协和医学杂志

2012, (01) 89-94

[打印本页] [关闭]
本期目录(Current Issue) | 过刊浏览(Past Issue) | 高级检索(Advanced Search)

心肌淀粉样变性的临床病理
Clinicopathological Features of Cardiac Amyloidosis

毛歆歆;赵大春;方全;田庄;程中伟;崔全才;
MAO Xin-xin1,ZHAO Da-chun1,FANG Quan2,TIAN Zhuang2,CHENG Zhong-wei2,CUI Quan-cai1 1Department of Pathology,2Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College,Beijing 100730,China

摘要(Abstract):

目的探讨心肌淀粉样变的临床病理特征、诊断及治疗。方法回顾性分析北京协和医院2006年9月至2010年4月63例心肌活检病例,其中17例经病理组织活检证实为心肌淀粉样变,分析其临床特征、诊断及治疗并复习文献。结果 17例心肌淀粉样变患者中(1)82.4%发病年龄>40岁,男女比例1:1;94%(16/17)有2个或以上系统受累,3个系统以上受累占52.9%(9/17);一般表现乏力者占41%(7/17)、皮肤瘀点瘀斑占23.5%(4/17)。(2)循环系统呈现低血压趋势患者占76.5%;超声心动图检查左室后壁厚(14.6±2.27)mm、室间隔或左室壁厚度>1.1mm者占82.4%(14/17),82.4%(14/17)的患者有左室舒张功能受损表现,76.5%(13/17)的患者发现颗粒样心肌回声增强;心电图异常者占82.4%(14/17)。(3)消化系统症状达47%(8/17),舌体肥大者占11.7%(2/17),肝脏肿大者占17.6%(3/17)。(4)41.2%(7/17)的患者表现为肾功能不全。(5)52.9%(9/17)的患者血和尿免疫固定电泳(immunofixation electrophoresis,IFE)发现单克隆免疫球蛋白轻链,其中λ轻链8例,κ轻链1例。(6)5.9%(1/17)患者出现神经系统表现。结论心肌淀粉样变呈多系统受累,心脏、消化系统、肾脏为常见的靶器官,本病预后不良,早期诊断尤为重要。
Objective To explore the clinical features of cardiac amyloidosis(CA).Methods The clinical data of 17 CA patients who were diagnosed in our hospital from September 2006 to April 2010 were retrospectively analyzed.Results Of these 17 patients,14(82.4%) were over 40 years old,and the ratio of male to female was 1:1.Sixteen patients(94%) had two or more systems involved,and 9 patients(52.9%) had three or more systems involved.Patients were manifested as fatigue(41%) and/or skin petechia/ecchymosis.In the circulation system,seven patients(41.2%) showed a trend of hypotension,and echocardiography showed 14 patients(82.4%) had thickened ventricular walls [(14.6±2.27) mm] and interventricular septal thickness/left ventricular wall thickness>1.1 mm.Fourteen patients(82.4%) had impaired diastolic function of ventriculus sinister.Thirteen patients(76.5%) had increased echogenicity of the myocardium with a granular appearance.Fourteen patients(82.4%) had abnormal electrocardiogram findings.Eight patients(47%) had gastrointestinal systems,which included glossal hypertrophy(11.7%) and enlargement of the liver(17.6%).Seven patients(41.2%) showed renal insufficiency.Immunofixation electrophoresis of blood and urine detected monoclonal light chain in 52.9% of patients(λ light chain,n= 8;κ light chain,n=1).One patient(5.9%) experienced nervous symptom.Conclusion CA can affect multiple systems,with heart,gastrointestinal system,and kidney as the most commonly involved target organs.

关键词(KeyWords): 心肌淀粉样变;临床特点;诊断;治疗
cardiac amyloidosis;clinical characteristics;diagonosis;treatment

Abstract:

Keywords:

基金项目(Foundation):

作者(Author): 毛歆歆;赵大春;方全;田庄;程中伟;崔全才;
MAO Xin-xin1,ZHAO Da-chun1,FANG Quan2,TIAN Zhuang2,CHENG Zhong-wei2,CUI Quan-cai1 1Department of Pathology,2Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College,Beijing 100730,China

Email:

DOI:

参考文献(References):

扩展功能
本文信息
服务与反馈
本文关键词相关文章
本文作者相关文章
中国知网
分享