Bijl E J, Bharwani K D, Houwen R H J, de Man R A
Department of Gastroenterology and Hepatology, Erasmus University, Rotterdam, the Netherlands.
Neth J Med. 2013 May;71(4):170-3.
Biliary atresia (BA) is a progressive inflammatory destructive process of the bile ducts occurring in about one of every 20,000 live births. If left untreated, biliary atresia can lead to liver failure. The only effective treatments for BA at the moment are the Kasai operation and liver transplantation. Kasai portoenterostomy increases the survival of children with BA and postpones subsequent liver transplantation. Because long-term survival is rare, there is not much known about the long-term efficacy of the Kasai operation.
The aim of this review was to study the outcome of patients with BA who survived more than 20 years on their native liver. We performed a systematic search on PubMed using MeSH terms for articles describing the long-term outcomes of patients with biliary atresia. We searched for patients who have lived at least 20 years with their native liver and we registered the number of complications. The endpoints identified in these articles were: death, cholangitis, portal hypertension and gastrointestinal bleeding.
From 53 articles we included 14 articles for analysis. In total 184 patients were above the age of 20 years. Of these 162 patients, 88% (162/184) were still alive with their native liver and 60.5% (98/162) were suffering from liver-related complications.
It is possible for patients with biliary atresia to survive more than 20 years on their native liver after undergoing the Kasai operation during early infancy. However, 60.5% of the long-term survivors alive on their native liver end up suffering from progressive liver-related complications.
胆道闭锁（BA）是一种发生于约每20000例活产儿中的胆管进行性炎症破坏性疾病。若不治疗，胆道闭锁可导致肝衰竭。目前治疗BA的唯一有效方法是Kasai手术和肝移植。Kasai肝门肠吻合术可提高BA患儿的生存率并推迟后续肝移植。由于长期存活者罕见，关于Kasai手术的长期疗效知之甚少。
本综述的目的是研究接受Kasai手术后在其自身肝脏上存活超过20年的BA患者的结局。我们使用医学主题词（MeSH）在PubMed上进行系统检索，以查找描述胆道闭锁患者长期结局的文章。我们搜索了至少在自身肝脏上存活20年的患者，并记录并发症的数量。这些文章中确定的终点为：死亡、胆管炎、门静脉高压和胃肠道出血。
从53篇文章中，我们纳入了14篇进行分析。共有184例患者年龄超过20岁。其中162例患者中，88%（162/184）仍依靠自身肝脏存活，60.5%（98/162）患有与肝脏相关的并发症。
婴儿早期接受Kasai手术后，胆道闭锁患者有可能依靠自身肝脏存活超过20年。然而，60.5%依靠自身肝脏存活的长期幸存者最终会出现进行性肝脏相关并发症。
