Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sedai 980-8574, Japan.
J Pediatr Surg. 2012 Dec;47(12):2179-83. doi: 10.1016/j.jpedsurg.2012.09.003.
Following the Kasai operation, a number of patients have developed liver failure, even after long-term postoperative courses. We assessed the clinical parameters to clarify the early risk factors affecting late-presenting liver failure in biliary atresia.
From 1955 to 1991, 277 patients underwent a Kasai operation. Among those patients, 92 survived with their native liver for more than 20 years, and 72 continue to survive with their native liver in good condition (Group 1). In 20 patients, persistent jaundice recurred after the age of 20 years (Group 2). The postoperative courses of these patients were assessed retrospectively, and the clinical parameters, including age at the time of the Kasai operation (AGE, days), the period required for jaundice to disappear (PJD, days), and the association with early cholangitis (CG), were compared between the 2 groups.
Of the 20 patients in Group 2, 8 survived after a liver transplantation (LTx). Eight patients had recurrent jaundice, including 4 on the waiting list for anLTx. Additionally, 2 patients died after anLTx at the ages of 22 and 39. Another patient died of liver failure at the age of 28. One patient died of massive esophageal variceal bleeding at the age of 29. Significant differences were confirmed with respect to AGE (Group 1<Group 2, p<.001), PJD (Group 1<Group 2, p<.001), and CG (Group 1: Group 2=47 %: 75 %, p=.028).
A considerable number of adult patients developed liver failure, even after the age of 20 years. AGE, PJD, and CG were found to be risk factors affecting late-presenting liver failure. Therefore, close patient follow-up is essential, especially for long-term survivors with a late operative age and early postoperative complications.
卡塞手术后，许多患者出现肝功能衰竭，甚至在术后长期随访中也会出现这种情况。我们评估了临床参数，以明确影响胆道闭锁患者迟发性肝功能衰竭的早期危险因素。
1955 年至 1991 年，277 例患者接受了卡塞手术。在这些患者中，92 例患者的自体肝存活时间超过 20 年，72 例患者的自体肝在良好状态下继续存活（第 1 组）。20 例患者在 20 岁后出现持续性黄疸复发（第 2 组）。回顾性评估这些患者的术后病程，并比较两组患者的年龄（AGE，天）、黄疸消失所需时间（PJD，天）以及与早期胆管炎（CG）的关系。
第 2 组 20 例患者中，8 例接受肝移植（LTx）后存活。8 例患者出现复发性黄疸，其中 4 例在等待 LTx 时出现黄疸。此外，2 例患者在 22 岁和 39 岁时因 LTx 死亡。另一名患者在 28 岁时死于肝功能衰竭。1 例患者在 29 岁时因大量食管静脉曲张出血死亡。AGE（第 1 组<第 2 组，p<.001）、PJD（第 1 组<第 2 组，p<.001）和 CG（第 1 组：第 2 组=47%:75%，p=.028）方面有显著差异。
相当数量的成年患者在 20 岁以后出现肝功能衰竭。AGE、PJD 和 CG 是影响迟发性肝功能衰竭的危险因素。因此，需要对患者进行密切随访，特别是对于手术年龄晚、术后早期并发症多的长期存活患者。
