成人胡桃夹综合征合并IgA肾病30例临床分析

目的分析胡桃夹综合征合并IgA肾病患者的临床特点,提高临床医师诊治水平。方法以30例成人胡桃夹综合征合并IgA肾病患者为Ⅰ组,同期90例已行左肾静脉多普勒检查后无合并胡桃夹综合征的成人IgA肾病患者为Ⅱ组,同期60例单纯胡桃夹综合征患者为Ⅲ组。回顾性分析该3组患者的临床、实验室检查、影像学及病理资料。结果Ⅰ组中男女比为1∶3.29,女性明显多于男性。Ⅰ组及Ⅲ组体质量指数(BMI)低于Ⅱ组,Ⅰ组血清C3水平明显低于Ⅱ组,Ⅰ组中患者低C3血症发生率为56.7%,高于Ⅱ组的26.7%(P0.01)。Ⅰ组及Ⅲ组左肾静脉的狭窄处内径明显窄于Ⅱ组左肾静脉的狭窄处内径(P0.01)。结论长期胡桃夹综合征合并低C3血症成人患者,建议行肾活检明确诊断;成人女性瘦长体型IgAN患者及伴血C3下降的IgAN患者均建议行左肾静脉彩色多普勒检查,以明确是否存在胡桃夹综合征。     

3D打印支架治疗胡桃夹综合征合并IgA肾病的疗效观察

目的 观察3D打印血管外支架治疗胡桃夹综合征合并IgA肾病的疗效。方法 回顾性分析2017年5月—2021年9月在空军军医大学唐都医院接受左肾静脉3D打印血管外支架植入术的19例胡桃夹综合征合并IgA肾病患者的资料,通过配对样本的统计学方法,分别对照术前、术后的影像学与临床检验结果,分析比较胡桃夹综合征合并不同Lee氏分级IgA肾病的治疗情况。结果 随访数据显示19例患者术后至最新随访或随访结束影像学结果均呈现稳定。其中Lee氏分级Ⅱ级以下的16例患者肉眼血尿与蛋白尿症状均明显改善;此外,Lee氏分级Ⅲ级的3例患者虽然尿红细胞定量、24 h尿蛋白定量较术前也明显好转,但依然出现明显的肉眼血尿与蛋白尿,故需实施肾内科干预。结论 3D打印血管外支架治疗胡桃夹综合征合并IgA肾病安全、有效,不同的Lee氏分级或可引导制定更加个性化的治疗方案。     

彩色多普勒超声对小儿左肾静脉受压综合征的诊断价值

左肾静脉受压综合征又称胡桃夹现象。胡桃夹现象发展机制是左肾静脉受机械性挤压后而致血管腔狭窄,回流障碍,最终因左肾静脉压力增高而引起反复血尿或蛋白尿。此病多见于儿童及青春期少年。特别是生长发育过速,体型瘦长者,以男性为多。本文应用彩色多普勒超声对小儿左肾静脉受压综合征患者进行观察,旨在探讨彩色多普勒超声对小儿左肾静脉受压综合征的诊断价值。     

胡桃夹综合征的诊断和手术治疗(附3例报告)

目的:探讨胡桃夹综合征(NCS)的诊断与治疗。方法:NCS患者男2例,女1例,年龄14～23岁。临床主要表现为反复发作的血尿、蛋白尿和腰痛,1例并发左侧精索静脉曲张。多普勒彩色超声及CT示腹主动脉与肠系膜上动脉夹角处左肾静脉受压明显。3例患者均行左肾静脉移位术。结果:3例患者术后血尿或蛋白尿均消失,无并发症发生。术后6个月行多普勒彩色超声及尿液检查均未见异常。结论:NCS临床表现为反复发作的血尿或蛋白尿。多普勒彩色超声在NCS的诊断中具有重要价值。左肾静脉移位术是治疗NCS的有效方法。     

