Polycystic kidney disease among 4,436 intracranial aneurysm patients from a defined population
Polycystic kidney disease among 4,436 intracranial aneurysm patients from a defined population
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DOI：
10.1212/wnl.0000000000004597
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发表时间：
2017-10-31
期刊：
影响因子：
9.9
通讯作者：
Lindgren, Antti E.
中科院分区：
文献类型：
--
作者：
Nurmonen, Heidi J.;Huttunen, Terhi;Lindgren, Antti E.
目的：明确常染色体显性遗传性多囊肾病(ADPKD)与动脉瘤性蛛网膜下腔出血(ASAH)和未破裂性颅内动脉瘤(IA)疾病的关系。方法：将Kuopio颅内动脉瘤数据库(n=4436例IA患者)和芬兰全国登记的53例ADPKD患者的数据融合成一系列基于人群的ADPKD患者，比较ADPKD患者和普通IA患者的动脉瘤和患者的特异性特征，并确定新生IA形成的风险。ADPKD组IAS破裂的中位数明显小于普通人群(6.00比8.00 mm)，而小IAS的比例则显著高于普通人群(31.00%比18%)。ASAH的中位年龄为42.8岁，比一般IA人群年轻10岁。45%的ADPKD患者存在多个IA，而在普通IA人群中这一比例为28%。每年新发IA形成的累积风险为1.3%(在普通IA人群中为0.2%)。在ADPKD患者中，新生动脉瘤形成的风险显著增加(COX回归风险比7.795%可信区间2.8-20；p<；0.0005)。结论：ADPKD患者发生蛛网膜下腔出血的年龄较小且来自较小的IAS，且De-nevo IAS的风险高于一般的芬兰东部人群。ADPKD应作为诊断为IAS的患者进行长期血管造影随访的指标。
Objective: To define the association of autosomal dominant polycystic kidney disease (ADPKD) with the characteristics of aneurysmal subarachnoid hemorrhage (aSAH) and unruptured intracranial aneurysm (IA) disease.Methods: We fused data from the Kuopio Intracranial Aneurysm database (n = 4,436 IA patients) and Finnish nationwide registries into a population-based series of 53 IA patients with ADPKD to compare the aneurysm-and patient-specific characteristics of IA disease in ADPKD and in the general IA population, and to identify risks for de novo IA formation.Results: In total, there were 33 patients with ADPKD with aSAH and 20 patients with ADPKD with unruptured IAs. The median size of ruptured IAs in ADPKD was significantly smaller than in the general population (6.00 vs 8.00 mm) and the proportion of small ruptured IAs was significantly higher (31% vs 18%). Median age at aSAH was 42.8 years, 10 years younger than in the general IA population. Multiple IAs were present in 45% of patients with ADPKD compared to 28% in the general IA population. Cumulative risk of de novo IA formation was 1.3% per patient-year (vs 0.2% in the general IA population). Hazard for de novo aneurysm formation was significantly elevated in patients with ADPKD (Cox regression hazard ratio 7.7, 95% confidence interval 2.8-20; p < 0.0005).Conclusions: Subarachnoid hemorrhage occurs at younger age and from smaller IAs in patients with ADPKD and risk for de novo IAs is higher than in the general Eastern Finnish population. ADPKD should be considered as an indicator for long-term angiographic follow-up in patients with diagnosed IAs.