十二指肠液γ-谷氨酰转肽酶的测定对新生儿阻塞性黄疸的鉴别诊断

探讨十二指肠液γ-谷氨酰转肽酶(γ-GT)活性对新生儿阻塞性黄疸的鉴别诊断价值.应用婴儿十二指肠引流管收集38例新生儿期阻塞性黄疸的十二指肠液,检测十二指肠液中γ-GT活性.结果显示,随访黄疸消退的22例新生儿肝炎患儿,胆汁中胆红素值≥8.5μmol/L),胆汁酸阳性,γ-GT≥20Iu/L;16例肝外胆道闭锁者无胆汁,十二指肠液胆红素值0～5 μmol/L,胆汁酸阴性,十二指肠液γ-GT缺如.测定十二指肠液胆红素、胆汁酸和γ-GT活性有助于早期鉴别诊断新生儿肝炎与肝外胆道闭锁.     

十二指肠液γ-谷氨酰转肽酶的测定对新生儿阻塞性黄疸的鉴别诊断

探讨十二指肠液γ-谷氨酰转肽酶(γ-GT)活性对新生儿阻塞性黄疸的鉴别诊断价值。应用婴儿十二指肠引流管收集38例新生儿期阻塞性黄疸的十二指肠液，检测十二指肠液中γ-GT活性。结果显示，随访黄疸消退的22例新生儿肝炎患儿，胆汁中胆红素值≥8．5μmol／／L)，胆汁酸阳性，γ-GT≥20Iu／L；16例肝外胆道闭锁者无胆汁，十二指肠液胆红素值0～5μmol／L，胆汁酸阴性，十二指肠液γ-GT缺如。测定十二指肠液胆红素、胆汁酸和γ-GT活性有助于早期鉴别诊断新生儿肝炎与肝外胆道闭锁。     

动态持续十二指肠液检查鉴别诊断婴儿期持续性阻塞性黄疸

目的　评价动态持续十二指肠液检查鉴别诊断婴儿肝炎综合征 (Infantilehepatitissyndrome,IHS)与胆道闭锁 (Biliaryatresia,BA)的价值 ,寻求简单、快速、正确的鉴别诊断方法。方法应用婴儿十二指肠引流管和引流技术进行动态十二指肠液检查 ,观察十二指肠液颜色、定量测定十二指肠液胆红素值、γ 谷氨酰转肽酶 (γ GT)活性和胆汁酸定性或定量测定。结果　 5 6 1例婴儿持续性黄疸首次十二指肠液检查 ,在插管后 3～ 8min内获黄色液体 342例 ,持续引流 2 4h获黄色液体 2 1例 ,间接引流 4 8～ 72h获黄色液体 16例。十二指肠液呈淡黄色者 71例 ,十二指肠液呈微黄或白色者 111例 ,经治疗后再次对淡黄色和微黄或白色液体者 182例行十二指肠液检查 ,十二指肠液呈黄色者 91例 ,十二指肠液呈白色者 89例 ,呈微黄者 2例。十二指肠液呈黄色和淡黄色者胆红素定量≥ 8 5 μmol/L ,γ GT >2 0Iu/L ,胆汁酸阳性或定量为 33～ 2 6 0 μmol/L ,十二指肠液无色者胆红素值 0～ 2 μmol/L ,十二指肠液微黄者 2例 ,胆红素分别为 5 .8μmol/L ,胆汁酸阴性 ,γ GT 0～ 5Iu/L。以十二指肠液胆红素≥8 5 μmol/L ,胆汁酸阳性 ,γ GT >2 0Iu/L诊断为IHS 4 70例 ,经随访黄疸完全消退。以十二指肠液 <8 5μmol/L ,胆汁酸阴性 ,γ GT <2     

