首页 > 儿科医学词汇大全 > Endocardial fibroelastosis
Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block.
在没有房室传导阻滞的情况下,与母体抗 Ro 和抗 La 抗体相关的心内膜弹性纤维病。

摘要

OBJECTIVES:This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies.
BACKGROUND:Neonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies.
METHODS:We identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls.
RESULTS:Two cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive.
CONCLUSIONS:These are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.

译文

目的: 本研究旨在记录心内膜弹性纤维病 (EFE) 和母体自身抗体的相关性。
背景: 新生儿红斑狼疮与母体抗 Ro 和抗 La 抗体经胎盘通过有关,导致完全性房室传导阻滞 (CAVB)。在某些情况下,CAVB 与 EFE 相关。分离的 EFE 可能与母体抗 Ro 和抗 La 抗体独立相关。
方法: 我们鉴定了三例 (一个胎儿和两个婴儿,都是女性) 在没有 CAVB 的情况下,由自身抗体阳性的母亲所生的婴儿中分离出的 EFE。回顾人口统计学、超声心动图和病理学。免疫组织化学分析免疫球蛋白 (Ig) G 、 IgM 、 IgA 、 T 细胞、 b细胞和末端脱氧核苷酸转移酶介导的 dUTP-生物素缺口末端标记 (TUNEL) (细胞凋亡测试) 在每种情况下对心脏的多个切片进行染色,并与阴性对照组进行比较。
结果: 2 例死亡,1 例接受心脏移植。所有三个病例均经组织学证实为 EFE。所有病例均表现出明显的弥漫性 IgG 浸润。在较小程度上,心肌组织的 IgM 、 CD43 和颗粒酶 B 也呈阳性。没有一个标本 TUNEL 呈阳性。
结论: 这些是在没有 CAVB 的情况下首次报道的与母体抗 Ro 和抗 La 抗体相关的孤立 EFE 病例。IgG 的扩散沉积和 T 细胞在整个心肌的浸润表明母体自身抗体的胎盘通过诱导心肌内的免疫反应,导致孤立的 EFE。自身抗体介导的 EFE 可能是胎儿和新生儿 “特发性” 扩张型心肌病病例的病因。

Endocardial fibroelastosis

儿科 小儿原发性心肌病 疾病
概述  :  

心内膜弹力纤维增生症(EFE)又名心内膜硬化症,为小儿原发性心肌病中较为常见的一种,又称原发性心内膜弹力纤维增生症。先天性心脏病如主动脉缩窄、主动脉瓣狭窄、主动脉瓣闭锁等并发心内膜弹力纤维增生症,称继发性心内膜弹力纤维增生症,其临床意义决定于原发的心脏畸形,不包括在该范围。病因①病毒感染:胎儿期或出生后病毒感染引起炎症反应所致。认为柯萨奇B组病毒、腮腺炎病毒及传染性单核细胞增多症病毒感染与本病有关。曾从心内膜弹力纤维增生症死亡患儿的心肌中分离出柯萨奇B组病毒,组织学上也见到心肌炎的改变。有人

endocardial 英 /,endə(ʊ)'kɑːdɪəl/ 美 /,ɛndo'kɑrdɪəl/

释    义   adj. 心内膜的;心脏内的

例    句   Endocardial cushion defect and the blood transferred between the atriums and the ventricles. 心内膜垫缺损,心房间和心室间左右血流交通。

 

fibroelastosis /`faɪbrəuɪ læs`təusɪs/

释    义   n. 纤维弹性组织增生

例    句   Diagnosis and Treatment of Endocardial Fibroelastosis Correlations between carotid intima-media thickness and coronary heart disease. 心内膜弹力纤维增生症的诊断与治疗颈动脉内中膜厚度与冠心病的相关性

请扫描右侧二维码,免费查看词汇专业知识背景