OBJECTIVES:ududStudies report that the risks of significant neurologic complications (including stroke, cerebral abscess, and migraine) and hemorrhagic sequelae are high in patients with hereditary hemorrhagic telangiectasia (HHT), and that life expectancy in this cohort is reduced. However, most published cohorts derive from specialist centers, which may be susceptible to bias.udMETHODS:ududWe used a population-based approach to estimate the risks of developing neurologic and hemorrhagic complications of HHT, the association of a diagnosis of HHT with common cardiovascular and malignant comorbidities, and also long-term survival of those with the disease.udRESULTS:ududFrom a UK primary care database of 3.5 million patients (The Health Improvement Network), we identified 675 cases with a diagnosis of HHT and compared them with 6,696 controls matched by age, sex, and primary care practice. Risks of stroke (odds ratio [OR] 1.8, 95% confidence interval [CI] 1.2-2.6), cerebral abscess (OR 30.0, CI 3.1-288), and migraine (OR 1.7, CI 1.3-2.2) were elevated over controls. Bleeding complications including epistaxis (OR 11.6, CI 9.1-14.7) and gastrointestinal hemorrhage (OR 6.1, CI 2.8-13.4) were more common in cases with HHT. Survival of cases with HHT was poorer than controls with a hazard ratio for death of 2.0 (CI 1.6-2.6) and a median age at death 3 years younger.udCONCLUSIONS:ududPatients with HHT are at substantially increased risk of serious neurologic and hemorrhagic complications of the disease. Because a diagnosis of HHT is associated with a significantly poorer survival compared with those who have no disease, evaluation of new strategies to improve clinical management is required.
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机译：目的： ud ud研究报告说，遗传性出血性毛细血管扩张症（HHT）患者存在重大神经系统并发症（包括中风，脑脓肿和偏头痛）和出血性后遗症的风险较高，并且该人群的预期寿命降低。但是，大多数已发表的队列研究均来自专家中心，可能会产生偏见。 ud方法： ud ud我们使用基于人群的方法来评估发生HHT的神经系统和出血性并发症（与HHT诊断相关的风险）具有常见的心血管疾病和恶性合并症，以及患有该疾病的患者的长期生存。 ud结果： ud ud来自英国350万患者的初级保健数据库（健康改善网络），我们确定了675例诊断为HHT并根据年龄，性别和初级保健实践将其与6696名对照进行了比较。与对照组相比，中风（风险比[OR] 1.8，95％置信区间[CI] 1.2-2.6），脑脓肿（OR 30.0，CI 3.1-288）和偏头痛（OR 1.7，CI 1.3-2.2）的风险均高于对照组。在HHT患者中，包括鼻出血（OR 11.6，CI 9.1-14.7）和胃肠道出血（OR 6.1，CI 2.8-13.4）的出血并发症更为常见。 HHT患者的生存率较对照组差，死亡风险比为2.0（CI 1.6-2.6），中位死亡年龄年轻3岁。 ud结论： ud udHHT患者发生严重神经系统疾病的风险大大增加和该病的出血并发症。与没有疾病的人相比，由于HHT的诊断与较差的生存率相关，因此需要评估改善临床管理的新策略。
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