Xu Dong, Kacha-Ochana Akadia, Morgan Gabrielle A, Huang Chiang-Ching, Pachman Lauren M
Cure JM Program of Excellence in Juvenile Myositis Research at Stanley Manne Children's Research Institute of Ann and Robert H., Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Department of Pediatrics, Division of Rheumatology, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Pediatr Rheumatol Online J. 2017 May 17;15(1):42. doi: 10.1186/s12969-017-0171-3.
A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM).
After obtaining informed consent, the EPC number from 34 fasting children with definite/probable JDM at various stages of therapy-initially untreated, active disease on medication and clinically inactive, off medication-was compared with 13 healthy fasting pediatric controls. The EPC number was determined by fluorescence activated cell sorting (FACS), CD34/VEGFR2/CD45dim, and assessed in conjunction with clinical variables: disease activity scores (DAS), duration of untreated disease (DUD), TNF-α allelic polymorphism (A/G) at the promoter region of -308, number of nailfold capillary end row loop (ERL) and von Willebrand factor antigen (vWF:Ag). Correlations of the EPC numbers with the clinical and demographic variables, including DAS Skin (DAS SK), DAS Weakness (DAS WK), DAS Total Score, DUD, Cholesterol, triglycerides, High-Density Lipoprotein (HDL) and Low-Density Lipoprotein (LDL), and ERL were calculated using the Pearson correlation coefficient. Tests of associations of EPC with gender (boy vs girl), TNF-α-308A allele (GA/AA vs GG), vWF:Ag (categorized by specific ABO type) as normal/abnormal were performed, using two-sample T- tests.
The EPC number for JDM was not significantly different from the healthy controls and was not associated with any of the clinical or cardiovascular risk factors tested.
The EPC for JDM were in the normal range, similar to adults with DM. These data support the concept that the normal EPC numbers in DM/JDM, irrespective of age, differs from adult PM, where they are decreased, perhaps reflecting a different pathophysiology.
一项初步研究，以确定幼年皮肌炎（JDM）患儿的内皮祖细胞（EPC）数量。
在获得知情同意后，将34名处于治疗不同阶段（初始未治疗、用药时疾病活动、临床无活动且停药）的空腹确诊/可能患有JDM的患儿的EPC数量与13名空腹健康儿科对照进行比较。通过荧光激活细胞分选（FACS）、CD34/VEGFR2/CD45dim测定EPC数量，并结合临床变量进行评估：疾病活动评分（DAS）、未治疗疾病持续时间（DUD）、-308启动子区域的肿瘤坏死因子-α等位基因多态性（A/G）、甲襞毛细血管终末排环（ERL）数量和血管性血友病因子抗原（vWF:Ag）。使用Pearson相关系数计算EPC数量与临床和人口统计学变量之间的相关性，包括皮肤DAS（DAS SK）、肌无力DAS（DAS WK）、DAS总分、DUD、胆固醇、甘油三酯、高密度脂蛋白（HDL）和低密度脂蛋白（LDL）以及ERL。使用两样本T检验对EPC与性别（男孩与女孩）、肿瘤坏死因子-α -308A等位基因（GA/AA与GG）、vWF:Ag（按特定ABO血型分类为正常/异常）之间的关联进行检验。
JDM患儿的EPC数量与健康对照无显著差异，且与所测试的任何临床或心血管危险因素均无关联。
JDM患儿的EPC数量在正常范围内，与成年皮肌炎患者相似。这些数据支持以下概念，即DM/JDM中正常的EPC数量与年龄无关，与成年多发性肌炎不同，成年多发性肌炎中EPC数量减少，这可能反映了不同的病理生理学。