黄蛭益肾胶囊联合泼尼松治疗以肾病综合征为主要表现的IgA肾病的临床研究

正IgA肾病是我国常见的肾小球疾病,在华东地区相关省份报道的肾活检人群中高达43.38%[1],是我国终末期肾脏病的常见病因。根据IgA肾病常见临床表现可将其分为肉眼血尿型、尿检异常型、肾病综合征型及肾衰竭型[2],国内文献报道肾病综合征型可达肾活检中IgA肾病的41.1%[3],为常见类型。我国现有文献报道的中成药治疗IgA肾病多是在非肾病综合征患者中的应用[4,5],针对肾病综合征型原发性IgA肾病应用     

肾络宁治疗IgA肾病的临床疗效及安全性评价

目的：观察疏利少阳标本兼治法之肾络宁治疗IgA肾病气阴两虚兼有湿热证的临床疗效及安全性。方法：将90例IgA肾病气阴两虚兼有湿热证患者随机分为治疗组和对照组,治疗组在基础治疗的同时,加服肾络宁;对照组在基础治疗的同时,加服肾炎康复片。治疗6个月后,观察两组患者治疗前后中医证候积分变化,以及尿常规,24 h尿蛋白定量,尿NAG、GAL,肝肾功能等实验室指标的变化。结果：（1）临床疗效比较：肾络宁组总有效率为86.67%,对照组为80%;中医证候疗效比较：肾络宁组总有效率为93.33%,对照组为86.67%。（2）对尿红细胞及尿蛋白的影响：肾络宁可明显降低患者尿中红细胞数量及减少尿蛋白,且在改善血尿方面疗效优于肾炎康复片组。（3）肾功能的影响：本研究选择的IgA肾病患者,其肾脏病理分级多在三级以下,肾功能基本正常,故未能反映出肾络宁在此方面的疗效。（4）观察期间,两组患者均未发生不良反应。结论：肾络宁治疗IgA肾病气阴两虚兼有湿热证的临床疗效肯定,安全性良好;能较好地改善患者症状,有效地减少患者血尿、尿蛋白,保护肾小管功能,且在减少血尿方面优于对照组。     

胡桃夹综合征研究进展

胡桃夹综合征是指左肾静脉受压引起的血尿、蛋白尿和左腰腹痛等临床症状 ;彩色双功能超声结合磁共振血管成像是首选的检查方法 ,诊断标准尚不一致 ;治疗以保守为主 ,介入治疗正逐步取代手术治疗成为首选的特殊治疗。     

肾功能不全的IgA肾病患者临床表现与病理特点分析

目的分析肾脏活体组织检查（简称：肾活检）时肾功能异常的IgA肾病患者的临床表现与病理特点。方法选择我院经肾活检确诊的190例IgA肾病患者为研究对象,以其患者血肌酐（SCr）130μmol／L为界分为2组：肾功能正常组（SCr〈130μmol／L）128例和肾功能异常组（SCr≥130μmol／L）62例。同时对其肾脏病理进行半定量评分,比较2组患者的临床病理特点,并且通过回归分析与其肾功损害相关的因素。结果与肾功能正常组相比,肾功能异常组男性比例明显增高（72．6％1;L28．9％,P〈0．01）,年龄更大[（34±10）岁比（30±9）岁,P〈0．01],病程更短[（11±17）]个月比（20±41）个月],同时收缩压更高[（141±19）比（123±17）mmHg,P〈0．01],24h尿蛋白定量增多[（3．31±2．70）g比（2．25±2．19）g,P〈0．01]。同时其患者肾脏病理反映慢性病变的指数均明显增高。多因素分析还显示,与肾活检时肾功能异常密切相关的危险因素包括男性,年龄增大,收缩压增高,24h尿蛋白定量增多,以及肾小管萎缩和间质纤维化指数增高。结论肾活检时肾功能异常的IgA。肾病患者临床表现和肾脏病理改变均明显加重,肾小管萎缩和间质纤维化指数增高与IgA肾病患者肾活检时肾功能异常独立相关。     

