PMID- 26919792 OWN - NLM STAT- MEDLINE DCOM- 20170717 LR - 20220331 IS - 1916-7075 (Electronic) IS - 0828-282X (Linking) VI - 32 IP - 10 DP - 2016 Oct TI - Evaluation of Proinflammatory Prognostic Biomarkers for Fabry Cardiomyopathy With Enzyme Replacement Therapy. PG - 1221.e1-1221.e9 LID - S0828-282X(15)01578-0 [pii] LID - 10.1016/j.cjca.2015.10.033 [doi] AB - BACKGROUND: Fabry disease (FD) causes progressive glycosphingolipid accumulation and damage in various organs, and several proinflammatory processes may be involved in this disease. Enzyme replacement therapy (ERT) can reduce the severity of Fabry cardiomyopathy (FC), but whether ERT could attenuate proinflammatory cytokines in FC remains unclear. In this study, we attempted to evaluate the efficacy of ERT on proinflammatory cytokines and vascular cell adhesion biomarkers. METHODS: We enrolled 25 patients with FC and administered ERT to them according to the present clinical guideline. We analyzed and compared echocardiographic and blood examination results between 25 patients with FD without left ventricular hypertrophy (LVH), 25 patients with FC with LVH who were receiving ERT, and 25 healthy age- and sex-matched controls. The parameters of cardiac function at baseline and 12 months after ERT were assessed through echocardiography, and the expression profiles of proinflammatory biomarkers were determined. RESULTS: Left ventricular mass (LVM), LVM index (LVMI), interventricular septal thickness at diastole, and serum levels of globotriaosylsphingosine (Gb3) were elevated in patients with FC. Meanwhile, several proinflammatory cytokines, including interleukin (IL)-6, IL-2, IL-1b, tumor necrosis factor-alpha, intercellular adhesion molecule, soluble vascular cell adhesion molecule, and monocyte chemoattractant protein-1 (MCP-1) were concomitantly increased. ERT significantly reduced these transthoracic echocardiographic parameters and lyso-Gb3 and proinflammatory cytokine levels. The changes in IL-6, MCP-1, and lyso-Gb3 levels were positively correlated with the change in LVMI. CONCLUSIONS: Our study has revealed that proinflammatory biomarkers, particularly IL-6 and MCP-1, may represent effective biomarkers for evaluating ERT outcomes in patients with FC. CI - Copyright (c) 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved. FAU - Chen, Kuan-Hsuan AU - Chen KH AD - Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan. FAU - Chien, Yueh AU - Chien Y AD - Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan; Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Pharmacy, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Wang, Kang-Ling AU - Wang KL AD - School of Medicine, National Yang-Ming University, Taipei, Taiwan; Divison of Cardiology and Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Leu, Hsin-Bang AU - Leu HB AD - Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan; Health Care and Management Center, Taipei Veterans General Hospital, Taipei, Taiwan; Divison of Cardiology and Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Hsiao, Chen-Yuan AU - Hsiao CY AD - Department of Surgery, National Yang-Ming University Hospital, Taipei, Taiwan. FAU - Lai, Ying-Hsiu AU - Lai YH AD - Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Wang, Chien-Ying AU - Wang CY AD - School of Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Chang, Yuh-Lih AU - Chang YL AD - Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan; Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Lin, Shing-Jong AU - Lin SJ AD - Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan; Divison of Cardiology and Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Niu, Dau-Ming AU - Niu DM AD - Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Pediatrics, Taipei, Veterans General Hospital, Taipei, Taiwan. FAU - Chiou, Shih-Hwa AU - Chiou SH AD - Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan; Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan; Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Yu, Wen-Chung AU - Yu WC AD - School of Medicine, National Yang-Ming University, Taipei, Taiwan; Divison of Cardiology and Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan. Electronic address: wcyu@vghtpe.gov.tw. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20151110 PL - England TA - Can J Cardiol JT - The Canadian journal of cardiology JID - 8510280 RN - 0 (Biomarkers) RN - 0 (Cytokines) RN - 0 (Glycolipids) RN - 0 (Sphingolipids) RN - 126550-86-5 (globotriaosyl lysosphingolipid) RN - EC 3.2.1.22 (alpha-Galactosidase) SB - IM MH - Adult MH - Biomarkers/blood MH - Case-Control Studies MH - Cytokines/*blood MH - Echocardiography MH - *Enzyme Replacement Therapy MH - Fabry Disease/complications/*therapy MH - Female MH - Glycolipids/blood MH - Heart Ventricles/diagnostic imaging MH - Humans MH - Hypertrophy, Left Ventricular/blood/etiology/*therapy MH - Male MH - Middle Aged MH - Prognosis MH - Sphingolipids/blood MH - alpha-Galactosidase/*therapeutic use EDAT- 2016/02/28 06:00 MHDA- 2017/07/18 06:00 CRDT- 2016/02/28 06:00 PHST- 2015/07/31 00:00 [received] PHST- 2015/10/12 00:00 [revised] PHST- 2015/10/28 00:00 [accepted] PHST- 2016/02/28 06:00 [pubmed] PHST- 2017/07/18 06:00 [medline] PHST- 2016/02/28 06:00 [entrez] AID - S0828-282X(15)01578-0 [pii] AID - 10.1016/j.cjca.2015.10.033 [doi] PST - ppublish SO - Can J Cardiol. 2016 Oct;32(10):1221.e1-1221.e9. doi: 10.1016/j.cjca.2015.10.033. Epub 2015 Nov 10.