Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?

OBJECTIVES: The prognosis of hepatoportoenterostomy (the Kasai operation) for biliary atresia worsens when the age at surgery increases. This study examines whether the Kasai operation remains justified after 3 months of life. STUDY DESIGN: Records for all patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed, and patients were classified into 3 groups: group 1 (n = 30), no contraindication to the Kasai operation, but orientation to de novo transplantation; group 2 (n = 380), age at Kasai operation <90 days; and group 3 (n = 60), age at Kasai operation > or =90 days. Survival with native liver, survival after liver transplantation, and overall survival (Kaplan-Meier method) were compared by using the log-rank test. RESULTS: Five-year (10-year) survival with native liver was 35% (30%) in group 2 and 25% (22%) in group 3 (P =.03). Five-year overall survival was 57%, 74%, and 55% in groups 1, 2, and 3, respectively (P =.003). Poor results in groups 1 and 3 were mainly due to increased pre-transplantation mortality, but survival after transplantation was not significantly different in the 3 groups. CONCLUSIONS: Performance of the Kasai operation after 3 months of age is justified in selected cases, because it may obviate liver transplantation. Preoperative evaluation should exclude patients with advanced liver disease for whom liver transplantation should not be delayed.     

Biliary atresia: perspective on transplantation

In North America, long-term, jaundice-free survival following Kasai's operation for biliary atresia is 25%–35%; thus, the majority of patients require liver transplantation for salvage. The timing of organ replacement is crucial. Patients without bile drainage because of either surgical election or operative failure should be referred immediately. Patients with ongoing but inadequate biliary drainage should be referred at a time that coincides with a plateau of the growth curve or if cholangitis is recalcitrant. Portal hypertension itself is not an indication for transplantation irrespective of its manifestations. Ascites, however, when primarily due to hypoalbuminemia, is a solid indication for transplantation. Other tests of liver function are generally unreliable. Factors found to be unimportant or of questionable adverse impact on transplant outcome include previous operation, coexisting infection limited to the liver, nonpatent portal vein, and/or abnormal liver function tests. The only factor that was found to significantly influence outcome was patient size and age. Thus, bile drainage after Kasai portoenterostomy, by permitting growth, improves the chances for a favorable outcome. Transplant 1-year survival is from 60% to 88%. Liver replacement is not without complications, namely technical errors, infection, and rejection. Nonetheless, in a majority of cases hepatic transplantation provides the only opportunity for high-quality long-term survival. Offprint requests to: R. J. Hall     

胆道闭锁Kasai术后肝脏纤维化及生化指标变化趋势的横断面研究

目的 研究胆道闭锁患儿Kasai术后肝脏纤维化及生化指标的变化趋势.方法 采用横断面研究方法,对2013年1月至2013年12月于首都医科大学附属北京儿童医院普外科随访的胆道闭锁Kasai术后患儿进行复查资料收集,术后随访时间6个月以上,资料完整的121例纳入研究.按照Kasai术后胆红素降至正常的时间,以术后3个月、6个月为界分为优、良、差三组,获取患儿术时年龄、术前和术后Fibroscan肝脏硬度测量值及生化结果,绘制趋势图并进行统计学分析研究各组患儿Kasai术后肝脏纤维化及生化指标的变化趋势.结果 纳入本研究胆道闭锁患儿共121例,男69例(57.0％),女52例(43.0％);其中优组63例(52.1％),良组34例(28.1％),差组24例(19.8％).三组患儿术时年龄差异无统计学意义.三组患儿Fibroscan肝脏硬度测量值在术后1年内波动进展,1年后趋于稳定;单因素方差分析及秩和检验结果显示优良组差异无统计学意义,优良组与差组患儿Fibroscan肝脏硬度测量值在术后1年内差异均有统计学意义.优良组患儿总胆红素在术后前3个月下降较快,随后缓慢下降,至术后6个月稳定于正常值,而差组患儿总胆红素术后前2个月下降较快,但随后出现较大波动.三组患儿ALT趋势图均在术后1个月出现波峰,优良组术后1～6个月有所下降,但之后持续波动不能稳定,差组持续波动;三组患儿AST术后6个月内有所下降,但之后波动较大不能稳定.三组患儿CGT术后1个月达到最高峰,至术后6个月下降较快,随后逐渐趋于稳定小幅波动.结论 ①胆道闭锁患儿Kasai术后1年内肝脏纤维化波动性进展,自肝存活超过1年者肝纤维化情况进展缓慢趋于稳定,优组患儿在2年后更加稳定;②Kasai术后1个月内胆红素下降最快,3～6个月胆红素可降至正常的患儿,其胆红素水平可维持长期正常;③肝脏炎症、胆管反应、功能损害在术后仍继续进展,术后1个月开始减轻,术后6个月趋于稳定.     