IgA肾病中医辨证分型治疗新探

IgA肾病（IgAN）系指肾小球系膜区以IgA为主的免疫球蛋白沉积为免疫病理特征的一组肾小球疾病,是我国最常见的原发性肾小球疾病,约占肾活检病例的1／3,在确诊后5～25年内有20％-40％的患者可发展为慢性肾衰竭。IgA肾病临床表现多数以血尿为主,可伴有少量蛋白尿、大量蛋白尿或肾病综合征,少数病人可见急进性肾炎综合征。     

210例IgA肾病肾间质血管病变的临床病理分析

目的 探讨肾间质血管病变在IgA肾病中的临床病理意义.方法 总结本院肾内科1999年1月～2010年6月肾活检证实210例IgA肾病患者临床病理资料,分析肾血管病变与患者临床表现、病理改变之间的关系.结果 210例IgA肾病中有各种肾血管病变者71例,占33.8％,139例患者无血管病变,占66.2％.两者相比,前者高血压发生率较高(73.2％vs24.5％,P＜0.05),血肌酐水平较高[(132.5±20.8)μmol/L vs (86.4±15.2)μmol/L,P＜0.05],肾小球滤过率较低[(59.7±9.2)ml/min vs (83.1±12.6) ml/min,P＜0.05],两组间差异有统计学意义.在IgA肾病各亚型中血管病变发生率分别为:Ⅰ型6.0％;Ⅱ型43.9％;Ⅲ型6.9％;Ⅳ型40.8％;V型93.1％.x2检验表明其不同分布的差异有统计学意义(P＜0.05),并提示随着IgA肾病的组织病理加重,肾间质血管病变增多.结论 肾间质血管病变在IgA肾病中较为常见,并与IgA肾病的临床表现及病理改变密切相关,提示肾间质血管病变是影响IgA肾病进展的重要因素.     

IgA肾病中足细胞损伤及其与蛋白尿的关系

目的 观察 IgA肾病（IgAN）患者足细胞损伤的各种表现，探讨其与蛋白尿的关系。 方法 收集35例伴有明显蛋白尿[尿蛋白量（24 h）>1.0 g]的IgAN患者肾活检组织作研究；以8例肾错构瘤患者术后切除肾和肾癌患者术后远离癌旁肾组织为正常对照。免疫组化方法观察肾组织细胞周期调节蛋白（p21、p27）、足细胞结构蛋白（nestin）、足细胞数目 （WT1）。用显微切割方法取出肾小球，通过实时定量PCR方法检测整合素（integrin）β1、nephrin和α辅肌动蛋白4（α-actinin 4）水平。电镜观察足细胞超微结构的改变。根据足细胞数目密度(Nv, n×106/μm3)将35例IgAN患者分为足细胞数目减少组（ Nv<52.49×106/μm3，n = 15）和足细胞数目正常组（Nv≥52.49×106/μm3，n = 20）。随访蛋白尿的转归情况，共18个月。 结果 （1）与正常对照组比较，IgAN患者肾小球内个别足细胞重新表达p21，而足细胞p27的表达明显降低（0.71±0.12比0.91±0.07，P < 0.05）。（2）IgAN患者足细胞nestin 蛋白表达比正常对照显著降低（13.40%±0.04%比 17.60%±0.04%，P < 0.05）；肾小球内integrin-β1 mRNA表达显著升高（12.54±5.20比1.02±0.30，P < 0.05），而nephrin及α-actinin4 mRNA无明显改变。（3）电镜下观察到明显的足突融合和足细胞从基底膜脱落。（4）IgAN患者足细胞数目密度比正常对照组显著减少(161.27±225.92比323.22±138.12，P < 0.05)，且与Lee氏分级相关。（5）足细胞数目密度、integrin-β1 mRNA与肾穿刺当时的尿蛋白量（24 h）呈负相关(r = -0.4483、-0.840, 均P < 0.05)。足细胞数目减少组较足细胞数目正常组的蛋白尿下降程度明显减少（P < 0.05）。 结论 伴蛋白尿的IgAN中存在足细胞的损伤，表现为足细胞周期调节蛋白、结构蛋白的改变，足突的融合及足细胞数目的减少，而足细胞损伤及足细胞数目减少会影响蛋白尿的发生和发展。     

