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.     

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.     

胆道闭锁患儿Kasai术后早期胆管炎相关危险因素分析

目的 探讨影响胆道闭锁患儿(biliary atresia,BA)经典Kasai术后早期胆管炎发生的相关因素.方法 对本组中35例BA患儿的临床资料应用二项分类logistic同归分析方法作回顾性分析.结果 胆道闭锁患儿术后近期胆管炎发生与术后胆汁引流效果、术后辅以激素治疗及术中预留胆支长度有显著相关性,而与患儿性别、手术日龄、术前总胆红素及肝功能、手术前后辅以熊去氧胆酸和苯巴比妥利胆退黄治疗、术中设置防反流瓣及术后抗感染力度等因素无关.结论 胆道闭锁患儿Kasai术后胆汁引流效果好,辅以激素治疗,同时术中预留胆支长度充分的BA患儿,其术后不易发生早期胆管炎;反之,患儿发生早期胆管炎风险增加.

Abstract：

Objective To evaluate the factors affecting the early post-operative cholangeitis after classical Kasai operation for biliary atresia (BA) in children. Methods The clinical data of thirty-five BA children from pediatric surgery department of Nanjing Childrens Hospital was retrospectively analyzed using binary logistic regression analysis. Results The occurrence of early cholangitis after classical Kasai operation was found to correlate with post-op biliary drainage effect, hormone therapy and the length of Roux-loop in operation. No correlation could be established between early cholangeitis and sex,age, total bilirubin and pre-op liver function, ursodeoxycholic and luminal therapy, the installation of anti-refluxing valve in operation and the dosage of antibiotics. Conclusions Good biliary drainage,hormone therapy and sufficient length of Roux-loop were associated with decreased risk of early cholangitis.     

A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy

A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3±7.2 mg/dl versus 3.5±3.1 mg/dl), ALT (136±89 U/l versus 92±88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group ( P <0.05). A score of 8 had a high sensitivity (96.9%) and specificity (89.5%) for predicting the need for liver transplant. Conclusion:based on easily available clinical information, our scoring system can predict which patients with biliary atresia who have already undergone a Kasai procedure should be considered for liver transplantation.Abbreviations EHBA extrahepatic biliary atresia - ROC receiver operating characteristic     

胆道闭锁Kasai术后早期并发症的分析

目的 分析胆道闭锁Kasai术后早期并发症与激素抗生素治疗方案、生存预后的关系.方法 回顾性分析1994年8月至2008年8月Ⅲ型胆道闭锁Kasai术后281例临床资料.根据治疗方案分为一般治疗A组和术后大剂量激素和抗生素治疗B组.随访术后2年生存,分析早期并发症与手术日龄、术前胆红素、术后激素抗生素治疗方案、生存预后之间的关系.结果 术后共93例(33.1%)发生早期并发症,急性胆管炎最常见.无并发症与并发症相比,术前总胆红素及手术日龄无差异;危险因素分析,急性胆管炎、上消化道出血分别与术前胆红素及手术日龄无明显相关.B组并发症较A组明显降低(25.7%比63.6%,P＜0.01),其中反流性胆管炎较A组明显降低(23.9%比56.4%,P＜0.01),但术后早期消化道出血无统计学差异.2年随访率92.5%,术后2年以上存活率51.2%,早期并发症2年生存率较无早期并发症明显降低(34.4%比59.6%,P＜0.01).急性胆管炎2年生存率亦明显降低(32.9%比59.2%,P＜0.01).B组2年生存率高于A组(54.0%比40.0%,P=0.043＜0.05).结论 胆道闭锁Kasai术后早期急性胆管炎是影响术后生存的危险因素;术后大剂量应用激素和抗生素疗法明显降低早期急性胆管炎发生率,提高术后2年生存率.

