胆道闭锁的主要影像诊断方法及各自的优缺点

胆道闭锁（biliary atresia,BA）是肝内外胆管部分或全部闭锁,其中以肝外胆道闭锁常见。BA多见于母乳喂养的新生儿,出生时体质量正常,女性多于男性,其肝脏病理变化同一般的胆汁性肝硬化,如果治疗不及时,不可避免地会发展为肝硬化、肝衰竭甚至死亡。因此,早作出正确诊断,尽快进行手术,尤为重要。肝穿刺活检是诊断BA的金标准,其灵敏度、特异性和准确性均高于95%。     

胆道闭锁早期诊断的研究进展

胆道闭锁(BA)是以肝内外胆管进行性纤维化为特征的小儿外科常见严重疾病,如能在早期明确诊断并且施行手术可显著改善患儿预后,否则预后大多不良,因此胆道闭锁的早期诊断尤为重要。术中胆道造影是诊断BA的金标准,但是创伤性较大,目前仍然需要非损伤性的、特异性和敏感性高的指标来筛查诊断BA。本文将从血液、尿液来源的实验室标记物、粪便比色卡和超声检查进行综述。     

超声诊断胆道闭锁的研究进展

胆道闭锁（BA）是婴儿期持续性黄疸的主要原因,可快速发展为肝硬化而导致死亡。及时手术治疗可减少患儿的死亡率,故早期诊断及鉴别诊断尤为重要。BA的诊断主要依靠患儿的临床表现、肝穿刺活检及高频超声、磁共振胰胆管造影（MRCP）、核素肝胆显像等影像学方法,其中高频超声的敏感度及特异度均较高。本文对BA的超声影像学诊断及鉴别诊断进展进行综述。     

99mTc-EHIDA肝胆显像在小儿先天性胆道闭锁中的价值

先天性胆道闭锁(Biliary atresia,BA)是新生儿及婴儿持续性黄疸最常见的原因之一,如能早期诊断、及时手术治疗,能够提高患儿的生存率,改善患儿的生存质量,延期治疗则可能引起肝硬化和肝功能衰竭,甚至死亡.放射性核素肝胆显像和腹部B超是早期诊断BA的最常用的影像学检查方法.我们自2005年3月～2008年3月对32例持续性黄疸患儿行99mTc-EHIDA肝胆显像,现将结果报道如下.     

超声高频凸阵探头在婴儿胆道闭锁诊断中的价值

目的:应用高频凸阵探头对梗阻性黄疸婴儿进行检查,评价其对胆道闭锁(BA)患儿的诊断价值.方法:婴儿梗阻性黄疸患儿空腹4h后进行腹部高频超声检查,同时加彩色血流显像(CDFI)观察、存图,对经手术证实的超声诊断为BA的59例患儿的图像进行总结分析.结果:以术后病理检查结果为金标准,高频超声诊断59例BA中58正确,1例误诊.肝内外胆管未探及、无胆囊及胆囊形态异常、肝门部纤维块对BA诊断的敏感度分别为93.2％、88.1％、78.0％,3种征象结合判断敏感度达98.3％,肝门部孤立小囊出现率不高,但伴上述征象时其对BA诊断的准确率很高.肝动脉内径增宽、流速增快可用于判断BA患儿的肝损伤程度.结论:高频凸阵探头频率高、体积小、操作灵活,更清晰地显示肝门部的形态结构,特别是可对肝内外胆管直接显示,显著提高超声对婴儿BA诊断的准确性,优于以往间接通过胆囊及肝门部纤维块变化对BA的诊断.     

