新生儿红斑狼疮综合征4例报告

新生儿红斑狼疮综合征 (ＮＬＥＳ)是一种罕见的发生在新生儿或小婴儿的暂时性皮肤红斑狼疮 ,临床少见报道 ,为了加强对本病的认识 ,作者总结了 4例并分析如下。1　临床资料1.1　一般资料4例均为女性 ,出生胎龄 30～ 38周 ,体重 12 0 0～ 2 5 0 0 ｇ;均系剖宫产出生 ,除 1例出生     

新生儿红斑狼疮概述

新生儿红斑狼疮（neonatal lupus erythematosus,NLE）是一组包括皮肤狼疮、先天性心脏传导阻滞（congenital heart block,CHB）和（或）多系统表现的综合征。1954年McCuistion和Schoch首先报道,国外于1981年以后对NLE的研究进一步深入,国内20世纪80年代后期开始报道,至今报道了24例。因此,随着对NLE认识的提高,估计其病例将会逐步增多。     

母亲自身免疫性疾病对新生儿影响研究进展

自身免疫性疾病(systemic autoimmune disease,AD)在女性中发病率较高,尤其是育龄期妇女。患有全身性红斑狼疮、干燥综合征、类风湿关节炎或其他AD的孕母体内的IgG同型自身抗体可通过胎盘对胎儿产生抗体介导的免疫损伤[1-2]。部分具有自身抗体但无症状的孕妇对胎儿也可能产生影响。相对于正常妊娠,母亲AD造成胎儿发生早     

新生儿狼疮综合征并室性心动过速1例

对华中科技大学同济医学院附属协和医院儿科收治的1例新生儿狼疮综合征并室性心动过速伴发育异常患儿的临床资料进行回顾性分析。患儿,女,3 d;临床特点为室性心律失常、皮疹、黄疸、发育异常;查体:脉率快,皮肤黄染,宽眼距、内眦赘皮、高鼻梁,右侧通贯掌;抗干燥综合征抗原A(SSA)抗体、抗Ro-52抗体阳性,心电图示阵发性室性心动过速(伴室房逆传),染色体核型分析示46,XX。予心电监护、抗心律失常、抗感染、保护心功能、免疫球蛋白、退黄等治疗,住院11 d后病情好转出院;定期随访至出生8个月,患儿恢复可。新生儿狼疮综合征临床表现不典型,心脏损害较为突出,除心脏传导阻滞外,也可能导致其他心律失常,如室性心动过速。     

17例新生儿红斑狼疮临床分析

目的总结新生儿红斑狼疮的临床特点, 提高对该病的认识。方法对郑州大学第一附属医院2015年2月至2021年9月收治的17例新生儿红斑狼疮患儿的临床资料进行回顾性分析, 并复习相关文献。结果 17例患儿中, 男9例, 女8例。出现皮肤损害13例, 心脏损害8例, 其中房室传导阻滞4例, QT间期延长合并房性早搏1例, 房间隔缺损3例, 室间隔缺损1例, 动脉导管未闭2例, 心包积液1例, 肺动脉高压1例, 心功能不全2例。1例患儿同时出现皮肤损害及心脏传导阻滞。血液系统损害15例, 肝胆系统损害11例。血清学指标中, 抗ANA抗体阳性17例, 抗SSA-60阳性12例, 抗SSA-52阳性13例, 抗SSB阳性7例, 抗Sm阳性1例, 抗RNP阳性3例。其中4例房室传导阻滞患儿抗SSA-60、抗SSA-52均为阳性。皮肤损害患儿多预后良好, 仅1例遗留褐色瘢痕, 1例Ⅱ°Ⅱ型房室传导阻滞患儿及1例QT间期延长合并房性早搏患儿复查心电图正常, 2例仍存在Ⅲ°房室传导阻滞, 另1例于1岁2个月安装起搏器, 且遗留痉挛性脑瘫。患儿母亲共16例行血清学检测, 产前发现结缔组织抗体异常10例, 产...     

