Neto Bárbara, Borges-Dias Mariana, Trindade Eunice, Estevão-Costa José, Campos José Miguel
Department of Pediatric Surgery, Centro Hospitalar São João, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
Department of Pediatrics, Centro Hospitalar São João, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
GE Port J Gastroenterol. 2018 Mar;25(2):68-73. doi: 10.1159/000480708. Epub 2017 Oct 5.
Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João.
Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status.
Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively.
The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis.
In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.
胆道闭锁是导致儿童肝衰竭死亡的主要原因，也是儿童肝移植的主要适应症。本研究旨在分析2000年至2015年期间在圣若昂医院接受治疗的该诊断患者群体。
描述性、观察性和回顾性研究，纳入2000年1月1日至2015年12月31日期间诊断并接受治疗的胆道闭锁患者。我们分析了流行病学、临床、生化和影像数据，以及记录的并发症和现状。
评估了18例患者。接受葛西肝门肠吻合术时的中位年龄为63天，72天前接受手术的患者预后较好。该手术在2/3的病例中成功。复发性胆管炎与生存率之间存在显著关联。记录了5例移植病例和2例死亡病例，其中1例在移植后死亡。随访1年、5年和10年时，自体肝生存率分别为77.8%、72.2%和64.2%。
患者的表现及病情演变与其他研究相似。然而，随访5年和10年时的手术成功率和生存率高于大多数系列研究。手术年龄和胆管炎复发是仅有的与预后显著相关的因素。
尽管患者数量较少（每年1125例），但我们的结果与其他参考中心相似。