Long-term prognosis of patients with biliary atresia: a 25 year summary

OBJECTIVE: The purpose of this study was to delineate the long-term prognosis of biliary atresia (BA) in Taiwan. STUDY DESIGN: From 1976 to 2000, 185 children were diagnosed with BA, 22 underwent exploratory laparotomy without Kasai operation, and 163 underwent Kasai operation, of which 141 cases had long-term follow-up and formed the basis of this study. The outcome was analyzed. RESULTS: Among the 141 BA children studied who underwent Kasai operation, 115 (81.6%) had recoloration of stools, and 86 (61.0%) became jaundice-free (bilirubin <34 micromol/L) [corrected]. The resolution of jaundice and the absence of repeated cholangitis contributed to better outcome. Five and 10 year survival rates with native liver were 35% and 31%, respectively. Liver transplantation was performed in 19 patients (all but 2 with a living-related donor), and 15 (79%) survived. Five and 10 year overall survival rates for BA patients were 41.9% and 40.2%, respectively. CONCLUSIONS: The study delineated the long-term outcome of BA in an Asian country other than Japan. Survival with native liver after a Kasai operation in Taiwan was similar to that in the American and European series. Limited donors for liver transplantation in the years of the study accounted for the poor overall prognosis of BA patients in this series.     

Long-term results of biliary atresia in the era of liver transplantation

Purpose

The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival.

Methods

Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed.

Results

Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002).

Conclusions

Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.     

Early cytomegalovirus infection and the long-term outcome of biliary atresia

Aim:  To study the impact of ongoing cytomegalovirus (CMV) infection at presentation of biliary atresia (BA) on the long-term outcome after Kasai procedure.
Methods:  Twenty-eight patients with BA born 1988–1997 were included and followed-up until 2007. Eleven patients (group A) had ongoing CMV infection at presentation and were compared to the remaining 17 patients (group B). Median age at Kasai procedure was 75 days in group A and 70 days in group B (p = 0.12).
Results:  Including all patients, survival with native liver was 50% and 36% at 4 and 10 years of follow-up respectively. At the end of follow-up, it was 25% and overall survival was 68%. When comparing groups A and B, neither difference in survival with native liver (p = 0.67, log-rank test) nor in survival after liver transplantation was detected.
Conclusion:  Survival with native liver after Kasai procedure is comparable to that of other centres. CMV positive patients may present with a later onset, alternatively the detection of CMV infection could delay the referral of BA patients. No significant differences in long-term outcome were detected with regard to early CMV infection.     

Analysis of the prognostic factors of long-term native liver survival in survivors of biliary atresia

Purpose

Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure.

Methods

This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis.

Results

The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors.

Conclusions

The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.

     

Liver transplantation for biliary atresia: a systematic review

Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan. The 1-, 5-, 10-, 15- and 20-year survival rates for the patients and grafts undergoing living donor LT were 91.6, 91.5, 87.1, 85.4 and 84.2 and 90.5, 90.4, 84.6, 82.0 and 79.9%, respectively. LDLT was able to be performed even in patients weighing less than 5 kg with early liver failure following a Kasai operation using a reduced left lateral segments. As LT has been revealed to increase the donor pool and decrease the waiting list mortality with an excellent long-term graft survival, early referral to a transplant center should be considered when at least one complication of cirrhosis occurs during its natural history, especially in adolescents.     