Abstract：

Objective The purpose of this study was to evaluate the relation between early complications of Kasai operation and high-dose steroids and antibiotics and to demonstrate the prognosis of biliary atresia(BA). Methods 281 patients diagnosed with biliary atresia(BA) type Ⅲ,admitted from Aug. 1994 to Aug 2008 were retrospectively reviewed and followed up for two years. The patients were divided into two groups. Patients in Group A were controls while patients in Group B were treated with high-dose steroids and antibiotics. We evaluated the relation between early complications and age at operation (days), baseline bilirubin, different treatments and 2 year survival. Results 93 patients had early complications,and cholangitis is the most common, There is no statistics difference of complications in age at operation and baseline bilirubin. The complication rate was 63. 6% in group A, and 25. 7% in group B(P＜0. 01). The cholangitis rate was 56. 4% in group A and 23. 9% in group B (P＜0. 01). Two groups showed no significant difference in digestive tract bleeding. The visit frequency rate was 92. 5%, while 2 year survival rate was 51. 2%. Patients with early complications or cholangitis have a 34. 4% or 32. 9% 2 year survival rate, while those without have a 59. 6% or 59. 2%(P＜ 0. 01). Patients with high-dose treatment in group B have a better 2 year survival rate (54. 0% vs 40. 0% ,P＜0. 05). Conclusions Early cholangitis was the risk factor of outcome after Kasai operation. These data implied that the use of high-dose steroids and antibiotics can lower the occurrence of cholangitis and elevate 2 year survival rate.     

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.     

Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis

Purpose

Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis.

Methods

A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason.

Results

We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23–12.89, p?<?0.00001; odds ratio: 25.88, 95% CI 2.83–236.84, p?<?0.004; odds ratio: 15.05, 95% CI 2.70–83.93, p?=?0.002, respectively).

Conclusion

The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

     

Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy?

Kasai portoenterostomy has been the treatment of choice for neonates with biliary atresia since its introduction. With the advance in laparoscopic techniques, a few centers have reported the feasibility of performing laparoscopic Kasai portoenterostomy. However, the outcome of this new technique is not known. Here, we aim to evaluate, as the only referral center for liver transplantation, our experience with patients referred for transplantation after failed Kasai portoenterostomy. A retrospective study was carried out between October 1996 and September 2005. The records of all patients with the diagnosis of biliary atresia were retrieved. The type of procedure and clinical outcome of the patients were noted. Early failure of Kasai enterostomy was defined as the need for liver transplantation within 1-year post-Kasai operation. For the period studied, a total of 72 patients with biliary atresia were identified. Sixty-three of the 72 patients had their Kasai portoenterostomies performed openly while nine patients underwent laparoscopic Kasai portoenterostomy in a center experienced in laparoscopic surgery. Six of these patients were referred for transplantation within 1 year, giving the early failure rate of 66.6%. In comparison, the early failure rate for open Kasai procedure was 38.5%. Regarding post-operative complications, one patient who underwent laparoscopic Kasai procedure also suffered intestinal volvulus after initial surgery and another was found to have internal herniation of the Roux loop. Laparoscopic Kasai portoenterostomy seems to be associated with more post-operative complications and worse early clinical outcome. As a result, we remain guarded about the present-day technique of laparoscopy for biliary atresia.     

胆道闭锁多中心综合诊断治疗方案研究

目的 探讨胆道闭锁综合诊疗方案应用的有效性和安全性.方法 根据具有地域代表性的四家儿童医院专家确定的胆道闭锁诊疗方案,2007年1月开始对胆道闭锁进行诊断及治疗.收集2004年1月至2006年12月四家医院经确诊为胆道闭锁并进行根治手术的病例作为对照组2007年1月至2009年12月病例作为观察组.使用标准化量表统计首次就诊年龄、手术年龄、术前及术后3个月,6个月和1年黄疸清除率,术后胆管炎发生率,2年自体肝生存率.结果 符合诊断标准498例,实施Kasai术456例,其中对照组184例,手术168例;观察组314例,手术288例.二组首次就诊年龄无明显差异[(33±27)d比(34±26)d,P＞0.05],使用标准化诊断方案后平均手术年龄由(64±24)d下降为(74±31)d,P＜0.05;观察组术后3个月、6个月及1年退黄率(53.9%、56.9%、58.0%)较对照组(38.9%、39.9%、43.4%)明显提高(P＜0.05);其术后胆管炎发生率亦显著降低(31.8%比47.8%,P＜0.05);对照组2年自体肝生存率39.3%,观察组56.1%(P＜0.05).结论 标准化胆道闭锁综合诊断治疗方案可降低手术年龄、提高术后黄疸清除率、减少术后反流性胆管炎,显著提高术后2年自体肝生存率.

