title: Metabolic pathways related to oxidative stress in patients with hemoglobin h disease and iron overload.
abstract: BACKGROUND
  Iron overload is a major complication in patients with hemoglobin H (Hb H) disease and causes damage of tissues.
  
  
  METHODS
  We investigated 26 Hb H patients and 75 controls to evaluate their oxidative stress and antioxidant statuses.
  
  
  RESULTS
  There were significantly increased levels of superoxide anion in leucocytes, nitrite (NO2-), and malondialdehyde (MDA) in plasma, and activities of superoxide dismutase (SOD), glutathione peroxidase (GPx), glutathione reductase (GRx) and oxidized glutathione (GSSG) in erythrocytes, decreased levels of nitrate (NO3-) and vitamin C in plasma, and reduced glutathione (GSH) in erythrocytes, in addition to the abnormal iron status in the patients when compared with those in the controls. Meanwhile, levels of serum ferritin were positively correlated with serum iron, plasma MDA, and erythrocyte SOD in the patients. In addition, the activities of SOD were positively correlated with those of GPx and GRx, and the levels of GSSG and MDA, but negatively correlated with those of GSH. Furthermore, the levels of MDA were negatively correlated those of vitamin C.
  
  
  CONCLUSIONS
  These results demonstrate the presence of oxidative stress and decreased levels of antioxidants; moreover, the related metabolic antioxidant pathway is active in Hb H patients with iron overload.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Division of Hematology-Oncology, Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
authors.forename: Shyh-Shin
authors.initials: SS
authors.lastname: Chiou
authors.forename: Chao-Jung
authors.initials: CJ
authors.lastname: Tsao
authors.forename: Shih-Meng
authors.initials: SM
authors.lastname: Tsai
authors.forename: Ye-Ru
authors.initials: YR
authors.lastname: Wu
authors.forename: Yu-Mei
authors.initials: YM
authors.lastname: Liao
authors.forename: Pei-Chin
authors.initials: PC
authors.lastname: Lin
authors.forename: Li-Yu
authors.initials: LY
authors.lastname: Tsai
mesh_terms: D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood; D000975:Antioxidants / Q000378:metabolism; D016022:Case-Control Studies; D003402:Creatine Kinase / Q000097:blood; D004912:Erythrocytes / Q000201:enzymology; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000097:blood; D019190:Iron Overload / Q000097:blood / Q000150:complications / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D008315:Malondialdehyde / Q000097:blood; D053858:Metabolic Networks and Pathways; D018384:Oxidative Stress; D013482:Superoxide Dismutase / Q000378:metabolism; D055815:Young Adult; D017085:alpha-Thalassemia / Q000097:blood / Q000150:complications / Q000378:metabolism* / Q000473:pathology*
chemical_list: D000975:Antioxidants; D008315:Malondialdehyde; D005293:Ferritins; D007501:Iron; D013482:Superoxide Dismutase; D000410:Alanine Transaminase; D003402:Creatine Kinase
keywords: Hb H disease; antioxidant status; iron overload; malondialdehyde; oxidative stress; superoxide dismutase