常染色体显性多囊肾病合并颅内动脉瘤的临床特征及其随访研究

目的:探讨常染色体显性多囊肾病(Autosomal dominant polycystic kidney disease,ADPKD)合并颅内动脉瘤的临床特征及其预后情况。方法:选择355例2007年11月至2008年11月上海长征医院收治的被诊断为ADPKD的患者为研究对象,分析其临床资料及头部动脉瘤MRA筛查的结果,并对合并颅内动脉瘤的患者进行随访。结果:355例ADPKD患者颅内动脉瘤的发病率为12.4%,发病率随年龄的增加而升高,60-69岁组的发病率为23.3%,有脑卒中家族史的患者发病率明显高于无明确脑卒中家族史的患者(P0.05)。大部分动脉瘤较小(平均直径3.85±3.25mm),且都位于前循环,其中颈内动脉最常见(占48.1%)。对44位合并颅内动脉瘤的患者进行随访,共随访21位患者(27枚动脉瘤),平均随访43.5±4.3月,未发现新生动脉瘤;2枚动脉瘤有明显增大,扩大率为7.4%,其余25枚动脉瘤无明显增大,无动脉瘤破裂。结论:年龄≥30岁和具有脑卒中家族史的ADPKD患者易并发颅内动脉瘤,大多直径较小且位于前循环,随访期间大多无明显增大或破裂。

Observation on the Clinical Characteristics and Follow-up of Autosomal Dominant Polycystic Kidney Combined with Intracranial Aneurysms

Objective:To Iinvestigate the clinical characteristics and prognosis of autosomal dominant polycystic kidney disease (ADPKD) with intracranial aneurysm(ICA).Methods:Patients who were diagnosed as ADPKD fromNovember 2007 to November 2008 in CHANG ZHENG hospital were selected as research objects. Their clinical data and MRI data were collected and analyzed, and patients with ICA were followed up (recheck MRI and collect clinical data).Results:355 patients with ADPKD were found. The incidence rate was 12.4%, which increased as the age increased, reaching a peak value of 23.3% in the 60-69 year age group. The incidence rate of ICA in patients with ADPKD with a positive family history of hemorrhagic stroke was higher than that lack such family history (P<0.05). Most aneurysms were small (median diameter 3.85± 3.25 mm) and all in the anterior circulation, the most common location was in internal carotid artery (48.1%).21 patients, 27 aneurysms had imaging follow-up. During cumulative imaging follow-up of 43.5± 4.3 months, no de novo UIA was detected and two ICA grew obviously with enlargement rate was 7.4%. No evident change was detected in the remaining 25 aneurysms, no aneurysm ruptured.Conclusion:Patients whose age were older than 30 or had family history of hemorrhagic stroke were pretended to suffer from ICA. Most ICAs were small and located in the anterior circulation, during the duration of followed-up, the majority of ICA hadn''t enlarged and ruptured.