关键词: de novo disease development domino liver transplantation domino‐auxiliary partial orthotopic liver transplantation metabolic diseases multidisciplinary approach perioperative management

来  源:   DOI:10.1111/pan.14967

Abstract:
Domino liver transplantation and domino-auxiliary partial orthotopic liver transplantation are emerging techniques that can expand the liver donor pool and provide hope for children with liver disease. The innovative technique of domino liver transplantation has emerged as a pioneering strategy, capitalizing on structurally preserved livers from donors exhibiting single enzymatic defects within a morphologically normal context, effectively broadening the donor pool. Concurrently, the increasingly prevalent domino-auxiliary partial orthotopic liver transplantation method assumes a critical role in bolstering available donor resources. These advanced transplantation methods present a unique opportunity for pediatric patients who, despite having structurally and functionally intact livers and lacking early signs of portal hypertension or extrahepatic involvement, do not attain priority on conventional transplant lists. Utilizing optimal clinical conditions enhances posttransplant outcomes, benefiting patients who would otherwise endure extended waiting periods for traditional transplantation. The perioperative management of children undergoing these procedures is complex and requires careful consideration of some factors, including clinical and metabolic conditions of the specific metabolic disorder, and the need for tailored perioperative management planning. Furthermore, the prudent consideration of de novo disease development in the recipient assumes paramount significance when selecting suitable donors for domino liver transplantation, as it profoundly influences prognosis, mortality, and morbidity. This narrative review of domino liver transplantation will discuss the pathophysiology, clinical evaluation, perioperative management, and prognostic expectations, focusing on perioperative anesthetic considerations for children undergoing domino liver transplantation.
摘要:
多米诺肝移植和多米诺辅助部分原位肝移植是新兴的技术,可以扩大肝脏供体库,为肝病患儿提供希望。多米诺肝移植的创新技术已经成为一种开创性的战略,利用在形态正常情况下表现出单酶缺陷的供体结构保存的肝脏,有效地扩大捐赠池。同时,越来越普遍的多米诺辅助部分原位肝移植方法在支持可用供体资源方面发挥着关键作用。这些先进的移植方法为儿科患者提供了独特的机会,尽管肝脏结构和功能完整,但缺乏门静脉高压或肝外受累的早期迹象,在常规移植名单上没有获得优先权。利用最佳临床条件可增强移植后结果,受益的患者会忍受延长的等待期传统移植。接受这些手术的儿童的围手术期管理是复杂的,需要仔细考虑一些因素,包括特定代谢紊乱的临床和代谢状况,以及需要量身定制的围手术期管理计划。此外,在选择合适的多米诺肝移植供体时,对受体新生疾病发展的谨慎考虑具有至关重要的意义,因为它深刻影响预后,死亡率,和发病率。本文叙述了多米诺肝移植的病理生理学,临床评估,围手术期管理,和预后预期,重点关注接受多米诺肝移植的儿童围手术期麻醉注意事项。
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