Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy.
Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy.
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DOI：
10.1038/s41598-021-90860-w
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发表时间：
2021-05-27
影响因子：
4.6
通讯作者：
Wong KKY
中科院分区：
文献类型：
--
作者：
Chung PHY;Chan EKW;Yeung F;Chan ACY;Mou JWC;Lee KH;Hung JWS;Leung MWY;Tam PKH;Wong KKY
我们提出一个37年的经验，在处理胆道闭锁（BA）和讨论长期并发症后，加塞门肠吻合术（KPE）。从1980年至2017年，对来自三个三级儿科外科中心的231名开放性KPE患者进行了回顾性全港性研究。结果参数为黄疸清除率（COJ）、自体肝存活率（NLS）和长期并发症。分析影响手术效果的因素。中位随访时间为17.5（IQR：13.5-22）年。超过66%的患者在KPE后1年时黄疸消失。70例患者（30.3%）接受了肝移植（LT），中位年龄为6.2（IQR：4.3-8.4）岁。10年和20年的NLS率分别为70.7%和61.5%，在研究期间无显著变化。KPE时的中位年龄为59（IQR：49-67）天。在70天前进行KPE与成功引流的较高比值相关，但KPE的年龄对长期NLS没有影响。在所有自体肝脏存活者（n = 153）中，中位胆红素水平为24（IQR：16-36）µmol/L。门静脉高压症（PHT）和复发性胆管炎分别占51.6%和27.5%。警惕的后续计划，超过60%的BA患者可以保持稳定的疾病，并实现长期生存没有LT。虽然胆汁淤积，门静脉高压症和复发性胆管炎是常见的长期NLS，与一个全面的后续管理策略，他们并不总是需要LT。我们的研究作为一个例子，死者捐赠器官是稀缺的国家，由于捐赠率非常低。
We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5–22) years. Over 66% of patients became jaundice-freed at 1 year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3–8.4) years. The NLS rates at 10 and 20 years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49–67) days. KPE performed before 70 days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n = 153), the median bilirubin level was 24 (IQR: 16–36) µmol/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.
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影响因子：
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作者：
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通讯作者：
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DOI：
10.1097/mpg.0000000000002555
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DOI：
10.1007/s00464-019-07108-y
发表时间：
2020-08-01
影响因子：
3.1
作者：
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