Lee Sanghoon, Park Hyojun, Moon Suk-Bae, Jung Soo-Min, Kim Jong Man, Kwon Choon Hyuck David, Kim Sung Joo, Joh Jae-Won, Seo Jeong-Meen, Lee Suk-Koo
Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, 135-710, Korea.
Pediatr Surg Int. 2013 Dec;29(12):1297-301. doi: 10.1007/s00383-013-3366-9. Epub 2013 Aug 15.
The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival.
Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed.
Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002).
Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.
本研究旨在分析 Kasai 手术和肝移植（LT）后胆道闭锁（BA）患者的生存率，并分析影响生存的因素。
对 1995 年 4 月至 2009 年 12 月期间诊断为 BA 的 72 例患者进行手术，并进行回顾性分析。
72 例患者中，59 例行 Kasai 手术，13 例未先行 Kasai 手术直接接受 LT。27 例患者在 Kasai 手术后接受 LT。10 年时自体肝生存率为 39%。随着 LT 的应用，BA 患者的总体 10 年生存率为 94.9%。Kasai 手术后自体肝存活的患者中，14 例（58.3%）至少有一种与胆汁性肝硬化和门静脉高压相关的并发症。根据多因素分析，进行 Kasai 手术时的年龄（60 天）和术后胆红素正常化是自体肝生存的独立危险因素（HR 2.90，p = 0.033；HR 9.89，p = 0.002）。
在 LT 时代，BA 患者的生存率有了显著提高。然而，许多 Kasai 手术后自体肝存活的患者仍有胆汁性肝硬化和肝功能异常的迹象。