Abstract：

Objective To evaluate the efficacy and safety of a standardized protocol for the diagnosis and treatment of biliary atresia (BA) in children.Methods This study enrolled 498 patients from 4 hospitals diagnosed with BA from January 2004 to December 2009.The BA patients of the standardized protocol group,who enrolled in this study after January 2007,were diagnosed and treated using the standardized protocol.The rest of the patients were taken as controls.The patients were followed up for 2 years.The age of the first diagnosis,the age at operation,jaundice clearance,the occurrence of cholangitis and two year survival rates with native liver were retrospectively reviewed and compared between the 2 groups.Results Among the 498 patients,456 patients underwent Kasai procedure.Age of the first diagnosis between the two groups showed no difference (33 ± 27 vs 34 ± 26 days,P＞0.05),but the patients of the standardized protocol group received surgeries at earlier ages (64 ± 24 vs 74 ± 31 days,P＜0.05).The jaundice clearance rates of 3,6 and 12 months after operation in the patients of the standardize protocol group were higher than those of controls (53.9%,56.9%,58.0% vs 38.9%,39.9%,43.4%,P＜0.05).Patients of the standard protocol group had lower occurrence of cholangitis (31.8% vs 47.8%,P＜0.05),and better two years survival rate (56.1% vs 39.3%,P＜0.05).Conclusions The standardized protocol for the diagnoses and treatment of BA improves the clinical outcomes of the children with BA.     

Surgery for biliary atresia--is there a European consensus?

Initial surgical therapy of biliary atresia (BA) consists of the radical excision of extrahepatic remnants and portoenterostomy. However, despite this procedure, which was introduced by Kasai, BA remains the commonest indication for paediatric liver transplantation. The goal of the work group on the technical aspects of Kasai portoenterostomy procedures during the European Biliary Atresia Registry Conference 2007 was to achieve consensus on various operative and perioperative aspects relevant for paediatric surgeons. Although there is still disagreement regarding some of the technical details of the Kasai operation, a remarkable consensus has emerged in most areas of the actual surgery. No consensus was achieved on the role of postoperative drainage, the routine application of steroids, the use of oral prophylactic antibiotics, and the treatment of postoperative cholangitis. Nevertheless, the wide variation in reported results, which was a feature of this conference, suggests that there are still areas where improvement in outcomes can be anticipated by changes in technique or practice.     

Kasai术后肝内囊肿形成的危险因素分析及对预后的影响

目的探讨Kasai术后肝内囊肿形成的危险因素、临床特点及其对预后的影响。方法回顾性分析2011年11月至2018年11月间天津市儿童医院外科Kasai手术后及准备行肝移植124例患儿的临床资料。包括Kasai手术年龄、术后的黄疸清除、胆管炎发作情况、术后肝功能、术后影像学检查、自体肝生存时间。其中符合正态分布的资料使用独立样本t检验进行分析,非正态分布的资料使用中位数、第25和第75百分位数表示,采用非参数的秩和检验(Mann-Whitney U)进行分析,计数资料行卡方检验,生存分析采用K-M生存分析。结果共收集124例患儿资料,其中男52例,女72例。Kasai手术日龄为11~158d(中位日龄:60.5d),自体肝生存时间为2.2~125.5个月(中位自体肝生存时间10.7个月)。术后形成肝内囊肿的患儿46例(37%)。Kasai术后女孩更易形成肝内囊肿(P<0.001),差异具有统计学意义。囊肿患儿的频发胆管炎、早期胆管炎的发生率高于无囊肿患儿(P<0.05),差异具有统计学意义。囊肿患儿相对于无囊肿患儿的自体肝生存率明显下降,但不同类型囊肿患儿在术后肝功能及自体肝生存时间上差异并无统计学意义。结论Kasai术后肝内囊肿形成与术后早期、频发胆管炎密切相关并且是预后不良的标志。     