高频超声在胆道闭锁诊断中的应用价值

目的探讨高频超声在胆道闭锁(BA)早期诊断中的价值。方法回顾性分析2008年1月至2009年11月37例(行中频超声检查)及2009年12月至2010年12月16例(行高频超声检查)均经手术确诊的BA患儿的术前超声检查资料并进行比较。结果中频超声检查组中提示BA2例(2/37,5.4%);胆囊显示异常25例(25/37,67.6%),其中胆囊腔未显示22例(22/37,59.5%),壁或形态异常2例(2/37,5.4%),小胆囊2例(2/37,5.4%);胆囊腔可显示且未提示异常12例(12/37,32.4%);显示肝门异常高回声2例(2/37,5.4%)。高频超声检查组中,提示BA15例(15/16,93.8%),胆囊均显示异常,其中胆囊腔未显示3例(3/16,18.8%),胆囊壁或形态异常12例(12/16,75.0%),小胆囊1例(1/16,6.3%);胆囊腔可显示且未提示异常1例(1/16,6.3%);TC征(即肝门或左、右肝管汇合部团块状或带状高回声区)阳性14例(14/16,87.5%);提示肝动脉增宽15例(15/16,93.8%)。中频超声对BA的诊断符合率为5.4%(2/37),高频超声对BA的诊断符合率为(93.8%,15/16),两者之间的差异有统计学意义(χ2=40.1,P<0.01)。若将中频超声检查组的胆囊显示异常与肝门显示异常者也诊断为BA,则其诊断符合率(25/37,67.6%)与高频超声检查组的诊断符合率(15/16,93.8%)差异仍有统计学意义(χ2=4.1,P<0.05)。结论高频超声能更清晰地显示肝门结构和胆囊情况,大大提高了对小儿BA的早期诊断率。     

无创性影像学检查诊断胆道闭锁疾病

胆道闭锁(BA)是新生儿期特殊的疾病,需通过及时的外科手术阻止肝脏进行性损害,早期诊断此病尤为重要。无创性的影像学检查方法如超声、磁共振胰胆管成像、放射性核素肝胆动态显影均是临床诊断常用的方法。弹性成像新技术在诊断肝纤维化中有很好的应用价值。     

急性时相蛋白早期诊断新生儿败血症

新生儿败血症是新生儿期常见而死亡率较高的疾病.由于新生儿败血症症状缺乏特征性,诊断依赖血培养,但需时较长且易污染,不能早期诊断.近几年来国外特别是欧美各国和日本采用急性时相蛋白(Acute phase proteins)作为早期诊断新生儿败血症有效方法.急性时相蛋白是正常血清中蛋白成份,在肝内合成,当细菌感染时,机体除表现局部炎症反应外,这一类蛋白如C-反应蛋白、α_1-酸性糖蛋白、α_1-抗胰蛋白酶、触珠蛋白等在肝内合成显著增加,在血     

高频超声对婴儿胆道闭锁的鉴别诊断价值探讨

目的 应用高频超声对黄疸婴儿进行检查,评价其对胆道闭锁(BA)患儿的鉴别诊断价值.方法 43例黄疸患儿空腹4h后进行常规腹部超声和高频超声检查,记录每例患儿的肝脏、胆囊、胆管、肝右动脉、门静脉及TC (triangular cord)征的情况等,并初步诊断是否为胆道闭锁.同时收集患儿的各项化验结果,MRI、CT等影像资料,手术所见和肝穿组织学及术后病理报告.结果 以肝穿组织学及术后病理检查结果为金标准,高频超声正确诊断18例BA中的17例,并且25例非BA均未被误诊.TC征阳性,肝右动脉增宽,胆囊异常(无胆囊、胆囊壁异常或(和)形态异常)三者对诊断胆道闭锁具都有很高的价值.以TC征阳性诊断BA的敏感性,特异性及准确性分别为:88.9％、100％、95.3％;如以肝右动脉内径＞0.16 cm为诊断BA的理想截断值,其敏感性、特异性及准确性分别为:94％、84％、88.4％;以胆囊异常诊断BA的敏感性,特异性及准确性分别为:94％、100％、97.7％.综合考虑以上三者,诊断BA的敏感性、特异性及准确性分别为:94％、100％、97.7％.结论 高频超声通过清晰地显示肝门部的形态结构,显著提高超声对婴儿胆道闭锁诊断的准确性,其准确率为97.7％ (42/43).     

