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1.
IgA肾病(IgA nephropathy)是亚洲,特别是我国常见的原发性肾小球疾病。占全部肾脏活体组织检查(简称:肾活检)患者的30%~40%。它是临床上一组以免疫球蛋白IgA颗粒状或团块状沉积于肾小球系膜区以及毛细血管袢为特征的疾病。其病理改变复杂多样,轻至轻微病变肾小球疾病,重至硬化性肾小球肾炎不等,经常伴有严重的肾小管间质病变。临床表现多种多样,  相似文献   

2.
目的 研究肾组织中缺乏IgG沿肾小球毛细血管袢线样沉积的抗肾小球基底膜(GBM)病患者的临床、病理及预后特点。 方法 选取北京大学第一医院肾内科1991年至2008年确诊的抗GBM病患者93例,其中40例肾脏病理中冰冻切片直接免疫荧光检查缺乏IgG沿肾小球毛细血管袢沉积的患者为研究组(A组),53例肾组织中有IgG沿肾小球毛细血管袢线样沉积的经典抗GBM病患者为对照组(B组),比较两组患者的临床、病理和预后的差异。 结果 两组患者在性别、年龄、咯血、少尿或无尿和肉眼血尿的发生率、蛋白尿水平、贫血程度、循环中抗GBM抗体百分结合率、ANCA阳性率、肾脏病理肾小球中新月体的比例及组成成分、患者的生存率以及肾脏预后等方面,差异均无统计学意义(均P > 0.05)。肾组织中缺乏IgG沉积的患者,从起病到确诊所需的时间较长(68 d比36 d,P = 0.013);确诊时的血肌酐水平较低(716.0 μmol/L比896.8 μmol/L,P = 0.027)。其中4例患者肾组织石蜡切片行直接免疫荧光检查可以见到IgG沿GBM呈线样沉积。 结论 肾脏病理冰冻切片直接免疫荧光缺乏IgG线样沉积的抗GBM病患者,与经典抗GBM病患者相比,肾脏病变进展较慢,但其他临床及病理表现并无显著差异,肾脏预后及患者存活率亦无显著差异。因此,临床上应尽早检测血清抗GBM抗体,早期给予血浆置换治疗,以改善预后。  相似文献   

3.
IgA肾病(IgA nephropathy,IgAN)是我国最常见的原发性肾小球疾病,指IgA或以IgA为主的免疫球蛋白弥漫沉积在肾小球系膜区及毛细血管袢引起的一系列临床症状及病理改  相似文献   

4.
一、概述 膜性肾病(membranous nephropathy,MN)是病理形态学诊断名词,为肾病综合征常见的病理类型。MN又称膜性肾小球肾炎、膜上性肾小球肾炎、膜外性肾小球肾炎,也称为膜周性肾小球肾炎。Gluck等描述MN的病理特点是:肾小球毛细血管袢基底膜上皮侧有弥漫、均匀的颗粒状的免疫复合物沉积,一般不伴肾小球固有细胞增殖和局部炎症反应。  相似文献   

5.
原发性IgA肾病(IgA nephropathy,IgAN)首先由Berger在1968年报道,它不是单一疾病,而是一组以IgA为主的免疫球蛋白颗粒状弥漫沉积在肾小球系膜区及毛细血管袢的临床综合征。IgA肾病是目前国内外最常见的原发性肾小球肾炎,该病占原发性肾小球疾病的10%~30%,亚洲国家如中国、日本、  相似文献   

6.
2型糖尿病患者合并非糖尿病性肾损害的临床病理分析   总被引:44,自引:5,他引:39  
目的:了解2型糖尿病合并非糖尿病性肾损害的临床病理特点。方法:总结分析29例2型糖尿病合并非糖尿病肾损害的临床资料、病理改变及治疗反应。结果:2型糖尿病或糖尿病肾病可以合并多种非糖尿病肾损害,以各种类型的原发性及继发性肾小球疾病为主。原发性肾小球疾病常见病理类型有轻度系膜增生性肾小球肾炎、膜性肾病、IgA肾病和微小病变。这些患者具有以下不同于典型糖尿病肾病的特点:(1)糖尿病病程短于5年;(2)大量蛋白尿或肾功能不全时血压正常;(3)急性肾功能衰竭;(4)血尿明显。大部分肾病水平蛋白尿患者经糖皮质激素或糖皮质激素联合细胞毒类药物治疗后可完全缓解.结论:(1)2型糖尿病合并肾损害不等于糖尿病肾病;(2)2型糖尿病可以合并各种非糖尿病性肾损害;(3)当2型糖尿病伴肾脏受累者具有上述不符合糖尿病肾病特征时,应尽早行肾活检明确诊断;(4)在充分考虑患者 的临床特点、病理改变、严格控制血糖及血压的情况下,糖皮质激素或糖皮质激素联合细胞毒类药物治疗是安全有效的,可以改变患者的预后。  相似文献   

