新生儿红斑狼疮八例分析  

Neonatal lupus erythematosus： analysis of 8 cases

作　　者：杨群[1] 邵肖梅[1] 曹云[1] 陈超[1] 程国强[1] 施忆赟[2] 周利军[3] 

机构地区：[1]复旦大学儿科医院新生儿科,上海200032 [2]复旦大学儿科医院儿内科神经专业,上海200032 [3]复旦大学儿科医院儿内科肾脏专业,上海200032

出　　处：《中华儿科杂志》2008年第1期18-21,共4页Chinese Journal of Pediatrics

摘　　要：目的提高对新生儿红斑狼疮（neonatal lupus erythematosus，NLE）的认识。方法分析2003年9月至2006年2月诊断为NLE患儿的临床表现、狼疮相关血清学指标及头颅CT等辅助检查，并随访至正常或1．5岁。结果共8例患儿诊断为NLE，其中男3例，女5例，足月小样儿4例，临床表现为皮疹（8例）、肝脾肿大（5例）、血小板减少（4例）、神经系统异常（2例）、Ⅲ度房室传导阻滞（AVB）（1例）等。仅3例患儿的母亲产前诊断为系统性红斑狼疮。患儿均有抗核抗体（ANA）和／或抗干燥综合征A抗体（Ro／SSA）和／或抗干燥综合征B抗体（La／SSB）阳性，母亲均有ANA阳性。所有症状除Ⅲ度AVB外均消失，1例神经系统异常者1．5岁随访时也表现正常。结论需提高对NLE的认识才能避免漏诊，早期神经系统异常者不能轻易下有后遗症的结论。Objective Neonatal lupus erythematosus （NLE） is an uncommon autoinunune disease passively transmitted from the mother in which there is transplacental passage of maternal antibodies. It is often misdiagnosed as intrauterine infection or sepsis. The main purpose of this retrospective study was to improve the understanding of pathogenesis and clinical manifestations of NLE. Methods Clinical manifestations, results of the tests for antinuclear antibodies （ ANA）, anti-Ro/SSA, anti-La/SSB and anti- dsDNA antibodies in both infants with NLE （8 cases） and their mothers and head ultrasound and CT scans of the infants were analyzed. Follow-up was performed until one and ahalf years of age or all the abnormalities had been resolved. Results Totally 8 cases （3 males and 5 females） matched the criteria for diagnosis of NLE from September 2003 to February 2006, among whom 4 were small for gestational age and one was born prematurely. Mean gestational age was 38. 1 ± 1.9 weeks, mean birth weight 2 605 ± 420 grams, mean admission age 22. 4 ± 27.7 days （2 hours-72 days） and mean age of onset 9.4 ± 12. 1 days （0- 28 days）. The common clinical manifestations included cutaneous lupus lesions （8 infants） , neural system abnormalities （2 infants） and congenital heart block （2 infants）. The skin of the infants exhibited annular, erythematous or desquamative lesions. They all disappeared before 6 months of age. One patient presented with grade Ⅲ atrioventricular block and was delivered by cesarean section because of ＂fetal distress＂. He did not recover at one and a half years follow-up. One infant was hypotonic with delayed neuro-motor development initially and during follow-up with both abnormal neonatal behavioral neurological assessment （NBNA） and image findings. CT showed generalized low density involving periventricular area and deep white matter at one week of age. At the age of one and a half years, he presented with normal mental development index determined by CDCC infant intel

关 键 词：红斑狼疮 系统性 婴儿 新生 心脏传导阻滞 

分 类 号：R686[医药卫生—骨科学]