新生儿先天性梅毒早期诊断及防治的探讨

目的:探讨新生儿先天性梅毒早期诊断及防治的方法。方法:对28例产前孕母已诊断梅毒并治疗后娩出的新生儿与30例产后才诊断先天性梅毒的新生儿进行比较分析。结果:产前治疗组与产前未治疗组的新生儿在孕周、体重、皮肤损害、肝脾肿大、肺炎、胎盘异常等比较,差异有显著意义(P<0.001)。宫内规则治疗后,娩出的新生儿无症状,不治疗娩出的出现临床症状,不规则治疗、宫内治疗时间不足的可有可无症状。结论:对娩出时有皮肤损害、肝脾肿大、肺炎、早产、低出生体重儿、胎盘异常、窒息等新生儿应高度怀疑先天性梅毒,做梅毒确诊试验和母婴传播性疾病的检查。梅毒血清学检查阳性,均应治疗,青霉素治疗有效,规则治疗预后良好。建议孕期常规做梅毒筛查,使确诊者胎儿在宫内便进行早期驱梅治疗。     

胆道闭锁的超声诊断

目的：总结并分析20例胆道闭锁患儿的超声图像表现，探讨超声检查对胆道闭锁诊断的临床价值。方法：应用彩色多普勒超声仪观察和记录肝脏及脾脏大小、内部回声；空腹胆囊、餐后胆囊的变化；左右肝管汇合部纤维斑块存在与否。结果：10例左右肝管汇合部可见明显高回声斑块（厚径0-3～0．8cm）；3例高回声斑块较小（厚径0．2～0．26cm）。2例胆囊未显示；1例呈条索状，无囊腔；15例胆囊瘪小，胆囊发育及收缩不良；2例胆囊大小、形态正常，但餐后无收缩。全部病例肝脏不同程度增大及肝实质回声粗糙，9例腹腔存在少量积液，14例脾脏增大。结论：左右肝管汇合部纤维斑块具有特异性，可作为胆道闭锁超声诊断的客观指标，胆囊瘪小及收缩不良也具有重要的诊断和鉴别诊断意义。肝脏增大与肝脏回声增强、粗糙程度，及肝纤维化呈正比，可以提示病程长短和预后。     

彩色多普勒超声检侧不同程度肝纤维化胆道闭锁婴儿肝内血流动力学变化的价值

目的 运用彩色多普勒超声对不同程度肝纤维化胆道闭锁(BA)婴儿肝静脉(HV)、门静脉(PV)及肝固有动脉(HA)的血管内径、各项血流动力学指标进行检测,探讨各项指标诊断BA婴儿肝纤维化的价值.方法 选取22例确诊为BA的婴儿作为BA组,其中轻度肝纤维化(S1~S2期)9例,中度肝纤维化(S3期)7例,重度肝纤维化(S4期)6例;选取23例年龄、性别相匹配,排除肝脏、心肺病变的婴儿作为对照组.彩色多普勒超声观察两组婴儿血管频谱的形态,同时测量其肝静脉最大截面直径(HVD)、肝静脉最大流速(HVVmax)、肝静脉最小流速(HVVmin)、门静脉最大截面直径(PVD)、门静脉最大流速(PVVmax)、肝动脉最大截面直径(HAD)、肝动脉最大流速(HAVmax)、肝动脉平均流速(HAVtam)、肝动脉阻力指数(HARI),计算肝静脉衰减指数(HVDI)、肝动脉/门静脉最大流速比率(A/P).结果 BA组31.8%(7/22)的婴儿HV多普勒波形为HV0型,63.6%(14/22)为HV1型,4.5%(1/22)为HV2型;对照组39.1%(9/23)的婴儿HV多普勒波形为HV0型,47.8%(11/23)为HV1型,13.0%(3/23)为HV2型.BA组与对照组HV频谱形态差异无统计学意义(χ2=1.589,P＝0.452).BA组HV内径为(2.37±0.56)mm,与对照组的(2.58±0.53)mm比较差异无统计学意义(t=1.681,P>0.05);BA组HVDI为(0.79±0.07),较对照组的(0.55±0.20)高,差异有统计学意义(t=2.747,P<0.05).