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Hepatopulmonary syndrome.

机译:肝肺综合征。

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摘要

The hepatopulmonary syndrome (HPS) is an important and often under-recognized vascular complication of cirrhosis and portal hypertension characterized by pulmonary vascular dilatation, which results in hypoxemia. This syndrome is identified in as many as 20% of patients who are evaluated for orthotopic liver transplantation (OLT), and it has recently been found to increase mortality in affected patients, particularly when hypoxemia is severe. Currently, OLT is the only therapy established to reverse intrapulmonary vasodilatation, although postoperative mortality is increased in patients with severe hypoxemia. No randomized controlled trials of pharmacologic therapies have been undertaken, but supplemental oxygen improves oxygenation. Data derived from case reports, small studies, and experimental models suggest that pharmacologic therapies may be effective. In cirrhotic patients with HPS, particularly those with moderate hypoxemia (PaO2 < 60 mmHg), OLT should be considered prior to the development of severe deoxygenation. Supplemental oxygen should be given to patients with a PaO2 < 60 mmHg or those with exercise oxygen desaturation. For those patients with mild hypoxemia or those who are not OLT candidates, a trial of pharmacologic treatment may be considered.
机译:肝肺综合征(HPS)是一种重要的且常常被人们认识不到的肝硬化和门静脉高压症,其特征是肺血管扩张,导致低氧血症。在接受原位肝移植(OLT)评估的患者中,多达20%的患者发现了这种综合征,最近发现它会增加患病患者的死亡率,特别是在低氧血症严重时。目前,尽管重度低氧血症患者的术后死亡率会增加,但OLT是唯一可以逆转肺内血管舒张的疗法。没有进行药物治疗的随机对照试验,但是补充氧气可以改善氧合作用。来自病例报告,小型研究和实验模型的数据表明,药物治疗可能有效。对于HPS肝硬化患者,尤其是中度低氧血症(PaO2 <60 mmHg)的患者,应在严重脱氧发生之前考虑OLT。 PaO2 <60 mmHg或运动氧去饱和的患者应补充氧气。对于那些轻度低氧血症或不是OLT候选者的患者,可以考虑进行药物治疗试验。

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