7.
目的 探讨以弥漫性毛细血管内皮细胞增生为主要病理表现的紫癜性肾炎(DEP-HSPN)的临床、病理及预后。 方法 回顾性分析本院近10年来经肾活检确诊的8例DEP-HSPN患儿临床、病理和预后资料,并分别与同病理级别或具有同等蛋白尿水平(肾病水平蛋白尿)的非DEP-HSPN患儿进行比较。 结果 (1)DEP-HSPN起病急,临床表现重,8例患儿中,4例临床表现为肾炎性肾病,3例表现为肾病水平蛋白尿伴血尿,1例呈急性肾炎综合征,4例患儿合并有肉眼血尿。病理分级均为Ⅲ-b级,光镜主要表现为弥漫性毛细血管内皮细胞和系膜细胞增生,常合并毛细血管袢坏死及肾小球内炎性细胞浸润,4例患儿合并细胞性新月体。(2)与病理为Ⅲ-b级的非DEP-HSPN患儿比较,DEP-HSPN患儿病程较短,临床多见肉眼血尿,24 h尿蛋白量高,更多呈肾炎性肾病表现。病理上,DEP-HSPN肾小球毛细血管袢坏死更常见。与具有肾病水平蛋白尿的非DEP-HSPN患儿相比,DEP-HSPN合并新月体的比例较低。(3)8例患儿均采用口服泼尼松联合静脉滴注环磷酰胺(CTX)冲击,病程早期给予2个疗程甲泼尼龙冲击治疗方案。平均随防(7.00±2.20)月,1例临床痊愈,5例持续镜下血尿,2例微量蛋白尿及持续镜下血尿。两组患儿预后差异无统计学意义。 结论 DEP-HSPN起病较急,临床以大量蛋白尿或肾炎性肾病为主要表现,并且常合并肉眼血尿。病程早期给予积极的免疫抑制剂治疗常能取得较满意的近期疗效。  相似文献   

8.
儿童孤立性血尿207例病理分析   总被引:1,自引:0,他引:1  
目的:探讨小儿孤立性血尿的病理类型.方法:对207例符合孤立性血尿诊断标准的患儿行肾活检术,肾组织进行光镜、电镜及免疫荧光检查.结果:轻微病变72例(34.8%);正常肾小球61例(29.5%),其中局灶节段性肾小球透明样变性5例.IgA肾病36例(17.4%);薄基底膜肾病21例(10.1%),其中正常肾小球7例,伴轻微病变14例;系膜增生性肾小球肾炎13例(6.3%),其中伴薄基底膜肾病2例;局灶增生性肾炎4例(1.9%).结论:轻微病变与正常肾小球占第一位,IgA肾病是小儿表现为肉眼血尿的孤立性血尿的主要原因,但表现为孤立性血尿的IgA肾病病理变化相对较轻,为轻微病变或轻度系膜增生.所有病例均无新月体形成、肾小球硬化和小管间质受累.提示孤立性血尿患儿预后良好.  相似文献   

