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1.
Postoperative endoscopic examination of the porta hepatis through an exteriorized enterostomy was undertaken in 19 children with biliary atresia (BA). Endoscopic findings were classified into three types: (1) ductal (D) type with distinct bile-duct orifice and good bile drainage; (2) oozing (O) type without distinct bile-duct orifice but good bile flow; and (3) covered (C) type with no bile-duct orifice and no bile discharge. The postoperative course of the D-type patients was excellent with immediate disappearance of jaundice and good liver function. In the O type, all but 1 patient became jaundice-free, but the duration of disappearance of jaundice after the operation was significantly longer than for the D type (P <0.05). All of the patients with type C died within 3 years after portoenterostomy. Histologic studies of the extrahepatic biliary remnant showed no significant difference in the area of the largest bile-duct lumen or the total area of all bile duct openings among the three types. The age at operation was youngest in type D, and there was a significant difference between types D and C (P <0.05). Endoscopic examination of the porta hepatis reaffirmed the importance of early diagnosis and early operation in the treatment of BA. Type D may have an excellent prognosis while type C may require liver transplantation. Correspondence to: S. Matsuo at his present address  相似文献   

2.
胆小管增生诱导胆道闭锁早期肝纤维化   总被引:6,自引:0,他引:6  
目的观察门管区胆小管增生与肝纤维化的关系,进而探讨胆小管增生对胆道闭锁病程的作用和意义。方法选取在1997-2003年间经我科治疗的胆道闭锁(17例)和婴儿肝炎综合征(19例)患儿。术中肝活检标本石蜡切片行常规HE染色和免疫组化染色(Cytokeratin-7,Ki67),观察手术时年龄、肝纤维化、胆管增生、胆管增生活性和术前直接胆红素水平。结果在胆道闭锁组,不同肝纤维化级别的胆小管增生级别有相关关系(rs=0.561,P=0.019)。结论大量的胆管迅速增生,诱导产生大量的基质沉积于门管区,可能就是胆道闭锁患儿肝纤维化迅速进展的主要原因。  相似文献   

3.
目的 总结胆管闭锁术后肝内胆管囊性扩张的诊治经验,分析其对患儿预后的影响.方法 1998年6月至2008年3月,对胆管闭锁行葛西手术的患儿进行追踪随访.通过超声检查,发现8例患儿存在肝内胆管扩张.其中3例再行MRI检查,6例再行CT平扫加增强检杳以明确诊断.此8例患儿术后均有不同程度胆管炎症状.5例行PTCD显示肝内胆管囊性扩张.3例患儿行囊肿与空肠胆支再吻合术,2例患儿仪行PTCD置管引流,2例患儿暂未予任何处理.结果 3例经手术治疗的患儿,术后黄疸消退或减轻.仅行PTCD的患儿肝内胆管扩张长期存在.结论 对反复发作的胆管炎应定期行超声检查,尽早发现肝内囊肿;对肝内胆管囊性扩张,无论其影像学分型如何,应根据其具体临床表现进行相应的积极治疗;PTCD无法长期放置,仅是暂时性的治疗,但对手术时寻找囊肿有指示作用,使手术时囊肿的定位相对容易;囊肿的大小、位置与门静脉的关系,压迫的时间、治疗是否及时有效都可影响患儿的预后.  相似文献   

