Salzedas-Netto A A, Chinen E, de Oliveira D F, Pasquetti A F, Azevedo R A, da Silva Patricio F F, Cury E K, Gonzalez A M, Vicentine F P P, Martins J L
Universidade Federal de São Paulo (Unifesp), São Paulo, Brazil.
Transplant Proc. 2014 Jul-Aug;46(6):1781-3. doi: 10.1016/j.transproceed.2014.05.045.
Biliary atresia (BA) is the most common cause of liver transplantation in children. The earlier the treatment is done, the better the prognosis. The aim is to evaluate the impact of late diagnosis in children with BA, including the histopathological findings and success rate of biliary drainage in patients submitted to hepatic portoenterostomy (HPE).
A retrospective study of cases of BA in the Department of Pediatric Surgery, Federal University of São Paulo (UNIFESP) between 1998-2011. We found 63 cases of BA; of these, 42 underwent HPE and 21 were referred for liver transplantation. Clinic and pathologic data were evaluated.
The HPE was performed with a mean age of 86.5 days, with 16.6% having the operation at 60 days or earlier; 59.2% between 61 and 90 days; and 23.8% after 90 days. Successful biliary drainage occurred in 31% of surgeries, Mean days when HPE drained was 69.1 days, and 94.3 days when the surgery did not drain (P = .05). All patients who were successfully drained, did not have grade IV fibrosis on histology. In cases in which surgery was performed after 60 days that had not drained, 25% had grade IV fibrosis on biopsy (P = .0469).
The age of HPE relates to better prognosis of the disease. It was found that the rate of grade IV fibrosis is higher in no drainage patients. All patients with grade IV fibrosis had no biliary drainage.
胆道闭锁(BA)是儿童肝移植最常见的病因。治疗越早,预后越好。本研究旨在评估BA患儿延迟诊断的影响,包括肝门空肠吻合术(HPE)患者的组织病理学结果和胆汁引流成功率。
对圣保罗联邦大学(UNIFESP)小儿外科1998年至2011年间的BA病例进行回顾性研究。共发现63例BA病例;其中42例行HPE,21例转诊进行肝移植。对临床和病理数据进行评估。
HPE手术的平均年龄为86.5天,16.6%的患者在60天或更早接受手术;59.2%在61至90天之间;23.8%在90天后。31%的手术实现了成功的胆汁引流,HPE引流成功的平均天数为69.1天,未成功引流的为94.3天(P = 0.05)。所有成功引流的患者组织学上均无IV级纤维化。在60天后进行手术且未成功引流的病例中,25%的活检显示有IV级纤维化(P = 0.0469)。
HPE的手术年龄与疾病的较好预后相关。发现未成功引流的患者IV级纤维化发生率较高。所有IV级纤维化患者均未实现胆汁引流。