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胆道闭锁幸存者长期自体肝存活的预后因素分析。

Analysis of the prognostic factors of long-term native liver survival in survivors of biliary atresia.

作者信息

Sasaki Hideyuki, Tanaka Hiromu, Wada Motoshi, Kazama Takuro, Nakamura Megumi, Kudo Hironori, Okubo Ryoji, Sakurai Tsuyoshi, Nio Masaki

机构信息

Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.

出版信息

Pediatr Surg Int. 2016 Sep;32(9):839-43. doi: 10.1007/s00383-016-3934-x. Epub 2016 Jul 27.

Abstract

PURPOSE

Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure.

METHODS

This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis.

RESULTS

The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors.

CONCLUSIONS

The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.

摘要

目的

胆道闭锁(BA)的长期存活者有时会出现肝功能障碍。我们评估了接受葛西手术的BA患者长期自体肝存活(NLS)的预后因素。

方法

本研究纳入了1972年至1995年间接受葛西手术后黄疸消失且NLS超过10年的67例患者。我们回顾性评估了临床参数,包括BA的类型、葛西手术时的年龄、医疗状况和治疗方法。使用逻辑回归分析获得20年NLS的调整优势比(aOR)。

结果

患者接受葛西手术时的中位年龄为63天。在67例研究患者中,62例(92.5%)在20岁时实现了无黄疸NLS,4例患者在20岁前因肝衰竭死亡,1例患者接受了活体亲属肝移植。需要内镜注射硬化治疗的胃食管静脉曲张的存在是一个重要因素(aOR 33.8;p = 0.0033),而脾功能亢进和胆管炎未被确定为重要因素。

结论

有症状的门静脉高压的存在会影响接受葛西手术后的BA患者的长期NLS。对于此类患者,需要准确评估肝功能并对后遗症进行适当治疗。

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