动态持续十二指肠液检查对婴儿肝炎综合征与先天性肝外胆道闭锁的鉴别诊断价值探讨

目的　评价动态持续十二指肠液检查对婴儿持续性黄疸的鉴别诊断价值。方法　应用婴儿十二指肠引流管动态持续收集十二指肠液 ,观察颜色、测定总胆红素值和γ 谷氨酰转肽酶 (γ GT)活性。结果　 16 8例婴儿持续性黄疸中 133例十二指肠液呈黄色 ,总胆红素值≥ 8 5 μmol/L ,γ GT≥ 2 0IU/L ,经随访黄疸消退 ,诊断为婴儿肝炎综合征 ;35例十二指肠液无色或呈淡黄色 ,总胆红素值 <8 5 μmol/L ,γ GT <2 0IU/L ,经剖腹探查诊断为先天性肝外胆道闭锁。结论　动态持续十二指肠液检查能早期正确鉴别婴儿肝炎综合征与先天性肝外胆道闭锁     

婴儿肝炎综合征血清和胆汁成分变化的意义

目的探讨婴儿肝炎综合征（IHS）患儿血清及胆汁成分变化的临床意义。方法采用全自动生化分析仪检测42例IHS患儿及对照组16例婴儿的血清及胆汁中总胆红素（TB）、直接胆红素（DB）、ALT、γ-谷氨酰转肽酶（γ-GT）、总胆汁酸（TBA）水平,采用双抗体夹心ELISA法检测血清及胆汁中白细胞介素-6（IL-6）、肿瘤坏死因子-α（TNF-α）的水平。婴儿肝炎综合征患儿分为淤胆型组（18例）和肝炎型组（24例）。结果1.IHS组血清TB、DB、ALT、γ-GT、TBA、IL-6、TNF-α均明显升高（Pa〈0.05）;胆汁中TB、DB、γ-GT、TBA均明显降低（Pa〈0.05）,IL-6、TNF-α高于对照组（Pa〈0.05）。2.淤胆型血清TB、DB、γ-GT、TBA、IL-6、TNF-α均高于肝炎型（Pa〈0.05）;淤胆型胆汁中IL-6、TNF-α高于肝炎型（Pa〈0.05）,γ-GT、TBA则低于肝炎型（Pa〈0.05）。3.IHS患儿胆汁IL-6、TNF-α分别与血清DB、TBA、γ-GT呈正相关（Pa〈0.05）。结论IHS患儿均存在不同程度胆汁淤积,淤胆型IHS胆汁淤积程度更严重,胆汁IL-6、TNF-α可能参与IHS胆汁淤积的发生过程,且与疾病严重程度有关,检测胆汁中TB、DB、γ-GT、TBA、IL-6、TNF-α水平可为判断IHS患儿病情程度及估计预后提供依据。     

淤胆型婴儿肝炎综合征与胆道闭锁的早期鉴别诊断

淤胆型婴儿肝炎综合征（IHS）与肝外胆道闭锁（EHBA）早期鉴别诊断极为重要。二者早期鉴别诊断EHBA就能早期手术，重建胆道，恢复胆流；淤胆型HIS就能避免手术创伤导致的肝损害加重。淤胆型IHS与EHBA早期鉴别诊断需依靠临床及辅助的检查方法综合评估。     

评论

鉴别婴儿肝炎综合征(IHS)与胆道闭锁(BA)是临床重要且困难的课题.既无创伤性又能正确鉴别上述疾病的检查,可以使BA患儿早期手术,提高存活率,同时免去ISH患儿行剖腹探查术的危险.到目前为止,虽然不断出现先进的检查方法,但还没有一种方法能够取代综合检查.<动态持续十二指肠液检查鉴别诊断婴儿期持续性阻塞性黄疸>一文,以较大的样本数对动态持续十二指肠引液检查的临床应用进行了探索,取得了宝贵的经验.     

十二指肠内胆红素光吸收值监测在胆道闭锁诊断中的应用研究

目的本研究对〈4个月阻塞性黄疸婴儿十二指肠内的胆红素进行光吸收值动态监测，评价光吸收值在胆道闭锁诊断中的意义。方法研究对象分为胆道闭锁患儿（biliary atresia，BA）17例（男11例，女6例；年龄2～16周，平均5．7周）及婴儿肝炎综合征患儿（infantile hepatitis syndrome，IHS）20例（男14例，女6例；年龄3～7周，平均5周）。37例患儿接受了Bilitec十二指肠内胆红素光吸收值的监测，观察原始光吸收值曲线图，分析、比较最高光吸收值、光吸收值≥0．14U及≥0．25U的时间百分比。结果胆道闭锁组的光吸收值明显低于婴儿肝炎综合征组。结论十二指肠内胆红素光吸收值监测可为鉴别BA与IHS提供客观依据。     

