Guenther Rory A, Kemp Walter L
From the University of North Dakota School of Medicine and Health Sciences, Grand Forks, ND.
Am J Forensic Med Pathol. 2018 Dec;39(4):370-374. doi: 10.1097/PAF.0000000000000432.
While the characteristic features of nephrotic syndrome (ie, proteinuria, hypoalbuminemia, peripheral edema, and hyperlipidemia) are well known, the association of nephrotic syndrome and the risk of thromboembolic events is not as often appreciated and may be overlooked. This report describes a 10-year-old boy with focal segmental glomerulosclerosis who died following a saddle pulmonary thromboembolus, with near-complete occlusion of the left and right pulmonary arteries. The gross appearance of the thrombus suggested organization and histologic changes within the wall of the pulmonary artery indicated a period of at least 3 or more hours to a few days since the event occurred. Pulmonary thromboemboli in children are rare, and the number of cases in the medical literature discussing the occurrence of pulmonary thromboemboli in the background of renal disease in children is relatively small, and none apparently clearly describe saddle thromboemboli with a delay from time of occurrence until death. This case report serves as a good reminder for forensic pathologists to consider renal disease as an underlying etiology for pulmonary thromboembolus and how histologic features of the pulmonary artery may help determine a time frame for the event.
虽然肾病综合征的典型特征(即蛋白尿、低白蛋白血症、外周水肿和高脂血症)广为人知,但肾病综合征与血栓栓塞事件风险之间的关联却不那么为人所重视,可能会被忽视。本报告描述了一名10岁患有局灶节段性肾小球硬化的男孩,他因鞍状肺血栓栓塞死亡,左右肺动脉几乎完全闭塞。血栓的大体外观提示有机化,肺动脉壁的组织学变化表明自事件发生以来至少有3小时或更长时间至数天。儿童肺血栓栓塞很少见,医学文献中讨论儿童肾病背景下肺血栓栓塞发生情况的病例数量相对较少,且显然没有明确描述从发生到死亡有延迟的鞍状血栓栓塞。本病例报告很好地提醒法医病理学家,要将肾病视为肺血栓栓塞的潜在病因,以及肺动脉的组织学特征如何有助于确定事件的时间框架。