肝胆同位素动态显像和超声检查对婴儿肝外胆道闭锁的诊断价值

目的探讨99Tcm-二乙基乙酰苯胺亚氨二醋酸(99Tcm-EHIDA)肝胆平面+断层显像和肝胆超声检查对婴儿肝外胆道闭锁(EHBA)的诊断价值。方法临床表现高度怀疑EHBA的持续性黄疸患儿98例,予肝胆平面+断层显像和超声检查。参照临床最终诊断,评价两种检查方法对不同日龄段EHBA的诊断效能和联合应用价值。结果99Tcm-EHIDA肝胆平面显像诊断EHBA的灵敏度、特异度和准确性分别为92.6%、60.6%和68.4%;联合6～8h肝胆断层显像,分别提高到100.0%、77.5%和83.7%。对日龄<60d组患儿,肝胆平面+断层显像对EHBA灵敏度、特异度和准确性分别为100.0%、86.2%、89.7%,高于以胆囊大小、收缩率为诊断标准的超声检查(80.0%、69.0%、71.8%)和以肝门纤维块为诊断标准的超声检查(40.0%、93.1%、79.5%);而对日龄>60d者,三者对EHBA的效能分别为100%、71.4%、79.7%和88.2%、57.1%、66.1%和76.5%、92.9%、88.1%。其中,肝胆平面+断层显像持续未见肠道放射性出现的29例患儿,联合超声检查以三角形索带征为诊断标准,诊断E...     

肝组织病理学检查在肝外胆道闭锁与婴儿肝炎综合征鉴别诊断中的意义

目的 探讨肝组织病理学检查在肝外胆道闭锁和婴儿肝炎综合征鉴别诊断中的价值.方法 将1997年8月-2006年10月经手术或尸检后确诊的32例肝外胆道闭锁和16例婴儿肝炎综合征进行回顾性病理学检查.结果 肝外胆道闭锁以胆管增生和汇管区纤维化伴炎性细胞浸润为主要病变,而婴儿肝炎综合征以肝细胞变性坏死、肝巨细胞样变和髓外造血为主要病变,但少数病例部分病变具有重叠性.结论 肝外胆道闭锁和婴儿肝炎综合征具有某些相似的早期病理改变,但前者主要为胆管的病变,后者则以肝细胞病变为主,故肝组织病理学检查对于两者的鉴别诊断有重要意义.     

Implication of early‐onset biliary atresia and extrahepatic congenital anomalies

Background: The aim of the present study was to determine the rate of early‐onset biliary atresia (BA) and its implications, for embryonic‐type BA in Taiwan, a high‐prevalence area for BA. The relationship between the timing of disease onset and congenital extrahepatic anomalies was also identified. Methods: Medical records of 130 infants born in Taiwan with biliary atresia between January 1996 and December 2005 were reviewed retrospectively. The gold standard for the diagnosis of biliary atresia was intraoperative cholangiography. As well as medical records review, abdominal imaging and echocardiograms were performed to determine other structural anomalies. Early‐onset BA was defined as acholic stool and cholestatic jaundice observed before 2 weeks of age. Results: On review of onset of acholic stool and cholestatic jaundice before 2 weeks of age, 31 patients (23.8%) were defined as having early‐onset BA. Twenty patients (15.4%) had major congenital extrahepatic anomalies. One (0.7%) had biliary atresia splenic malformation syndrome (BASM). Both early‐onset and late‐onset BA may be associated with other structural anomalies. Patients with early‐onset BA had a higher probability of having major extrahepatic anomaly (9/31 vs 11/99, P= 0.046). Situs anomalies accompanying major gastrointestinal (GI) tract anomalies occurred only in early‐onset BA patients. Conclusions: After comprehensively investigating the timing of onset and associated congenital extrahepatic anomalies in BA patients in Taiwan, only one BASM with double spleen was detected. A total of 23.8% of patients had early‐onset BA, and this group of patients is prone to extrahepatic anomalies. Situs anomalies accompanying major GI tract anomaly may be indicative of embryonic‐type early‐onset BA.     