4.
目的 探讨胆道闭锁术中胆汁流量及成分变化与预后的关系.方法 本组对27例BA患儿行Kasai手术时,采用5 ml气体采样管收集和测量从肝门流出的胆汁.以17例胆总管囊肿患儿术中收集的胆汁作为对照组,其中测胆汁流量者7例.采用全自动生化仪检测胆汁中磷脂、总蛋白(TP)、胆汁酸(TBA)、总胆红素(TBIL)、直接胆红素(DBIL)、总胆固醇(TC)、谷草转氨酶(AST)、谷丙转氨酶(ALT)、谷氨酰胺转肽酶(GGT)、碱性磷酸酶(ALP)的浓度.术后随访1~6个月,随访内容有黄疸消退时间、术后血生化、术后胆管炎发生情况.结果 BA组胆汁流量显著低于对照组(P<0.05).手术日龄>60 d的BA患儿及术前血ALT>150 U/L的BA患儿术中胆汁流量均显著低于对照组(P<0.05).术中胆汁流量≥0.19 ml/min的BA患儿术后3个月退黄率显著高于术中胆汁流量<0.19 ml/min的BA患儿(P<0.05).BA组的胆汁磷脂、TBA及DBIL浓度均显著低于对照组(P<0.05);BA组的胆汁ALT浓度显著高于对照组(P<0.01).术后3个月黄疸消除者的胆汁AST及TC浓度均显著低于未消除者(P<0.05).结论 BA术中胆汁流量与预后有明显的关系,患儿术中胆汁流量越好,术后的预后越好,提示术中胆汁流量可以更早地预测患儿的远期效果.BA胆汁成分的变化说明BA患儿的肝功能较差,胆汁中磷脂、TBA和DBIL浓度较低可能是肝脏分泌功能较差的表现.胆汁中的AST和TC浓度与预后有关,胆汁AST和TC浓度较低的BA患儿术后预后较好,可能是由于肝功能较好和胆汁粘度较低利于引流所致.  相似文献   

5.
目的 探讨肝功能检查和门冬氨酸氨基转移酶/血小板指数(aspartate aminotransferase-to-platelet ratio index,APRI)与肝脏纤维化程度的关系,阐述其在BA肝纤维化评估中的临床价值.方法 收集2006年2月至2011年8月间在我院治疗的胆道闭锁患儿38例和胆汁淤积综合征患儿25例为研究对象.临床观察指标包括肝功能检查,肝脏活检切片,血小板指数;肝硬化程度采用Metavir分类,APRI的诊断性评估采用ROC曲线,应用SPSS 16.0统计学软件进行统计分析.并对本组患儿进行随访,随访时间是3~69个月(平均随访时间:20.7个月).结果 胆道闭锁组患儿ALP、γ-GT、DBIL(564.14±257.75、153.36±97.47、7.55±2.57)较胆汁淤积综合征组患儿存在明显升高(P<0.05);胆道闭锁肝硬化组患儿Age、ALT、AST、γ-GT(84.50±24.72、225.07±109.68、331.64±130.93、951.07±667.24)较非肝硬化组明显升高,两组差异具有统计学意义(P<0.05);胆汁淤积综合征肝纤维化组患儿Age、ALT、AST(84.76±14.28、159.92±61.76、238.15±62.60)较非肝纤维化组(54.17±11.17、98.92±58.08、151.17±41.44)明显升高,两组差异具有统计学意义(P<0.05).患儿绘制APRI的ROC曲线,用于判定肝硬化程度,胆道闭锁组敏感性为79%,特异性为88%;胆汁淤积综合征组敏感性为91%,特异性为79%.胆道闭锁中肝硬化组病死率显著高于非肝硬化组,且自体肝生存情况低于非肝硬化组.结论 肝功能检查可以作为胆道闭锁的初步判断指标,绘制APRI的ROC曲线对于评价胆道闭锁及胆汁淤积综合征患儿的肝脏纤维化情况均有较高准确性和可靠性,可用于预测预后和提早做好肝移植准备,因其简单、无创性可以在临床上广泛应用.  相似文献   

6.
目的 分析胆道闭锁( biliary atresia,BA)婴幼儿肝移植围术期各阶段内环境的变化特点,并探讨相应的液体管理措施.方法 2006年9月至2011年8月我院行胆道闭锁婴幼儿肝脏移植手术24例,围术期连续监测患儿血流动力学,定时采血检测酸碱平衡、乳酸、电解质、血糖等指标,记录液体治疗措施.结果 患儿术中血pH值及碱剩余(BE值)于无肝期即刻下降至术毕接近正常,分别为(7.38±0.09)、(-1.44±0.64),术后开始升高、至术后12h达峰,此后逐渐下降至正常;乳酸于无肝期开始升高(3.31 -±0.8) mmol/L,新肝期30 min达峰,术后6h明显下降,术后12h完全降至正常;血清钾浓度(K+)于新肝期后出现显著下降(3.21±0.52) re mol/L;血清钙浓度(Ca2+)自无肝期出现明显下降(1.03±0.12) m mol/L;血清钠浓度(Na+)于新肝期后出现逐渐升高趋势(141.2±2.9)mmol/l;血糖(BG)于无肝期后明显高于术前水平(7.4±2.8) mmol/L.术后患儿电解质及血糖水平有所波动,但与术后即刻比较无明显变化.24例患儿均顺利完成手术,围术期未发生脱水及心肺事件.术后ICU死亡4例,其余患儿均顺利回到病房.结论 胆道闭锁婴幼儿肝移植围术期内环境呈现规律性变化.术中液体治疗原则与一般腹部大手术有所不同;补液速度相似,但是液体比例以胶体液为主,占液体总量的50 %以上.术后即期液体治疗与普通外科手术相近.  相似文献   