婴儿肝炎综合征与胆道闭锁的早期鉴别诊断

本文对手术或尸检诊断的13例先天性胆道闭锁和随访黄疸消失的18例婴儿肝炎综合征的临床观察和阻塞性黄疸的几种检测方法的结果分析,显示:两者的早期鉴别仍需综合评估,若出生后血清胆红素值持续增加,大便持续白色,十二指肠液透明无色且胆红素缺如或<8.3μmol/L,血清γ-GT升高而十二指肠液中r-GT缺如,放射性核素显像肠道无显像者应早期剖腹探查。     

放射性核素肝胆显像联合动态十二指肠液检查对婴儿持续性黄疸鉴别诊断的意义

目的 探讨99mTc-EHIDA肝胆显像联合动态十二指肠液引流检查对婴儿期持续性黄疸鉴别诊断的意义.方法 应用SPECT仪对84例15～90 d持续性阻塞性黄疸息儿进行肝胆核素显像和动态十二指肠引流液检查.结果 核素肝胆显像对先天性胆道闭锁(EHBA)诊断灵敏度为100.0%(29/29),特异度为74.5%(41/55);核素肝胆显像联合动态十二指肠引流对EHBA诊断灵敏度为100.0%(29/29),特异度为100.0%(55/55).结论 ,99mTc-EHIDA肝胆显像是一种无创、安全、有效的检查方法,与动态十二指肠引流法结合对婴儿持续性黄疸鉴别诊断具有较高的临床价值.     

腹腔镜在婴幼儿胆管发育不良与胆汁淤积症诊治中的应用

目的 探讨腹腔镜在胆管发育不良与胆汁淤积症诊断和治疗中的应用。方法 回顾性分析近年18例婴幼儿胆管发育不良与胆汁淤积症的临床资料。结果 所有病例均经腹腔镜行胆道造影明确诊断，其中胆管发育不良10例，胆汁淤积8例。10例胆管发育不良中，7例中转开腹行扩大肝门肝肠吻合术，3例胆道外引流、置管冲洗术；8例胆汁淤积中，2例单纯胆道减压、冲洗，6例胆囊造瘘，术中术后分别灌洗。结论 腹腔镜探查、胆道造影是诊断胆管发育不良与胆汁淤积症的简便、准确、易行的方法，胆管发育不良可行扩大肝门肝肠吻合术，或胆道减压、胆管灌洗术。胆汁淤积症的患儿，可行胆囊造瘘、胆道灌洗，并可免除开腹手术。     

Isolated extrahepatic bile duct injury: diagnosis and surgical management

Isolated injuries of the extrahepatic bile ducts due to blunt trauma are rare. The diagnosis in these cases is often delayed and there are no firm guidelines in regard to the treatment. We present a 3-year-old child with partial disruption of the confluence of the left and right hepatic ducts. A hepatic scintiscan was used to confirm the diagnosis and identify the site of the bile leak. The available literature has been reviewed briefly to highlight the clinical features that should arouse suspicion of such injuries and the various surgical options are discussed.     

计算机辅助手术系统在先天性胆管扩张症诊治中的应用

目的研究计算机辅助手术系统在小儿先天性胆管扩张症诊断与治疗中的应用价值。方法回顾性分析2013年6月至2015年1月青岛大学附属医院收治的25例先天性胆管扩张症患儿的肝脏64排螺旋CT扫描原始数据,采用计算机辅助手术系统(Hisense CAS)进行肝脏及胆道三维重建,根据三维重建结果进行精准的术前诊断和病理形态分析,以及辅助手术规划,确定最佳手术方案。观察术中所见与术前规划的符合程度、术中出血量、并发症等。结果 25例中,囊肿型14例,梭状型11例;合并肝内胆管扩张18例;少见的复杂胆道畸形3例。肝脏及其内部管道系统的三维模型形态逼真、立体感强,清晰地显示胆管的分布走形及其与肝内三套血管系统的空间位置关系。术前手术方案与实际手术方式符合率为88%(22/25)。术中出血量中位数为12 mL,最少出血量为6 mL。术后无一例出现严重并发症或死亡。结论通过计算机辅助手术系统对CT数据进行三维重建,实现了胆道系统的数字化解剖,并能清晰显示胆道及其周围血管系统的空间位置关系,提高先天性胆管扩张症手术的精准性和手术安全性。     

