Seibold-Weiger K, Vochem M, Mackensen-Haen S, Speer C
Department of Neonatology, University Children's Hospital, Tübingen, Germany.
Am J Perinatol. 1997 Feb;14(2):107-11. doi: 10.1055/s-2007-994108.
We describe a newborn infant with veno-occlusive disease (VOD) of the liver. Prior to discharge from the hospital, the newborn, who had been treated for suspected neonatal infection, suddenly developed sepsis-like symptoms. The size of the liver as well as serum activity of hepatic enzymes increased progressively. Initial Doppler-flow studies demonstrated an absent flow in the vena portae, a finding that was compatible with vena portae thrombosis or occlusion of other hepatic veins. A therapy with recombinant tissue plasminogen activator (rt-PA) was initiated; due to extensive bleedings from various sides, the fibrinolytic therapy had to be withdrawn 12 hours later, when Doppler-flow examination revealed a reverse flow in hepatofugal direction. Despite supportive therapy, the general condition of the patient deteriorated continuously, finally resulting in liver and renal failure. Our patient died 19 days after birth. The autopsy demonstrated obliterative lesions of the centrilobular and sublobular hepatic veins, the classical signs of VOD of the liver. Despite extensive diagnostics and examinations, the etiology of VOD could not been elucidated in this newborn.
我们描述了一名患有肝静脉闭塞性疾病（VOD）的新生儿。在出院前，这名因疑似新生儿感染接受治疗的新生儿突然出现败血症样症状。肝脏大小以及肝酶的血清活性逐渐增加。最初的多普勒血流研究显示门静脉无血流，这一发现与门静脉血栓形成或其他肝静脉闭塞相符。开始使用重组组织型纤溶酶原激活剂（rt-PA）进行治疗；由于出现多处广泛出血，12小时后不得不停止纤溶治疗，此时多普勒血流检查显示出现向肝外方向的逆流。尽管进行了支持治疗，但患者的总体状况持续恶化，最终导致肝肾功能衰竭。我们的患者在出生后19天死亡。尸检显示小叶中心和小叶下肝静脉有闭塞性病变，这是肝VOD的典型体征。尽管进行了广泛的诊断和检查，但仍未能阐明该新生儿VOD的病因。