9.
汞中毒相关肾小球疾病的临床病理分析   总被引:6,自引:0,他引:6  
目的 了解汞中毒相关肾小球疾病患者的临床病理特点。 方法 回顾分析北京大学第一医院2005年1月至2010年5月经临床结合重金属检测诊断的汞中毒相关肾小球疾病患者7例的临床病理资料,包括接触含汞物质的特点、汞中毒及肾小球疾病的临床病理表现。 结果 所有患者均为女性,平均年龄(28.9±8.1)岁,均为接触含汞化妆品后5~8个月发病。发病时血汞27.0~98.0 μg/L,尿汞34.4~204.0 μg/L。初诊症状为水肿,出现不同程度的蛋白尿,血浆白蛋白下降,5例(5/7)达肾病综合征诊断标准。6例肾组织活检病理结果提示,3例为微小病变肾病,2例为膜性肾病,1例局灶节段性肾小球硬化症。全部患者接受驱汞治疗3~7个疗程,血、尿汞下降,尿蛋白于治疗后3~5周内转阴。 结论 本研究汞中毒相关肾小球疾病患者均为接触化妆品致病,出现轻度水肿及不同程度的蛋白尿,大部分为肾病综合征范围。肾脏病理为微小病变肾病、膜性肾病及局灶节段性肾小球硬化症。驱汞治疗后可获临床完全缓解。  相似文献   

10.
98例特发性局灶节段性肾小球硬化的临床病理分析   总被引:1,自引:0,他引:1  
目的:探讨温州地区特发性局灶节段性肾小球硬化(FSGS)的临床病理及流行病学特点。方法:对1993年2月~2007年9月间经我院肾内科病理室诊断的98例特发性FSGS进行回顾性总结,分析其临床表现、肾活检组织形态学及流行病学特点,进行临床与病理联系分析。结果:(1)98例特发性FSGS以20岁~45岁为发病高峰年龄(占59.1%),占同期原发性肾小球疾病的3.6%及原发性肾病综合征的4.3%。(2)临床表现以肾病综合征(NS)最常见,占43例(43.9%),发病时常并发高血压(49.0%)和慢性肾衰竭(35.7%)。肾衰竭组的肾病范围蛋白尿发生率、高血压的发生率及血尿酸水平明显高于肾功能正常组(P〈0.05)。(3)主要病理特征:76.5%患者伴有不同程度的肾小球球性硬化,其中球性硬化比例≥25%者占36.7%;82.7%患者伴不同程度的慢性肾小管间质病变,其中Ⅱ~Ⅲ级占15.3%。肾衰竭者Ⅱ~Ⅲ级肾小管间质病变比例较肾功能正常者高(28.6%vs10.0%,P〈0.05)。(4)肾小球球性硬化的比例与血清肌酐值、病程及年龄呈正相关(P〈0.05),肾小管间质病变程度与Ccr呈负相关(P〈0.05)。球性硬化比例与肾小管间质病变程度呈正相关(P〈0.01)。结论:特发性FSGS在占本地区同期肾活检原发性肾小球疾病的3.6%,该病常并发高血压和慢性肾衰竭,病理上常见明显的肾小球球性硬化及慢性肾小管间质损害,慢性肾小管间质病变是影响患者预后的重要因素。  相似文献   

11.
Aim: Identification of glomerulomegaly is a prerequisite for diagnosis of obesity‐related glomerulopathy, so measurement of glomerular size is of critical importance. Methods: A total 100 cases pathologically diagnosed as minor glomerular abnormalities or thin basement membrane nephropathy with normal body mass index and blood glucose level were selected as the normal value measurement group of glomerular size. The mean value of diameters of capillary tuft on the glomerular maximum profile was determined using the direct method and indirect method with the Motic Med 6.0 digital medical image analysis system. Meanwhile, 80 cases of different glomerular disease with normal body mass index and blood glucose level were also collected. Their glomerular diameters were measured and compared with those in the normal value measurement group. Results: The measurement results showed that gender and age had no effects on glomerular diameter. The normal value ranges of the diameter on glomerular maximum profile were as follows. (i) Pole‐containing glomerulus (the glomerulus with vascular pole or/and urinary pole): direct method, 101.3–184.9 µm; indirect method, 100.3–183.5 µm. (ii) Pole‐containing glomerulus plus non‐pole glomerulus (the glomerulus without poles, the maximum profile of which was larger than that in the smallest pole‐containing glomerulus): direct method, 108.3–185.9 µm; indirect method, 107.4–185.4 µm. The glomerular diameters of the 80 cases with different glomerular disease were all within the aforementioned normal value ranges. Conclusions: Both methods used in the present study are feasible to measure the glomerular diameter and the normal value range of glomerular diameter in Chinese adults is established.  相似文献   

