Modified stool color card with digital images was efficient and feasible for early detection of biliary atresia—a pilot study in Beijing,China

Background

The aim of this pilot study in Beijing, China, was to validate a screening system for early detection of biliary atresia (BA) by using a modified version of the stool color card (SCC).

Methods

From 2013 to 2014, a total of 29 799 live born infants were screened. SCC was distributed in maternal facilities. Guardians were asked to check their infants’ stool colors daily using SCC up until four months after birth. The screening results among 92.5% of participants were reported. Cases deemed as high risk were referred to a surgical department immediately.

Results

Based on the results reported by the guardians, 24 infants showed pale-pigmented stools, of which two males without obvious signs of jaundice were diagnosed with BA at 52 and 55 days of age, respectively. The sensitivity was 100% and specificity was 99.9%. Four infants were confirmed as having other diseases. Two female patients failed to be screened by the SCC because they had severe jaundice and were referred to the Neonatal Intensive Care Unit after birth. They were diagnosed as BA at 14 and 17 days after birth, respectively. The overall prevalence of BA in this study was 1.3 in 10 000 live births.

Conclusion

The modified SCC was effective and feasible for early detection of BA, especially for patients with no apparent jaundice.

     

Living donor liver transplantation for biliary atresia – An Indian experience

LT has played a significant role in improving the outcome of children with BA. We review our five‐yr experience of LDLT for children with BA. Records of all children who underwent LDLT in our institution over a five‐yr period (August 2010–June 2015) were reviewed and those with a primary diagnosis of BA were selected for our study. Data were extracted from a prospectively maintained database. Additional data were collected by review of case notes and imaging studies. Analysis was carried out using standard statistical means. One hundred and thirty‐two children underwent LDLT at our center over the study period, of which 58 children (31 females) had a primary diagnosis of BA. Thirty‐three (56.9%) children had undergone a prior KPE and 25 (43.1%) had a primary LT. Thirty‐four children had at least one post‐op complication, of which 13 had minor complications (Clavien grades I and II) and 21 had major complications (Clavien grade >II). Thirty‐day survival was 96.6% and one‐yr survival was 91.4%. Univariate analysis of variables comparing children who did and did not have a KPE prior to LT showed that age at LT, weight at LT, PELD, and GRWR were significantly different. LDLT provides excellent outcomes in children with BA. Primary LDLT and LT after KPE provide equivalent results, although the former is technically more challenging as the child is younger.     

切割闭合器在胆道闭锁Kasai手术中的应用

目的探讨切割闭合器对胆道闭锁手术疗效、可行性及安全性的影响。方法将2012年5月至2014年12月本院收治的118例胆道闭锁患儿随机分为常规手术组和使用闭合器组,每组各59例。所有病例均行肝门空肠吻合术(Kasai手术),常规手术组行手工肠吻合术,闭合器组术中使用微型切割闭合器进行肠道重建。比较两组在手术时间、麻醉复苏时间、术后肠道功能恢复、吻合口瘘、狭窄、早期粘连性肠梗阻、肝功能损害程度以及术后黄疸清除率、早期胆管炎发生率、麻醉并发症及手术费用等方面的差异。结果两组在吻合口瘘、狭窄、体温变化,早期粘连性肠梗阻、术后呼吸道感染、黄疸清除率方面比较,差异无统计学意义(P0.05);在手术时间、麻醉复苏时间、肠道功能恢复时间等方面比较,差异有统计学意义(P0.05),闭合器组优于常规手术组,但前者住院费用明显升高(P0.05);两组术后转氨酶较术前均明显升高(P0.05),但升高差异,两组间比较,无统计学意义(P0.05)。结论使用切割闭合器进行Kasai手术,可缩短手术及麻醉时间,促进术后复苏及肠功能恢复,不增加手术、麻醉并发症、肝功能损害及术后早期胆管炎的发生率,但费用较高,在条件许可的情况下,可做临床推广应用。     

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.     

