母亲抗Ro和抗La抗体相关的内心纤维延症
Lynne E Nield1, Earl D Silverman, Glenn P Taylor
1Department of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Toronto, Ontario, Canada.
Circulation
|February 21, 2002
概括
与先天性心脏阻塞相关的母体抗体可能会导致婴儿内心纤维延伸症 (EFE). 这种疾病的死亡率很高,即使有治疗.
科学领域:
- 心脏病学 心脏病学
- 免疫学 免疫学 免疫学
- 新生儿科学 新生儿科学
背景情况:
- 母亲的抗Ro和抗La抗体与先天性心脏阻塞 (CHB) 有关.
- 内心纤维生结症 (EFE) 很少被描述为自抗体介导的CHB,其自然史和病因不明.
研究的目的:
- 调查EFE在胎儿和患有CHB的婴儿的自然史和病变发生,这些婴儿的母亲有抗Ro或抗La抗体.
主要方法:
- 在5个中心对临床病史,心声和病理学的回顾性审查.
- 对13名患有EFE和CHB的胎儿和儿童的分析,这些胎儿和儿童的母亲对抗Ro或抗La抗体呈阳性.
- 免疫组织化学评估受影响心脏中的胎儿免疫反应.
主要成果:
- 在所有病例中都存在严重的心室功能障碍.
- 在某些情况下,EFE开发了CHB后诊断,尽管心室节奏.
- 由于EFE造成的高死亡率 (85%),有9例死亡和2例心脏移植.
- 病理学显示中度至严重的EFE,主要在左心室.
- 免疫组织化学显示IgG/IgM沉积和T细胞透,表明胎儿免疫反应.
结论:
- 内心纤维延展症 (EFE) 发生在自抗体介导的先天性心脏阻塞 (CHB) 中,即使有节奏.
- 与自身抗体相关的EFE在胎儿和产后生活中具有非常高的死亡率.
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