12.
Glomerular T-lymphocyte infiltration has recently been demonstrated to precede glomerular macrophage influx in a pre-immunized model of anti-glomerular basement-membrane antibody-induced glomerulonephritis (antiGBM-GN). In the current study, the functional role of these glomerular T-lymphocytes in directing macrophage localization was sought by measuring their production of macrophage migration inhibition factor (MIF). MIF activity in supernatants from cultured isolated glomeruli was measured in a conventional capillary tube bioassay. Glomerular T-lymphocytes (OX19 positive cells) were maximal (1.95 +/- 0.19 cells/glomerular cross section, c/gcs) 24 hours after injection of antiGBM antibody into sensitized animals. Seventy-two hours after antibody injection, T-lymphocyte numbers were reduced (1.02 +/- 0.14 c/gcs) while macrophage accumulation was maximal (at 24 hrs 4.2 +/- 1.3 macrophages/glomerulus (m/g), at 72 hrs 19.8 +/- 3.7 m/g). MIF activity was only detected in supernatants from T-lymphocyte infiltrated glomeruli (12 hrs 40.81 +/- 4.32% migration inhibition, 24 hrs 45.11 +/- 4.11% migration inhibition, 48 hrs 38.24 +/- 3.53% migration inhibition, 72 hrs 20.86 +/- 3.85% migration inhibition, all P less than 0.05). Control glomeruli from normal animals, pre-immunized animals given normal sheep globulin, pre-immunized animals given anti-GBM antibody and Cyclosporin A, and non-pre-immunized animals given antiGBM antibody did not contain glomerular T-lymphocytes, and their supernatants contained no MIF activity. This data indicates that the glomerular T-lymphocytes in pre-immunized antiGBM-GN are sensitized cells which release MIF and thus may direct glomerular macrophage localization in this model of antibody-induced glomerulonephritis.  相似文献   

13.
Glomerular size has been the subject of many studies and, in a number of settings, has a direct association with the development of glomerular sclerosis. However, the normal distribution of glomerular size has not been thoroughly evaluated in the general population in the United States. To address this issue, we analyzed the baseline biopsy specimens of 103 human donor kidneys to determine the maximal planar area (MPA) of the glomerular tuft in a heterogeneous human population. The MPA of each glomerulus was determined by measurement of sections through the vascular pole and/or origin of the proximal tubule, and was determined on each section by two methods: point counting and computer planimetry. There was very high agreement between these two methods. Multivariate analysis was used to identify significant correlates with MPA. Overall, younger donors had smaller glomeruli (P < 0.0001). Black donors had a larger MPA (23.4+/-8.6 mm2 x 10(-3)) than white donors (17.9+/-6.7 mm2 x 10(-3); P < 0.001), independent of donor age. MPA was not significantly different between genders. This heterogeneity in glomerular size may confound clinical studies if not recognized and may help explain differences in glomerular structure and function in response to injurious processes.  相似文献   

14.
Ham WS  Jeong HJ  Han SW  Kim JH  Kim DK 《The Journal of urology》2004,172(3):1108-1110
PURPOSE: Increasing clinical importance is being placed on the role of differential renal function (DRF) in the management of congenital ureteropelvic junction obstruction. Supranormal DRF of the hydronephrotic kidney on renal scan is a puzzling phenomenon and is hypothesized to be due to an increase in single nephron filtration or nephron volume without sound evidence. We studied the histopathological changes of hydronephrotic kidneys to determine whether glomerular hypertrophy underlies supranormal DRF. MATERIALS AND METHODS: We retrospectively evaluated the records of 3 females and 32 males with unilateral congenital hydronephrosis who underwent pyeloplasty. Mean patient age at operation was 12.6 months (range 0.1 to 144). Needle biopsies from 3 different sites at the lower pole of the kidney were performed during surgery. To evaluate the presence of glomerular hypertrophy, the maximal planar area of glomeruli was measured under light microscopy using an image analyzer. Tissue samples obtained from kidneys without a history of urinary tract disease at autopsy were used as controls. The mean glomerular areas of the patient and control groups were evaluated according to DRF and age. RESULTS: The mean glomerular area values of the patient group were smaller than those of the control group, except for 4 patients. The glomerular areas of the hydronephrotic kidneys with supranormal DRF were not significantly different from those of the control group. Instead, the probability of larger renal glomeruli increased with decreasing DRF (p = 0.1155). CONCLUSIONS: Increased nephron volume can be discounted as a cause of supranormal DRF.  相似文献   