Clinicopathological features and renal outcomes of IgA nephropathy with acute tubulointerstitial nephropathy

Objective To evaluate the clinicopathological characteristics and outcomes of IgA nephropathy (IgAN) with acute tubulointerstitial nephropathy (ATIN). Methods Patients who were diagnosed as IgAN with ATIN and IgAN without ATIN by renal biopsy in Peking University First Hospital were enrolled. There were 74 cases of IgAN with ATIN, and seventy-four cases of IgAN without ATIN were enrolled based on stratified sampling (chosen by 1∶1). The two groups were well matched with age, gender, follow-up time, mesangial hypercellularity(M), endocapillaryhypercellularity(E), segmental glomerulosclerosis(S), tubular atrophy/interstitial fibrosis(T) and cellular/fibrocellular crescent(C). The clinicopathological characteristics and outcomes of two groups were retrospectively analyzed. A composite end point, defined as 30% or 50% estimated glomerular filtration rate (eGFR) decline and end stage renal disease (ESRD) was used. Renal function and proteinuria during follow-up were observed. Renal survival was calculated by Kaplan-Meier survival analysis and risk factors of progression were analyzed by using univariate and multivariate Cox regression models. Results Seventy-four cases of IgAN with ATIN and seventy-four cases of IgAN without ATIN were enrolled. Serum creatinine [(185.6±83.2) μmol/L vs (146.3±69.2) μmol/L, P=0.010] and incidence of acute kidney disease (AKD) (31.1% vs 5.4%, P＜0.001) were higher in IgAN with ATIN group than those in IgAN without ATIN group. Patients in ATIN group received more immunosuppressive treatment (86.5% vs 58.1%, P＜0.001). During 1 year after biopsy, mean eGFR increased significantly in IgAN with ATIN group [(39.7±14.6) ml?min-1?(1.73 m2)-1 vs (47.2±19.9) ml?min-1?(1.73 m2)-1, P=0.017], but mean eGFR was not statistic different in IgAN without ATIN group [(60.0±30.5) ml?min-1?(1.73 m2)-1 vs (59.0±31.7) ml?min-1?(1.73 m2)-1, P=0.567]. Median follow-up was 23.0 months in IgAN with ATIN group, and Median follow-up was 30.0 months in IgAN without ATIN group. Incidence of composite end point had no significant differences between two groups. IgAN with ATIN was not the independent risk factor for end point. IgAN patients with ATIN were divided into two groups (with AKD and without AKD), then renal survival rate was higher (Log-rank test, χ2=5.293, P=0.021) and the risk for composite end point decreased by 79.2% (HR=0.208, 95%CI 0.046-0.939, P=0.041) in the group with AKD. Conclusions In IgAN, there is a subgroup of patients with the specific pathological phenotype combined with ATIN. Compared with those without AKD, the risk for composite end point of IgAN patients with ATIN and AKD showed a 79.2% decrease.     

血清低半乳糖化IgA1测定对鉴别IgA肾病的临床价值

目的 确定血清低半乳糖化IgA1对鉴别诊断IgA肾病的临床价值。 方法 以原发性肾小球疾病患者91例为研究对象，接受肾活检并留取血清；以健康体检者20例血清作为对照。血清标本先用装有耦联蚕豆凝集素的微球进行微量离心柱法分离并洗脱，获得低半乳糖化IgA1。再以凝集素HAA（Helix aspersa）用ELISA法定量检测异常糖基化IgA1（HAA-IgA1）。分析血清低半乳糖IgA1升高在鉴别诊断IgA肾病方面的临床价值。 结果 48例IgA肾病患者HAA-IgA1水平[（83.7±41.0） U]高于健康对照组[（52.6±22.9） U]及43例其他原发性肾小球疾病患者组[（49.2±27.3） U]（均P < 0.01）。而该43例中，非IgA系膜增殖性肾炎患者22例（51%）的HAA-IgA1水平[（47.6±21.5） U]亦显著低于IgA肾病患者。以肾穿刺病理诊断为金标准，所绘制ROC曲线面积为0.797，面积的标准误为0.047（P < 0.01）；鉴别诊断IgA肾病的灵敏度为72.9%，特异度为72.1%，准确度为72.5%。 结论 应用微量离心柱法联合ELISA法检测IgA肾病患者血清低半乳糖IgA1对于鉴别诊断IgA肾病具有一定临床价值。     

