The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival.
The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival.
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DOI：
10.1097/sla.0b013e3182300950
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发表时间：
2011-10
影响因子：
9
通讯作者：
Childhood Liver Disease Research and Education Network
中科院分区：
文献类型：
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作者：
Superina R;Magee JC;Brandt ML;Healey PJ;Tiao G;Ryckman F;Karrer FM;Iyer K;Fecteau A;West K;Burns RC;Flake A;Lee H;Lowell JA;Dillon P;Colombani P;Ricketts R;Li Y;Moore J;Wang KS;Childhood Liver Disease Research and Education Network
本研究的目的是描述接受Kasai门肠造口术（KPE）治疗胆道闭锁（BA）的婴儿的临床和解剖学特征，并检查这些参数与结果之间的关系。纳入前瞻性儿童肝病研究和教育网络的婴儿进行了KPE研究。参加盲法介入试验的患者被排除在生存分析之外。主要终点是手术引流成功（前三个月内总胆红素低于2 mg/dL）、无移植生存（Kaplan-Meier）和移植/死亡时间（Cox回归）。244例婴儿（女婴54%，平均年龄65±29天）行KPE。kpe后1年和2年的无移植生存率分别为53.7%和46.7%。45.6%的患者在kpe后3个月内成功进行胆汁引流，移植/死亡风险显著降低（HR 0.08, p<0.001）。肝门闭锁（Ohi II型和III型vs. I型；HR 2.03, p=0.030）、非通畅胆总管（Ohi b、c和d亚型vs. a型；HR 4.31, p=0.022）、BA脾畸形综合征（HR 1.92, p=0.025）、腹水bbb20 ml （HR=1.90, p=0.0230）、肝结节状与坚固性（HR=1.61, p=0.008）、KPE≥75天（HR 1.73, p<0.002）患者的移植/死亡风险增加。结果与胎龄、性别、种族、民族或肝门剥离程度无关。BA、BASM的解剖类型、KPE中腹水和肝结节的出现以及术后早期黄疸清除是无移植生存的重要预测因素。
The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA), and to examine associations between these parameters and outcomes. Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary end-points were successful surgical drainage (total bilirubin less than 2 mg/dL within the first three months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). KPE was performed in 244 infants (54% female; mean age 65± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR 0.08, p<0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi Type II and III vs. Type I; HR 2.03, p=0.030), non-patent common bile duct (Ohi Subtype b, c, and d vs. a; HR 4.31, p=0.022), BA splenic malformation syndrome (HR 1.92, p=0.025), ascites > 20 ml (HR=1.90, p=0.0230), nodular liver appearance compared to firm (HR=1.61, p=0.008), and age at KPE ≥ 75 days (HR 1.73, p<0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.