心内膜弹力纤维增生症的诊治体会

目的 总结心内膜弹力增生症的诊治经验.方法 分析16例心内膜弹力纤维增生症的临床资料.结果 死亡1例,出院随访15例,均未发现心衰,10例心电图及心脏彩超恢复正常.结论 早期诊断,及时、规律、长期合理治疗,能降低患者死亡率,并可改善生命质量.     

Endoderm contributes to endocardial composition during cardiogenesis

Heart formation commences from a single heart tube, which fuses from bilateral primordial heart fields. The developing heart tube is composed of outerlayer myocardial cells and innerlayer endocardial cells. Several distinct populations of precardiac cells contribute to cardiac morphogenesis. However, it still remains not very clear about the lineage of endocardium at gastrulation stage. Thereby, this study focused on ascertaining the correlation between the hypoblast in gastrulation and endocardium during cardiogenesis. Firstly, the fusing heart tube morphologically is closed to endoderm-derived pharynx floor, implying the possibility that pharynx floor might be wrapped into the formation of endoderm. Secondly, HNK1 is expressed in hypoblast strongly at gastrula stage and subsequently appeared in endocardium of cardiogenesis. Moreover, fate map data displayed that DiI labeled hypoblast was also present in endocardium later on. One more evidence is chickquail chimera of hypoblast transplantation, in which quailhypoblast derivative could be identified in endocardium of cardiogenesis by QCPN antibody. In sum, our current data suggests that endoderrn in gastrula contribute at least partly to the formation of endocardium of cardiogenesis.     

彩色多普勒超声对心内膜弹力纤维增生症的诊断价值

心内膜弹力纤维增生症（EFE）是小儿心血管较常见疾病之一,其临床主要表现为充血性心力衰竭,仅靠临床表现容易漏诊,X线检查缺乏特异性,为使本病能得到早期诊断,避免误诊或漏诊,彩色多普勒超声提供了可靠的依据,本文回顾了我院1992年3－1997年8月我院门诊及住院病人12例E血患儿的超声检查结果,并对其进行了分析,现报告如下。1临床资料1．1对象男4例,丈8例,年龄2月一l岁10例,l－3岁2例。患儿均以不同程度心衰为主要临床表现来院就诊,同时伴有呼吸道感染症状者Ic例。心脏听诊：心音低钝,心尖部11－m级收缩期杂音8例,余4例未…     

心内膜弹力纤维增生症的特征性超声表现及心功能评价

目的:用超声检测小儿心内膜弹力纤维增生症(简称心弹)的心内结构及心功能改变并探讨其临床价值。方法:心弹患儿22例,年龄50d～22个月,平均(7.8±3)个月。根据左室大小分为扩张组和缩窄组,另设年龄、性别、身高、体重一致的正常儿童22例作对照组。应用超声测量心脏房室的大小,心肌、心内膜的厚度及运动,各瓣膜血流频谱参数及心功能指标。结果:心弹患儿左室心内膜明显增厚呈区域性分布且回声增强;左心收缩、舒张功能减低。扩张组多见(18例,占81 8%);缩窄组少见(4例,占18 2%)。结论:心弹有特征性超声表现,心内膜不均匀增厚并反射增强对心弹的诊断更为有利。     

心内膜弹力纤维增生症的诊治体会

目的总结心内膜弹力增生症的诊治经验。方法分析16例心内膜弹力纤维增生症的临床资料。结果死亡1例，出院随访15例，均未发现心衰，10例心电图及心脏彩超恢复正常。结论早期诊断，及时、规律、长期合理治疗，能降低患者死亡率，并可改善生命质量。