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Retrospective, single‐center case series of neonatal lupus

机译:回顾性,单中心盒系列新生儿狼疮

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Abstract Background Neonatal lupus erythematosus (NLE) results from cross‐placental transfer of maternal autoantibodies. Neonates can present with cardiac, cutaneous, hepatobiliary, hematologic, and neurologic complications from antibody‐mediated organ toxicity. Scant evidence exists on long‐term clinical characteristics and outcomes of patients with neonatal lupus. Objectives To characterize the autoantibody profile, laboratory, and clinical features of patients with NLE. Materials/Methods This was a single‐site retrospective cohort study of patients at the Children's Hospital of Philadelphia with NLE. Data were collected on clinical, laboratory, and autoantibody profile at time of presentation, as well as long‐term complications. Results Thirteen patients were included. Congenital cardiac findings were reported in 3 patients, with 1 having persistent cardiac sequelae. Cardiac manifestations were correlated with anti‐Ro/SSA positivity in our cohort. Two patients had neurologic findings, with good long‐term outcomes. Cutaneous findings were present in all patients, and many resolved without topical steroid treatment. Hematologic and hepatobiliary findings were common, but uncomplicated, with complete resolution by 6?months after initial presentation in all. Maternal rheumatologic disease, treatment, and race were not associated with systemic manifestations. Conclusions Patients born to mothers with positive anti‐Ro/SSA titers may benefit from routine cardiac monitoring in utero and at birth. Routine EEG or head ultrasound monitoring in patients who are autoantibody positive for NLE may be unnecessary. Information regarding long‐term outcomes in NLE can be used to guide familial counseling and the use of serial laboratory testing.
机译:摘要背景新生儿狼疮红斑狼疮(NLE)由母体自身抗体的交叉胎盘转移结果。新生儿可以呈现心脏,皮肤,肝胆,血液学和抗体介导的器官毒性的神经系统。节约证据存在于新生儿狼疮患者的长期临床特征和结果。目的是表征Autoantibody剖面,实验室和NLE患者的临床特征。材料/方法这是一个单独的回顾队员对费城儿童医院的患者与NLE的患者。在介绍的临床,实验室和自身抗体谱上收集数据,以及长期并发症。结果包括十三个患者。先天性心脏成分在3名患者中报告,具有1例持续的心脏后遗症。心脏表现与我们的队列中的抗RO / SSA积极性相关。两名患者有神经系统发现,具有良好的长期结果。所有患者都存在皮肤发现,并且许多没有局部类固醇治疗的解决方案。血液学和肝胆调查结果是常见的,但简单,完整的分辨率在初步介绍后6个月。产妇风湿病疾病,治疗和种族与全身表现无关。结论患有阳性抗RO / SSA滴度的母亲出生的患者可能会受益于子宫和出生的常规心脏监测。对于NLE的自身抗体阳性患者的患者常规EEG或头部超声监测可能是不必要的。有关NLE中长期结果的信息可用于指导家庭咨询和串行实验室测试的使用。

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