胆道闭锁术后反复发作性胆管炎抗感染治疗的中长期随访

目的探讨先天性胆道闭锁肝门空肠吻合术(Kasai术)后反复发作胆管炎患儿经抗感染治疗后自体肝存活情况及行肝移植术的时机。方法对本院2002年至2011年Kasai术后反复发作胆管炎患儿18例进行抗感染治疗,并对这部分患儿进行中长期随访,了解其自体肝存活情况。结果 2例分别在胆管炎病程4个月和5个月后行肝移植术,其余16例均坚持抗感染治疗。12例在抗感染治疗6~15个月后症状消失。随访5~12年10例仍自体肝存活,但均有肝硬化症状;其余2例因继发腹水或消化道出血予肝移植。另外4例治疗中出现肝内胆管扩张,行经皮肝穿刺置管引流(PTBD)。引流后1例放弃治疗死亡;2例引流后仍无法控制感染行肝脏移植手术后感染消退;1例行肝门部再吻合后仍存在胆管炎反复发作,但经抗感染治疗15个月后症状缓解。自体肝生存至今6年。结论胆道闭锁术后反复发作性胆管炎患儿仍可自体肝长时间存活,合并肝内胆管扩张时提示预后不良,需考虑肝移植术。     

胆道闭锁Kasai术后近中期疗效及部分影响因素分析

目的 探讨胆道闭锁Kasai术后的近中期疗效及其影响因素.方法 回顾性分析2005年10月至2010年9月在本院行Kasai手术并获得随访的152例胆道闭锁患儿,男68例,女84例,手术时的中位日龄为75 d(31～528 d),平均(82.36±45.17)d,定期门诊复诊并电话随访,平均随访时间(20.11±16.11)个月(2～62个月),对其诊疗过程及随访情况进行分析,对黄疸消退情况、生存率及影响因素采用Kaplan-Meier生存分析、Cox regression分析及x2检验.结果 Kasai术后黄疸消退率为50％(74/149),Ⅰ、Ⅱ、Ⅲ型患儿的黄疸消退率分别为67％、67％、47％,差异无统计学意义(P=0.306);≤60 d、60～≤90d、91～120 d手术组患儿的黄疸消退率分别为54％、53％、46％,≥120 d手术组患儿的黄疸消退率明显降低(25％),但差异无统计学意义(P=0.310);胆管炎发生率57％(85/149),有无胆管炎发作的黄疸消退率分别为31％、75％,差异有统计学意义(P＜0.01).Kaplan-Meier生存分析Kasai术后2、4年的自体肝存活率分别为56％、49％,有胆管炎组和无胆管炎组的2年自体肝存活率分别为43％、77％,差异有统计学意义(P＜0.01).结论 Kasai手术是目前我国治疗胆道闭锁的首要方法,手术年龄和分型与Kasai术后的近中期效果无明显相关,胆管炎是影响Kasai术后效果的重要因素.     

Outcome of bowel perforation after pediatric liver transplantation

Abstract:  Bowel perforation is one of the causes of mortality after pediatric liver transplantation. The aim of this study was to evaluate the incidence, risk factors, clinical presentations, and outcomes of bowel perforation in pediatric liver recipients. This is a retrospective analysis of all pediatric patients who underwent liver transplantation at a single liver transplant center in Iran between 1999 and 2006. During this period 72 liver transplantations were performed in children <18 yr. Twenty-two children underwent 33 re-explorations after liver transplantation. Five bowel perforations occurred in four children (incidence, 6.9%). One patient required two re-explorations. The median time between liver transplantation and the diagnosis of the bowel perforation was seven days. All patients had abdominal distention before re-exploration. The sites of perforation were jejunum (n = 3) and ileum (n = 2), and simple repair was performed in all cases. Three children had a history of prior Kasai operation. One of them received high dose of methylprednisolone before bowel perforation. Two children expired after bowel perforation (mortality rate, 50%). Bowel perforation is relatively frequent after pediatric liver transplantation. Among risk factors, prior Kasai operation may have a role. We observed that abdominal distention is a sign of bowel perforation and a high index of suspicion is required for rapidly diagnosis of this complication. The outcome of bowel perforation is poor and its mortality is high. Further studies are needed to establish real risk factors for this complication.     

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.     

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.     

