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Treatment of severe neurological deficits with IgG depletion through immunoadsorption in patients with Escherichia coli O104:H4-associated haemolytic uraemic syndrome: a prospective trial.免疫吸附法 IgG 清除治疗产志贺样毒素大肠埃希菌 O104:H4 相关性溶血尿毒综合征患者的严重神经功能缺损:一项前瞻性试验。
Lancet. 2011 Sep 24;378(9797):1166-73. doi: 10.1016/S0140-6736(11)61253-1. Epub 2011 Sep 2.
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Management of an acute outbreak of diarrhoea-associated haemolytic uraemic syndrome with early plasma exchange in adults from southern Denmark: an observational study.丹麦南部成年人中急性腹泻相关性溶血尿毒综合征爆发的管理:一项观察性研究。
Lancet. 2011 Sep 17;378(9796):1089-93. doi: 10.1016/S0140-6736(11)61145-8. Epub 2011 Aug 24.
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Systemic infections mimicking thrombotic thrombocytopenic purpura.模拟血栓性血小板减少性紫癜的系统性感染。
Am J Hematol. 2011 Sep;86(9):743-51. doi: 10.1002/ajh.22091.
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Eculizumab in severe Shiga-toxin-associated HUS.依库珠单抗治疗重症志贺毒素相关性溶血尿毒综合征
N Engl J Med. 2011 Jun 30;364(26):2561-3. doi: 10.1056/NEJMc1100859. Epub 2011 May 25.
5
Long term risk for hypertension, renal impairment, and cardiovascular disease after gastroenteritis from drinking water contaminated with Escherichia coli O157:H7: a prospective cohort study.饮用受大肠杆菌 O157:H7 污染的水导致的肠胃炎后,高血压、肾功能损害和心血管疾病的长期风险:一项前瞻性队列研究。
BMJ. 2010 Nov 17;341:c6020. doi: 10.1136/bmj.c6020.
6
Efficacy and safety of rituximab in adult patients with idiopathic relapsing or refractory thrombotic thrombocytopenic purpura: results of a Spanish multicenter study.利妥昔单抗治疗成人特发性复发或难治性血栓性血小板减少性紫癜的疗效和安全性:一项西班牙多中心研究的结果
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7
How I treat patients with thrombotic thrombocytopenic purpura: 2010.如何治疗血栓性血小板减少性紫癜患者:2010 年观点
Blood. 2010 Nov 18;116(20):4060-9. doi: 10.1182/blood-2010-07-271445. Epub 2010 Aug 4.
8
Pulling the trigger in atypical hemolytic uremic syndrome: the role of pregnancy.引发非典型溶血性尿毒症综合征:妊娠的作用
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Long-term, sub-clinical cardiac and renal complications in patients with multiple relapses of thrombotic thrombocytopenic purpura.血栓性血小板减少性紫癜多次复发患者的长期亚临床心脏和肾脏并发症。
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随诊:伴有肾功能不全的微血管病性溶血性贫血。

Attending rounds: microangiopathic hemolytic anemia with renal insufficiency.

机构信息

University of Western Ontario, Department of Medicine, London, Ontario, Canada.

出版信息

Clin J Am Soc Nephrol. 2012 Feb;7(2):342-7. doi: 10.2215/CJN.07230711. Epub 2011 Dec 22.

DOI:10.2215/CJN.07230711
PMID:22193233
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3280030/
Abstract

The classification of thrombotic microangiopathy has evolved and expanded due to treatment and advances in understanding of the diseases associated with this clinical presentation. The three clinical forms of thrombotic microangiopathy-thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and disseminated intravascular coagulation-encompass a wide range of disorders that can be classified as either primary (idiopathic) or secondary to another identifiable disease or clinical context. Identification of an inhibitor to a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in the idiopathic and acute forms of TTP, recognition of the absence of ADAMTS13 inhibition in diarrheal HUS, identification of complement abnormalities in atypical HUS, and a better understanding of the role of plasma therapy, rituximab, and eculizumab therapy have all had a major effect on current understanding of the thrombotic microangiopathies. In this Attending Rounds, a patient with a thrombotic microangiopathy is presented, along with discussion highlighting the difficulty of differentiating TTP from HUS and disseminated intravascular coagulation, the need for a prompt diagnosis, and the role for plasma therapy in appropriately selected patients. The discussion attempts to provide a simple clinical approach to the diagnosis, treatment options, and future course of adults and children suffering from a thrombotic microangiopathy.

摘要

由于治疗方法的改进和对与该临床表现相关疾病的认识的提高,血栓性微血管病的分类已经演变和扩展。血栓性微血管病的三种临床形式——血栓性血小板减少性紫癜(TTP)、溶血尿毒综合征(HUS)和弥散性血管内凝血——涵盖了一系列广泛的疾病,可以分为原发性(特发性)或继发性于另一种可识别的疾病或临床情况。在特发性和急性 TTP 中发现了一种对整合素和金属蛋白酶与血小板反应蛋白 1 型基序,成员 13(ADAMTS13)的抑制剂,在腹泻性 HUS 中发现了缺乏 ADAMTS13 抑制,在非典型 HUS 中发现了补体异常,以及对血浆治疗、利妥昔单抗和依库珠单抗治疗作用的更好理解,所有这些都对当前对血栓性微血管病的理解产生了重大影响。在本次主治医生查房中,提出了一名患有血栓性微血管病的患者,并讨论了区分 TTP 与 HUS 和弥散性血管内凝血的困难、快速诊断的必要性以及血浆治疗在适当选择的患者中的作用。讨论试图为成人和儿童患有血栓性微血管病的诊断、治疗选择和未来病程提供一种简单的临床方法。