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目的研究肝外胆道闭锁及巨细胞病毒性肝炎患儿肝组织中的多药耐药蛋白3(MDR3)的表达,探讨MDR3与胆汁淤积的关系。方法收集2000年1月至2009年10月广西医科大学第一附属医院儿童病例,分为肝外胆道闭锁(EHBA)组20例,CMV肝炎组12例,正常对照组10例,采用免疫组化法检测肝组织中MDR3的表达情况,通过图像分析技术进行定量分析。结果 MDR3在正常对照组、EHBA和CMV肝炎组患儿肝组织均有不同程度表达,以肝细胞膜表达最为明显,MDR3在肝组织的阳性表达随γ-GT的增高而增强,呈正相关(r=0.438,P=0.003),CMV肝炎组、EHBA组和正常对照组MDR3表达吸光度值分别为0.13±0.02、0.18±0.06和0.10±0.03,MDR3在EHBA肝组织中的表达强度明显高于CMV肝炎组和对照组(P<0.05)。结论 MDR3蛋白水平与胆汁淤积程度密切相关,EHBA肝组织MDR3的上调可能为机体的适应性反应,以利于增强磷脂的转运,减轻对胆管上皮的损伤。     

Bleeding as the first symptom of extrahepatic biliary atresia

Four cases of extrahepatic biliary atresia are reported. Each of these infants presented with haemorrhagic phenomena rather than with prolonged jaundice. The increased bleeding tendency was due to a vitamin K deficiency, probably caused by cholestasis-induced malabsorption. Therefore extrahepatic biliary atresia should be considered in each infant with a bleeding diathesis.Abbreviations Hb haemoglobin - SGOT aspartate transaminase - SGPT alanine aminotransferase - ^99mTc-IDA ^99mTechnetium-labelled iminodiacetic acid     

Elevation of serum stem-cell factor in postoperative biliary atresia

BACKGROUND: Biliary atresia (BA) is one of the most common causes of neonatal cholestasis. Stem-cell factor (SCF) has been implicated in the development of fibrosis in various diseases. The objective of the present study was to examine the significant role of SCF in BA. METHODS: Fifty-seven pediatric patients with BA after Kasai operation and 30 healthy children were recruited. The mean ages of BA patients and controls were 6.1 +/- 0.6 years and 6.1 +/- 0.7 years, respectively. The patients were categorized into two groups according to their serum levels of total bilirubin (TBil < 2 mg/dL, no jaundice vs TBil > or = 2 mg/dL, persistent jaundice) and alanine aminotransferase (ALT < 100 vs ALT > or = 100 U/L). The serum SCF levels were determined on commercially available enzyme-linked immunosorbent assay. RESULTS: The mean serum SCF level of the BA children was higher than that of normal controls (748.3 +/- 17.9 pg/mL vs 582.2 +/- 17.3 pg/mL; P < 0.001). Subsequent analysis demonstrated that the BA patients with serum ALT > or = 100 U/L had significantly greater levels of serum SCF compared to those with serum ALT < 100 U/L (796.5 +/- 22.6 pg/mL vs 694.7 +/- 25.0 pg/mL, respectively; P = 0.002). In addition, serum SCF levels were significantly elevated in the patients with portal hypertension (PH) compared with those without PH (810.0 +/- 18.8 pg/mL vs 634.1 +/- 20.1 pg/mL, P < 0.001). CONCLUSION: The current study showed that BA patients had higher serum SCF levels compared with controls. The significant elevation in SCF levels is associated with the presence of PH and the degree of hepatic injury. These findings suggest that SCF may play a part in the pathogenesis of hepatic fibrosis in BA patients after Kasai procedure.     

