Living donor liver transplantation for biliary atresia

Biliary atresia is the most common cause of chronic cholestasis in infants and children. The incidence is estimated at 3.7:10,000 among Taiwanese infants. Kasai hepatoportoenterostomy helps children survive beyond infancy. Liver transplantation is indicated when the Kasai procedure fails to work or when patients develop progressive deterioration of liver function despite an initially successful Kasai operation. Living donor liver transplantation was developed to alleviate organ shortage from deceased donors. It has decreased the waiting time for transplantation and, therefore, improves patient survival. One hundred living donor liver transplantations have been performed for biliary atresia at Chang Gung Memorial Hospital-Kaohsiung Medical Center with both 98% 1-year and 5-year actual recipient survival.     

A new operation for noncorrectable biliary atresia

An improved operative technique to transect the fibrous cord by dividing the ligamentum venosum (Arantius' canal) is described for noncorrectable biliary atresia. The Arantius' canal is situated cranial and posterior side to the bifurcation of the umbilical portion and the portal branch of the Quinous' segment 3. The portal vein is fully mobile and the porta hepatis can be widely exposed by dividing the Arantius' canal. The fibrous cord of the porta hepatis can easily be dissected posteriorly and laterally where there is an extensive number of bile ducts. Eight patients with biliary atresia underwent this procedure. Jaundice resolved completely (serum total bilirubin concentration: < or = 1 mg/dl) in 7 patients within 40 days. Postoperative cholangitis did not occur. By dividing the Arantius' canal, the portal vein comes free from the portal fissure to make the hepatic hilum wider, and surgeons are able to work within a larger porta hepatic space without causing portal vein compression. Free drainage of the bile from the porta hepatis may prevent postoperative cholangitis and promote resolution of jaundice.     

Hepatic function following portoenterostomy for extrahepatic biliary atresia.

Liver structure and function in 10 patients with extrahepatic biliary atresia were studied after portoenteric anastomosis (Kasai operation). Bile flow adequate to reduce the serum bilirubin concentration was established in five patients (improved group), three of whom became anicteric. The serum bilirubin concentration did not decrease in the remaining five patients (unimproved group). Hepatic effluent collected postoperatively from both groups contained small amounts of cholesterol and bilirubin; bile salts, however, were present in the hepatic effluent of only the improved patients. Liver biopsy specimens obtained postoperatively from the five improved patients showed partial (in two) or complete (in three) relief of cholestasis; hepatic fibrosis, however, was unchanged (in one) or worse (in four). The serum concentrations of bile salts were markedly elevated, despite normal excretion of sodium sulfobromophthalein and rose bengal, in two anicteric patients studied 14 and 24 months postoperatively. It is concluded that neither structure nor function of the liver is normalized by portoenterostomy even in clinically well, anicteric patients.     

Forty-four living donor liver transplantations for children with biliary atresia

<正>Objective To observe the outcomes of living donor liver transplantation ( LDLT) for children with biliary atresia ( BA) and to summarize clinical experiences. Methods Forty - four BA patients ( 26 boys and 18 girls) underwent LDLT between October 2006 and December 2010. Mean ( SD) and median ( range) age at     

先天性胆道闭锁肝内毛细胆管超微结构与临床预后关系探讨

目的　探讨先天性胆道闭锁 (CBA)肝内毛细胆管超微结构与临床预后的关系。方法　用PHILIPSCM10透射电镜观察肝内毛细胆管超微结构 ,比较肝组织电镜切片中发育良好与发育不良的毛细胆管数目 ,并与临床预后作比较。结果　 2 5例CBA患儿中 ,肝内毛细胆管发育良好为主的 13例 ,其中 12例术后生存 ,生存率为 92 3% (12 / 13) ;毛细胆管发育不良为主的 12例中 ,仅有 4例生存 ,生存率为 33 3% (4 / 12 ) ,两组生存率有显著性差异 (P <0 0 5 )。结论　肝内毛细胆管的发育情况可作为初步判断术后临床预后指标之一。     

110例胆道闭锁患儿临床资料分析

目的 回顾性分析110例胆道闭锁患儿的临床特征以为胆道闭锁的诊断和治疗提供更多证据.方法 收集2017年8月至2020年8月期间在深圳市儿童医院收治的110例胆道闭锁患儿的临床资料,对患儿的一般资料和实验室检查资料进行总结分析.结果 本研究纳入的110例胆道闭锁患儿中,男49例(39％),女67例(61％).在所有患儿...     

