Lanari M, Lazzarotto T, Papa I, Venturi V, Bronzetti G, Guerra B, Faldella G, Corvaglia L, Picchio F M, Landini M P, Salvioli G P
Department of Preventive Pediatrics and Neonatology, University of Bologna, Bologna, Italy.
Pediatrics. 2001 Dec;108(6):E114. doi: 10.1542/peds.108.6.e114.
Thrombotic disease is rare in neonates. The main risk factors at this age are perinatal asphyxia, maternal diabetes, sepsis, polycythemia, dehydration, a low cardiac output, and in primis the catheterization of central lines. Another important risk factor is inherited thrombophilia. Arterial thrombosis is even more rare than venous thrombosis and less related to most of the risk factors listed above; it occurs more frequently in the iliac, femoral, and cerebral arteries but very rarely in the aorta. Most of the described cases of aortic thrombosis are associated with the catheterization of an umbilical artery and involve the descending tract and the renal arteries; very few relate to the ascending tract and the aortic arch. The possible role of virus-induced primary vascular endothelium damage in the etiopathogenesis of neonatal arterial thrombosis has been previously hypothesized. Herpesviruses, particularly human cytomegalovirus (HCMV), can infect endothelial cells and directly damage intact vascular endothelium, altering its thromboresistant surface as a result of procoagulant activity mediated by specific viral surface phospholipids, necessary for the coagulation enzyme complex assembly that leads to thrombin generation. We describe a case of congenital aortic arch thrombosis. The clinical, laboratory, and virologic pictures; the anatomopathologic findings (fully compatible with viral infection); the detection of HCMV in various tissues (including the aorta); and the absence of other causes of aortic thrombosis make it possible to attribute the case to a severe congenital HCMV infection with multiple organ involvement, after the primary infection of the mother. The hemostatic system disorders and hemodynamic disturbances related to viral cardiac damage explain the clinical features of the case and indicate that congenital HCMV infection should be included among the causes of neonatal aortic thrombosis.
血栓形成性疾病在新生儿中较为罕见。这个年龄段的主要危险因素包括围产期窒息、母亲糖尿病、败血症、红细胞增多症、脱水、心输出量低,以及首先是中心静脉置管。另一个重要的危险因素是遗传性血栓形成倾向。动脉血栓形成比静脉血栓形成更为罕见,且与上述大多数危险因素的相关性较小;它更常发生在髂动脉、股动脉和脑动脉,但很少发生在主动脉。大多数已描述的主动脉血栓形成病例与脐动脉置管有关,累及降主动脉和肾动脉;与升主动脉和主动脉弓相关的病例极少。病毒诱导的原发性血管内皮损伤在新生儿动脉血栓形成的发病机制中的可能作用此前已有推测。疱疹病毒,尤其是人巨细胞病毒(HCMV),可感染内皮细胞并直接损伤完整的血管内皮,由于特定病毒表面磷脂介导的促凝活性改变其抗血栓表面,而这种磷脂是凝血酶原复合物组装从而导致凝血酶生成所必需的。我们描述了一例先天性主动脉弓血栓形成病例。临床、实验室和病毒学表现;解剖病理学发现(与病毒感染完全相符);在各种组织(包括主动脉)中检测到HCMV;以及不存在主动脉血栓形成的其他原因,使得该病例有可能归因于母亲原发性感染后发生的严重先天性HCMV感染伴多器官受累。与病毒性心脏损伤相关的止血系统紊乱和血流动力学障碍解释了该病例的临床特征,并表明先天性HCMV感染应被纳入新生儿主动脉血栓形成的病因之中。