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伴有肝移植难治性低氧血症的严重肝肺综合征:体外膜肺氧合支持67天后康复

Severe hepatopulmonary syndrome with hypoxemia refractory to liver transplant: Recovery after 67 days of ECMO support.

作者信息

Piltcher-da-Silva Rodrigo, Chedid Marcio Fernandes, Grezzana Filho Tomaz Jesus Maria, Leipnitz Ian, de Araújo Alexandre, Gazzana Marcelo Basso, Saueressig Mauricio Guidi, Lorenzi William, Cardoni Mario Gurvitez, Bellaver Priscila, Alvares-da-Silva Mario Reis, Feier Flavia Heinz, Chedid Aljamir Duarte, Kruel Cleber Rosito Pinto

机构信息

Liver transplantation, Hospital de Clinicas de Porto Alegre, Porto Alegre, Brazil.

Hospital de Clinicas de Porto Alegre, Porto Alegre, Brazil.

出版信息

Int J Artif Organs. 2022 Jan;45(1):121-123. doi: 10.1177/0391398821989067. Epub 2021 Jan 21.

Abstract

Hepatopulmonary syndrome (HPS) is a complication of end stage liver disease (ESLD) and is manifested by severe hypoxemia, which usually responds to liver transplantation (LT). As compared to patients undergoing LT for other etiologies, patients with HPS present an increased risk of postoperative morbidity and mortality. There is no effective treatment for patients whose hypoxemia does not respond to LT. This subset of patients is at a highly increased risk of death. There are very few reports on the use of extracorporeal membrane oxygenation (ECMO) in this setting with rapid response. However, there is no prior report of ECMO utilization for longer than 4 weeks. We present the case of a 17 year-old male patient who underwent LT for ESLD secondary to chronic portal vein thrombosis and HPS. He received a liver from a deceased donor and presented with severe HPS after LT, requiring ECMO support for 67 days. The patient was discharged home and is breathing in ambient air. He is currently asymptomatic and has a normal liver function.

摘要

肝肺综合征(HPS)是终末期肝病(ESLD)的一种并发症,表现为严重低氧血症,通常对肝移植(LT)有反应。与因其他病因接受肝移植的患者相比,HPS患者术后发病和死亡风险增加。对于低氧血症对肝移植无反应的患者,没有有效的治疗方法。这部分患者的死亡风险极高。关于在这种情况下使用体外膜肺氧合(ECMO)且反应迅速的报道非常少。然而,此前尚无ECMO使用超过4周的报道。我们报告一例17岁男性患者,他因慢性门静脉血栓形成继发ESLD和HPS接受了肝移植。他接受了一位脑死亡供者的肝脏,肝移植后出现严重HPS,需要ECMO支持67天。患者出院回家,在室内空气中呼吸。他目前无症状,肝功能正常。

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