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小儿门静脉高压症经颈静脉肝内门体分流术的压力梯度、实验室检查变化及预后

Pressure gradients, laboratory changes, and outcomes with transjugular intrahepatic portosystemic shunts in pediatric portal hypertension.

作者信息

Slowik Voytek, Monroe Eric J, Friedman Seth D, Hsu Evelyn K, Horslen Simon

机构信息

Division of Gastroenterology and Hepatology, Department of Pediatrics, University of Washington School of Medicine/Seattle Children's Hospital, Seattle, Washington.

Department of Radiology, Seattle Children's Hospital, Seattle, Washington.

出版信息

Pediatr Transplant. 2019 May;23(3):e13387. doi: 10.1111/petr.13387. Epub 2019 Apr 1.

Abstract

INTRODUCTION

Indications for TIPS are well described in adults and involve complications of PHTN. Complications from PHTN are associated with PSG of > 12 mm Hg in adults. It is unclear if these parameters apply to children with PHTN.

OBJECTIVE

To assess whether adult criteria for TIPS placement can be utilized in children, describe laboratory changes over time, and report outcomes.

METHODS

We performed a retrospective review of 34 pediatric patients who underwent TIPS, examining indications, radiology, PSG reductions, laboratory changes, and outcomes.

RESULTS

Most patients had PHTN due to parenchymal liver disease including congenital hepatic fibrosis (n = 5), biliary atresia (n = 5), cystic fibrosis-related liver disease (n = 3) and cavernous transformation of the portal vein (n = 6). Indications for TIPS included variceal bleeding, recurrent ascites, and maintenance of portal vein flow following thrombolysis. Variceal bleeding was observed in six children with PSG < 12 mm Hg. Minor complications occurred in eight subjects. Continued bleeding occurred in one patient. Six patients were successfully bridged to transplantation, and three patients died secondary to end-stage disease. Standard laboratory tests stabilized after TIPS placement and hematocrit increased.

CONCLUSION

TIPS placement in pediatric patients was performed for complications of PHTN. Unlike adult series, a substantial proportion of our cases treated extrahepatic PHTN from cavernous transformation of the portal vein. Children presented with sequelae of PHTN with PSG below 12 mm Hg, below the adult standard. We found TIPS in pediatrics to be safe and effective with laboratory stabilization and improvement in hematocrit.

摘要

引言

经颈静脉肝内门体分流术(TIPS)的适应症在成人中已有详尽描述,涉及门静脉高压症(PHTN)的并发症。成人PHTN并发症与肝静脉压力梯度(PSG)>12 mmHg相关。目前尚不清楚这些参数是否适用于儿童PHTN患者。

目的

评估成人TIPS置入标准是否可用于儿童,描述随时间变化的实验室指标变化,并报告治疗结果。

方法

我们对34例行TIPS的儿科患者进行了回顾性研究,检查适应症、放射学检查、PSG降低情况、实验室指标变化及治疗结果。

结果

大多数患者因实质性肝病导致PHTN,包括先天性肝纤维化(n = 5)、胆道闭锁(n = 5)、囊性纤维化相关肝病(n = 3)和门静脉海绵样变性(n = 6)。TIPS的适应症包括静脉曲张出血、反复腹水以及溶栓后门静脉血流的维持。6例PSG<12 mmHg的儿童出现静脉曲张出血。8名受试者出现轻微并发症。1例患者持续出血。6例患者成功过渡到肝移植,3例患者因终末期疾病死亡。TIPS置入后标准实验室检查指标稳定,血细胞比容升高。

结论

儿科患者行TIPS是为了治疗PHTN的并发症。与成人病例不同,我们的病例中有很大一部分是治疗门静脉海绵样变性引起的肝外PHTN。儿童PHTN后遗症患者的PSG低于12 mmHg,低于成人标准。我们发现儿科TIPS安全有效,可使实验室指标稳定并提高血细胞比容。

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