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镰状细胞病:更新。

Sickle cell disease: an update.

机构信息

University Hospitals Sussex NHS Foundation Trust, Brighton, UK emma.conwayo'

University Hospitals Sussex NHS Foundation Trust, Brighton, UK.

出版信息

Clin Med (Lond). 2022 May;22(3):218-220. doi: 10.7861/clinmed.2022-0143.

Abstract

Sickle cell disease is a common inherited disorder that is characterised by chronic haemolysis and vaso-occlusive episodes, resulting in severe pain and end-organ damage. The most frequent acute manifestation of sickle cell disease is a painful vaso-occlusive crisis, which can, in some cases, develop into a sickle chest crisis: a life-threatening complication of sickle cell disease that requires early recognition and prompt intervention to prevent progressive respiratory failure. In addition to the acute complications, patients with sickle cell disease are also at risk of a number of chronic complications that require multidisciplinary specialist input.

摘要

镰状细胞病是一种常见的遗传性疾病,其特征是慢性溶血和血管阻塞发作,导致严重疼痛和终末器官损伤。镰状细胞病最常见的急性表现是疼痛性血管阻塞危象,在某些情况下,可发展为镰状胸危象:镰状细胞病的一种危及生命的并发症,需要早期识别和及时干预,以防止进行性呼吸衰竭。除了急性并发症外,镰状细胞病患者还存在多种需要多学科专家参与的慢性并发症的风险。

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