新生儿胆道闭锁的预警筛查与早期诊断

胆道闭锁是婴儿期的严重慢性肝病,表现为破坏性炎性阻塞性胆管病。近期研究显示对30 d内胆道闭锁患儿实施早期Kasai手术最有可能延缓或防止肝移植,但目前国内外Kasai手术时间多在60 d左右,主要原因在于早期诊断面临很多困难和挑战,如30 d前行γ-谷氨酰基转肽酶筛查要充分考虑年龄分层的因素,且胆道系统早期病变程度轻微,导致影像学检查（如超声）的准确度降低,给精准医疗和改善预后带来挑战。本文对胆道闭锁新生儿早期诊断方式进行综述,旨在为30 d内的早期识别提供参考,并对未来潜在发展前景和研究方向进行分析。     

高频超声对肝外囊肿型胆道闭锁与胆总管囊肿的鉴别诊断

目的：探讨高频超声对肝外囊肿型胆道闭锁（CBA）与胆总管囊肿（CC）的鉴别诊断价值。方法：回顾性分析行高频超声检查及手术治疗的32例肝门部囊性病变患儿的临床资料,根据病理结果分为CBA组10例和CC组22例,对比分析2组的超声图像特点。结果：CBA组胆囊长径、前后径均明显小于CC组（均P<0.01）;CBA组囊壁厚度>1.0 mm的比例明显高于CC组（P<0.01）;CBA组胆囊形态异常与肝门部纤维块（≥4.0 mm）的发生率均明显高于CC组（均P<0.01）;CBA组肝内胆管扩张、囊内胆泥沉积的发生率均明显低于CC组（均P<0.01）;CBA组肝动脉内径明显大于CC组（P<0.01）。结论：高频超声对CBA与CC的鉴别诊断有重要的临床价值,结合临床症状和相关实验室检查,可准确诊断。     

胆道闭锁的影像学诊断研究进展

胆道闭锁（BA）是导致／bIL梗阻性黄疸的常见疾病之一。患儿在40～60d内行手术治疗效果好,超过90d因发展为肝硬化、肝衰竭而导致病情难于逆转,因此该病的早期诊断非常重要。BA的影像学诊断方法主要有腹部超声、放射性核素肝胆动态显像、磁共振胰胆管造影、胆管造影等,它们既有各自独特的优势,也有其相应不足。该文将对BA的影像学诊断方法进行综述。     

胆道闭锁的MRI诊断

目的:分析胆道闭锁的MRI表现,评价其诊断价值.材料和方法:回顾性分析7例确诊为胆道闭锁患儿的MRI资料,观察其肝内外胆道、肝门部结构及胆囊的显示情况.结果:所有的患儿均未见到完整的肝外胆道.其中,3例冠状面T2WI可见肝门部三角形高信号影;5例门静脉周围增宽,肝门部出现条索状长T2信号;6例可见小胆囊,1例胆囊未显示.结论:胆道闭锁的MRI表现具有一定的特征性,多方位观察均未发现完整的肝外胆道时,应高度怀疑胆道闭锁.     

超声诊断先天性胆道闭锁1例

患儿，女，6个月。足月顺产，生后混合喂养，吃奶量尚可．生后1月皮肤出现黄染，进行性加重．并排陶土样便，近20日来黄染加重，化验肝功能：ALT568U／L，AST〉850U／L,TB237μLmol／L，DB184μmol／L，查体：周身皮肤粘膜及巩膜中度黄染。超声见：肝脏上界位于右锁骨中线第6肋间．肋下长3．1cm，肝实质回声增强粗糙，门脉系统管壁增厚，回声增强。     

超声形态学及血流动力学指标诊断新生儿胆道闭锁临床价值

目的 探讨超声形态学及血流动力学指标诊断新生儿胆道闭锁（BA）的临床价值。方法 选择2020年5月至2023年5月于我院治疗的138例黄疸新生儿。所有新生儿均进行超声检查。分析病理诊断结果,统计BA患儿与非BA患儿的胆囊形态异常及三角条索征（TC）征情况,并以病理诊断结果作为金标准,计算胆囊形态异常及TC征诊断BA灵敏度、特异度,比较BA患儿与非BA患儿的肝胆血流参数,采用受试者工作特征曲线（ROC）分析肝胆血流参数诊断BA的价值。结果 138例黄疸新生儿中经手术病理及肝穿刺组织学检查证实BA 68例,非BA 70例;BA组胆囊形态异常及TC征发生率均高于非BA组,差异有统计学意义（P<0.05）;胆囊形态异常诊断BA阳性66例,阴性72例,误诊0例,漏诊2例,诊断灵敏度为97.06%(66/68)、特异度为100.00%(70/70);TC征诊断BA阳性63例,阴性75例,误诊0例,漏诊5例,诊断灵敏度为92.65%(63/68)、特异度为100.00%(70/70);BA组门静脉前后径（Dha）、脾静脉阻力指数（RIspa）及门静脉阻力指数（RIha）均高于非BA组,差异有统...     

