Natural Course of Moyamoya Disease in Patients With Prior Hemorrhagic Stroke Clinical Outcome and Risk Factors

Natural Course of Moyamoya Disease in Patients With Prior Hemorrhagic Stroke Clinical Outcome and Risk Factors
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既往出血性中风患者烟雾病的自然病程临床结果和危险因素

DOI:
10.1161/strokeaha.118.022771
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发表时间:
2019-05-01
期刊:
影响因子:
8.3
通讯作者:
Zhao, Ji-zong
Zhao, Ji-zong
中科院分区:
医学1区
文献类型:
--
作者:
Kang, Shuai;Liu, Xingju;Zhao, Ji-zong

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背景和目的--关于出血性烟雾病自然病史的证据仍然不足。我们调查了烟雾病患者反复颅内出血的发生率、死亡率和再出血的危险因素。方法:共有128名有出血表现和完整的随访资料的保守治疗患者纳入研究。记录长期随访期间反复出血的情况。通过Kaplan-Meier生存分析得出出血的年发生率和累积发生率,用Logistic回归分析进行危险因素分析。结果:中位随访时间为10.1(1-27)年。在总共1300.7个病人年中,47名患者(36.7%)经历了59次再出血,年平均发病率为4.5%。其中9例(19.1%)死于再出血,12例严重伤残(改良Rankin评分表>=3)。5年再出血的累积风险为7.8%,10年为22.6%,15年为35.9%。只有4名患者(3.1%)经历了缺血性中风,平均年发病率为0.3%。多因素分析显示吸烟是再出血的独立危险因素(优势比为4.85;P=0.04)。再出血(风险比11.04;P=0.02)和高血压(风险比4.16;P=0.04)与死亡率增加相关。年龄、首次出血类型、数字减影血管造影分期、家族史和合并脑动脉瘤与再出血风险增加无关。结论-再出血事件在出血性烟雾病患者中很常见,是死亡的主要原因。在长期随访期间,再出血的风险稳步增加。吸烟是再次出血的危险因素,高血压与死亡率增加有关。
Background and Purpose-Evidence on the natural history of hemorrhagic moyamoya disease is still insufficient. We investigated the incidence of recurrent intracranial bleeding, mortality, and risk factors for rebleeding in patients with moyamoya disease.Methods-A total of 128 conservatively managed patients with hemorrhagic presentation and complete follow-up data were included. Recurrent hemorrhages during long-term follow-up were documented. Annual and cumulative incidence rate of bleeding was generated via Kaplan-Meier survival analysis, and risk factors were analyzed using logistic regression analysis.Results-The median follow-up time was 10.1 (1-27) years. During a total of 1300.7 patient-years, 47 (36.7%) patients experienced 59 occurrences of recurrent hemorrhages, rendering an average annual incidence of 4.5%. Among them, 9 patients (19.1%) died from rebleeding and 12 patients sustained severe disability (modified Rankin Scale score of >= 3). The cumulative risk of rebleeding was 7.8% at 5 years, 22.6% at 10 years, and 35.9% at 15 years. Only 4 (3.1%) patients experienced ischemic stroke, yielding an average annual incidence of 0.3%. Multivariate analysis showed that smoking (odds ratio, 4.85; P=0.04) was an independent risk factor of rebleeding. Rebleeding (hazard ratio, 11.04; P=0.02) and hypertension (hazard ratio, 4.16; P=0.04) were associated with increased mortality. Age, type of initial bleeding, digital subtraction angiography staging, family history, and coexisting cerebral aneurysms were not associated with increased risk of rebleeding.Conclusions-Rebleeding events were common and the main cause of death in patients with hemorrhagic moyamoya disease. The risk of rebleeding steadily increased during long-term follow-up. Smoking was a risk factor for rebleeding, and hypertension was associated with increased mortality.