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镰状细胞病中的白三烯通路:靶向治疗的潜在靶点。

Leukotriene pathway in sickle cell disease: a potential target for directed therapy.

机构信息

Department of Internal Medicine, Washington University School of Medicine, St Louis, MO, USA.

出版信息

Expert Rev Hematol. 2009 Feb;2(1):57-68. doi: 10.1586/17474086.2.1.57.

DOI:10.1586/17474086.2.1.57
PMID:21082995
Abstract

Sickle cell disease (SCD) is characterized by recurrent episodes of vaso-occlusion, resulting in tissue ischemia and end-organ damage. Inflammation is critical to the pathogenesis of vaso-occlusion and has been associated with SCD-related morbidity and mortality. Despite the impact of inflammation, no directed anti-inflammatory therapies for the treatment or prevention of vaso-occlusive events currently exist. Among individuals with SCD, asthma is a comorbid inflammatory condition that increases the risk of pain episodes, acute chest syndrome and death. Inflammation associated with asthma could augment the proinflammatory state of SCD, increasing episodes of vaso-occlusion. Leukotrienes are inflammatory mediators that play a prominent role in the pathogenesis of asthma and have been associated with SCD-related morbidity. Targeting inflammatory mediators, such as leukotrienes, is a promising approach for the development of novel therapies for the treatment of SCD. This review will examine the relationship between inflammation and vaso-occlusion, with particular focus on the leukotriene pathway.

摘要

镰状细胞病(SCD)的特征是反复发作的血管阻塞,导致组织缺血和终末器官损伤。炎症对于血管阻塞的发病机制至关重要,并且与 SCD 相关的发病率和死亡率有关。尽管炎症有影响,但目前尚无针对血管阻塞性事件的治疗或预防的靶向抗炎治疗。在 SCD 患者中,哮喘是一种合并的炎症性疾病,会增加疼痛发作、急性胸部综合征和死亡的风险。与哮喘相关的炎症可能会加剧 SCD 的促炎状态,增加血管阻塞的发作。白三烯是炎症介质,在哮喘发病机制中起重要作用,并与 SCD 相关的发病率有关。针对炎症介质,如白三烯,是开发治疗 SCD 的新型治疗方法的有前途的方法。这篇综述将研究炎症与血管阻塞之间的关系,特别关注白三烯途径。

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