轻度、中度、重度肝纤维化BA婴儿HV内径分别为(2.52±0.61)、(2.24±0.69)、(2.30±0.30) mm,HVDI分别为(0.75±0.09)、(0.78±0.13)、(0.79±0.05);不同程度肝纤维化BA婴儿HV内径差异无统计学意义(P>0.05),而HVDI差异有统计学意义,且高度肝纤维化组HVDI高于轻度肝纤维化组(χ2=-2.401,P＝0.016),差异有统计学意义,其余不同程度肝纤维化BA婴儿之间比较差异无统计学意义(P>0.05).BA组PV内径为(3.93±0.76)mm,PVVmax为(23.6±8.0)cm/s,与对照组的(3.79±0.66)mm、(23.1±5.5)cm/s比较,差异均无统计学差异(t=0.659、0.685,P>0.05).轻度、中度、重度肝纤维化BA婴儿PV内径分别为(3.91±0.82)、(4.00±0.62)、(3.88±0.95) mm,PVVmax分别为(22.6±7.1)、(24.2±8.0)、(24.4±10.0)cm/s,不同程度肝纤维化BA婴儿PV内径、PVVmax差异均无统计学意义.BA组HA内径、HAVmax、HAVtam分别为(2.01±0.32)mm、(108.3±49.7)cm/s、(51.6±24.6)cm/s,高于对照组的(1.24±0.38)mm、(47.5±18.4)cm/s、(22.9±8.3)cm/s,差异均有统计学意义(t=8.908、5.181、5.018,P<0.05);BA组HARI为(0.81±0.06),与对照组的(0.77±0.06)比较差异无统计学意义(t=1.910,P>0.05);BA组A/P为(6.76±5.17),低于对照组的(2.26±0.95),差异有统计学意义(t=3.434,P<0.05).轻度、中度、重度肝纤维化BA婴儿HA内径分别为(2.00±0.82)、(91.84±0.27)、(2.22±0.38)mm,HAVmax分别为(114.4±48.1)、(98.1±16.9)、(111.2±78.1)cm/s,HAVtam分别为(56.4±27.7)、(48.9±16.9)、(47.5±29.9)cm/s,HARI分别为(0.78±0.06)、(0.80±0.06)、(0.86±0.04),A/P分别为(8.55±6.71)、(4.37±1.41)、(6.88±5.82),不同程度肝纤维化BA婴儿HA内径、HAVmax、HAVtam、A/P差异无统计学意义(χ2=0.999、0.602、1.175、1.233,P均>0.05),而HARI差异有统计学意义(χ2=7.891,P=0.019),且重度肝纤维化组HARI高于轻度肝纤维化组,差异有统计学意义(χ2=-2.911,P=0.004),其余不同程度肝纤维化BA婴儿之间比较差异无统计学意义(P>0.05),BA组的HA波形比对照组高、尖.结论 在BA婴儿中,HA内径增宽、流速增高对于肝纤维化具有诊断价值,HARI可反映肝纤维化严重程度;HVDI有助于肝纤维化的诊断;PV血流参数对于BA婴儿肝纤维化的诊断意义不大.     