57例胆道闭锁术后近期疗效及部分影响预后因素分析

为了解胆道闭锁术后近期疗效及部分影响预后的因素，对1990年1月－2001年12月收治的胆道闭锁患儿57例进行分析。男32例，女25例；平均手术年龄81天（33－223天）；36例行Kasai术。测定13例患儿血清巨细胞病毒的DNA、抗CMV－lgG和lgM抗体和肝活检组织和CMV抗原表达。结果：Kasai术后总的胆汁排出率为53．8％，总的黄疸消退率为33．3％。激素治疗有促进胆汁引流作用。60天以内手术组疗效明显好于90天以后手术组。CMV感染组术后疗效较非感染相差。提示在目前国内尚未广泛开展肝移植的情况下，早期诊断、及早手术及加强术后抗感染和抗病毒治疗对提高其生存率意义重大。     

Biliary atresia--a fifteen-year review of clinical and pathologic factors associated with liver transplantation

Purpose

The aim of this study was to identify clinical and pathologic factors associated with liver transplantation in infants with biliary atresia initially treated with Kasai hepatic portoenterostomy (KHPE).

Methods

Institutional Review Board approval was obtained. Records of patients with biliary atresia diagnosed between January 1986 and December 2000 were reviewed. Patients were divided into those who never required transplantation, those who underwent transplant in the first year after KHPE, and those who required transplantation later in childhood. Analysis of variance (ANOVA) compared multiple factors among the 3 groups. Proportional analysis compared those who required transplantation against those who did not. Statistical significance was considered achieved if P was less than .05.

Results

Forty-five patients were identified. Survival after KHPE was 96% (43 of 45). Sixteen (37%) never required transplantation, 13 (30%) underwent transplant within 1 year after KHPE, and 14 (33%) underwent transplant more than 1 year after KHPE. ANOVA comparison showed that the duration of jaundice before KHPE as a predictor for liver transplantation approached significance (P = .082). Proportional analysis found that a longer initial duration of jaundice before KHPE (P = .016) and failure to establish biliary flow (P = .033) were also significant predictive factors. An initial requirement for phototherapy (P = .057) and ductules less than 200 μm in diameter (P = .060) showed a trend toward predictor of liver transplantation.

Conclusions

A longer duration of jaundice before KHPE, failure to establish bile flow, requirement for phototherapy in the neonatal period, and ductules smaller than 200 μm are associated with liver transplant after KHPE.     

Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia

Almost a half-century since Kasai described the portoentersotomy for extrahepatic biliary atresia (EHBA), some questions about the management of this condition have been resolved and many are unanswered. The most useful diagnostic steps to aid in the diagnosis are debated. Sonography can be helpful but its sensitivity and selectivity are arguable with strong advocates for its effectiveness. Likewise, the magnetic resonance imaging has forceful advocates but also has not been universalized. The liver biopsy, done commonly before an operation for cholestasis, is often not discriminating. The radionuclide scan hepatobiliary iminodiacetic acid (HIDA) scan after phenobarbital stimulation is helpful if negative, but false positive results are common. It is agreed to proceed expeditiously to the operation in the cholestatic infant after a prompt investigation. The proposal to avoid this step and provide liver transplantation as initial management for EHBA has been suppressed by several clinical findings. The Kasai procedure has not worsened the outcome of eventual liver transplantation. The Kasai, even if it eventually fails, will often buy time and allow the child to grow before transplantation is needed. Multiple reoperations prior to the transplant are discouraged. Revisions to improve bile flow have not gained wide popularity. Use of a stoma to divert the bile has been largely abandoned. The need for frozen section examination of the liver at the site chosen for the portoenterostomy is no longer demanded. The preferred type of intestinal conduit is argued. Unanswered questions about the post-operative management include the role of steroids and of prophylactic antibiotics. The Biliary Atresia Research Consortium, a multi-institutional National Institutes of Health (NIH)-supported project, will address many of the unanswered issues.     

Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome

 Bacterial cholangitis (BC) is a common complication in patients with biliary atresia (BA) and is characterized by fever, acholic stools and positive blood cultures. The diagnosis is often empirical because the yield of blood cultures is low. It is difficult to differentiate BC from other febrile episodes. In order to characterize the clinical and laboratory features of BC in patients with BA, identify risk factors, and correlate cholangitis with outcome, 37 patients with BA from 1993 to 1998 who underwent a Kasai operation in our hospital were studied. The follow-up period ranged from 6 to 59 months. A total of 107 febrile episodes were documented in these patients. The diagnostic criteria for cholangitis were fever, increased jaundice, or acholic stools. The clinical features, laboratory data, results of bacterial cultures, and outcomes were analyzed retrospectively. A total of 107 febrile episodes, including 78 bouts of cholangitis and 29 non-cholangitis infections, were found in 34 patients. Patients with BC had higher postoperative bilirubin levels (P=0.02) and less frequent use of prophylactic antibiotics (P=0.05) than those with non-cholangitis infections. Abnormal white blood cell counts (>12,000 or <4,000 mm³) tended to be present in patients with BC (P=0.08). There were no statistical differences in the risk factors and laboratory data between culture-positive (n=16) and -negative (n=62) cholangitis cases. The occurrence of cholangitis significantly reduced survival in both patients with good (P=0.03) and inadequate bile flow (P=0.03). All 9 patients who had never had cholangitis survived during the follow-up period. Repeated attacks of BC further decreased survival probability. The responsive organisms were mainly enteric bacteria, including Escherichia coli, Enterobacter cloacae, Klebsiella pneumoniae, Pseudomonas aeruginosa, Acinetobacter baumanni, and Salmonella typhi. The sensitivity tests justified empirical therapy with ceftriaxone. The effectiveness of prophylactic trimethoprim-sulfamethoxazole or neomycin warrants further studies. BC was a highly prevalent postoperative complication in patients with BA, especially those with inadequate bile drainage. It significantly affected early mortality. Aggressive and complete treatment with empirical ceftriaxone was appropriate.     

Timing of liver transplantation in biliary atresia-results in 71 children managed by a multidisciplinary team

Background

Kasai portoenterostomy (KP) remains the initial surgical therapy for biliary atresia (BA). Liver transplantation (LTx) is offered after a failed KP or if KP is not feasible. The timing of LTx in these children is not well established. We attempted to define factors that may help choose the optimal timing for LTx in children with BA managed by a multidisciplinary team including a pediatric surgeon, hepatologist, and liver transplant surgeon.

Methods

Records of children who underwent LTx for BA at our institution between January 1998 and December 2006 were reviewed. Clinical data such as pre-LTx pediatric end-stage liver disease (PELD) score, location of KP, and outcome were evaluated.

Results

Seventy one children underwent 77 liver transplants for BA at an average age of 25 months (range, 3-216 months). Sixty-one had a previous KP, 30 at our institution. Ten had LTx without KP. The overall patient survival was 94.4% and overall graft survival was 87% at median follow-up of 58 months (range, 6-111 months). Four patients died, 1 because of vascular thrombosis despite repeat LTx, 1 because of fungal infection after LTx, and 2 because of causes unrelated to LTx. Six children required retransplantation. Living donor liver transplantation was performed in 32 of these children with 91% patient and graft survival. Fifty-three children had a PELD score of 10 or higher with patient and graft survivals of 92% and 86%, respectively. Eighteen children had a PELD score of less than 10 with patient and graft survivals of 100%. For the 30 children who underwent KP at our institution, the median age at LTx was 9 months (range, 3-168 months), and patient and graft survival were both 93%.

Conclusions

Outcome of LTx for BA is excellent. Children with higher PELD scores (≥10) at LTx may have worse outcome. Children with a PELD score of less than 10 survived with their original grafts. In children with BA, the PELD score should be monitored and may help stratify patients for eventual LTx. When a child with BA is deemed a candidate for LTx, the PELD score should be determined. A PELD score that approaches 10 should trigger discussion of LTx and living donor liver transplantation with the family.