7.
新生儿巨细胞病毒感染与胆道闭锁肝脏纤维化的相关研究   总被引:11,自引:1,他引:11  
目的探讨新生儿巨细胞病毒感染与胆道闭锁肝脏损伤的关系。方法回顾我院2004年1月-2005年1月收治21例胆道闭锁患儿临床资料。对肝组织纤维化和肝细胞变性坏死程度分级,同时进行肝脏和肝门纤维块巨细胞病毒-pp65免疫荧光染色。根据血清及病毒学检查结果将病人分组,比较两组患儿肝功能,肝脏纤维化,肝细胞破坏程度。结果巨细胞病毒感染组13例,非感染组8例。两组肝功能各指标除总胆红素,γ-谷氨酰转肽酶外无明显差异。巨细胞病毒感染组肝脏纤维化程度重于非感染组(P〈0.05)。结论巨细胞病毒活动性感染加重胆道闭锁患儿胆汁淤积和肝脏的纤维化。  相似文献   

8.
目的通过解剖胆道闭锁患者肝脏肝门组织,探讨Kasai术后肝门改变与肝内病理改变的差异,为改进Kasai手术提供理论依据。方法收集Kasai术后因胆汁淤积性肝硬化而行肝移植的胆道闭锁患儿13例,将其中术后胆汁引流情况较差者作为引流不良组,切取肝门及肝内组织。收集3例Kasai术后因肝门部囊肿,反复发作胆管炎而行肝移植的胆道闭锁患儿,3例术后胆汁引流较好,为引流良好组,自吻合口开始,沿左右肝管分别间隔1 cm连续取材。结果引流不良组:肝内病理改变以纤维化加重、胆管增生及血管增生为主,增生胆管直径较小,肝门淤胆程度较肝内相对重。引流良好组:靠近肝门部位胆管增生较肝内轻,并以大胆管为主。肝左叶胆管直径较大(最大750μm),具有引流功能;肝右叶胆管增生明显,胆管直径较小(最大390μm)。结论 Kasai术后胆汁引流较好的病例均呈现左叶胆管直径较大,可能提示解剖肝门广度需要增加。  相似文献   

9.
The aims of this study were to demonstrate the anatomic relationship between portal vein (PV) branches and hepatic ducts in the porta hepatis in individuals with preduodenal portal vein (PDPV) and to examine the validity of portoenterostomy (Kasai procedure) for patients with biliary atresia (BA) and PDPV. The porta hepatis of four subjects with PDPV without BA was dissected. Similar dissection supplemented by computer-aided three-dimensional reconstruction of the hilar structures was undertaken in a patient with BA and PDPV who underwent an unsuccessful Kasai procedure and died at 5 months of age. In three of the four subjects with PDPV alone, PV branches and extrahepatic bile ducts were abnormally arranged in the porta hepatis, some of the hepatic ducts entering the liver substance posterior, and occasionally lateral, to the main PV branches. The three-dimensional study showed that the original right main intrahepatic bile duct was located anterolateral to the portoenterostomy, which had been placed just anterior and to the right of the PV bifurcation. These findings strongly suggest that the PV may not be a reliable landmark for the Kasai procedure in patients with BA and PDPV.  相似文献   

10.
Situs inversus (SI) and the polysplenia syndrome (PS) occur relatively frequently in patients with biliary atresia, the largest subgroup of pediatric liver transplantation patients. We present two cases of orthotopic liver transplantation (OLT) in pediatric patients with SI. One had SI totalis, where a normal liver was placed in the left upper quadrant (LUQ) with the right lobe overlying the vertebral column. The second had PS and isolated SI of the liver, and a living-related left-lateral-segment graft was placed in the LUQ. Although multiple, often unpredictable vascular and intestinal anomalies occur frequently in association with SI, particularly in the setting of the PS, these cases, as well as several others recently reported, reveal that these anomalies can be managed successfully by a variety of technical modifications of the standard OLT technique. Likewise, concerns about the placement of a situs solitus liver in the midline or LUQ position of a SI abdomen have proved to be unfounded. Accepted: 24 June 1997  相似文献   

