Nguyen Trung, Hall Mark, Han Yong, Fiedor Melinda, Hasset Andrea, Lopez-Plaza Ileana, Watson Scott, Lum Lucy, Carcillo Joseph A.
Departments of Critical Care Medicine (Drs. Nguyen, Hall, Han, Fiedor, Watson, and Carcillo) and the Pathology (Dr. Lopez-Plaza), University of Pittsburgh School of Medicine, Pittsburgh, PA; the Institute for Transfusion Medicine, Pittsburgh, PA (Dr. Hasset); and the Department of Pediatrics, University of Malaya, Kuala Lampur, Indonesia (Dr. Lum). E-mail:
Pediatr Crit Care Med. 2001 Jul;2(3):187-196. doi: 10.1097/00130478-200107000-00001.
To discuss the current rationale for the use of specific and nonspecific therapies for thrombotic microangiopathy in thrombocytopenia-associated pediatric multiple organ failure syndromes. Methods: Pertinent PubMed and MEDLINE citations and proceedings of recent critical care meeting presentations were reviewed. RESULTS: Critical care clinicians have reported using antithrombin III concentrate, protein C concentrate, activated protein C, prostacyclin and its analogues, heparin, tissue factor pathway inhibitor concentrate, plasma infusion, plasma exchange, whole blood exchange, pentoxifylline, tissue plasminogen activator, urokinase, and streptokinase with perceived therapeutic benefits in patients with thrombocytopenia-associated multiple organ failure, including those with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, disseminated intravascular coagulation syndrome, and secondary thrombotic microangiopathy syndrome without prolonged prothrombin time/activated partial thromboplastin time. CONCLUSION: Assuming that underlying disease is remediable, a consensus has developed that thrombotic microangiopathy is a therapeutic target in children with thrombocytopenia-associated multiple organ failure syndromes. Studies are warranted to delineate efficacious use of specific and nonspecific therapies to prevent and reverse thrombotic microangiopathy in these patients.
探讨在血小板减少相关的儿童多器官功能衰竭综合征中使用特异性和非特异性疗法治疗血栓性微血管病的当前理论依据。方法：回顾了相关的PubMed和MEDLINE引文以及近期危重病会议报告的相关内容。结果：危重病临床医生报告称，在血小板减少相关的多器官功能衰竭患者中，包括血栓性血小板减少性紫癜/溶血尿毒症综合征、弥散性血管内凝血综合征以及凝血酶原时间/活化部分凝血活酶时间未延长的继发性血栓性微血管病综合征患者，使用抗凝血酶III浓缩物、蛋白C浓缩物、活化蛋白C、前列环素及其类似物、肝素、组织因子途径抑制剂浓缩物、血浆输注、血浆置换、全血置换、己酮可可碱、组织型纤溶酶原激活剂、尿激酶和链激酶具有明显的治疗益处。结论：假设潜在疾病可治愈，目前已形成共识，即血栓性微血管病是血小板减少相关多器官功能衰竭综合征患儿的治疗靶点。有必要开展研究以明确特异性和非特异性疗法在预防和逆转这些患者血栓性微血管病方面的有效应用。
