Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus.
Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus.
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DOI：
10.1007/s11926-007-0003-4
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发表时间：
2007-05-01
影响因子：
5
通讯作者：
Buyon, Jill P
中科院分区：
文献类型：
--
作者：
Friedman, Deborah M;Rupel, Ann;Buyon, Jill P
新生儿狼疮综合征是一种被动获得性自身免疫的模型，其中孕妇的血清含有52或60 kd SSA/Ro和/或48 kd SSB/La的特异性抗体，其穿过胎盘并与胎儿中先天性心脏传导阻滞和/或新生儿中短暂皮疹或各种肝和血细胞异常的发展相关。迄今为止，先天性心脏传导阻滞是一种永久性疾病，会导致严重的发病率和死亡率，几乎所有受影响的婴儿都需要心脏起搏器，3岁时的累积生存概率为80%。一个密集的搜索是对特定的病因病理生理学和新的临床工具，以接近和治疗这种疾病。
Neonatal lupus syndrome is a model of passively acquired autoimmunity in which the pregnant woman's serum contains specific antibodies to 52 or 60 kd SSA/Ro and/or 48 kd SSB/La, which cross the placenta and are associated with the development of congenital heart block in the fetus and/or a transient rash or various liver and blood cell abnormalities in the newborn. To date, congenital heart block is a permanent condition that entails significant morbidity and mortality, with nearly all affected infants requiring pacemakers and with an 80% cumulative probability of survival at 3 years of age. An intensive search is on for the specific etiopathophysiology and for new clinical tools to approach and treat this disease.