11.
目的综合分析胆道闭锁Kasai术后的超声图像,明确超声检查对于临床随访的应用价值。方法回顾性分析2014年3月至2015年9月在本院进行随访的胆道闭锁患儿超声检查结果,对部分阳性图像及参数与天冬氨酸转移酶-血小板比值(APRI)进行比较,根据黄疸消退情况进行分组,分析各组间超声随访指标的差异。。结果不均质肝脏的APRI[0.36(0.21~0.65)]明显高于均质肝脏[0.11(0.06~0.14)]和欠均质肝脏[0.19(0.12~0.32)],P0.05;包膜欠光整和包膜高低不平患儿的APRI[分别为0.33(0.18~0.58)]和0.44(0.27~0.67)],明显高于包膜光滑者[0.19(0.10~0.31)],P0.05;肝脏半定量评分与APRI之间呈正相关,并且有统计学意义(ρ=0.42,P0.05)。肝内胆管囊性扩张患儿APRI[0.46(0.35~0.65)]高于阴性患儿[0.23(0.13~0.42)],两者差异有统计学意义(P0.05)。术后时间6~12个月及≥12个月组中,肝静脉衰减指数(HVDI)与APRI之间均具有明显的正相关性(ρ分别为0.65及0.49,P0.05)。脾肿大患儿APRI[0.33(0.19~0.57)]高于脾脏大小正常患者[0.14(0.09~0.21)],P0.05。黄疸消退C组的肝脏评分[5(4~7)]明显高于A组和B组(P0.05);B组和C组的脾肋下长度[34.9(20~45)mm,35(23~40)mm]均明显大于A组[18(0~30mm)],P0.05。C组中的脾肿大、腹水阳性、单相波比例均明显大于A组(P0.05),C组的三相波比例明显小于A组(P0.05)。结论将肝实质回声、肝表面包膜进行半定量评分可用于评估肝纤维化和手术预后;肝内胆管囊状扩张者肝纤维化程度会更为严重;对于术后6个月以上的患儿,肝静脉呈单相波或波形平坦的双相波对于提示肝硬化具有参考价值;脾肿大(特别是脾肋下长度≥20 mm)和患儿的手术预后较差相关。  相似文献   

12.
目的研究胆道闭锁肝组织MMP-1、TIMP-11和TGF-β_1的表达与肝纤维化的关系,探讨胆道闭锁肝纤维化机制。方法利用免疫组化法(S-P法)检测33例胆道闭锁肝组织中MMP-1、TIMP-1、TGF-β_1蛋白的表达,通过图像分析技术进行定量研究。结果胆道闭锁肝组织中MMP-1、TIMP-1、TGF-β_1蛋白均有不同程度表达,以肝细胞胞浆、胞膜表达最为明显。TGF-β_1、TIMP-1蛋白在肝组织中的阳性表达随肝纤维化程度的加重而增强,有正的相关性;MMP-1蛋白在肝组织中的表达,随肝纤维化程度的加重无明显变化,无相关性;TGF-β_1蛋白表达与TIMP-1间有相关性,与MMP-1间无相关性。TGF-β_1、TIMP-1在胆道闭锁肝组织的表达强度明显高于对照组(P<0.01),MMP-1在两组间的表达差异无显著性意义(P>0.05)。结论MMP-1、TIMP-1、TGF-β_1与胆道闭锁肝纤维化的发生、发展有密切联系,TGF-β_1与TIMP-1有协同表达和调控作用,通过抑制肝组织TIMP-1、TGF-β_1蛋白表达,或促进MMP-1的表达,可成为抑制胆道闭锁肝纤维化的有效方法。  相似文献   

