新疆单中心淀粉样变性患者临床特点及预后研究  

Clinical features and prognosis of amyloidosis patients in Xinjiang single center

作　　者：曲悦 王顺[1] 戴晨[1] 韩媛媛[1] 刘健[1] 李素华[1] QU Yue;WANG Shun;DAI Chen;HAN Yuanyuan;LIU Jian;LI Suhua(Department of Nephropathy,the First Affiliated Hospital of Xinjiang Medical University,Urumqi 830054,China)

机构地区：[1]新疆医科大学第一附属医院肾病科,乌鲁木齐830054

出　　处：《新疆医科大学学报》2018年第4期414-418,共5页Journal of Xinjiang Medical University

基　　金：国家科技支撑计划项目(2015BAI12B05)

摘　　要：目的分析本中心淀粉样变性患者临床特点及预后相关危险因素。方法纳入自2007年1月-2017年1月新疆医科大学第一附属医院明确诊断的淀粉样变性患者,分析其临床特点,利用Kaplan-Meier法计算患者的累积生存率,利用COX回归模型分析相关危险围素。结果本研究共纳入患者114例,平均年龄(54.07±13.26)岁,其中男性77例,女性37例。患者以水肿及蛋白尿为主要首发症状,肾脏(52.63%)及心脏(49.10%)为最常受累器官。患者总体中位生存时间为34.81个月,1a、2a、3a、5a生存率分别为69.8%、62.2%、49.4%和42.6%,亚组分析发现肾脏、心脏受累及两个器官以上受累患者生存时间缩短,而经治疗的患者预后改善。COX多因素分析提示确诊时的肌酐水平、纽约心功能分级(NYAH)3~4级为患者预后的独立危险因素,而接受治疗为患者预后的独立保护性因素。结论淀粉样变性常累及肾脏及心脏,肌酐升高及进展至心功能衰竭提示患者预后不良,而积极接受治疗可能改善患者预后。Objective To analyze clinical features and prognostic risk factors of patients with amyloidosis in Xinjiang single center.Methods 114 patients biopsy-proven amyloidosis were included from January 2007 to January 2017 in the First Affiliated Hospital of Xinjiang Medical University,using Kaplan-Meier method to calculate the patient's survival rate,using COX regression model analysis related risk factors.Results The patients were included in this study with the mean age was(54.07±13.26)years old.They were 37 females and 77 males.Edema and proteinuria were the major clinical features of the patients and the kidneys(52.63%)and the heart(49.10%)were the most frequently affected organs.Patients with median overall survival time was 34.81 months,1 a,2 a,3 a,5 a survival rate were:69.8%,62.2%,49.4%and 42.6%respectively.The patients with renal,cardiac involvement and more than two organs involvement had shortened survival rates,but the treatment could improve the prognosis of patients.COX multivariate analysis showed the creatinine level and grade 3-4 of New York Heart Function Assessment(NYAH)were independent risk factors,and treatment was an independent protective factor for the prognosis of the patients.Conclusion Amyloidosis patients usually combined with renal and cardiac involvement,the high level of serum creatinine and progression to cardiac failure implied poor prognosis,while receiving treatment may improve the prognosis of patients.

关 键 词：淀粉样变性 预后 危险因素 

分 类 号：R597.2[医药卫生—内科学]