15.
BACKGROUND: Various methods have been used to determine mean glomerular volume, some requiring measurement of over 30 glomerular profiles for a satisfactory estimate. Needle biopsies are useful diagnostically, but if small, provide insufficient tissue for the use of such methods. METHODS: We performed glomerular volume measurements on renal biopsies from 10 normotensive, non-uraemic patients with Type 1 diabetes. Sections were taken at 10 microm intervals through 10 glomeruli per biopsy and points landing on glomerular tuft counted under light microscopy. Volume was calculated from the measured cross-sectional area and known section thickness using the Cavalieri principle. RESULTS: Estimating the volume of 10 glomeruli per biopsy gave an overall mean glomerular volume of 4.21x10(6) microm(3) and standard deviation between patient means 1.23x10(6) microm(3.) Using a sample size of five glomeruli per biopsy only increased the standard deviation between patient mean values by 3%. Using sections taken at 20 microm intervals made little difference to the mean glomerular volume and standard deviation estimates (MGV 4.20x10(6) microm(3)+/-1.24). Further increases in the sectioning interval resulted in an appreciable increase in the variance of the estimate. CONCLUSIONS: The results suggest that a satisfactory estimate of mean glomerular volume can be obtained from a sample size of five glomeruli per biopsy using a sectioning interval of 20 microm. This represents a great saving in analysis time and effort, making widespread use of this method of glomerular volume measurement in renal disease more practicable, in both research and clinical settings.  相似文献   

16.
Estimation of glomerular volume: a comparison of four methods.   总被引:13,自引:0,他引:13  
Methods for estimating glomerular volume were compared in Zenker-fixed, paraffin-embedded biopsies from 10 patients with insulin-dependent diabetes mellitus and 6 normal kidney donors. Two methods of measurement of individual glomerular volumes were used: the Cavalieri method (considered the "gold standard") and the maximal profile area (MPA) method. Also studied were the method of Weibel and Gomez and a method based on the disector principle; both estimate mean volume (VG). MPA and Cavalieri showed strong correlation (r = 0.93; P less than 0.001), although the MPA method consistently overestimated the true volume; six glomeruli were necessary for a reliable estimate of VG. The disector method did not correlate with VG determined by Cavalieri. Weibel-Gomez did correlate with Cavalieri (r = 0.68; P less than 0.05), but overestimated VG. At least 15 profiles were needed to provide a dependable estimate of VG by Weibel-Gomez. The Cavalieri, MPA, and Weibel-Gomez methods all can provide reliable estimates of VG, the latter two with appropriate correction factors. The individual glomerular volume methods, while more time consuming, provide information on variation and distribution of the glomerular population and are the methods of choice for studies of glomerular volume.  相似文献   

17.
BACKGROUND: To assess the effects of aging and gender on glomerular size and global glomerulosclerosis (GS) and evaluate the relationship between glomerular size and GS in normal Caucasian donor kidneys. METHODS: All baseline graft biopsies between 1990 and 1998 were reviewed and sections with tissues containing at least 15 glomeruli were selected for morphometric analyses using a Digital Imaging Analyzer. Glomerular volumes (GV) were measured using the maximal profile area (MPA) method. The frequency of glomeruli with totally sclerotic lesions representing degree of global GS was expressed as a percentage of total number of glomeruli counted. RESULTS: 102 donor specimens (M/F, 46/56; mean age, 37.4 +/- 17.3 yrs) were analyzed showing mean MPA of 27.8 +/- 6.6 (x 10(-3)mm2), mean GV of 3.6 +/- 1.2 (x 10(6)microm3) and mean GS% of 6 +/- 10%. GV was increased with increasing age (r2 = 0.32, p < 0.0001). There was a progressive increase in glomerular size between infancy and adolescence (2-18 years old). The rate of MPA growth over childhood appeared to be linear (n = 13, r2 = 0.66, p = 0.0004). However, MPA increased with age at a slower rate (n = 99, r2 = 0.66, p = 0.0004) in adults (excluding data for patients < 18 years) than in infancy and adolescence. There wasn't significant gender difference in GV. Age (r = 0.47, p < 0.0001) and MPA (r = 0.31, p < 0.05) were both positively correlated with global GS. CONCLUSIONS: 1. Aging contributes to enlargement of glomerular size and global GS. 2. Gender had no significant effect on glomerular size. 3. Enlargement of glomerular size was associated with global GS in normal Caucasians.  相似文献   