13.
目的 探讨胆道闭锁术中胆汁成分变化与预后的关系。方法 本组对62例BA患儿在Kasai术中收集从肝门流出的胆汁,以25例胆总管囊肿患儿术中收集的胆汁作为对照组。采用全自动生化仪检测胆汁中总蛋白(TP)、胆汁酸(TBA)、总胆红素(TBIL)、直接胆红素(DBIL)、总胆固醇(CHO)、谷草转氨酶(AST)、谷丙转氨酶(ALT)、谷氨酰胺转肽酶(GGT)、碱性磷酸酶(ALP)的浓度。术后随访2~16个月,随访内容有黄疸消退时间,术后血生化,术后胆管炎发生情况及术后1年存活情况。结果 BA组胆汁中ALT(210.13±127.75) U/L、GGT(319.03±206.73) U/L、TP(45.86±18.16)g/L、CHO (0.08±(0.04) mmol/L浓度显著高于对照组,ALT:(69.39±44.55) U/L; GGT:(73.22±99.29)U/L; TP:(21.78±15.36)g/L; CHO:(0.02±0.03) mmol/L(P<0.05); BA组胆汁中TBA( 189.81±107.81) μmol/L和DBIL (61.31±25.53 )μmol/L浓度显著低于对照组TBA:(735.06±199.75)μmol/L; DBIL:(90.43±27.18)μmol/L(P=0.000和P=0.035)。术前肝功能较好组胆汁中ALT(86.24±65.63) U/L、AST(801.64±516.50) U/L和GGT (205.82±112.25) U/L 浓度显著低于术前肝功能较差组,ALT:(244.67±175.69) U/L; AST:(1409.52±927.05) U/L;GGT:(362.05±235.57)U/L(P<0.05);术前肝功能较好组胆汁中TBA (346.04±279.33)μmol/L 浓度显著高于术前肝功能较差组(192.47±93.55)μmbl/L(P=0.029)。术后3个月黄疸消退者胆汁TBA显著高于未消退者(251.18±131.83)μmol/L比(121.43±80.42) μmol/L,(P=0.007);术后3个月黄疸消退者胆汁DBIL(72.08±32.51 )μmol/L显著高于未消退者(40.22±23.47) μmol/L (P=0.022)。术后1年存活者胆汁TBA浓度较术后1年死亡者显著增高(258.63±105.63)μmol/L比(110.63±46.08) μmol/L,P= 0.015);术后1年存活者胆汁ALT浓度较术后1年死亡者显著降低(8.72±8.83)U/L比(95.15±44.18)U/L,P=0.048。结论 BA术中胆汁成分与预后有明显的关系,胆汁中TBA较高、DBIL较高的BA患儿术后3个月黄疸消退较好;胆汁中TBA较高、ALT较低的BA患儿术后1年存活情况较好。胆汁TBA和ALT浓度可作为评价BA短期预后和中期预后的可靠指标。  相似文献   

14.
目的 探讨胆道闭锁Kasai术后的近中期疗效及其影响因素.方法 回顾性分析2005年10月至2010年9月在本院行Kasai手术并获得随访的152例胆道闭锁患儿,男68例,女84例,手术时的中位日龄为75 d(31~528 d),平均(82.36±45.17)d,定期门诊复诊并电话随访,平均随访时间(20.11±16.11)个月(2~62个月),对其诊疗过程及随访情况进行分析,对黄疸消退情况、生存率及影响因素采用Kaplan-Meier生存分析、Cox regression分析及x2检验.结果 Kasai术后黄疸消退率为50%(74/149),Ⅰ、Ⅱ、Ⅲ型患儿的黄疸消退率分别为67%、67%、47%,差异无统计学意义(P=0.306);≤60 d、60~≤90d、91~120 d手术组患儿的黄疸消退率分别为54%、53%、46%,≥120 d手术组患儿的黄疸消退率明显降低(25%),但差异无统计学意义(P=0.310);胆管炎发生率57%(85/149),有无胆管炎发作的黄疸消退率分别为31%、75%,差异有统计学意义(P<0.01).Kaplan-Meier生存分析Kasai术后2、4年的自体肝存活率分别为56%、49%,有胆管炎组和无胆管炎组的2年自体肝存活率分别为43%、77%,差异有统计学意义(P<0.01).结论 Kasai手术是目前我国治疗胆道闭锁的首要方法,手术年龄和分型与Kasai术后的近中期效果无明显相关,胆管炎是影响Kasai术后效果的重要因素.  相似文献   

