sensory conduction velocity measured from the nervus medianus improved after transplantation. Autonomic neuropathy was observed in 50% of the patients and was unaffected by transplantation. Glycaemic control did not improve significantly during follow-up (HbA1, 10.6 +/- 0.5% before and 9.5 +/- 0.6% 3 years after transplantation). Body weight increased in both diabetic and non-diabetic patients within 3 years after transplantation (from 68 +/- 2 to 77 +/- 6 kg in diabetics, P less than 0.01; from 167 +/- 4 to 77 +/- 6 kg in non-diabetics, P less than 0.01). Subcutaneous fat thickness measured from computer tomography scans of the calf increased in diabetic patients from 5.0 +/- 0.6 to 6.1 +/- 0.9 mm (P less than 0.05). However, the cross-sectional areas of triceps and calf muscles did not increase, suggesting that the increase in body weight was solely due to an increase in fat. It is clear that diabetes-related complications continue to progress and are not influenced by a successful kidney transplant.     

肾移植术后肺部蠊缨滴虫感染--附4例报道

目的：回顾性分析4例。肾移植术后肺部蠊缨滴虫感染患者的临床表现及预后。方法：4例。肾移植术后肺部感染患者经纤维支气管镜和支气管肺泡灌洗液检查确诊蠊缨滴虫感染，分析其临床特点，影像学特征和治疗、预后。结果：（1）4例。肾移植术后肺部蠊缨滴虫感染均在术后2-3个月出现，其中2例合并其他病原体感染（细菌和巨细胞病毒）。（2）病程早期均表现为单纯发热，胸片和CT显示肺门轮廓模糊、密度增高、结构不清，部分支气管影增粗，紊乱；病程中可出现咳嗽、咯痰、胸闷、气短。（3）4例经甲硝唑治疗及撤减免疫抑制剂后4-7天3例体温降至正常，1例降至37．5℃；胸片和CT上的渗出性改变明显吸收消散；2例患者治疗后行重复支气管镜检查，肺泡灌洗液镜检可见大量死亡的蠊缨滴虫。结论：（1）肺部蠊缨滴虫感染早期表现隐匿，对于病原学诊断不明确和治疗反应不佳的术后肺部感染患者，需注意蠊缨滴虫感染的可能；（2）支气管肺泡灌洗液镜检是诊断蠊缨滴虫肺部感染的确切手段，撤减免疫抑制剂同时进行甲硝唑治疗可以迅速有效地缓解病情。     

Laparoscopic and robotic donor pancreatectomy for living donor pancreas and pancreas–kidney transplantation

Pancreas transplantation is a widely accepted procedure that can efficiently restore euglycemia and prevent progression of complications. In most instances, the limiting factor for deceased donor organ transplantation is the availability and quality of the available organs. Living donor pancreas transplant was introduced at the University of Minnesota in 1979. Because of the potential risks for the donor and the technical challenges in the recipient operation, this procedure has not become very popular since then. In 1999, in the attempt to decrease the morbidity associated with open distal pancreatectomy, the first laparoscopic donor distal pancreatectomy with hand-assisted technique was performed at the same institution. In 2000, the FDA approved the robotic surgical system Da Vinci for general use. Since then, the system has been extensively used at our institution to perform living donor nephrectomy. The only case reported worldwide of robotic distal pancreatectomy and nephrectomy for living donor pancreas–kidney transplantation was successfully performed by our team in 2006 at the University of Illinois at Chicago and proved as a promising technique. The application of minimally invasive techniques has allowed an increased acceptance of the procedure among potential donors and may, therefore, increase the number of donors for this life-saving transplant. The initial results are encouraging and clearly prove feasibility.     

进展型(重症)IgA肾病12例临床与病理分析

目的通过对12例进展型(重症)IgA肾病患者临床和病理资料的分析,试图发现IgA肾病进展的危险因素。方法选取我院近年来行肾穿刺活检时即出现肾功能减退和(或)随访过程中肾功能进行性减退的IgA肾病患者12例进行分析。结果12例患者中就诊原因分别为眼睑、下肢水肿,尿中泡沫增多或反复发作无痛性肉眼血尿或体检发现蛋白尿和(或)高血压及肾功能减退。病程20天~9年,平均病程3年。所有患者均有蛋白尿、其中肾病综合征5例;病程中反复发作肉眼血尿5例,其中伴肾病综合征和大量蛋白尿3例;蛋白尿 镜下血尿6例。肾功能损害轻度~重度,CKD(慢性肾脏疾病)1~4级;Ccr94ml/min~25.9ml/min;病理所见均为IgANⅣ级,以增生、硬化及新月体为主。完全缓解1例,进展为终末期肾衰竭2例,部分缓解3例,1例5年前肾活检时肾功能基本正常,病理改变较轻,失访5年后再次就诊时肾功能已减退,其余病例正在治疗随访中。结论部分IgA肾病患者病情进展迅速,尤其是反复发作肉眼血尿伴大量蛋白尿和(或)肾病综合征(NS)及免疫复合物在血管襻沉积者,可能是IgA肾病进展的危险因素。一旦确诊,应根据病理所见进行相应的免疫抑制治疗。积极治疗可以有效的控制IgA肾病的进展。     