Extrahepatic biliary atresia: from diagnosis to liver transplantation

Thirty infants were diagnosed with extrahepatic biliary atresia (EBA) from July 1978 to July 1989; 28 have undergone a Kasai or Lilly-Altman modification of the Kasai portoenterostomy; 2 were excluded from surgery because they presented after 3 months of age and had advanced biliary cirrhosis. Immediate postoperative drainage (>30 ml/day was achieved in 24 patients (86%), with 14 (50%) surviving free of jaundice. The average follow-up was 2.25 years (range 3 months to 10 years); the longest survivor is 10.3 years old. The overall survival was 64%, and 5-year survival 50%. Of 24 infants operated on at <12 weeks of age, 14 are free of jaundice (58%), 4 are alive with jaundice (17%), and 6 have died (25%). No correlation existed between subsequent bile drainage and duct size at the porta hepatis, presence or absence of hepatic fibrosis, giant-cell transformation, or hepatic inflammation. Complications included cholangitis (57%), progressive hepatic failure (39%), portal hypertension (21%), gastrointestinal bleeding (21%), esophageal varices (18%), stomal hemorrhage (11%), seizures (7%), rickets (3.5%), biliary calculus in the Roux-en-Y (3.5%), and hepatic abscess (3.5%). Five infants required revision, with 1 patient undergoing six reoperations, each followed by successful re-establishment of bile drainage. One infant had a successful liver transplant, and 2 are currently candidates for transplantation. Based on this analysis, an algorithm has been formulated for the diagnostic evaluation and subsequent surgical management from initial portoenterostomy to orthotopic liver transplantation for infants diagnosed with EBA. From our review of this experience and the literature on EBA and orthotopic liver transplantation, we have concluded that portoenterostomy has a substanial chance (P <0.05) of providing bile drainage and survival is comparable to that after liver transplantation. Although ultimate failures occur at a steadily increasing rate, portoenterostomy delays transplantation until children are older and can more easily undergo transplantation with its attendant risks of immunosuppression and complications.     

生物标记物粪便胆汁酸的测定在过敏性紫癜患儿诊治中的意义

目的 探讨生物标记物粪便胆汁酸浓度在过敏性紫癜(HSP)患者中的变化及其在诊治中的临床意义。方法 选取2014~2016年确诊为HSP的19例患儿为HSP组,另选取27例健康儿童为健康对照组。采集HSP组患儿急性期、恢复期及健康对照组儿童粪便标本,应用液相质谱技术检测各组儿童粪便胆汁酸水平。结果 HSP组患儿恢复期胆酸水平均高于健康对照组和HSP组急性期 (P < 0.016)。HSP组患儿恢复期鹅脱氧胆酸水平高于健康对照组 (P < 0.016)。HSP组患儿急性期和恢复期脱氧胆酸、石胆酸水平均低于健康对照组 (分别P < 0.05、P < 0.016)。各组间熊去氧胆酸水平比较差异均无统计学意义 (P > 0.05)。结论 HSP患儿急性期粪便次级胆汁酸脱氧胆酸和石胆酸低于健康对照组,这可能与HSP的发病或转归有关。     

Zellweger's syndrome (cerebro-hepato-renal syndrome)--its clinical picture, morphology and biochemical diagnosis

The cerebro-hepato-renal (Zellweger) syndrome is characterised by dysmorphic features, severe muscular hypotonia, hepatic dysfunction and early death in infancy. Recently it has been shown that the disease is an inborn error of metabolism with an unusual variety of metabolic disturbances affecting pipecolic acid, bile acids, plasmalogens and very long chain fatty acids. Ultrastructural and biochemical findings confirming the diagnosis are illustrated. The syndrome is inherited as an autosomal recessive trait, prenatal diagnosis has become possible.     