Rapid steroid discontinuation for pediatric renal transplantation: a single center experience

To determine the outcomes of pediatric renal transplant recipients who received immunosuppression consisting of early withdrawal of corticosteroids at a single Northern California center. Protocols using minimal steroid exposure have been recently reported in adult transplant recipients with successful results. We examined the outcomes of pediatric renal transplant recipients who were managed at our center using a protocol with very early discontinuation of steroids after renal transplantation. We retrospectively studied the medical records of all renal transplant recipients followed at the Children's Hospital at the University of California, Davis Medical Center from 01/2004 to 12/2005. All patients were less than 18 yr of age at the time of transplantation. The immunosuppressive protocol included three tapering daily doses of methylprednisolone, together with five doses of thymoglobulin followed by maintenance therapy with tacrolimus and MMF. Eight patients with equal numbers of males and females were transplanted during this time period. There were equal numbers of Caucasians, African-Americans, Hispanics, and Asians. A total of 37.5% (3/8) of the subjects received preemptive transplantation, 25% (2/8) received peritoneal, and 37.5% (3/8) received hemodialysis before transplantation. The median (range) age at transplantation was 12.3 (3.1-16.0) year with a follow-up of 1.7 (0.9-2.8) year. At one yr post-transplantation, 57% (4/7) of patients still required anti-hypertensives. Three children required erythropoietin supplementation after transplantation. The mean delta height standard deviation score at 12 months was 0.20 +/- 0.56. There were no episodes of clinical acute rejection. One patient switched from tacrolimus to sirolimus due to biopsy-proven CAN. No patient became diabetic or required hypoglycemic agents. Surveillance biopsies showed no subclinical acute rejection in any patient. Steroid-free immunosuppression is safe in children after renal transplantation. Larger number of patients and longer follow-up are required to further confirm the effectiveness and safety of immunosuppression with rapid steroid discontinuation.     

Marked hyperbilirubinemia caused by acute cellular rejection combined with preservation injury in an infant undergoing living-related liver transplantation

Acute cellular rejection (ACR) and preservation injury (PI) are major complications occurring early after liver transplantation (LTx) and sometimes lead to severe graft dysfunction. We herein report an infant who developed marked hyperbilirubinemia as a result of combined ACR and PI in addition to ischemic damage from bleeding, but recovered without re-LTx. In a 1-yr-old-girl undergoing living-related LTx, liver biopsy (LBx) on post-operative day 7 showed ballooning of the hepatocytes, i.e. microvesicular steatosis (30%) and occasional centrolobular necrosis, consistent with mild-to-moderate PI and ACR with a rejection activity index (RAI) of 2.0. Because drainage of an intra-abdominal abscess was performed and a systemic infection was considered severe, the tacrolimus level was decreased and steroids were discontinued. The total bilirubin level started to rise, thereafter, reaching a maximum of 47.6 mg/dL at 1 month after LTx. LBx showed an RAI of 6.0 and steatosis of 10%. With good response to an increase of tacrolimus level and administration of steroid, the total bilirubin level gradually decreased, finally reaching the normal range in 3 months, and LBx showed no steatosis or finding of ACR. From this experience, ACR, if combined with mild-to-moderate PI or other ischemic damage, may lead to severe clinical manifestations after LTx, which, however, could remit with conservative therapy and without re-LTx.     

Growth of long-term survivors of liver transplantation.

OBJECTIVE: To assess growth in survivors of liver transplantation. STUDY DESIGN: Growth was studied in 105 children up to seven years after liver transplantation. RESULTS: At transplantation, mean height standard deviation score (zH) was -1.22 but 19% of patients were severely growth retarded (height below 0.4th centile). Growth and pubertal retardation were seen in the first six months after liver transplantation. Significant catch up in growth and puberty continued for more than five years. At five years, mean zH was -0.95 and at seven years -0.84. The mean zH of patients at final height was -0.55. zH at six months was predicted by zH and bilirubin at the time of transplantation and prednisolone dose at six months. At four years, zH was predicted by zH at the time of transplantation and the cumulative prednisolone dose. There was no association between zH and age at transplantation, sex, or diagnosis, although those with biliary atresia and those undergoing transplantation under 2 years of age showed more initial growth delay and subsequent catch up. Average age at menarche was 14.2 years. CONCLUSIONS: The mean height of the group to have reached final height after liver transplantation was on the 27th centile. Those transplanted earlier in childhood are likely to achieve more normal final heights. High steroid dose, poor liver function, and retransplantation are associated with poorer height outcomes. Persisting severe short stature is largely confined to children with severely retarded growth at the time of transplantation. Transient delay in puberty and menarche occur early after transplantation, although appropriate pubertal progress is resumed after two to three years.