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[镰状细胞病——常见且危险的并发症]

[Sickle cell disease - common and dangerous complications].

作者信息

Björkman Hedvig, Fager Ferrari Marcus, Arvanitakis Alexandros, Kjellander Christian

机构信息

ST-läkare, medicin-kliniken, Capio S:t Görans sjukhus, Stockholm.

med dr, ST-läkare, , hematologiska kliniken, Skånes universitetssjukhus Malmö.

出版信息

Lakartidningen. 2024 Jul 12;121:23174.

Abstract

Sickle cell disease is a genetic disorder affecting hundreds of thousands of people worldwide. This article will focus on the most common and dangerous acute complications to sickle cell disease. Vaso-occlusive crisis is the most common manifestation of sickle cell disease. Rapid pain relief and treating the underlying cause are cornerstones of the treatment. Acute chest syndrome is the most common cause for intensive care unit admission and death among adult patients with sickle cell disease. The condition is treated as vaso-occlusive crises, but patients often need blood transfusions and respiratory support. Patients with sickle cell disease have an increased risk of stroke. Treatment follows the usual guidelines for stroke with the addition of blood- or exchange transfusions. Splenic sequestration is a rare but acute and life-threatening complication, most commonly seen in children. The condition can quickly lead to hypovolemic shock and the treatment aims to maintain adequate circulation and to treat the underlying cause.

摘要

镰状细胞病是一种影响全球数十万人的遗传性疾病。本文将聚焦于镰状细胞病最常见且危险的急性并发症。血管阻塞性危机是镰状细胞病最常见的表现形式。快速缓解疼痛和治疗潜在病因是治疗的基石。急性胸部综合征是成年镰状细胞病患者入住重症监护病房和死亡的最常见原因。该病症的治疗方法与血管阻塞性危机相同,但患者通常需要输血和呼吸支持。镰状细胞病患者中风风险增加。治疗遵循中风的常规指南,并增加输血或换血治疗。脾隔离症是一种罕见但急性且危及生命的并发症,最常见于儿童。这种情况可迅速导致低血容量性休克,治疗目的是维持充足的循环并治疗潜在病因。

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