The changing pattern of diagnosis of infantile cholestasis

OBJECTIVE: Cholestatic liver disease in infancy is caused by a wide range of conditions. This study reviews the pattern of diagnosis of infants with cholestasis presenting to a tertiary referral paediatric hospital in Sydney, Australia, during a 12-year period (1985-96). METHODOLOGY: Infants aged less than 6 months with cholestasis were identified retrospectively from hospital records and data retrieved from the medical records. RESULTS: There were 205 infants identified as having cholestatic liver disease. The aetiology of the cholestasis was idiopathic in 25%, metabolic/genetic in 23%, and due to obstruction in 20%, parenteral nutrition in 20%, infection in 9% and bile duct hypoplasia in 3%. CONCLUSIONS: This study highlights the changing patterns of diagnosis of cholestatic liver disease in infants at a tertiary paediatric facility, demonstrating that up to 50% of cases are now due to genetic/metabolic diseases or parenteral nutrition, and a high proportion are due to idiopathic disease.     

Hyaluronic acid: Additional biochemical marker in the diagnosis of biliary atresia

BACKGROUND: The purpose of the present paper was to evaluate the value of biochemical markers, including conventional liver function tests, gamma-glutamyl transferase (GGT), and hyaluronic acid (HA), in the diagnosis of neonatal cholestasis. METHODS: Infants with neonatal jaundice were consecutively enrolled during 1 year period. The patients were diagnosed as having biliary atresia (BA) if there was either bile ductular proliferation in the portal tracts, atretic common bile duct/gallbladder, or evidence of bile duct obstruction demonstrated by liver pathology or intraoperative cholangiography, respectively. Serum HA was measured using an enzyme-linked immunosorbent assay-based test. RESULTS: A total of 25 patients diagnosed as having BA (n = 10), neonatal hepatitis (NH; n = 9), choledochal cyst (n = 3) and parenteral nutrition-induced cholestasis (n = 3), were studied. The age at diagnosis was not significantly different between groups. Only GGT and HA were significantly elevated in the patients with BA when compared to NH (P = 0.02, P = 0.03, respectively). In BA, the median value of serum HA was 514 ng/mL (range 19-4476 ng/mL), compared to 50 ng/mL (range 19-315 ng/mL) in NH. Additionally, the serum HA level was much higher in children with choledochal cyst. CONCLUSION: HA could be considered as a complementary biochemical marker for evaluating infants with prolonged jaundice.     

3beta-hydroxy-delta5 -C27-steroid dehydrogenase deficiency: diagnosis and treatment

The aim of this study was to evaluate the effects of bile acid treatment and to obtain further information about the pathway of bile acid biosynthesis in a patient with 3beta-hydroxy-delta5-C27-steroid dehydrogenase/isomerase (3beta-HSD) deficiency by gas chromatography-mass spectrometry. Results showed that at 2 months of age, 3beta-hydroxy-5-cholen-24-oic acid (3.0 micromol/mmol Cr, 7.9%) was detected in the urine in essentially the same relative amount as 3beta,7alpha-dihydroxy- and 3beta,7alpha,12alpha-trihydroxy-5-cholen-24-oic acids (3.7 micromol/mmol Cr, 9.8%) during ursodeoxycholic acid treatment combined with prednisolone. As a result, diagnosis was delayed until 18 months of age. One month later with substitution of chenodeoxycholic acid treatment, urinary 3beta,7alpha-dihydroxy- and 3beta,7alpha,12alpha-trihydroxy-5-cholen-24-oic acids decreased significantly, and subsequent improvement of liver dysfunction was accelerated. Chenodeoxycholic acid treatment is useful in 3beta-HSD deficiency. However, in the diagnosis of this disease in early life, it should be noted that the acidic pathway may be the major route for bile acid biosynthesis in the neonatal period. Diagnosis of 3beta-HSD deficiency may have been delayed by administration of ursodeoxycholic acid, resulting in prolonged diagnostic investigation in this child with cholestasis. Further, use of prednisolone may have been contraindicated.