Monitoring of hepatic artery resistance index and optimal timing of liver transplantation for biliary atresia

Purpose Biliary atresia (BA) is diagnosed by ultrasound (US) examination of the hepatic artery and gallbladder. This study was designed to assess the usefulness of the hepatic artery resistance index (HRI) measured by Doppler ultrasonography (DUS) for prognostication of liver viability in children with BA. Methods Seventeen patients with non-correctable BA were examined by US and DUS before and after hepatoenterostomy to evaluate hepatic artery dynamics. Results Dilatation of the hepatic artery was demonstrated in all 17 patients. US of the gallbladder showed hypogenesis in seven patients. Preoperatively, all 17 BA patients had an HRI of <1.0. Six of the 17 patients underwent liver transplantation. Four of the six had an HRI of >0.9, and the HRI decreased after the procedure. All patients were alive at the time of writing. Conclusions Regular US examinations are helpful for diagnosis of BA and for detecting patients at high risk. A prospective study is required to determine the optimal frequency of assessment.     

彩色多普勒超声在胆道闭锁患儿肝硬化诊断中的价值

目的探讨彩色多普勒超声在胆道闭锁患儿肝硬化诊断中的价值,寻找可靠的超声形态学与血流动力学参数。 方法选取2014年1月至2017年4月广州市妇女儿童医疗中心行胆道造影＋肝活检术或胆道造影＋Kasai手术＋肝活检术的胆道闭锁患儿93例。结合术中肝脏标本病理学检查结果,依据肝脏纤维化Ohkuma′s分级标准,将患儿分为肝硬化组（4级）21例和非肝硬化组（0~3级）72例。回顾性分析和比较2组患儿术前腹部彩色多普勒超声检查资料：肝实质回声、肝缘情况、肝表面、脾长径、肝动脉最大截面直径（HAD）、肝动脉峰值流速（HAVmax）、肝动脉阻力指数（HARI）、门静脉主干最大截面直径（PVD）,有无腹水、脐静脉有无重开情况。 结果21例肝硬化组患儿均表现为肝实质回声增粗增强、肝缘变钝、肝表面呈波浪状改变以及出现腹水,14例出现脐静脉重开,各超声征象所占比例均显著高于非肝硬化组,差异均有统计学意义（χ²=73.78、69.90、93.04、62.97、56.51,P均<0.01）;肝硬化组脾长径、HAD、HAVmax、HARI均显著高于非肝硬化组,差异有统计学意义（t=13.972、2.984、5.643、6.930,P均<0.01）;2组患儿PVD比较,差异无统计学意义（t=0.516,P>0.05）。 结论脾大、肝实质回声增强增粗、肝缘变钝、肝表面呈波浪状改变、肝动脉增宽,HARI、HAVmax增高,以及腹水、脐静脉重开对诊断胆道闭锁患儿肝硬化具有重要价值。     

超声漏诊婴儿胆道闭锁特征

目的 分析超声漏诊的婴儿胆道闭锁(BA)的特征。方法 回顾性分析72例超声漏诊BA婴儿(假阴性组,A组)、72例超声诊断正确BA(真阳性组,B组)及72例无BA胆汁淤积症婴儿(对照组,C组),对比其临床资料及超声特征;行logistic回归分析,探讨诊断及漏诊BA的影响因素。结果 A、C组间总胆红素、直接胆红素、γ-谷氨酰转移酶(GGT)及谷草转氨酶(GOT),以及B、C组间总胆红素、直接胆红素及GGT差异均有统计学意义(P均<0.05)。A、B组间小胆囊、胆囊壁僵硬、胆囊收缩功能差及肝门部纤维斑块发生率,A、C组间胆囊未探及或囊变、胆囊壁僵硬、胆囊收缩功能差、肝门部纤维斑块及肝门部囊肿发生率,以及B、C组间胆囊未探及或囊变、小胆囊、胆囊壁僵硬、胆囊壁增厚、胆囊收缩功能差及肝门部纤维斑块发生率差异均有统计学意义(P均<0.05)。3组间两两比较胆总管显影率差异均有统计学意义(P均<0.05)。肝门部纤维斑块及胆囊收缩功能差发生率均较低为A组漏诊BA,GGT更高、肝门部纤维斑块及胆囊收缩功能差发生率均更高为A组诊断BA的影响因素(P均<0.05)。结论 超声漏诊B...     

Concentrations of copper and zinc in liver and serum samples in biliary atresia patients at different stages of traditional surgeries

Many patients with biliary atresia (BA) have impaired metabolism of copper (Cu) and zinc (Zn) because of the obstruction of bile ducts. An excessive Cu accumulation is cytotoxic and results in fibrosis in hepatic tissues. Since Zn works antagonistically to Cu, lower Zn concentrations may deteriorate liver damage. In the 1980's, we performed a series of surgeries on BA patients for the construction and alteration of the bile flow route, which is the major excretion route for Cu. We obtained liver and serum samples at each surgery, and measured Cu and Zn concentrations by inductively coupled plasma atomic emission spectrometry. Hepatic Cu concentration decreased with the improvement of cholestasis after the establishment of bile excretion. Conversely, when cholestasis persisted or recurred, increases in hepatic and serum Cu concentrations were noted. Hepatic Zn concentration was lower than previously reported normal values. High hepatic and serum Cu concentrations due to persistent or recurrent cholestasis and low hepatic Zn concentration may deteriorate hepatic fibrosis and liver cirrhosis.     

