遗传性出血性毛细血管扩张症患者脑动静脉畸形的出血率
Hemorrhage rates from brain arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.
作者信息
Kim Helen, Nelson Jeffrey, Krings Timo, terBrugge Karel G, McCulloch Charles E, Lawton Michael T, Young William L, Faughnan Marie E
机构信息
From the Departments of Anesthesia and Perioperative Care (H.K., J.N., W.L.Y.), Epidemiology and Biostatistics (H.K., C.E.M.), and Neurological Surgery (M.T.L., W.L.Y.), University of California, San Francisco; Division of Neuroradiology, Department of Medical Imaging (T.K., K.G.T.), Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada; and Division of Respirology, Keenan Research Centre, and Li Ka Shing Knowledge Institute, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada (M.E.F.).
出版信息
Stroke. 2015 May;46(5):1362-4. doi: 10.1161/STROKEAHA.114.007367. Epub 2015 Apr 9.
BACKGROUND AND PURPOSE
Hereditary hemorrhagic telangiectasia (HHT) is a systemic disease characterized by mucocutaneous telangiectasias, epistaxis, and arteriovenous malformations (AVMs). Intracranial hemorrhage (ICH) rates in this population are not well described. We report ICH rates and characteristics in HHT patients with brain AVMs (HHT-BAVMs).
METHODS
We studied the first 153 HHT-BAVM patients with follow-up data enrolled in the Brain Vascular Malformation Consortium HHT Project. We estimated ICH rates after BAVM diagnosis.
RESULTS
The majority of patients were women (58%) and white (98%). The mean age at BAVM diagnosis was 31±19 years (range, 0-70), with 61% of cases diagnosed on asymptomatic screening. Overall, 14% presented with ICH; among symptomatic cases, 37% presented ruptured. During 493 patient-years of follow-up, 5 ICH events occurred yielding a rate of 1.02% per year (95% confidence interval, 0.42-2.44%). ICH-free survival differed significantly by ICH presentation (P=0.003); ruptured cases had a higher ICH rate (10.07%; 95% confidence interval, 3.25-31.21%) than unruptured cases (0.43%; 95% confidence interval, 0.11-1.73%).
CONCLUSIONS
Patients with HHT-BAVM who present with hemorrhage are at a higher risk for rehemorrhage compared with patients with BAVM detected presymptomatically.
背景与目的
遗传性出血性毛细血管扩张症（HHT）是一种全身性疾病，其特征为黏膜皮肤毛细血管扩张、鼻出血和动静脉畸形（AVM）。该人群的颅内出血（ICH）发生率尚无充分描述。我们报告了患有脑动静脉畸形的HHT患者（HHT-BAVM）的ICH发生率及特征。
方法
我们研究了参加脑血管畸形联盟HHT项目且有随访数据的首批153例HHT-BAVM患者。我们估计了BAVM诊断后的ICH发生率。
结果
大多数患者为女性（58%）且为白人（98%）。BAVM诊断时的平均年龄为31±19岁（范围0-70岁），61%的病例在无症状筛查时被诊断。总体而言，14%的患者出现ICH；在有症状的病例中，37%表现为破裂出血。在493患者年的随访期间，发生了5次ICH事件，年发生率为1.02%（95%置信区间，0.42-2.44%）。无ICH生存情况因ICH表现不同而有显著差异（P=0.003）；破裂病例的ICH发生率（10.07%；95%置信区间，3.25-31.21%）高于未破裂病例（0.43%；95%置信区间，0.11-1.73%）。
结论
与症状前检测到BAVM的患者相比，出现出血的HHT-BAVM患者再次出血的风险更高。
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