Friedland-Little Joshua M, Gajarski Robert J, Schumacher Kurt R
Division of Pediatric Cardiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
Division of Pediatric Cardiology, Nationwide Children's Hospital, Ohio State University, Columbus, OH, USA.
Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12925. Epub 2017 Mar 30.
PLE is an important cause of morbidity and mortality in patients who have undergone Fontan palliation. While multiple PLE therapies have been reported, none has proved consistently effective. Patients who do not respond to "standard" PLE therapies face poor long-term outcomes. We report here a significant response to dopamine infusion in three patients with chronic, refractory PLE. We hypothesize that this response may be at least partially due to a dopamine effect on lymphatic receptors rather than to an augmentation of cardiac output.
在接受Fontan姑息手术的患者中，蛋白丢失性肠病（PLE）是发病和死亡的重要原因。虽然已报道了多种PLE治疗方法，但没有一种被证明始终有效。对“标准”PLE治疗无反应的患者面临不良的长期预后。我们在此报告3例慢性难治性PLE患者对多巴胺输注有显著反应。我们推测，这种反应可能至少部分归因于多巴胺对淋巴受体的作用，而非心输出量的增加。