Biliary atresia and liver transplantation: results and thoughts for primary liver transplantation in select patients

Biliary atresia (BA) is one of the most common indications for liver transplantation in children. Despite advances in biliary atresia surgical techniques, most children will ultimately require liver transplantation. Possible pre-operative predictors of outcome after the Kasai operation are: 1. Age at operation 2. Presence of the biliary atresia splenic malformation syndrome (BASM) 3. Center specific factors 4. Liver histology and 5. Anatomic pattern of bile ducts found at surgery.Age at surgery is considered a strong predictor of success after portoenterostomy. In a recent study, age of 75 days or more at surgery was associated with less frequent resolution of jaundice and decreased transplant free survival. Similarly, the Ohi type II or III anatomy was associated with a higher risk of transplantation or death than type I. Inflammatory findings on pre-operative biopsy predicted a pooreroutcome after a Kasai procedure than obstructive changes. Nodularity of the liver at surgery as well as ascites was associated with a poorer prognosis.Primary transplantation is rarely done despite excellent outcome. Deaths on the waiting list also have improved with routine use of split and live donor transplantation. The Kasai operation has the highest failure rate in its stated objective than any other operation in pediatric surgery. Failure to achieve any improvement in jaundice occurs in over 30% of all cases, even in the best of hands, and transplantation or listing for transplantation occurs in over half the children with type II and III BA by one year of age in countries where liver transplantation is readily available.There are almost no studies in children with BA that compare the outcome after liver transplantation for BA with or without a prior Kasai procedure. It is postulated that a prospective trial in children predicted to have a poor prognosis after the Kasai procedure based on anatomic pattern, liver histology and presence of BASM, would yield improved care, spare some infants needless surgery, and quite possibly result in diminished morbidity and mortality following liver transplant.     

Early predictors of success of Kasai operation in children with biliary atresia.

AIM: Aim of the study was the evaluation of early predictive parameters of event-free survival (not listed for liver transplantation, not transplanted, no death) in children suffering from biliary atresia after hepatoportoenterostomy (Kasai procedure) in order to optimize pretransplant management. PATIENTS AND METHODS: Sixty-seven infants were treated with the Kasai operation at our institution over a 20-year period from 1978 until 1998. Median age at time of operation was 51 days after birth (range 19 - 180 days). Of these 67 infants, 24 children with complete datasets and an observation time of at least one year were evaluated retrospectively using a Cox regression model. The response variable was event-free survival after a median observation time of 4.9 years (1.11- 10.37 years). Six variables were entered as covariates: alanine aminotransferase (ALAT), cholinesterase activity, bilirubin, age at the time of Kasai operation and tracer excretion and uptake during hepatobiliary scintigraphy (HBSS). All variables were evaluated six weeks after operation. For subsequent cut-off determination, a receiver operating analysis (ROC analysis) was carried out. RESULTS: Tracer excretion shown by HBSS showed the highest prognostic power to predict event-free survival after Kasai operation (log rank 18.68, p < 0.0001) followed by bilirubin and ALAT as further significant parameters in the first univariate step of the Cox regression model. In the subsequent multivariate step, the prognostic power of HBSS was improved only by bilirubin (log rank 24.6, p < 0.0001). The ROC analysis determined a cut-off for bilirubin concentrations of 57 micromol/l for event-free survival with a sensitivity of 80 % and a specificity of 78.6 %. The five-year event-free survival-rate was 100 % in the group with good tracer excretion and a bilirubin concentration of 57 micromol/l and 27 % for the other group (log rank test, p < 0. 0001). CONCLUSION: Early predictors of success of the Kasai operation in children with biliary atresia are free tracer excretion as shown by HBSS and a serum bilirubin concentration < 57 micromol/l six weeks after the operation. Thus, children with bilirubin concentrations above this level should be carefully and frequently monitored with regard to a transplantation requirement in order to optimize pretransplant management.     