Jaundice clearance and cholangitis in the first year following portoenterostomy for biliary atresia

Sixty-one patients with biliary atresia, who underwent portoenterostomy (PE) between 1992 to 1998 in the Institute of Pediatrics, Kuala Lumpur and were followed for a period of one year, were studied to analyze the factors associated with jaundice clearance and cholangitis following PE. Sex distribution was equal. Majority of patients were Malays. Mean age in days at admission to the surgical ward was 66.90 +/- 23.36 and mean age at PE was 75.85 +/- 24.05. At the end of one-year follow-up, six patients (10%) had died, 35 (57%) developed one or more attacks of cholangitis, 35 (57%) had portal hypertension, eight (13%) liver failure and six patients had esophageal variceal bleeding. Thirty-three patients (54%) had jaundice clearance with a mean clearance time of 85 days after PE. The study shows that when the ductules in the porta hepatis were < 150 mu in size, persistence of jaundice after PE and the incidence of cholangitis in the first post-operative year were higher; patients with cholangitis in the first year had lower rate of jaundice clearance. Jaundice clearance was achieved in more patients when their postnatal age at the time of PE was lower but the relationship was not linear. Age at PE also did not have a linear temporal relationship to the incidence of cholangitis and the size of portal ductules. Prospective, multi-center based local studies on a bigger patient population are needed to identify other indicators of successful outcome following PE. This would help to define the indications for primary liver transplantation in the local population.     

胆管闭锁婴儿术前可溶性黏附分子的状况

目的:肝脏和残余胆管中的细胞间黏附分子(ICA M)的表达是胆管闭锁(BA)的炎症性特征表现。循环中这种可溶性黏附分子(SAM)的水平能够反映肝内疾病,也是评价BA的一个有用的辅助因子。研究设计:在实施肝门-肠吻合术时,对BA患儿血清ICA M-1(sICA M-1)、血清血管细胞黏附因子(sVCAM-     

Implication of innate immunity in the pathogenesis of biliary atresia

Biliary atresia (BA) is a complex disorder for which the etiology is still far from clear. Newborn infants that develop BA may carry certain genetic defects, resulting in susceptibility to uncertain pathogens with characteristic pathogen-associated molecular patterns (PAMPs). The pathogens with their characteristic PAMPs in turn lead to activation of the innate immune system by triggering pattern recognition receptors on the immune cells. Toll-like receptors (TLRs) are the most recognized pattern recognition receptors and TLR signaling is the telltale sign of activation of innate immunity. The activation of TLR and the innate immune system in BA is demonstrated by the up-regulation of TLR7 and by the association of promoter polymorphism of CD14 with BA. The antimicrobial peptide hepcidin and MxA, a protein downstream of TLR7 signaling, which is also known as a highly specific marker for type I IFN signaling, are also found highly expressed in the early stage of BA. This review examines the known components of innate immunity involved in BA and outlines the potential role of the innate immune system, in cooperation with adaptive immunity, in the pathogenesis of BA.     

Characteristics of Malaysian infants with biliary atresia and neonatal hepatitis

Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created.     

术前营养状况对胆道闭锁Kasai术后自体肝生存的影响

目的:评估胆道闭锁（BA）患儿的营养状况,探讨术前营养不良对Kasai术后自体肝生存的影响。方法:回顾性收集93例行Kasai手术BA患儿临床资料并进行营养状况评估。采用多因素回归分析筛选影响Kasai术后24个月自体肝生存的因素,评估营养不良对24个月自体肝生存的预测价值。结果:93例患儿出现营养不良23例（24.7%）,多因素回归模型显示肝纤维化分级（OR=10.803）、术前总胆红素（TB）（OR=1.005）、术后1周γ-谷氨酰转肽酶(γ-GT）（OR=1.002,95%CI:1.000～1.003）、营养不良（OR=4.026,95%CI:2.091～7.751）、肝功能恢复（OR=0.124,95%CI:0.050～0.307）及黄疸清除（OR=0.334,95%CI:0.147～0.757）为24个月自体肝生存的独立影响因素（均P<0.05）。术前营养不良对24个月自体肝生存的特异度最高（94.9%）,但敏感性较低（38.9%）。结论:BA患儿术前营养不良可能与Kasai术时日龄大及肝脏纤维化分级高相关,并影响Kasai术后的自体肝生存时间。     

A comparison of spur valve and percutaneous enterostomy in Roux-Y portoente rostomy for biliary atresia

目的　对肝门空肠吻合加空肠胆支造瘘术与加胆支防返流瓣成形术 ,这两种术式的优缺点进行比较。方法　A组 2 4例胆道闭锁行肝门空肠Roux Y吻合 ,空肠胆支造瘘术。B组为另外 2 4例行肝门空肠Roux Y吻合 ,空肠胆支防返流瓣成形术。对这两组术后情况进行回顾性分析。结果　A组 10例存活 ,无黄疸 9例 ,最大的已 9岁 ,1例出现肝脾肿大、食道静脉曲张。 1例带黄疸存活并出现门脉高压 ,现已 8岁。B组存活 10例 ,其中无黄疸存活 8例。结论　两组病例术后排胆汁 (或排黄绿色大便 )时间、胆管炎发生次数等临床表现进行了比较。排胆汁时间相似 ,无显著差异。术后胆管炎发生率也无显著差异。两种手术都有防返流作用 ,而空肠胆支防返流瓣免除了经皮肤的空肠胆支造瘘。