Chiang Li Wei, Lee Chuan Yaw, Krishnaswamy Gita, Nah Shireen Anne, Kader Ajmal, Ong Christina, Low Yee, Phua Kong Boo
Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
J Paediatr Child Health. 2017 Apr;53(4):412-415. doi: 10.1111/jpc.13379. Epub 2016 Nov 8.
Biliary atresia (BA) has preponderance in Asian populations with Kasai portoenterostomy (KP) regarded as the first-line standard of care. Yet reports from Southeast Asia remain scant. This study reviews the demographics, short- and medium-term outcomes for our cohort, and evaluates prognostic factors for outcome.
All patients diagnosed with BA between January 1997 and December 2013 were included. Clinical data were obtained from medical records. Jaundice clearance was defined as total bilirubin < 20 µmol/L within 6 months after KP. Two-year and 5-year native liver survival (NLS) were determined. Prognostic factors examined included gender, ethnicity, associated anomalies, age at KP, post-KP cholangitis and clearance of jaundice within 6 months.
Of 58 patients studied, 31(53.4%) were male. Median age at time of KP was 53 days (range: 28-127). Ethnic distribution showed 32 (55.2%) Chinese, 16 (27.6%) Malays and 10 (17.2%) others. Twenty-one (36.2%) patients achieved jaundice clearance by 6 months. Two-year NLS rate was 36 out of 50 (72%), while 5-year NLS rate was 16 out of 35 (45.7%). Only clearance of jaundice within 6 months had a significant association with NLS (P = 0.006). All other factors showed no significant impact on outcome.
Our short- and medium-term outcomes after KP for BA are comparable with those reported by most international centres. However, prognostic factors such as age at KP, cholangitis episodes and associated anomalies did not show significant correlation; only clearance of jaundice within 6 months was significantly predictive of NLS.
胆道闭锁（BA）在亚洲人群中更为常见，肝门空肠吻合术（KP）被视为一线标准治疗方法。然而，东南亚地区的相关报道仍然很少。本研究回顾了我们队列的人口统计学特征、短期和中期结果，并评估了预后因素。
纳入1997年1月至2013年12月期间所有诊断为BA的患者。临床数据从病历中获取。黄疸清除定义为KP术后6个月内总胆红素<20µmol/L。确定2年和5年的自体肝生存率（NLS）。检查的预后因素包括性别、种族、相关畸形、KP时的年龄、KP后胆管炎以及6个月内黄疸清除情况。
在研究的58例患者中，31例（53.4%）为男性。KP时的中位年龄为53天（范围：28 - 127天）。种族分布显示，32例（55.2%）为中国人，16例（27.6%）为马来人，10例（17.2%）为其他种族。21例（36.2%）患者在6个月内实现黄疸清除。50例患者中2年NLS率为36例（72%），35例患者中5年NLS率为16例（45.7%）。只有6个月内黄疸清除与NLS有显著相关性（P = 0.006）。所有其他因素对结果均无显著影响。
我们对BA进行KP后的短期和中期结果与大多数国际中心报道的结果相当。然而，诸如KP时的年龄、胆管炎发作次数和相关畸形等预后因素并未显示出显著相关性；只有6个月内黄疸清除对NLS有显著预测作用。