Biliary atresia: the Croatian experience 1992–2006

The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and treated at a single university center were reviewed. Survival rates were calculated with the Kaplan–Meier method. Twenty-nine patients with biliary atresia were identified. Incidence was one in 23,600 live births (95% confidence interval 1/17,400–1/27,200). The median age at Kasai operation was 66 days (range 22–192). Median follow-up was 2.65 years (range 0.2–14.3). Overall survival rates for the patients who underwent Kasai portoenterostomy, including those six (20%) who subsequently underwent liver transplantation, were 75.6%. Five and 10-year native liver survival rates were 51.7% and 38.8%, respectively (median survival time was 7.88 years). Survival rate curves in two groups of patients according to the biliary atresia phenotype (fetal or perinatal form) were divergent. Survival was 87.7% in perinatal form and 43.8% in fetal form (Breslow chi-square 8.082, p < 0.01). Overall survival rates of patients with biliary atresia in Croatia compared unfavorably with current international standards; this could be improved with earlier referral for liver transplantation. Results of Kasai operation (native liver survival rates) compared favorably with results reported elsewhere.     

Evaluation of a standardized protocol in the use of steroids after Kasai operation

Although the Kasai operation is still the treatment of choice for infants with biliary atresia, the long-term success rate, as defined by survival without transplantation, is only about 25-40%. It has been proposed that post-operative inflammatory changes affect the bile flow and eventually lead to cholangitis and liver failure. Recent case reports have suggested that the administration of steroids post-operatively can improve outcomes. Since 2004, our unit has adopted a strict protocol for the use of post-operative steroids for patients who undergo Kasai operation. The aim of this study is to access the early outcomes of these patients. A retrospective analysis was carried out for all patients who received Kasai operation between 1996 and 2006. For the treatment group, patients all received prednisolone at 4 mg/kg 1 week after operation as guided by protocol. The demographics and outcomes, including post operative bilirubin level, episodes of cholangitic attack, the need for early liver transplantation (transplant within 1 year of Kasai), and transplantation-free survival, were noted. Statistical analysis was done using Fisher's exact test and unpaired t-test when appropriate. A value of P < 0.05 was considered to be statistically significant. Kasai operation was performed in 30 patients (11 boys and 19 girls) during the study period. Thirteen patients received post-operative prednisolone according to protocol. The average age at operation and the mean preoperative bilirubin levels for the steroid and non-steroid group were not significantly different. A normal post-operative bilirubin (defined as bilirubin level less than 20 mumol/L) was achieved at 6 months in 7 (53.9%) patients who received steroid and 8 (47.0%) patients who did not (P = 0.71). A statistically significant reduction in the post-operative bilirubin level was also seen at 3 and 6 months in the steroid group. Early liver transplantation was required in 5 (38.5%) patients with steroid and 5 (29.4%) patients without it (P = 0.60). No significant difference in terms of cholangitic attack was observed. There was also no steroid-associated complication reported. We conclude that lower post-operative bilirubin level can be achieved with the routine use of prednisolone. However, there is no statistical improvement in terms of early liver transplantation and cholangitis. This may be attributed to the small sample size of our study population. Based on this pilot study, a multi-centre randomized trial is needed.     

Laparoscopic versus open Kasai portoenterostomy in infant with biliary atresia: a retrospective review on the 5-year native liver survival

Purpose

Laparoscopic Kasai portoenterostomy was reported to be a safe and feasible procedure in infant with biliary atresia. We aimed to investigate the long-term results after laparoscopic portoenterostomy as such data in the literature are lacking.

Methods

Sixteen infants underwent laparoscopic Kasai portoenterostomy from 2002 to 2006. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20?μmol/L within 6?months of portoenterostomy), the native liver survival at 2 and 5?years after the operation were reviewed. The results were retrospectively compared with 16 consecutive infants who underwent open Kasai portoenterostomy before 2002.

Results

All infants had type III biliary atresia. The early clearance of jaundice rate at 6?months was 50?% (8/16) after laparoscopic operation and was 75?% (12/16) after open operation (p?=?0.144). Two?years after the operation, the native liver survival was 50?% (8/16) in the laparoscopic group and was 81?% (13/16) in the open group (p?=?0.076). Five?years after the operation, the native liver survival rate was 50?% (8/16) in the laparoscopic group and was 81?% (13/16) in the open group (p?=?0.076). The jaundice-free native liver survival rate at 5?years was 50?% (8/16) in laparoscopic group and was 75?% (12/16) in the open group. In the laparoscopic group, all patients with early clearance of jaundice survived and remained jaundice freed 5?years after the operation.