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??Abstracts??Objective Different diagnostic methods of biliary atresia ??BA?? and infantile intrahepatic cholestasis disease were investigated in order to find a simple?? fast??practical??economic and non invasive differential diagnostic method. Methods A total of 584 cases of infantile cholestasis were collected from May 2006 to June 2012 for persistent jaundice??pale yellow or white shit who lived in Department of Pediatric Digestion and Infection?? Tongji Hospital?? Tongji Medical College?? Huazhong University of Science & Technology??HUST??. Seven methods including clinical diagnosis??blood biochemical tests?? liver and gallbladder ultrasonography?? dynamic continuous duodenal liquid bile check?? nuclide hepatic imaging?? magnetic resonance imaging and histology were applied for differential diagnosis and the results were analyzed. Results The correctness of clinical diagnosis method was 74.5%?? sensitivity 81.6%??specificity 69.9%?? liver size: 49.0%??89.0%?? and76.9%?? stool color:83.2%??96.1% and96.7%??blood total bilirubin:63.0%??93.1%and91.2% ?? serumγ-GT:79.7%?? 71.1%and78.7% ?? dynamic duodenal liquid color check:93.3%??91.7% and92.7%?? bile acid of duodenal liquid:97.8%??100.0% and 100.0%??B graphy :89.7%??91.7%and94.3%??porta fibre block check:72.1%??29.4%and 68.7%??nuclide hepatic imaging :60.5%??100.0% and 100.0%??MRCP:88.3%??96.5%and94.4%??liver pathology :97.4%??98.2% and 94.9%. Conclusion Differential diagnosis in 1 week can help differentiate biliary atresia from infantile intrahepatic cholestasis. B-ultrasonography and dynamic duodenal fluid test are simple?? fast practical??economic and noninvasive as differential diagnostic methods.     

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.     

Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia

Two children with incidentally-diagnosed ectopic pancreatic tissue in the jejunum at surgery for extrahepatic biliary atresia (EHBA) and choledochal cyst (CC) are reported. No case has been reported in the literature describing the association of a CC with ectopic pancreas, and only one case of EHBA associated with ectopic pancreas has been reported. We believe that incidentally-detected ectopic pancreatic tissue should be excised, even though the patient is symptom-free, in order to prevent the risk of serious complications due to either the mass effect or the potential for acute pancreatitis, cystic degeneration, or malignant transformation at a later date. Accepted: 12 December 2000     

Urinary sulfated bile acid analysis for the early detection of biliary atresia in infants

Background: Measurement of urinary sulfated bile acid (USBA) is a non‐invasive method to detect bile congestion. Our aim was to evaluate the feasibility of USBA analysis for the early detection of biliary atresia (BA). Methods: We determined the USBA‐to‐creatinine ratio (USBA/cr) in 1148 infants at 10–40 days after birth. All infants were followed until the 3‐ to 4‐month postnatal routine health check. The cutoff value for USBA/cr was 55.0 µmol/g creatinine. Results: Among the infants tested, 47 (4.10%) had USBA/cr ratios that exceeded the cutoff value. Two of these 47 infants had liver disease; one was diagnosed with neonatal hepatitis syndrome, and the other was diagnosed with BA. The BA patient underwent USBA analysis for the first time on day 18 after birth and hepatoportoenterostomy on day 49. No other infants were diagnosed with hepatobiliary disease during the follow‐up period. Conclusion: This USBA analysis provided the correct assessment without fail and identified a case of BA. This approach could be used for the screening and early detection of BA when the false‐positive rate is decreased by improving the methods for sample collection and urine storage.     

Pre-operative time course changes in liver function tests in biliary atresia: Its usefulness in the discrimination of biliary atresia in early infancy

In order to investigate the possibility of early discrimination of extrahepatic biliary atresia from other cholestatic diseases, a series of results of liver function tests in infants with cholestatic diseases were reviewed. The results of routine liver function tests (LFT) recorded in patients' charts were reviewed within 12 weeks after birth in 47 infants with extrahepatic biliary atresia (BA), 10 infants with neonatal hepatitis (NH) and 130 age-matched control infants (CO) without cholestatic diseases. The mean of each test value for each week after birth was derived from the actual data examined in each infant. No differences were observed between BA and CO in the levels of aminotransferases within 2 weeks after birth. Total bilirubin and direct bilirubin levels were significantly different between BA and CO within 1 week after birth (16.1 ± 3.2 mg/dL vs 11.1 ± 4.5 mg/dL, 4.6 ± 2.6 mg/dL vs 0.7 ± 0.3 mg/dL, respectively) The direct bilirubin-total bilirubin ratio exceeded 25% within the first week in BA. The individual values of direct bilirubin (DB) exceeded 2 mg/dL within the first week in all infants with BA, while none of the individual values exceeded 1.6 mg/dL in CO. Gamma-glutamyl transpeptidase levels were significantly different between BA and CO at 4 weeks (432 ± 272 IU/L vs 79 ± 43 IU/L) and thereafter; and were significantly different between BA and NH at 6 weeks (314 ± 232 IU/l vs 69 ± 58 IU/L) and thereafter. These data suggest that the determination of direct bilirubin within 1 week after birth can detect extrahepatic biliary atresia patients from those with physiologic jaundice, and γ-glutamyl transpeptidase levels may discriminate BA from NH at no later than 6 weeks of age.     