MRCP诊断先天性胆道闭锁的临床应用

目的:探讨MRCP诊断先天性胆道闭锁的临床应用价值。方法:回顾性分析21例经手术证实为胆道闭锁的MRCP图像。所有患儿均行常规T2WI和MRCP序列扫描。结果:21例患儿均未见完整肝外胆道系统。7例胆囊瘪小,10例胆囊未成像,3例左右肝管成像,21例胆总管无成像。结论:肝外胆管在多序列或多方位图像上不显示是胆道闭锁的直接征象,小或无胆囊及肝门区片状高信号间接提示胆道闭锁。MRCP无创、无需对比剂,可多方位清晰显示肝内外胆道系统,对先天性胆道闭锁的诊断有重要临床价值。     

彩色超声诊断胎儿食管闭锁1例

1 病例简介 孕妇29岁,孕1产0.曾在外院行常规产前检查,孕23周检查提示羊水多,孕28周检查提示羊水多并胃泡小,未提示其他异常所见.于孕32周到兰州军区乌鲁木齐总医院产前诊断中心进行检查,超声见:双顶径7.7cm,头围28.3cm,腹围27.3cm,肱骨5.5cm,股骨6.4cm;胃泡最大范围约1.26cm×0.87cm(图1),于食管上段主动脉弓水平可见周期性出现的范围约2.77cm×1.19cm的囊状回声,会咽部可见2.20cm×1.13cm的液区,两者相通(图2),CDFI未见血流信号;羊水指数27.3cm.超声诊断:①胎儿食管闭锁;②胎儿胃泡小;③羊水多.引产后尸体解剖证实胎儿食管闭锁.     

小儿先天性胆道闭锁漏诊1例

病例患儿,男,出生22d,因“呕血,面色苍白1d,气促1h”入院。患儿于入院的前1d晨起时吐奶,奶中带血3次,黑便3次,且有进行性苍白。入院前1h起气促。入院查体：T36％、P150次／min、R55次／min,神清,精神萎,呻吟,重度贫血貌,肢端暖,呼吸稍促,皮肤轻度黄染,针刺部位出血;前囟饱满2cm&#215;2cm,张力高,颅缝分离5mm,后囟未闭,颈软;心率150次／min,律齐,心音低钝,无杂音;     

先天性胆道闭锁2例

胆道闭锁是一种危及患儿生命的严重疾病。我科近年来收治2例，其中1例经手术证实，报告如下。例1男，72d。生后曾一度出现黄疸，14d后黄疸再现并进行性加重伴尿色深黄，大便渐呈白陶土样，但精神、哺乳均可。至2月龄时住某医院，拟诊“婴儿肝炎综合征”，“新生儿感染”。经抗感染等治疗，后又以强的松7．5mg／d，一周后黄疸依然，自动出院，前来我院。查体：巩膜明显黄染，全身皮肤呈暗黄色，心肺（一），腹部较膨隆，肝助下3cm，脾助4．5cm，质中偏硬，腹水征（一），实验室检查：总胆红素（TBIL）90．23～93．78μmol／L，直接胆红素（…     

胆道闭锁和婴儿肝炎综合征影像学诊断和鉴别诊断

胆道闭锁（BA）预后与手术日龄密切相关,早期诊断并同婴儿肝炎综合征（IHs）等鉴别极为重要。主要影像学诊断方法是超声显像（US）、磁共振成像（MRCP）、胆管造影及放射性核素肝胆显像（HBS）等。这些检查方法既有各自独特的优势,也有其相应不足。US对BA的特异性和对IHS灵敏度较高,但对BA的灵敏度较低;而肝胆显像对BA的诊断具有极高的灵敏度,其特异性受胆汁淤积等影响较大;MRCP能显示胆管结构,但受其条件、设备影响;胆管造影虽为金标准,但难度和创伤性较大。本文将BA和IHS影像学诊断、鉴别诊断及其进展综述如下。     