Conclusion

The 5-year native liver survival rate after laparoscopic portoenterostomy was 50?%. Apparently superior result was observed in the open group (81?%) although the figures did not reach statistical difference because of the small sample size. A larger scale study is required to draw a more meaningful conclusion.     

Indications for the Kasai operation: experience with 103 Suruga II modifications

103 patients with biliary atresia were treated by the Suruga II operation. A close correlation was found between degree of liver fibrosis, the size of the ductuli and prognosis. Thus, it is often possible to predict the prognosis at the initial operation by evaluating frozen sections. In group I (large ductuli, mild fibrosis) a Kasai operation or a modification is recommended. For group II (smaller ductuli, moderate fibrosis) the necessity of liver transplantation is increased. Kasai operation without stoma is the appropriate procedure. For group III (small ductuli, marked fibrosis), Kasai operation should be avoided, but liver transplantation has to be considered. Offprint requests to: T. Miyano     

Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation

Purpose

This study was conducted to reappraise the efficacy of redo-Kasai (or revision) in the era of liver transplantation as a treatment option in those patients with recurrent jaundice after initially successful Kasai procedure.

Methods

We studied ten patients that received redo-Kasai, among a total of 102 patients diagnosed with biliary atresia after receiving Kasai operation from 1986 to 2011.

Results

Kasai operation was done at a median age of 55?days and redo-Kasai at 150?days. The bilirubin levels returned to normal in six patients after the procedure. Four of six enjoyed jaundice-free survival with native liver till the time of last follow-up. Three patients died and three received liver transplantation (LT). Only one out of seven patients with three or more episodes of cholangitis survived with native liver, while all the three patients with 1 or 0 episode survived with native liver. The difference was significant (P?=?0.033). Re-do Kasai did not result in more blood loss or operative time during LT.

Conclusion

Redo-Kasai is still valuable in the era of LT and the episodes of cholangitis are the decisive factors affecting the outcome of the procedure.     

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.     

胆道闭锁Kasai手术后自体肝生存时间小于2年的危险因素分析

目的探讨胆道闭锁(biliary atresia,BA)Kasai手术后自体肝生存(native liver survival,NLS)时间小于2年的危险因素。方法回顾性分析山西省儿童医院2009年1月至2017年6月行Kasai手术的BA患者临床资料及随访资料。根据术后NLS时间是否小于2年分为死亡组(n=78)和生存组(n=46)。将BA分型、性别、巨细胞病毒(cytomegalovirus,CMV)感染、手术年龄、术后使用抗生素方案、术后使用激素方案、是否合并早期胆管炎及术后黄疸是否消退作为自变量,采用Kaplan-Meier法绘制生存曲线并进行Log-rank检验,采用Cox比例风险模型(逐步前进法)筛选BA患者Kasai手术后NLS时间小于2年的危险因素。结果共纳入124例BA患者,其中生存组46例,死亡组78例,2年NLS率为37.1%。单因素分析结果显示合并早期胆管炎及术后黄疸消退是影响BA患者术后2年NLS率的因素(P<0.05)。Cox比例风险模型分析结果显示术后3个月内黄疸未消退为BA患者Kasai手术后NLS时间小于2年的独立危险因素,术后3个月内黄疸未消退组患者NLS时间小于2年的风险是黄疸消退患者的5.65(95%CI:2.45~13.04)倍。结论BA患者Kasai手术后3个月内黄疸未消退是NLS时间小于2年的危险因素。     