Outcome of biliary atresia in Malaysia: A single-centre study

Aim:   This study aimed to determine the outcome of the operation of children with biliary atresia (BA) at a tertiary paediatric referral centre in Malaysia.
Methods:   A prospective study on all patients with BA referred to the University of Malaya Medical Centre (UMMC), Kuala Lumpur, from 1996 to 2005 was conducted. Survival with native liver, liver transplantation (LT) or death at 2 years of age was determined.
Results:   The median age at referral of the 57 patients with BA seen at University of Malaya Medical Centre was 62 days. Kasai procedure was not performed in nine patients who were all referred late (median age of referral 180 days). The median age at hepatoportoenterostomy (HPE) of the remaining 48 patients was 70 days. A total of 53 (93%) patients had type 3 BA, while only 1 (2%) patient had BA splenic malformation. At 2 years, the survival rate with native liver for the 48 patients who had HPE was 37%, while the overall survival (native liver and LT) rate was 40%. Two of the four patients who had LT survived with a liver graft at 2 years.
Conclusions:   The 2-year survival with native liver following corrective surgery for BA in UMMC, Malaysia, compares favourably with other international figures, but the overall survival rate was adversely affected by a lack of timely LT. The outcome of BA in Malaysia may be further improved by increasing the awareness among child-health professionals on the importance early referral for appropriate surgery in infants suspected of having BA.     

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.     

Relationship between prognosis of biliary atresia and infection of cytomegalovirus

Background  The etiology of biliary atresia is still unknown. It is generally accepted that virus infection may be one of the important causes that lead to biliary atresia. This study aimed to illustrate the relationship between infection of cytomegalovirus and prognosis of biliary atresia. Methods  From January 2002 to March 2004, 27 patients who had undergone Kasai’s procedure because of biliary atresia were investigated for cytomegalovirus-IgG, IgM and pp65, and their mothers were also examined for confirmation of cytomegalovirus infection. The patients were divided into three groups: infection free group, cytomegalovirus positive group and cytomegalovirus infection group. The rate of jaundice disappearance and the incidence of reflux cholangitis were analyzed statistically. The histopathological changes of the liver were also analyzed. Results  The positive expression of cytomegalovirus-IgM and cytomegalovirus-pp65 in the patients was higher than that in their mothers (48% versus 14.81% and 37% versus 3.78%, respectively). Compared with the other two groups (80% in the infection free group, and 82% in the cytomegalovirus positive group), the rate of jaundice disappearance after operation in the cytomegalovirus infection group (36%) was significantly lower (P<0.05), and the incidence of reflux cholangitis was higher (P<0.05). Histopathological examination also showed that the degree of liver fibrosis and inflammation was more serious (P<0.05). Conclusions  There is a strong correlation between cytomegalovirus infection and a lower rate of jaundice disappearance, also a higher post-operational reflux cholangitis. Liver fibrosis seems to be more severe in biliary atresia patients with cytomegalovirus infection.     

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??Abstract?? Objective??To evaluate the value of biochemical markers in the diagnosis of biliary atresia ??BA?? and neonatal intrahepatic cholestasis caused by citrin deficiency ??NICCD??. Methods??Totally 77 infants in hospital with infantile hepatitis syndrome ??IHS?? were enrolled from December 1?? 2008 to March 31?? 2009. Totally 27 patients were diagnosed as having BA and 11 with NICCD. Biochemical markers were compared between groups including alanine transaminase ALT?? aspartate transaminase AST ?? alkaline phosphatase ALP?? γ-glutamyl transpeptidase γ-GT?? total bilirubin TB?? direct bilirubin DB?? total bile acid TBA?? total cholesterol TC??to compute ALT/AST??ALP/γ-GT and glucose GLU?? lactic acid LAC?? total protein TP?? albumin ALB in the NICCD group. The data were analyzed by T test and ROC curve with SPSS10.0. Results??γ-GT was significantly elevated in the infants with BA when compared to non-BA group ??P = 0.003???? cut-off point was 332.5U/L. ALP/γ-GT was significantly lower in the patients with BA??and cut-off point was 1.93. The infants with NICCD had significantly different biochemical markers including GLU?? LAC?? TP?? ALB?? ALT/AST and γ-GT. Conclusion??Biochemical markers could be considered as complementary diagnosis of BA and NICCD for differentiating infants with IHS.     

