常染色体显性多囊肾病脑血管并发症的特征及预后  

Clinical features and prognosis of autosomal dominant polycystic kidney disease with cerebrovascular complications

作　　者：朱淑华 陶磊 李喆 许书添 高洁 王笑亮 高二志 李世军 ZHU Shuhua;TAO Lei;LI Zhe;XU Shutian;GAO Jie;WANG Xiaoliang;GAO Erzhi;LI Shijun(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China;Department of Medical Imaging,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China;Department of Neurology,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China;Department of Neurosurgery,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China)

机构地区：[1]东部战区总医院,国家肾脏疾病临床医学研究中心,全军肾脏病研究所,南京210016 [2]东部战区总医院医学影像科,南京210016 [3]东部战区总医院神经内科,南京210016 [4]东部战区总医院神经外科,南京210016

出　　处：《肾脏病与透析肾移植杂志》2020年第6期501-507,共7页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：江苏省临床医学中心项目(YXZXA2016003);东部战区总医院院管课题(2016027)。

摘　　要：目的:探讨常染色体显性多囊肾病(ADPKD)合并脑血管并发症的临床和影像学特征及预后,提高对ADPKD患者筛查脑血管疾病的认识。方法:本研究纳入2001年1月至2020年1月东部战区总医院重症监护中心(ICU)收治的ADPKD合并脑出血(ICH)、蛛网膜下腔出血(SAH)以及未破裂颅内动脉瘤(UIA)的患者,分析ADPKD合并脑血管并发症患者的临床表现、影像学特点和远期预后。结果:本研究共纳入29例患者,其中男性17例、女性12例,中位年龄48.5(41.25,54.0)岁,其中12例脑出血,12例蛛网膜下腔出血,5例发现未破裂颅内动脉瘤。随访期间累计死亡8例患者,与长期存活的21例患者相比,死亡组入院时的格拉斯哥昏迷量表(GCS)评分较低(P=0.029),血清肌酐、尿素氮明显升高(P<0.001)。结论:脑血管疾病是ADPKD常见的合并症,可导致重度残疾或死亡。因此,ADPKD患者需要注意监测血压和筛查颅内血管疾病。Objective:This retrospective cohort study was intended to evaluate the clinical and imaging characteristics and prognosis of autosomal dominant polycystic kidney disease(ADPKD)with cerebrovascular complications,and to improve the awareness of those patients.Methodology:We retrospectively analyzed the 29 ADPKD patients complicated with intracerebral hemorrhage(ICH),subarachnoid hemorrhage(SAH)or unruptured intracranial aneurysms(UIA)who were admitted to the Intensive Care Unit(ICU)of Jinling Hospital from January 2001 to January 2020.We analyzed the clinical manifestations and imaging characteristics of ADPKD patients with cerebrovascular complications and followed up the long-term prognosis.Results:Among the 29 patients,17 males and 12 females,with an average age of 48.5(41.25,54.0)years old were included in this study,including 12 cases of ICH,12 cases of SAH,and 5 cases of UIA.The 8 patients who died during follow-up had a lower GCS score on admission(P=0.029)and significantly higher serum creatinine and blood urea nitrogen(P<0.001)compared with the other 21 patients with long-term survival.Conclusion:Cerebrovascular diseases are common in ADPKD which may lead to severe disability or death.Therefore,ADPKD patients need to monitor blood pressure and screen for intracranial vascular diseases.

关 键 词：常染色体显性遗传多囊肾病 颅内动脉瘤 脑出血 蛛网膜下腔出血 

分 类 号：R692[医药卫生—泌尿科学] R743[医药卫生—外科学]