摘要
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 ret- rospectively 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 peteehia/eeehy- mosis. In the circulation system, seven patients （41.2%） showed a trend of hypotension, and echocardio- graphy showed 14 patients （82.4%） had thickened ventricular wails [ （ 14.6 ＋ 2.27 ） mini and interventricu- lar septal thickness/left ventricular wall thickness 〉 1.1 mm. Fourteen patients （ 82.4% ） had impaired dias- tolic 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 pa- tients （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 nlonoclonal light chain in 52.9% of patients （k light chain, n = 8; ~ 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.
出处
《协和医学杂志》
2012年第1期89-94,共6页
Medical Journal of Peking Union Medical College Hospital
关键词
心肌淀粉样变
临床特点
诊断
治疗
cardiac amyloidosis
clinical characteristics
diagonosis
treatment