肝移植治疗胆道闭锁:附16例报告

目的 探讨肝移植治疗胆道闭锁的疗效.方法 对16例胆道闭锁的患儿实施肝移植手术,其中7例为劈离式肝移植,9例为活体部分肝移植.术后所有患儿均给予抗排斥、抗感染、抗凝及营养支持等治疗,密切监测移植肝血流及肝功能变化,观察术后并发症及预后.结果 16例胆道闭锁患儿全部手术存活,术后早期主要并发症为感染,以细菌感染为主,感染部位主要为腹腔及肺部.死亡2例,1例术后9 d死于感染性休克,1例术后1年死于严重EB病毒感染、溶血性黄疸;其余14例患儿随访1 ～ 4.5年(至2010年12月),检测肝功能良好.结论 肝移植是治疗胆道闭锁安全有效的方法.     

胆道闭锁肝内外胆系组织病理形态学分析

目的 通过胆道闭锁（BA）肝门纤维块、肝脏组织的病理及其超微结构观察，对肝门成纤维细胞分化程度进行评分，并与肝纤维化分级进行相关分析。方法 选取BA患儿作为研究对象，术中取肝门纤维块及肝脏组织标本；研究同期选取疑似BA经术中胆道造影除外BA，诊断为胆汁淤积综合征和先天性胆管扩张症患儿作为对照组，留取肝脏组织标本。在光镜和电镜下观察标本的病理改变，以及肝细胞、毛细胆管和肝门成纤维细胞的超微结构。采用SPSS 14.0软件，半定量比较BA与对照组肝脏纤维化的差异，检验肝门纤维块成纤维细胞活跃程度与肝纤维化分级的相关性。结果 2005年7月至2006年5月复旦大学附属儿科医院收治的21例BA Kasai根治术病例，手术平均年龄（66±20）d；对照组为5例胆汁淤积综合征和10例先天性胆管扩张症患儿。BA组肝组织病理改变主要是肝内门脉区胆管炎症及纤维化形成，肝纤维化程度明显高于同年龄胆汁淤积综合征和先天性胆管扩张症患儿；肝门纤维块毛细胆管增生，部分管腔闭锁、狭窄，腔内炎细胞浸润及部分淤胆，大量间质成分增生；电镜下肝门成纤维细胞活跃、肝脏毛细胆管上皮微绒毛缺失、肝细胞及肝血窦内电子致密物质增多及部分毛细胆管扩张；肝门成纤维细胞分化程度与肝组织纤维化程度相关（P=0.04）。结论 BA肝组织病理改变主要是肝内门脉区胆管炎症及严重纤维化形成；超微结构改变提示肝门部成纤维细胞活跃，其分化程度与肝纤维化程度相关。     

晚发性维生素K缺乏所致颅内出血8年回顾与分析

目的 　了解晚发性维生素K缺乏所致的严重出血 ,特别是颅内出血 ,预后凶险 ,如治疗不及时 ,不仅病死率高 ,而且存活者可能有不同程度的神经系统后遗症等临床特点及如何防治。方法 　从 1 996年 1月～ 2 0 0 3年 1 2月 ,8年间大连市儿童医院共救治 1 1 9例患儿 ,现进行临床总结。结果 　早期住院治疗应用维生素K1者 ,凝血酶原时间恢复正常的时间短、易治愈。本组 6 1例治愈 (占 51 2 % ) ;因经济困难好转4 0例 (占 33 6 % ) ,放弃治疗 1 1例 (占 9 3% )。因发病时间长、就诊时间晚 ,失去抢救时机而死亡 7例 (占 5 9% )。结论 　本病病因是维生素K缺乏 ,母乳喂养易发生本病。慢性腹泻、维生素K依赖是常见的临床症状之一。当体内凝血酶原减低时 ,可见出血倾向 ,颅内出血以蛛网膜下腔出血为主。如果早期就医、确诊 ,是可以治愈的。如果早期预防 ,在出生后、1个月、3个月各注射 1次维生素K11～ 3mg效果最佳 ,可以减少本病的发生。