新生儿急性呼吸窘迫综合征研究进展

新生儿急性呼吸窘迫综合征（ARDS）是新生儿常见的临床危重症,是新生儿致死、致残的主要原因之一。新生儿ARDS病因及发病机制复杂,与各种病理因素导致的继发性肺表面活性物质（PS）缺乏有关,具有炎症性特点。新生儿ARDS作为一种临床综合征,与其他疾病难以鉴别。该病的治疗目前缺乏特效手段,仍是以呼吸支持、PS替代、体外膜肺氧合治疗、营养支持及液体管理等对症及综合治疗为主。该文就新生儿ARDS的病因分类、临床特点、诊断治疗策略等方面的研究进展做一综述。     

新生儿精神药物撤药综合征的研究进展

妊娠期应用精神药物可导致20％-30％的新生儿出现撤药症状,临床表现主要包括中枢神经系统兴奋症状、胃肠道功能失常症状、呼吸系统症状、自主神经方面症状体征等.由于新生儿精神药物撤药综合征临床表现无特异性,容易误诊,所以应详细询问母亲病史,特别是孕期用药史,严密观察临床症状,采用临床量表进行评定,进行有关的实验室及其他检查,并排除其他疾病.治疗上支持疗法至关重要,病情严重时可给予苯巴比妥等药物.     

11例新生儿红斑狼疮临床分析

新生儿红斑狼疮(neonatal lupus erythematosus,NLE)是一种由母亲自身抗体通过胎盘进入胎儿体内导致的被动性获得性自身免疫病,临床以一过性皮肤损害和/或永久性先天性心脏传导阻滞(CHB)为主要表现.国内报道较少,本研究回顾性分析我院近4年确诊NLE病例11例,就其临床特征,自身抗体和预后等作一综合分析.     

Neonatal lupus syndromes]

Children born from mothers positive for autoantibodies against SSA/Ro and/or anti-SSB/La ribonucleoproteins may develop heart conduction tissue damage resulting in atrioventricular block and/or transient skin rash, liver enzyme abnormalities and anaemia/thrombocytopenia. Additional transient electrocardiographic abnormalities (sinus bradycardia, QT interval prolongation) have been reported. Such clinical and laboratory manifestations are included in the so-called neonatal lupus syndromes, independently whether the mother is suffering from a systemic autoimmune disease or is totally asymptomatic. The prevalence of the congenital heart block is around 2%, of neonatal rash around 20%, while laboratory abnormalities in asymptomatic babies can be detected in up to 40% of cases. The risk of recurrence of complete heart block is almost 10 times higher in the following pregnancies. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Their long-term outcome is generally more reassuring than previously assumed. Serial echocardiograms and obstetric sonograms, performed at least every 2 weeks, starting from 16 weeks gestation, are recommended in anti-Ro/SSA positive pregnant women: the goal is to detect early fetal abnormalities, that might precede complete atrioventricular block and that might be a target for preventive therapy. Transplacental passage of maternal anti-SSA/Ro -SSB/La IgG is thought to be pivotal in inducing tissue damage. However, the discordant appearance of the syndrome in twins does suggest a role also for fetal or environmental factors.     

Neonatal lupus syndrome

Neonatal lupus syndrome is an autoimmune disease associated with transplacental passage of maternal autoantibodies to SSA/Ro and SSB/La antigens. Characterised by cutaneous lesions and cardiac involvement, the most important clinical manifestation is complete heart block. Rarely, hepatic and haematological abnormalities occur. Although occurring in only 2% of affected mothers, regular fetal monitoring is mandatory in all mothers with anti-SSA/Ro or anti-SSB/La antibodies from approximately 16 weeks of gestation to detect evidence of cardiac conduction defects. If present, maternal corticosteroid therapy may be indicated. Careful postnatal monitoring is essential to identify cardiac and other manifestations that may yet develop. Skin, hepatic and haematological manifestations are self-limiting and resolve. Cardiac disease can be fatal. Heart block is permanent, often requiring cardiac pacing. Mothers of infants with neonatal lupus syndrome may be asymptomatic but often develop autoimmune diseases, including systemic lupus erythematosus or Sjogren's syndrome. Affected infants may be at increased risk of subsequent autoimmune disease.     

Neonatal lupus syndrome

Neonatal lupus erythematosus is a rare syndrome occurring in the first six months of life. It is characterized by the presence of Ro antibodies in the mother and affected infant, and at least one clinical manifestation--cardiac, hematologic, or dermatologic. If present, congenital heart block is irreversible and may be life-threatening in the neonatal period. Mothers of infants with neonatal lupus erythematosus may be asymptomatic when the affected infant is born but may later develop connective-tissue disease. The affected infants are at risk to develop systemic lupus erythematosus during adolescence or adulthood.     

Neonatal lupus erythematosus

We describe case report of a 45 days old male baby with neonatal lupus erythematosus, who presented with 3rd degree congenital heart block and depigmented skin lesions on face and upper part of body. Diagnosis of the baby was confirmed by anti nuclear levels and skin biopsy.