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结缔组织病、抗核糖核蛋白抗体与先天性心脏传导阻滞。

Connective-tissue disease, antibodies to ribonucleoprotein, and congenital heart block.

作者信息

Scott J S, Maddison P J, Taylor P V, Esscher E, Scott O, Skinner R P

出版信息

N Engl J Med. 1983 Jul 28;309(4):209-12. doi: 10.1056/NEJM198307283090403.

DOI:10.1056/NEJM198307283090403
PMID:6866034
Abstract

The relation between congenital heart block and maternal connective-tissue disease was studied by antibody screening of serum samples obtained in connection with 45 cases of isolated congenital complete heart block. Serum was available from 41 mothers (17 who had connective-tissue disease and 24 who were healthy) and 21 children. Thirty-four mothers had antibody to a soluble tissue ribonucleoprotein antigen called Ro(SS-A), which was identified by immunodiffusion. Anti-Ro(SS-A) was found in seven of eight serum samples collected from affected children when they were less than three months old but in none of 13 samples obtained when these children were older. It appears that maternal anti-Ro(SS-A) antibody crosses the placenta and is a marker for risk of congenital complete heart block; its absence from maternal serum suggests that a child is unlikely to be affected. Anti-Ro(SS-A) or a related antibody is probably involved in the pathogenesis of congenital complete heart block.

摘要

通过对45例孤立性先天性完全性心脏传导阻滞病例相关血清样本进行抗体筛查,研究了先天性心脏传导阻滞与母体结缔组织病之间的关系。有41位母亲(17位患有结缔组织病,24位健康)和21名儿童的血清可供检测。通过免疫扩散法鉴定,34位母亲的血清中有针对一种名为Ro(SS-A)的可溶性组织核糖核蛋白抗原的抗体。在受影响儿童小于3个月时采集的8份血清样本中,有7份检测到抗Ro(SS-A),但在这些儿童年龄较大时采集的13份样本中均未检测到。看来母体抗Ro(SS-A)抗体可穿过胎盘,是先天性完全性心脏传导阻滞风险的一个标志物;母体血清中不存在该抗体表明孩子不太可能受到影响。抗Ro(SS-A)或一种相关抗体可能参与了先天性完全性心脏传导阻滞的发病机制。

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