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学科主题: 神经病学
题名:
法布里病患者多器官病变的临床特点分析
其他题名: Investigation of multiple organ involvement in Fabry disease
作者: 张巍; 康曼德; 赵亚雯; 李凡; 舒俊龙; 左越焕; 刘靖; 黄一宁; 袁云
关键词: 法布里病 ; 神经痛 ; 肾病 ; 高血压 ; 肥厚性心肌病 ; 卒中 ; Fabry disease ; Neuralgia ; Kidney disease ; Hypertension ; Hypertrophic cardiomyopathy ; Stroke
刊名: 中华医学杂志
发表日期: 2015
DOI: 10.3760/cma.j.issn.0376-2491.2015.23.009
卷: 95, 期:23, 页:1829-1832
收录类别: 中国科技核心期刊 ; 中文核心期刊 ; CSCD
文章类型: Journal Article
摘要: 目的 总结我国法布里病患者多器官病变的发生率和临床表现特点.方法 收集北京大学第一医院神经内科2001-2014年依据病理改变、α-半乳糖苷酶活性A测定和GLA基因突变检查确诊的31个法布里病家系发病者的临床资料,统计其神经痛、肾脏病变、心脏病变、高血压病和脑卒中的发生率和起病时间,使用SPSS 17.0统计软件对男女患者不同器官损害的发生率进行x2检验,比较男女患者上述病变发生率的差异.结果 31个法布里病家系中共151例发病(男∶女=82∶ 69),其中出现神经痛91例(60.26%)、肾脏病变62例(41.06%)、心脏病变33例(21.85%)、高血压病33例(21.85%)、脑卒中21例(13.91%).神经痛的发病高峰期在10 ~ 20岁、肾病为30~40岁,心脏病和脑卒中均为40 ~ 50岁,高血压无发病高峰年龄.女性患者在肾脏病变的发生率低于男性,差异具有统计学意义(P<0.05),其他多器官损害的发生率与男性差异均无统计学意义.结论 我国法布里病的多器官损害发生率和起病时间具有一定规律性,仅在肾脏病变发生率方面具有性别差异. Objective To investigate incidence and clinical features of multiple organ involvement in Chinese patients with Fabry disease.Methods We collected 151 patients of 31 families with Fabry disease,all of whom were confirmed by classic pathology,decreased α-galactosidase A activity or GLA mutation from the year of 2011 to 2014 in Department of Neurology,Peking University First Hospital.The clinical data included incidence and onset of neuralgia,renal dysfunction,heart disease,hypertension and cerebral stroke.We calculate the incidence of these symptoms and analyze their gender difference using chisquare with SPSS 17.0 software.Results Among 31 families with Fabry disease,151 patients (82 males and 69 females) were affected,including 91 patients (60.26%) with neuralgia,62 patients (41.06%) with renal disease,33 patients (21.85%) with heart disease,33 patients (21.85%) with hypertension,and 21 patients (13.91%) with cerebral stroke.The peak incidence of neuralgia was from 10 to 20 years old,followed by renal disease from 30 to 40 years old,heart disease and stroke from 40 to 50 years old.There is no peak period for hypertension.Except for males with higher incidence of renal dysfunction than females (P < 0.05),there were no gender difference in the incidence of other organ involvement.Conclusion The incidence and onset of multiple organ dysfunction in Chinese patients with Fabry disease displayed special regularity.Gender difference appeared only in renal disease.
语种: 中文
原文出处: 查看原文
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内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/41454
Appears in Collections:北京大学第一临床医学院_神经内科_期刊论文

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作者单位: 100034,北京大学第一医院神经内科
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