TY - JOUR
T1 - Endothelial progenitor cell number is not decreased in 34 children with Juvenile Dermatomyositis
T2 - A pilot study
AU - Xu, Dong
AU - Kacha-Ochana, Akadia
AU - Morgan, Gabrielle A.
AU - Huang, Chiang Ching
AU - Pachman, Lauren M
PY - 2017/5/17
Y1 - 2017/5/17
N2 - Objective: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM). Methods: 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. Results: 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. Conclusion: 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.
AB - Objective: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM). Methods: 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. Results: 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. Conclusion: 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.
KW - Endothelial Progenitor Cell (EPC)
KW - Fluorescence Activated Cell Sorting (FACS)
KW - Juvenile Dermatomyositis (JDM)
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U2 - 10.1186/s12969-017-0171-3
DO - 10.1186/s12969-017-0171-3
M3 - Article
C2 - 28514969
AN - SCOPUS:85019636636
VL - 15
JO - Pediatric Rheumatology
JF - Pediatric Rheumatology
SN - 1546-0096
IS - 1
M1 - 42
ER -