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镰状细胞贫血儿童和成人的出血性脑卒中:STOP 后队列。

Hemorrhagic Stroke in Children and Adults With Sickle Cell Anemia: The Post-STOP Cohort.

机构信息

Department of Neurology, University of California San Francisco (C.K.F., L.L., N.K.H., H.J.F.).

Children's Hospital of Philadelphia, Division of Hematology and Perelman School of Medicine, Department of Pediatrics, University of Pennsylvania (J.L.K.).

出版信息

Stroke. 2022 Nov;53(11):e463-e466. doi: 10.1161/STROKEAHA.122.038651. Epub 2022 Oct 7.

DOI:10.1161/STROKEAHA.122.038651
PMID:36205141
Abstract

BACKGROUND

Hemorrhagic stroke in young patients with sickle cell anemia remains poorly characterized.

METHODS

The Post-STOP (Stroke Prevention Trial in Sickle Cell Anemia) retrospective study collected follow-up data on STOP and STOP II clinical trial cohorts. From January 2012 to May 2014, a team of analysts abstracted data from medical records of prior participants (all with sickle cell anemia). Two vascular neurologists reviewed data to confirm hemorrhagic strokes defined as spontaneous intracerebral, subarachnoid, or intraventricular hemorrhage. Incidence rates were calculated using survival analysis techniques Results: Follow-up data were collected from 2850 of 3835 STOP or STOP II participants. Patients (51% male) were a median of 19.1 (interquartile range, 16.6-22.6) years old at the time of last known status. The overall hemorrhagic stroke incidence rate was 63 per 100 000 person-years (95% CI, 45-87). Stratified by age, the incidence rate per 100 000 person-years was 50 (95% CI, 34-75) for children and 134 (95% CI, 74-243) for adults >18 years. Vascular abnormalities (moyamoya arteriopathy, aneurysm or cavernous malformation) were identified in 18 of 35 patients with hemorrhagic stroke.

CONCLUSIONS

The incidence rate of hemorrhagic stroke in patients with sickle cell anemia increases with age. Structural vascular abnormalities such as moyamoya arteriopathy and aneurysms are common etiologies for hemorrhage and screening may be warranted.

摘要

背景

镰状细胞贫血症年轻患者的出血性卒中仍特征不明。

方法

Post-STOP(镰状细胞贫血症卒中预防试验)回顾性研究收集了 STOP 和 STOP II 临床试验队列的随访数据。从 2012 年 1 月至 2014 年 5 月,一组分析人员从先前参与者的病历中提取数据(均患有镰状细胞贫血症)。两名血管神经病学家审查数据以确认出血性卒中有症状的颅内、蛛网膜下腔或脑室内出血。使用生存分析技术计算发生率。

结果

从 2850 名 STOP 或 STOP II 参与者中收集了随访数据。患者(51%为男性)在最后一次已知状态时的中位年龄为 19.1 岁(四分位距,16.6-22.6)。总的出血性卒中发生率为每 100000 人年 63 例(95%CI,45-87)。按年龄分层,每 100000 人年的发生率为儿童 50 例(95%CI,34-75),18 岁以上成人 134 例(95%CI,74-243)。35 例出血性卒中患者中有 18 例存在血管异常(烟雾病、动脉瘤或海绵状畸形)。

结论

镰状细胞贫血症患者的出血性卒中发生率随年龄增长而增加。烟雾病和动脉瘤等结构性血管异常是出血的常见病因,可能需要进行筛查。

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