15.
儿童终末期肝病(pediatric end-stage liver disease,PELD)评分系统是根据客观的实验室检查数据评估儿童肝脏疾病严重程度的模型,国际上用它来分配日益减少的供肝给儿童肝移植受者.对于Kasai手术效果不佳的胆道闭锁(biliary atresia,BA)患儿来说,肝移植成为唯一的选择.本文通过分析PELD评分系统与经典的临床肝功能Child分级、BA肝移植手术时机的选择、活体肝移植(living donor liver transplantation,LDLT)、肝纤维化、葛西手术(Kasai procedure,KP)以及肝移植手术预后之间可能存在的关联,旨在较全面的了解PELD评分在BA患儿肝移植中所发挥的作用,力求使其应用更加科学、合理.  相似文献   

16.
Background and purpose  Many post-operative patients with biliary atresia (BA) suffer from liver dysfunction, such as chronic inflammation even without jaundice after a Kasai’s hepatic portoenterostomy. Methods  The presence and degree of oxidative stress were evaluated in the post-operative patients with BA. Twelve outpatients who underwent a Kasai’s hepatic portoenterostomy were evaluated. The active oxygen products, the rate of bioantioxidant, the markers of oxidative stress, and the degree of hepatic oxidative stress were examined by immunohistochemical staining of biopsied specimens. Results  All of the oxidative stress markers in the post-operative patients with BA increased in comparison to those in the controls. Moreover, 8-OHdG immunohistochemical staining was positive in 84 ± 4.8% in hepatic cells in the portal area in the post-operative patients with BA. Conclusion  The post-operative patients with BA were under increased oxidative stress, even if their liver dysfunction was mild without jaundice. Antioxidant therapy might be necessary to decrease of oxidative stress in the post-operative patients with BA.  相似文献   

17.
胆道闭锁(biliary atresia)是以进行性肝内外胆管纤维化为特征的疾病,是导致婴幼儿终末期肝病的重要原因,目前病因及发病机制尚不明确.研究表明,胆汁酸可以用于胆道闭锁早期筛查、预后评估及胆管炎诊断.本文对近些年胆汁酸在胆道闭锁中的相关研究进展进行综述.  相似文献   

18.
Sixty-eight childrenwith biliary atresia (BA) were admitted to the Department of Pediatric Surgery, Kyushu University Faculty of Medicine and fukuoka Municipal Children's Hospital, from 1971 to 1986. In 11 (16.2%) there was a tendency toward bleeding associated with obstructive jaundice and breast feeding. In 4 of the 11 cases, vitamin K deficiency (VKD) was diagnosed by coagulation studies. The bleeding tendency improved after administrationn of vitamin K in all 4 cases; the other 7 were also suspected of having VKD. The usefulness of the Normotest [7] for the diagnosis of VKD and for mass screening of infantile hepatobiliary disorders, particularly BA, is discussed.  相似文献   

19.
We retrospectively investigated the use of periodic bile cultures and irrigation of the external jejunostomy for prevention or treatment of postoperative cholangitis in 11 infants who had undergone the Suruga II modification with external jejunostomy for biliary atresia. Periodic cultures obtained from bile in the external jejunostomy were done weekly in all patients. Staphylococci were first cultured 1 week after operation, followed by intestinal flora such as enterococci, Pseudomonas, Klebsiella, or Enterobacter. Most of the cultures revealed two or more microorganisms. Susceptibility testing indicated the effective antibiotics for the treatment of each case of cholangitis. Therefore, periodic bile cultures and irrigation of the external jejunostomy provide important information for prevention or treatment of postoperative cholangitis, especially in the early postoperative period.  相似文献   

20.
小分子核糖核酸(microRNA,miRNA,miR)是一类约22个核苷酸的单链非编码RNA,在转录后水平调节基因的表达.miRNA广泛存在于人体各个组织器官中,在细胞增殖、分化、凋亡、胚胎发育、信号传导、肿瘤发生及代谢等过程中发挥着重要作用.近年来研究表明,某些miR-NA表达的改变在胆道闭锁的发生发展中起着重要作用,在该过程中体现出极其复杂的调控机制,同时miRNA的研究也为胆道闭锁的早期诊断和治疗带来了新的希望和思路.本文就miRNA在胆道闭锁中的研究进展做一综述.  相似文献   

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