阴性法64层CT胆胰管成像在胆道闭锁中的诊断价值

目的：总结分析胆道闭锁的阴性法64层CT胆胰管成像（N—CTCP）的影像表现,探讨其诊断价值。方法：回顾性分析临床疑诊为胆道闭锁的52例患儿的影像资料,全部患儿行腹部增强CT门脉期扫描后采用容积重建（VR）、多平面重建（MPR）及最小密度投影（MinlP）等技术进行重建,观察并比较患儿肝内外胆道、肝门部结构、胆囊、肝脏、脾脏大小等征象的显示情况。结果：32例经手术病理证实为胆道闭锁（BA组）,20例临床确诊为婴儿肝炎综合征（IHS组）。胆道闭锁的N—CTCP影像特征包括：无或小胆囊、肝门区三角形低密度区、门静脉间隙增宽,可见“双边征”或“靶征”。BA组无1例显示胆总管,4例左、右肝管可见,但管道不连续,20例出现肝、脾肿大。小胆囊、三角征及门静脉间隙增宽在IHS组与BA组中差异有统计学意义（P〈O．05）,肝外胆管不显示、肝脾肿大在IHS组与BA组间差异无统计学意义（P〉0．05）。结论：胆道闭锁的N—CTCP表现具有一定特征性,肝门区三角形低密度影、小胆囊、门静脉间隙增宽出现“双边征”或“靶征”是诊断BA的重要征象。     

Hepatic ADC value correlates with cirrhotic severity of patients with biliary atresia

Introduction

At least 40% of survivors of biliary atresia have progressive cirrhosis even after undergoing Kasai operation. The values of hepatic apparent diffusion coefficient and apparent-diffusion-coefficient-related indices were applied to biliary atresia patients and correlated with cirrhotic severity scores of model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte, and Child-Pugh systems.

Materials and methods

Thirty-three biliary atresia patents (mean = 1140, 61–4314 days of age) received magnetic resonance image examinations due to complications of biliary atresia from April 2008 to August 2009. Two non-breath-hold diffusion weighted imaging sequences were performed with motion-probing gradients in three directions with two b values: 0/100 and 0/500 s/mm²; 1000 ms/61.1 ms, time to repeat/time to echo; number of excitation, 1.0; 8 mm section thickness; 40 cm × 40 cm field of view; 128 × 256 matrix in all biliary atresia patients and 18 control subjects. We used the Spearman rank correlation test to analyze the relationship among the scores of model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte and Child-Pugh scores and right hepatic apparent diffusion coefficients, apparent diffusion coefficient using b factor of 500-albumin product and alanine transaminase/apparent diffusion coefficient with b factor of 500 ratio.

Results

The right hepatic apparent diffusion coefficient using b factor of 100, apparent diffusion coefficient with b factor of 500 and product of apparent diffusion coefficient with b factor of 500-albumin level were significantly negatively correlated (p ≤ 0.0125) with model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte, and Child-Pugh scores of biliary atresia patients. The ratio of alanine transaminase level/right hepatic apparent diffusion coefficient with b factor of 500 was also significantly (p ≤ 0.0251), moderately correlated with Child-Turcotte and Child-Pugh scores (rho = 0.5256 and 0.7518, respectively).

Conclusion

Right hepatic apparent diffusion coefficient with b factor of 500 and alanine transaminase/right hepatic apparent diffusion coefficient with b factor of 500 can be useful for long-term follow-up of cirrhotic severity in biliary atresia patients.     

Quantitative cholescintigraphy and bile abnormalities in patients with acalculous biliary pain

Acalculous biliary pain has been related to gallbladder dysfunction that produces a gallbladder emptying defect—a condition which favours the development of lithiasis. It is therefore probable that microlithiasis is present in patients with gallbladder dysfunction. The aims of this study were to measure gallbladder emptying and investigate bile abnormalities in patients with acalculous biliary pain. In 92 consecutive patients, gallbladder emptying was assessed by quantitative cholescintigraphy (abnormal ejection fraction 40%). In 64 patients, a microscopic study was performed on duodenal bile, defining abnormality as the presence of cholesterol crystals in any amount and/or calcium bilirubinate granules and/or microspheroliths at a rate of >10 per slide. The ejection fraction was abnormal in 45 patients (49%) (median 25.1%, range 6.8–39.3%) and normal in the remaining 47 cases (median 71.3%, range 41.0–96.1%). Bile was abnormal in 32 of 64 patients (50%), the most frequent finding being calcium bilirubinate granules. In the patients with bile abnormalities, abnormal ejection fraction was more frequent (20 of 32) and the median ejection fraction was lower (30.9%, range 12.0–94.1%) than in the patients with normal bile (16 of 32 with an abnormal ejection fraction; median ejection fraction 50.7%, range 6.8–96.1%). Abnormal bile was frequent (55.5%) in patients with reduced ejection fraction, but was not uncommon in patients with normal ejection fraction (33.3%). Fewer patients showed no alteration (25%). It is concluded that in most patients, acalculous biliary pain coexists with gallbladder dysfunction or abnormal bile, the combination of both alterations being common.     

