title: Amelioration of serum 8-OHdG level by enzyme replacement therapy in patients with Fabry cardiomyopathy.
abstract: The level of 8-hydroxy-2-deoxyguanosise (8-OHdG) is a marker of oxidative stress. The objective of this study was to evaluate the effect of enzyme replacement therapy (ERT) on the level of 8-OHdG in patients with Fabry cardiomyopathy and the clinical evolution of Fabry cardiomyopathy.
  
  
  
  We measured the serum levels of 8-OHdG in 20 healthy control and 22 patients with Fabry cardiomyopathy before and after ERT.
  
  
  
  The mean lysoGb3 and 8-OHdG levels was significantly increased in patients with Fabry cardiomyopathy compared with that of control subjects (lysoGb3, 3.6 ± 1.1 nM vs. 0.4 ± 0.1 nM, p < 0.01; 8-OHdG, 4.5 ± 0.5 ng/mL vs. 3.4 ± 0.4 ng/mL, P < 0.05). The mean lysoGb3 and 8-OHdG levels was significantly reduced after ERT for 14.2 months (lysoGb3, 3.6 ± 1.1 nM vs. 2.9 ± 1.1 nM, P < 0.05; 8-OHdG, 4.5 ± 0.5 ng/mL to 4 ± 0.4 ng/mL, P < 0.05). These changes were accompanied by decreases in LVM and LVMI.
  
  
  
  We demonstrated that the serum 8-OHdG levels is increased in patients with Fabry cardiomyopathy (FC) and that successful management of FC with ERT is associated with a decrease in this oxidative stress marker. Serum 8-OHdG levels can be used not only as a noninvasive biomarker of oxidative stress in patients with FC but also an objective and quantitative parameter in the follow-up of patients during ERT.
authors.affiliation: Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pharmacy, Taipei, Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Kuan-Hsuan
authors.initials: KH
authors.lastname: Chen
authors.affiliation: Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pharmacy, Taipei, Veterans General Hospital, Taipei, Taiwan, ROC; School of Pharmacy, Taipei Medical University, Taipei, Taiwan, ROC.
authors.forename: Yueh-Ching
authors.initials: YC
authors.lastname: Chou
authors.affiliation: Department of Surgery, National Yang-Ming University Hospital, Taipei, Taiwan, ROC.
authors.forename: Chen-Yuan
authors.initials: CY
authors.lastname: Hsiao
authors.affiliation: Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Yueh
authors.initials: Y
authors.lastname: Chien
authors.affiliation: School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC; Division of Cardiology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Kang-Ling
authors.initials: KL
authors.lastname: Wang
authors.affiliation: Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Ying-Hsiu
authors.initials: YH
authors.lastname: Lai
authors.affiliation: Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pharmacy, Taipei, Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Yuh-Lih
authors.initials: YL
authors.lastname: Chang
authors.affiliation: Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pediatrics, Taipei, Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Dau-Ming
authors.initials: DM
authors.lastname: Niu
authors.affiliation: School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC; Division of Cardiology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC. Electronic address: wcyu@vghtpe.gov.tw.
authors.forename: Wen-Chung
authors.initials: WC
authors.lastname: Yu
mesh_terms: D000080242:8-Hydroxy-2'-Deoxyguanosine; D000368:Aged; D015415:Biomarkers / Q000097:blood; D009202:Cardiomyopathies / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D016022:Case-Control Studies; D003849:Deoxyguanosine / Q000031:analogs & derivatives* / Q000097:blood; D056947:Enzyme Replacement Therapy; D000795:Fabry Disease / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006017:Glycolipids / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged; D008991:Monitoring, Physiologic; D018384:Oxidative Stress; D013107:Sphingolipids / Q000097:blood*; D016896:Treatment Outcome; D000519:alpha-Galactosidase / Q000627:therapeutic use*
chemical_list: D015415:Biomarkers; D006017:Glycolipids; D013107:Sphingolipids; C063288:globotriaosyl lysosphingolipid; D000080242:8-Hydroxy-2'-Deoxyguanosine; D000519:alpha-Galactosidase; D003849:Deoxyguanosine
keywords: Cardiomyopathy; ERT; Fabry disease; Oxidative stress