title: Decreased nitrite levels in erythrocytes of children with β-thalassemia/hemoglobin E.
abstract: Nitrite anion is bioactive nitric oxide (NO) species circulating in blood, and represents the NO bioavailability and endothelial function. In this study, we aimed to investigate the nitrite levels and the correlation with hemolysis and severity in β-thalassemia/hemoglobin E (β-thal/HbE). 38 Children (12.0±1.9 years of age) with a diagnosis of mild, moderate and severe β-thalassemia were enrolled in the study. The blood nitrite levels and potential plasma NO consumption were measured by the chemiluminescence method. The nitrite levels in whole blood and erythrocytes of the severe thalassemia subjects were lower than those of the control subjects. At day 7 after transfusion of packed erythrocytes, the nitrite levels in erythrocytes increased. The plasma hemoglobin and NO consumption increased in the severe thalassemia subjects. The nitrite levels in erythrocytes inversely correlated with plasma hemoglobin, lactate dehydrogenase activity, potential NO consumption, and lipid peroxidation. Our studies demonstrate the decreased NO bioavailability in thalassemia, which could result from endothelial dysfunction, the increased potential NO consumption in plasma by cell-free hemoglobin and oxidative stress.
authors.affiliation: Department of Pharmacology, Faculty of Science, Mahidol University, Bangkok 10400, Thailand.
authors.forename: Thitiwat
authors.initials: T
authors.lastname: Suvachananonda
authors.forename: Amara
authors.initials: A
authors.lastname: Wankham
authors.forename: Sirada
authors.initials: S
authors.lastname: Srihirun
authors.forename: Pansakorn
authors.initials: P
authors.lastname: Tanratana
authors.forename: Supeenun
authors.initials: S
authors.lastname: Unchern
authors.forename: Suthat
authors.initials: S
authors.lastname: Fucharoen
authors.forename: Ampaiwan
authors.initials: A
authors.lastname: Chuansumrit
authors.forename: Nongnuch
authors.initials: N
authors.lastname: Sirachainan
authors.forename: Nathawut
authors.initials: N
authors.lastname: Sibmooh
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D016022:Case-Control Studies; D002648:Child; D004912:Erythrocytes; D005260:Female; D006446:Hemoglobin E / Q000378:metabolism*; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D008297:Male; D009569:Nitric Oxide / Q000097:blood; D020030:Nitric Oxide Donors / Q000008:administration & dosage / Q000493:pharmacokinetics; D009573:Nitrites / Q000097:blood*; D009603:Nitroso Compounds / Q000008:administration & dosage / Q000493:pharmacokinetics; D017086:beta-Thalassemia / Q000097:blood* / Q000188:drug therapy / Q000378:metabolism
chemical_list: D006454:Hemoglobins; D020030:Nitric Oxide Donors; D009573:Nitrites; D009603:Nitroso Compounds; C094210:2,2'-(hydroxynitrosohydrazono)bis-ethanamine; D009569:Nitric Oxide; D006446:Hemoglobin E
keywords: Cell-free hemoglobin; Erythrocytes; Nitric oxide; Nitrite; Thalassemia