Clostridium difficile colitis in patients after kidney and pancreas–kidney transplantation

Limited data exist about Clostridium difficile colitis (CDC) in solid organ transplant patients. Between 1/1/99 and 12/31/02, 600 kidney and 102 pancreas-kidney allograft recipients were transplanted. Thirty-nine (5.5%) of these patients had CDC on the basis of clinical and laboratory findings. Of these 39 patients, 35 have information available for review. CDC developed at a median of 30 days after transplantation, and the patients undergoing pancreas-kidney transplantation had a slightly higher incidence of CDC than recipients of kidney alone (7.8% vs. 4.5%, P>0.05). All but one patient presented with diarrhea. Twenty-four patients (64.9%) were diagnosed in the hospital, and CDC occurred during first hospitalization in 14 patients (40%). Treatment was with oral metronidazole (M) in 33 patients (94%) and M+oral vancomycin (M+V) in 2 patients. Eight patients had recurrent CDC, which occurred at a median of 30 days (range 15-314) after the first episode. Two patients (5.7%) developed fulminant CDC, presented with toxic megacolon, and underwent colectomy. One of them died; the other patient survived after colectomy. CDC should be considered as a diagnosis in transplant patients with history of diarrhea after antibiotic use, and should be treated aggressively before the infection becomes complicated.     

糖尿病对肾移植预后影响的研究

目的探讨肾移植患者合并糖尿病对预后的影响。方法回顾性分析1988年4月至2008年3月中国人民解放军总医院第二附属医院收治的507例肾移植患者手术前后相关临床资料和随访资料。结果507例共行539例次肾移植术(多次手术29例),其中52例合并糖尿病[其中男30例,年龄(51.4±7.1)岁;女22例,年龄(51.6±4.9)岁],移植前糖尿病16例,移植后糖尿病36例;因糖尿病肾病致肾衰竭性肾移植治疗占糖尿病的30.8%。未合并糖尿病肾移植455例[其中男293例,年龄(38.7±12.3)岁;女126例,年龄(43.5±10.1)岁]。合并糖尿病组的各种并发症患病率均显著高于非糖尿病组。合并糖尿病组病死率17.3%(9/52),同期非糖尿病肾移植组病死率7.7%(35/455),尤以移植前糖尿病病死率最高。结论肾移植合并糖尿病者病死率高、合并症多,需加强对肾移植前后糖尿病的控制。     

IgA肾病并发肉眼血尿相关急性肾损伤临床及病理分析

目的分析IgA肾病(IgAN)患者发生肉眼血尿相关急性肾损伤(AKI)的临床、病理特点及其转归,探讨其对预后的影响及相关因素。方法对2004年1月至2007年8月在北京大学人民医院肾内科确诊为IgAN且同时伴发肉眼血尿相关AKI患者的临床、病理资料进行分析,并对这些患者进行随访。肾脏病变的程度根据KatafuchiIgAN评分标准进行评价。结果5例经临床和肾脏病理证实的IgAN患者发生肉眼血尿相关AKI,占同期确诊IgAN患者的1.3%,占发作肉眼血尿患者的13.5%。肾脏病理提示均存在大量红细胞管型伴不同程度的肾小管损伤。其中3例患者血清肌酐(Scr)在14d内完全恢复正常,1例患者Scr在1个月后明显好转,418d后恢复正常。另1例患者至最后一次随访(20个月)时Scr仍未恢复正常。未恢复者与恢复者相比,年龄较大,肉眼血尿持续时间较长,肾小球球性硬化积分和小管-间质病变积分较高。结论发作性肉眼血尿可以引起AKI的发生,且并非所有IgAN患者发生的肉眼血尿相关AKI都能完全恢复。影响肉眼血尿相关AKI患者预后的因素可能有年龄、肉眼血尿持续时间、小管-间质损伤程度以及硬化肾小球比例。     

Hepatitis E virus infection as a new probable cause of de novo membranous nephropathy after kidney transplantation

Hepatitis E virus (HEV) has been identified as a cause of chronic viral hepatitis in immunocompromised patients. Some glomerular diseases were found to be associated with this infection. We report the first case, to our knowledge, of a kidney transplant recipient who developed an HEV infection and de novo membranous nephropathy (MN) concomitantly. The patient displayed a hepatic cytolysis first and a nephrotic syndrome occurred 3 months later. HEV infection was diagnosed upon positive polymerase chain reaction on plasma and stool samples, and renal allograft biopsy revealed de novo MN. Typical causes of MN were definitively excluded. A 3‐month course of ribavirin monotherapy allowed the patient to mount a sustained viral response that was rapidly followed by complete remission of the nephrotic syndrome. The chronology of the onset and remission of both diseases is highly suggestive of a causal relationship between hepatitis E and MN.