Vaso-occlusive crisis in sickle cell disease: a vicious cycle of secondary events.
Vaso-occlusive crisis in sickle cell disease: a vicious cycle of secondary events.
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DOI：
10.1186/s12967-021-03074-z
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发表时间：
2021-09-20
影响因子：
7.4
通讯作者：
Lim SH
中科院分区：
文献类型：
--
作者：
Jang T;Poplawska M;Cimpeanu E;Mo G;Dutta D;Lim SH
疼痛性血管闭塞危象（VOC）仍然是镰状细胞病（SCD）患者急诊和住院的最常见原因。尽管食品和药物管理局已经批准了两种新的药物用于治疗SCD，但它们都旨在减少VOC的频率。迄今为止，调查各种治疗和缩短VOC的方法的研究结果普遍令人失望。在本文中，我们将总结复杂的病理生理学和下游事件的VOC，并讨论了可能的原因，令人失望的结果，使用单药治疗。我们将提出探索一些目前可用的药物的基本原理，以保护未参与血红蛋白聚合过程的红细胞免受继发性事件诱导的镰状化，或针对VOC复杂下游途径的多管齐下的组合方法。
Painful vaso-occlusive crisis (VOC) remains the most common reason for presenting to the Emergency Department and hospitalization in patients with sickle cell disease (SCD). Although two new agents have been approved by the Food and Drug Administration for treating SCD, they both target to reduce the frequency of VOC. Results from studies investigating various approaches to treat and shorten VOC have so far been generally disappointing. In this paper, we will summarize the complex pathophysiology and downstream events of VOC and discuss the likely reasons for the disappointing results using monotherapy. We will put forward the rationale for exploring some of the currently available agents to either protect erythrocytes un-involved in the hemoglobin polymerization process from sickling induced by the secondary events, or a multipronged combination approach that targets the complex downstream pathways of VOC.
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影响因子：
6.5
作者：
Gaartman AE;Sayedi AK;Gerritsma JJ;de Back TR;van Tuijn CF;Tang MW;Heijboer H;de Heer K;Biemond BJ;Nur E
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通讯作者：
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