| 罕见病眼痉挛-肌肉痉挛-共济失调综合征合并神经母细胞瘤患儿血清中6种自身抗体表达及意义 |
| 投稿时间：2016-07-21 修订日期：2016-07-21 点此下载全文 |
| 引用本文：丁绪,韩炜,杨维,秦红,王静,王焕民,王旭.罕见病眼痉挛-肌肉痉挛-共济失调综合征合并神经母细胞瘤患儿血清中6种自身抗体表达及意义[J].医学研究杂志,2017,46(3):51-55 |
| DOI：
10.11969/j.issn.1673-548X.2017.03.014 |
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| 基金项目:北京市优秀人才培养资助青年骨干个人项目（2015000021469G204）；首都医科大学基础-临床科研合作基金资助项目（15JL70，16JL20） |
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| 中文摘要:目的 探讨罕见病眼痉挛-肌肉痉挛-共济失调综合征（OMS）合并神经母细胞瘤（NB）患儿血清中差异性自身抗体及OMS合并NB患儿术前、术后自身抗体浓度变化，有助于研究OMS疾病机制及治疗。方法 采集单纯NB患儿术前血清6例，OMS合并NB患儿术前、术后血清各4例。ELISA法检测血清中普肯耶细胞抗体1型（PCA-1）、普肯耶细胞抗体2型（PCA-2）、抗坍塌反应调节蛋白5（CRMP5）抗体、抗神经元核抗体1型（ANNA-1）、抗神经元核抗体2型（ANNA-2）、抗神经元核抗体3型（ANNA-3）的浓度。结果 与单纯NB患儿相比，OMS合并NB患儿血清中除ANNA-3无差异，其余5种自身抗体浓度均明显高于单纯NB患儿。OMS合并NB患儿血清中PCA-2、抗CRMP5抗体、ANNA-1、ANNA-2浓度与OMS症状评分呈正相关。与术前比较，术后7天OMS合并NB患儿血清中除ANNA-2浓度降低，其余5种自身抗体均无改变。结论 在所研究人群范围内，OMS可以导致自身抗体PCA-1、PCA-2、抗CRMP5抗体、ANNA-1浓度增加，可能是OMS的发病机制之一。 |
| 中文关键词:眼痉挛-肌肉痉挛-共济失调综合征 神经母细胞瘤 自身抗体 |
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| Six Kinds of Autoantibodies in Children with Opsoclonus-myoclonus Syndrome and Neuroblastoma from Beijing Children's Hospital |
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| Abstract:Objective To investigate the types and doses of autoantibodies in the serum of children with OMS and NB compared with children with NB only, and detect the changes in autoantibodies in children with OMS and NB before and after operation treatment. Methods This study included 6 children with NB aged 3-6 years old and 4 children with OMS and NB with similar age from Beijing children's hospital from Nov. 2015 to July 2016. Purkinje cell cytoplasmic autoantibody type 1 (PCA-1), Purkinje cell cytoplasmic autoantibody type 2 (PCA-2), anti-collapsin response mediator protein 5 (CRMP5) autoantibody, anti-neuronal nuclear antibodies type 1 (ANNA-1), anti-neuronal nuclear antibodies type 2 (ANNA-2), anti-neuronal nuclear antibodies type 3 (ANNA-3) were detected using ELISA assay. Results Six kinds of autoantibodies were all found in serum in children with NB and in children with OMS and NB. Compared to the children with NB, the level of PCA-1, PCA-2, anti-CRMP5 autoantibody, ANNA-1 and ANNA-2 were all obviously higher in children with OMS and NB. And the concentration of PCA-2, anti-CRMP5 autoantibody, ANNA-1, ANNA-2 are correlated with the OMS symptom scores. In addition, the dose of five kinds of autoantibodies did not significantly change before and after surgical resection of NB in the serum of OMS and NB children. Conclusion The higher level of autoantibodies may play an important role in OMS. |
| keywords:Opsoclonus-myoclonus syndrome Neuroblastoma Autoantibody |
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