摘要
心内膜弹力纤维增生症(EFE)的典型病理过程为心内膜的弹力纤维和胶原纤维明显增生,最终引起充血性心力衰竭。大多数EFE病例在1岁以内发病,突然出现左心衰竭的症状,进行性加重。在部分EFE病例中,自身免疫过程可能起着主要的作用。对于基因突变的研究是寻找EFE的发病机制和开发新治疗的一个方向。心脏磁共振可以更加精确地诊断和评价EFE。卡维地洛和IVIG在EFE治疗中的作用需要更多的临床研究。
The typical pathological process of endocardial fibroelastosis (EFE) is hyperplasia of elastic fiber and collagen fiber in the endocardium, eventually causing congestive heart failure. Most EFE cases happened within one year old,suddenly presenting with the left heart failure, and progressively worsening. In part of the EFE cases, autoimmune process may play a main role. The research on gene mutation is a direction to find its pathogenesis and develop new treatments. Cardiac magnetic resonance can be more accurate to diagnose and evaluate EFE cases. The role of carvedilol and IVIG in the treatment of EFE needs more clinical studies to test.
出处
《兰州大学学报(医学版)》
CAS
2016年第5期68-71,共4页
Journal of Lanzhou University(Medical Sciences)