胆总管未闭锁型胆道闭锁的手术治疗及疗效分析

目的 分析24例胆总管未闭锁型胆道闭锁的临床疗效,旨在指导临床对此类胆道闭锁的手术方式进行选择.方法 回顾分析我院1995至2006年间收治的24例胆总管未闭锁型胆道闭锁患儿手术、术后病理及随访资料.对空肠肝门吻合(A组)与胆囊肝门吻合(B组)在自体肝生存率、黄疸清除率、反流性胆管炎发生率及临床疗效等方面比较分析;并对胆道闭锁胆囊病理标本及正常对照组胆囊进行胶原纤维Van Gieson染色和CD68免疫组化染色,比较其纤维化及炎性反应的差异.结果 平均(51±29.02)个月随访期内,自体肝生存率为66.7%.A组自体肝1年生存率为78.6%,3年生存率78.6%;B组1年生存率为66.7%,3年生存率33.2%,两组3年生存率差异有统计学意义(P＜0.05);A组术后6个月黄疸清除率明显高于B组(86.7%比33.3%,P＜0.05);术后1年胆管炎发生率,A组为60.0%,B组为44.4%(P＞0.05).胆道闭锁患儿胆囊表现为明显纤维化和CD68阳性细胞浸润.结论 胆囊肝门吻合治疗胆总管未闭锁型胆道闭锁的疗效较肝门空肠吻合差,可能与胆囊肝门吻合后残余胆道存在纤维化及炎症细胞浸润有关,对其治疗推荐采用肝门空肠吻合.     

Biliary atresia in Turkish children

AIM: To evaluate surgical success, survival rate and relationship of outcome parameters with time at diagnosis and operation of extrahepatic biliary atresia (EHBA) patients in Izmir, Turkey. METHODS: Clinical and laboratory data were reviewed from case reports of 27 EHBA patients. Twenty-five patients were operated on using Kasai procedure and two cases received liver transplants without portoenterostomy due to decompansated liver cirrhosis on diagnosis. Post operational success was defined as clearance of jaundice (bilirubine level <2 mg/dL). Patients were studied in two groups: I (jaundice free and/or compensated liver disease with liver transplant if needed after 3 years of age) and II (progressive liver disease with death or liver transplant if needed before 3 years of age). Kasai success, age at diagnosis and operation, survival and correlation of outcome with age, preoperative liver functions were evaluated. Eight patients received liver transplants. RESULTS: Median age at diagnosis was 63.5 days (21-212) and portoenterostomy was performed at median age of 67.5 days (25-220). Kasai operation was successful in two cases (8%) and the operation was performed at 35 and 42 days. Age at diagnosis (P = 0.13) and operation (P = 0.2) was not different between the two groups. Group I and II consisted of seven (28%) and 18 (72%) infants. Pre-existing ascites, serum alanine aminotransferase (ALT) and globulin levels were significantly higher in group II patients (P = 0.008, P = 0.04, P = 0.017, respectively). CONCLUSIONS: The results of the present study indicate that general practitioners should pay close attention to the evaluation of infants with prolonged jaundice in Turkey. Because of frequent late diagnosis presenting with cirrhosis at admission and also because of the low organ donation rates in Turkey, living related liver transplantation is an option and is currently undergoing detailed ethical consideration.     

Different polyunsaturated fatty acid profiles in patients with biliary atresia after successful Kasai operation and liver transplantation

Background  Although Kasai operation eliminates jaundice in patients with biliary atresia, inflammation at Glisson’s area persists and fibrosis advances slowly to liver cirrhosis. Profiles of polyunsaturated fatty acids, on which metabolic products have an immunoregulative effect, were investigated in this study. Methods  Blood samples were obtained from patients including 21 jaundice-free patients with biliary atresia after Kasai operation (native liver group) and 18 patients in whom Kasai operation failed and liver transplantation was performed (transplantation group). All of these patients are without any complications for more than 6 months. Blood samples from 18 patients with inguinal hernia were assigned as normal controls (control group). Profiles of fatty acids in whole serum lipids were measured using gas chromatography. The n-3/n-6 ratio of polyunsaturated fatty acids was compared among the three groups. Results  The n-3/n-6 ratio was 0.114 in the native liver group, which was significantly lower than 0.145 in the transplantation group (P = 0.009) and 0.158 in the control group (P = 0.004). There was no significant difference between the transplantation and control groups (P = 0.83). Results  The n-3/n-6 ratio was abnormal after Kasai operation despite normal liver function. This seems to be one factor of persisting inflammation in Glisson’s area.