IgA肾病患者血清IgA1与系膜细胞共培养上清诱导足细胞凋亡

目的 探讨IgA肾病(IgAN)患者血清IgA1与系膜细胞共培养上清对足细胞凋亡的影响。 方法 用Jacalin 亲和层析柱和Sephacryl S-200 分子筛纯化蛋白。单体IgA1（mIgA1）热聚合为聚合体IgA1（aIgA1）。实验分为患者上清组、健康上清组和对照组，系膜细胞分别与IgAN患者的aIgA1、健康对照的aIgA1和5%胎牛血清共培养，收集上清，与同步化的足细胞作用。流式细胞仪检测细胞凋亡情况。实时定量PCR 检测凋亡相关基因Bcl-2、Bax、Fas和Fas-L表达情况。 结果 患者上清可诱导足细胞凋亡，其凋亡率显著高于健康上清组和对照组[（28.5±5.9）%比（22.5±5.8）%、（20.5±4.5）%， 均P < 0.05]。患者上清可诱导足细胞Fas mRNA 升高，为对照组的1.89倍（P < 0.05）， 而Bcl-2 mRNA下调为对照组的72%（P < 0.05）。患者上清组的AngⅡ和TGF-β1水平均高于健康上清组[（13.2±3.4） ng/L比（8.2±2.3） ng/L，P < 0.05；（15.4±3.4） ng/L比（10.8±3.2） ng/L，P < 0.05]。 结论 IgAN患者血清IgA1与系膜细胞共培养上清可诱导足细胞凋亡，可能参与IgAN的进展。     

儿童无症状尿检异常IgA肾病的临床病理和预后分析

目的 探讨儿童无症状尿检异常的IgA肾病的临床病理特征和预后。 方法 对54例IgA肾病儿童的临床和病理特征进行分析。根据起病时有无临床症状分为无症状尿检异常组和有症状肾炎组。组织病理学分级参照Lee氏和Katafuchi氏半定量积分法。 结果 无症状尿检异常组18例，有症状肾炎组36例。有症状肾炎组尿蛋白量（24 h）明显高于无症状尿检异常组[（2.3±2.2） g比（0.4±0.3） g，P < 0.05]。无症状尿检异常的IgA肾病儿童表现为镜下血尿者，87％有尿微量白蛋白增高。无症状尿检异常IgA肾病患儿病理表现以Lee 氏Ⅰ~Ⅱ级为主，2例表现为Lee氏Ⅳ~Ⅴ级和 5例发生Katafuchi Ⅱ~Ⅲ级肾小管间质病变。有症状肾炎组Lee氏病理分级以Ⅱ~Ⅲ级为主，两者病理分级分布差异无统计学意义（P > 0.05）。全组患儿平均随访（26.9±8.8）月后，1例病理为Lee 氏Ⅴ级患儿进入终末期肾衰竭，其余患儿Scr均无升高1倍以上。 结论 无症状尿检异常的儿童IgA肾病虽临床症状轻微，但可出现病理损害严重的病例，并影响其预后。     

Acute poststreptococcal glomerulonephritis superimposed on IgA nephropathy

Superimposition of poststreptococcal glomerulonephritis (PSGN) on the course of IgA nephropathy (IgAN) is uncommon. A case of PSGN during IgA nephropathy is presented. A 30-year-old man who had alternating gross and microscopic hematuria for 7 months underwent a renal biopsy. The first renal biopsy revealed IgAN with mesangial deposits of IgA and C3. Two months later, the patient suffered generalized edema, proteinuria, hematuria, an increased ASO titer and a decreased C3 level. A second renal biopsy revealed diffuse endocapillary proliferative glomerulonephritis with epimembranous hump-like electron-dense deposits of C3, but the original mesangial IgA deposits had disappeared. A diagnosis of acute PSGN was indicated. Two months after the onset of acute nephritic syndrome, the patient remained asymptomatic, except for microscopic hematuria and proteinuria. Some cases with persistent proteinuria or hematuria after PSGN are probably related to preexisting IgAN.     