高频超声与MRI鉴别诊断胆管闭锁

目的 探讨高频超声与MRI对胆管闭锁及非胆管闭锁性黄疸的鉴别诊断价值。方法 对47例黄疸患儿进行高频超声检查,观察TC征、肝右动脉和胆囊等;对其中23例患儿行MR检查,观察胆总管、肝总管和胆囊。以肝穿刺组织学检查、术后病理检查结果或临床痊愈为金标准,对比两种方法诊断小儿黄疸的敏感度、特异度及准确率。结果 47例患儿中,胆管闭锁19例、非胆管闭锁28例。高频超声观察指标中,TC征阳性、肝右动脉增宽和异常胆囊对诊断胆管闭锁的诊断价值较高,以其中两项阳性诊断胆管闭锁的敏感度、特异度及准确率分别为94.47%(18/19)、92.86%(26/28)和93.62%(44/47)。MRI观察指标中,以胆总管、肝总管未显示或不连续(伴或不伴胆囊未显示)诊断胆管闭锁的敏感度、特异度及准确率分别为44.44%(8/18)、80.00%(4/5)和52.17%(12/23)。结论 高频超声对鉴别胆管闭锁及非胆管闭锁性黄疸有较高的准确率,可作为首选检查方法。     

彩色多普勒超声对胆道闭锁的诊断价值

目的总结分析胆道闭锁患儿早期声像图特点及超声诊断注意事项。方法回顾性分析58例经手术病理证实的胆道闭锁婴儿肝胆超声表现。结果 58例胆道闭锁患儿中,胆囊形态异常15例(25.9%),胆囊收缩功能异常56例(96.6%);门静脉前方不规则或索条状回声(TC征)增强36例(62.1%);肝动脉扩张49例(87.5%),33例(56.9%)患儿血流阻力指数增高;肝肋下间距40 mm者50例(86.2%),肝脾肿大32例(55.2%),腹水18例(34.6%)。结论超声是诊断胆道闭锁首选的影像学检查方法,综合应用胆囊形态及收缩功能异常、TC征阳性及肝动脉增宽等指标可提高胆道闭锁的诊断准确率。     

Hepatic uptake and intestinal absorption of bile acids in the rabbit

Abstract The existence of transporters for bile acids (BA) in liver and intestine has been well documented, but information is still needed as to their respective transport capacity. In the present investigation, we compared the hepatic and intestinal transport rates for BA, using perfused livers and intestines. The livers and intestines were separately perfused and dose-response curves (0·25–10 mM) for tauroursodeoxycholate, taurocholate and taurodeoxycholate were obtained. The intestinal and mesenteric concentration and bile acid pattern were also evaluated in six non-fasting rabbits. Taurocholic, tauroursodeoxy-cholic and taurodeoxycholic acid ileal absorption showed saturation kinetics in the intestine as in the liver; the maximal uptake velocity for each bile acid in the liver was tenfold higher than the respective maximal transport velocity in the intestine; the Km values obtained in the liver were of the same order of magnitude, i.e. in the millimolar range. Taurocholic, tauroursodeoxycholic and taurodeoxycholic acid transport differences in the liver paralleled those in the intestine. Although the intestine was not homogeneously filled, the bile acid concentration in the ileal content fell into the range of the Km for the three studied bile acids, while the portal blood total bile acid concentration was inferior to the observed Kms of liver uptake. Therefore, both the hepatic and intestinal systems do not operate at their maximal transport rates at the prevailing concentrations in portal blood and luminal content, and the hepatic transport occurs at its highest efficiency (below the Km values) in physiological conditions.     

An analysis of data on human hepatic bile. Relationship between main bile components,serum cholesterol and serum triglycerides

Hepatic bile samples were taken from the common duct during interval operations for gallstone disease, performed under standardized conditions. Prior to operation serum cholesterol and triglycerides levels were determined. The concentrations of Cholesterol (Chol), phospholipids (Lip P) and of the three major bile acids (BA) were determined in ninety-seven samples. The data were subjected to statistical analysis. A highly significant rank correlation was found between the Chol- and the Lip P molar fractions. The rank correlation between the Chol molar fraction and the BA/Lip P ratio was highly significantly negative. Reasons are given why the observed intraindividual differences are interpreted as reflecting interindividual changes. The conclusion is that just as in the animal model, a rise of Chol in human hepatic bile is accompanied by a decrease of the BA/Lip P ratio. A significant correlation was found between chenodeoxycholic acid (CDCA), and the ratio between the two other main bile acids (CA/DCA). High lithogenicity was associated with low CDCA- and high DCA values. In a mathematical representation valid in the sense of a rank correlation, the bile/serum Chol ratio rose with increasing DCA values combined with increasing absolute differences between the two other acids. Serum triglycerides were negatively correlated with the BA molar fraction and with the absolute concentration of BA. As a result there was an association between serum triglycerides and lithogenicity.