肝移植术后并发胆道狭窄和胆泥淤积影像诊断及介入治疗

目的:评价肝移植术后胆道狭窄和胆泥淤积影像诊断及介入治疗的价值。方法:对39例肝移植术后并发胆道狭窄和胆泥淤积的影像诊断及介入治疗进行回顾性分析。结果:超声、T型管胆道造影、CT和MRI检查诊断胆道狭窄伴胆泥形成39例,38例介入治疗后胆道梗阻症状明显缓解;1例介入治疗后胆道梗阻症状未明显改善,后行外科胆管修补术。结论:T型管胆道造影或直接经皮胆道造影对肝移植术后胆道狭窄和胆泥淤积诊断特异性及敏感性最高,放射介入和内镜介入技术对其均发挥重要的治疗作用。     

~(99m)Tc-MIBI肝胆动态显像对先天性胆道闭锁的诊断价值

目的评价99m锝-甲氧基异丁基异腈(99mTc-MIBI)肝胆动态显像在先天性胆道闭锁(BA)诊断中的临床应用价值。方法 52例患儿静脉注射99mTc-MIBI后,用SPECT进行肝胆动态显像,并经手术病理和临床随访结果证实。结果在17例最终诊断为BA的患儿中,99mTc-MIBI肝胆显像全部检出,灵敏度、特异度和准确性分别为100%、80.9%、92.3%。结论 99mTc-MIBI肝胆动态显像是无创、安全、有效的检查方法,对于BA的诊断,有较高的临床应用价值。     

Biliary ileus: preoperative diagnosis by US. A report of two cases

The preoperative diagnosis of biliary ileus was correctly made by US in two cases (87 and 80-year-old females) referred to US studies because of nonspecific abdominal symptoms and to rule out possible acute appendicitis, respectively. Plain-film findings were unremarkable in one patient, small-bowel obstruction was detected in the latter. In both cases sonography revealed a small amount of air within the thick-walled gallbladder; the presence of mechanical ileus, and a gallstone impacted within the ileal loop. These features, together with collapsed bowel lumen distally to the stone, enabled a reliable diagnosis of biliary ileus to be made. This was confirmed by ensuing laparotomy, when a large cholesterol stone was removed in both cases. Correspondence to: V. Simonovský     

磁共振与超声在胆道系统疾病诊断中的价值

 目的 评价磁共振与超声在胆道系统疾病诊断中的价值.方法 收集手术治疗的病例92例,并将其术前的磁共振胆胰管造影(MRCP)及超声结果与手术结果作对比,利用ROC曲线进行分析.结果 超声诊断胆囊炎、胆囊结石价值比磁共振高,但磁共振对胆总管结石的检出率较高.结论 超声在胆道系统病变诊断中应作为首选,而对胆总管直径超过8 mm的病例术前应做MRCP以便正确选择手术方式.     

MRCP对胆系梗阻性疾病的诊断及鉴别诊断研究

目的 探讨胆系梗阻性疾病的MRCP表现及其临床诊断价值.方法 对2002年12月至2004年12月共342例胆系梗阻性疾病进行MRCP检查,所有病例经手术病理或其他影像学和临床资料所证实.使用GE signa MRI/echo speed超导型1.5TMR扫描机,常规行上腹部T1WI和T2WI轴位扫描,MRCP采用单次激发快速自旋回波序列进行冠状位扫描.结果 342例胆系梗阻性疾病的定位诊断正确率为100%,结合常规平扫及其他影像学检查定性诊断正确率可达到92.4%.良性病变（包括胆管结石、胆总管囊肿及胆管炎性狭窄）140例,占所有病例的41%,诊断正确率达95.7%.医源性肝外胆管狭窄21例,占6%,诊断正确率达95.2%.恶性病变（包括胆管癌、胰头癌及壶腹癌）181例,占53%,诊断正确率达89.5%.结论 MRCP作为一种无创性检查手段,结合磁共振平扫和其他临床资料可对良性梗阻性病变及壶腹以上部位的恶性胆道梗阻做出较准确的定性、定位诊断;但对于壶腹周围梗阻性病变的准确诊断有待于进一步研究和改进.