Hematuria and proteinuria in a mass school urine screening test

A total of 1,044 school children identified with hematuria and/or proteinuria during a mass school urine screening test were referred to pediatric nephrologists at 13 hospitals in Korea. These children had isolated hematuria (IH) (60.1%), isolated proteinuria (IP) (26.4%: transient, 19.6%; orthostatic, 4.9%; persistent, 1.9%) or combined hematuria and proteinuria (CHP) (13.5%). The patients history, physical examination, laboratory tests, kidney ultrasound and Doppler ultrasonography were obtained. Renal biopsies were performed on 113 children who showed severe proteinuria, hypertension, abnormal renal function, family history of chronic renal disease, systemic diseases or persistent hematuria and/or proteinuria for more than 12 months. IgA nephropathy (IgAN), thin basement membrane nephropathy (TBMN), membranoproliferative glomerulonephritis (MPGN), focal segmental glomerulosclerosis (FSGS), other GN, Alport syndrome and lupus nephritis were detected. IgAN and TBMN were the most common causes in the CHP group and IH group, respectively. Abnormal findings on the renal ultrasound with or without Doppler ultrasonography were noted in 147 cases (suspected nutcracker phenomenon, 65; increased parenchymal echogenicity, 40; hydronephrosis, 15). This study showed that the use of a mass school urine screening program can detect chronic renal disease in its early stage and recommends that more attention should be paid to identifying those children with CHP and massive proteinuria. A school urine screening program can detect chronic renal disease in its early stage. When mass screening is used, the initial aggressive diagnostic procedures such as renal biopsy are not needed. In addition, a regular follow-up for those children with IH and IP is certainly warranted.     

固本通络冲剂治疗IgA肾病的疗效特点分析

目的：研究中药复方固本通络冲剂治疗IgA肾病的疗效特点。方法：对肾活检证实的IgA肾病息随机分为固本通络冲剂治疗组(50例)及潘生丁对照组(30例)进行疗效特点分析，疗程3个月。结果：其疗效特点为对病理损害轻，临床表现为持续镜下血尿伴少量蛋白尿，中医辨证为气阴两虚的病人疗效最好；而对病理损害重，临床表现为肾病综合征，中医辨证为脾肾气虚疗效差。结论：固本通络冲剂对临床表现为持续镜下血尿伴少量蛋白尿，中医辨证属气阴两虚更为适宜。     

Clinicopathological features of paediatric renal biopsies in Shanghai over a 31 year period

Aim: To identify the variations in paediatric renal biopsy pathology and clinicopathological features during the past 31 years. Methods: A retrospective analysis of paediatric renal biopsies performed at a single institution in Shanghai from January 1979 to December 2009 was conducted. Results: The major pathologies included minor glomerular abnormalities (MGA, 26.1%), IgA nephropathy (IgAN, 17%) and mesangial proliferative glomerulonephritis (MsPGN) without IgA deposition (11.3%). The major clinical presentations included nephrotic syndrome (NS, 39.4%), haematuria with proteinuria (24.4%) and persistent microscopic haematuria (15.1%). MGA accounted for 46.9% of the cases in NS. IgAN and HSN accounted for 24% and 28.9% of patients with concomitant haematuria and proteinuria, and thin basement membrane nephropathy accounted for 51.2% of cases with persistent microscopic haematuria. The frequency of IgAN (78.6%) was much higher than that of TBMN (29.0%) in patients with persistent microscopic haematuria with abnormal urinary albumin. Conclusion: Minor glomerular abnormalities and IgAN were the major renal diseases in our study population, and the focus of our paediatric nephrologists. The high proportion of TBMN suggested that there should be limited use of renal biopsy for patients with persistent microscopic haematuria and renal biopsy should be performed in the presence of proteinuria or abnormal levels of urinary albumin.