血栓性微血管病。

Q4 Medicine

Nephrologe Pub Date : 2021-01-01 Epub Date: 2021-02-03 DOI:10.1007/s11560-021-00487-1

Anja Gäckler, Oliver Witzke

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引用次数: 5

摘要

血栓性微血管病(TMA)的特点是动脉和静脉微血管中内皮损伤相关的血小板凝块形成。伴随的缺血会导致严重的器官功能障碍，并可能严重危及生命。TMA的潜在病因显示出非常不均匀的疾病谱。除了血栓性血小板减少性紫癜，其特征是具有血小板反应蛋白1型基元的崩解素和金属蛋白酶活性大大降低，成员13 (ADAMTS13)，感染相关的经典溶血性尿毒症综合征(HUS)和补体介导的非典型溶血性尿毒症(aHUS)，进一步的非常罕见的疾病或继发性形式可能存在。鉴别诊断分类是关键，因为不同的治疗方法是必要的。新的特异性药物治疗方法的开始极大地改善了TMA的预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

[Thrombotic microangiopathy].

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[Thrombotic microangiopathy].

Thrombotic microangiopathy (TMA) is characterized by an endothelium injury-associated formation of platelet clots in arterial and venous microvessels. Concomitant ischemia causes severe organ dysfunction and can be acutely life threatening. The underlying etiology of TMA shows a very heterogeneous disease spectrum. In addition to thrombotic thrombocytopenic purpura, which is characterized by a greatly reduced activity of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), infection-associated classical hemolytic uremic syndrome (HUS) and complement-mediated atypical HUS (aHUS), further very rare diseases or secondary forms can be present. The differential diagnostic classification is pivotal as different treatment approaches are necessary. Initiation of novel specific pharmacotherapy methods has greatly improved the prognosis of TMA.

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来源期刊

Nephrologe UROLOGY & NEPHROLOGY-

CiteScore

0.40

自引率

0.00%

发文量

期刊介绍： Zielsetzung der Zeitschrift Der Nephrologe bietet aktuelle und kompakte Fortbildung für alle nephrologisch tätigen Ärzt*innen in Klinik und Praxis. Inhaltlich werden sämtliche Bereiche der angewandten Nephrologie und Hypertensiologie praxisnah abgedeckt. Das Spektrum reicht von der Prävention, diagnostischen Vorgehensweisen und Komplikationsmanagement bis hin zu modernen Therapiestrategien. Umfassende Übersichtsarbeiten zu einem aktuellen Schwerpunktthema sind das Kernstück jeder Ausgabe. Im Mittelpunkt steht dabei gesichertes Wissen zu Diagnostik und Therapie mit hoher Relevanz für die tägliche Arbeit. Beiträge der Rubrik "CME Zertifizierte Fortbildung" bieten gesicherte Ergebnisse wissenschaftlicher Forschung und machen ärztliche Erfahrung für die tägliche Praxis nutzbar. Nach Lektüre der Beiträge können die Leser*innen ihr erworbenes Wissen überprüfen und online CME-Punkte erwerben. Die Rubrik orientiert sich an der Weiterbildungsordnung des Fachgebiets. Aims & Scope Der Nephrologe offers up-to-date information for all nephrologists working in practical and clinical environments and scientists who are particularly interested in issues of nephrology. The content covers all areas of applied nephrology and hypertensiology. The topics range from prevention to diagnostic approaches and management of complications to current therapy strategies. Comprehensive reviews on a specific topical issue provide evidenced based information on diagnostics and therapy. Review articles under the rubric ''Continuing Medical Education'' present verified results of scientific research and their integration into daily practice. Review All articles of Der Nephrologe are reviewed. Declaration of Helsinki All manuscripts submitted for publication presenting results from studies on probands or patients must comply with the Declaration of Helsinki.