18.
The most reliable method for estimation of mean glomerular volume (MGV), the disector/Cavalieri method, is technically demanding and time consuming. Other methods suffer either from a lack of precise correlation with the gold standard or from the need for a large number of glomeruli in the sample. Here, a new method (the 2-profile method) is described; it provides a reliable estimate of MGV by measuring the profile area of glomeruli in two arbitrary parallel sections. MGV was estimated in renal biopsies from 16 diabetic patients and 13 normal subjects using both the Cavalieri and the 2-profile methods. The range of individual glomerular volumes based on the Cavalieri measurements was 0.31 to 4.02 x10(6) micro m(3). There was a high correlation between the two methods for MGV (r = 0.97; P < 0.0001). However, the 2-profile method systematically overestimated MGV (P = 0.0005, paired t test). This overestimation was corrected by introducing a multiplication factor of 0.91, after which statistical criteria of interchangeability with the Cavalieri method were met. The optimal distance between two sections was determined as 20 micro m with a coefficient of variation of 7.4% in repeated measurements of MGV. On the basis of findings that values for MGV stabilize after ten glomeruli are measured by the disector/Cavalieri method, it was determined that the accuracy of MGV by the 2-profile method obtained by eight glomeruli was less than 7% different from ten in all cases. Thus, the 2-profile method is a practical alternative to the disector/Cavalieri method for estimating MGV, especially in small samples and blocks with limited residual tissue.  相似文献   

19.
BACKGROUND: Eicosanoids are believed to play a role in the pathophysiology of several models of glomerular disease. The cyclooxygenase inhibitor indomethacin reduces proteinuria in patients with focal segmental glomerulosclerosis (FSGS) or other glomerular diseases. We have shown that sera of some patients with FSGS significantly increase glomerular albumin permeability (Palb) in an in vitro assay. METHODS: To determine the role of eicosanoids in the increased Palb caused by the FSGS factor, glomeruli were isolated from normal rats, preincubated with indomethacin, then incubated with FSGS serum or normal serum and Palb was calculated. To study the direct effect of individual eicosanoids on Palb, glomeruli were incubated with prostaglandin E2, prostaglandin F2alpha or a thromboxane A2 mimetic, and Palb was calculated. In the final set of experiments, normal glomeruli were preincubated with the thromboxane synthase inhibitor furegrelate, incubated with FSGS serum, and Palb was calculated. RESULTS: Preincubation of isolated glomeruli with either the cyclooxygenase inhibitor indomethacin or the thromboxane synthase inhibitor furegrelate protected glomeruli from the increase in Palb caused by FSGS serum. Each of the three principal glomerular eicosanoids significantly increased Palb of isolated glomeruli. CONCLUSIONS: These studies implicate a product of the cyclooxygenase pathway of arachidonic acid metabolism as mediating the increased Palb caused by FSGS serum in our in vitro assay and possibly the proteinuria seen in patients with FSGS.  相似文献   

20.
The purpose of the present study was to evaluate the correlations among expression of intercellular adhesion molecule 1 (ICAM-1) in glomeruli, levels of soluble ICAM-1 (sICAM-1) in sera, and renal injuries in patients with IgA nephropathy. The levels of sICAM-1 in sera from 27 patients with IgA nephropathy and 7 healthy controls were measured by the human soluble ICAM-1 immunoassay. The expression of ICAM-1 in glomeruli was detected by indirect immunofluorescence. We observed marked expression of ICAM-1 in glomerular capillary walls and mesangial areas in patients with advanced-stage, but not in those with mild IgA nephropathy. Since the histopathological changes in the advanced stage of this disease were characterized by diffuse mesangial cell proliferation and tubulointerstitial injury, the expression of ICAM-1 in the glomeruli may be of value in evaluating the degree of renal lesions in patients with IgA nephropathy. However, there was no significant change in the levels of serum sICAM-1 among mild-stage and advanced-stage patients and healthy controls. It appears that the measurement of serum sICAM-1 is not useful in evaluating the degree of renal injuries in patients with IgA nephropathy.  相似文献   

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