Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study

• Published in: Turkish journal of pediatrics Vol. 64; no. 5; pp. 876 - 881
• Main Authors: Tasar, Sercin, Guven, Ayse Gul, Olgac, Asburce, Neselioglu, Salim, Erel, Ozcan, Alioglu, Bulent
• Format: Journal Article
• Language: English
• Published: Ankara Akdema Informatics and Publishing 01-09-2022; Hacettepe University Faculty of Medicine
• Subjects: Analysis; Antioxidants; Blood coagulation factor VIII; Chemical bonds; Children; Health aspects; Hematology; Hemophilia; Independent sample; Mann-Whitney U test; Normal distribution; Oxidation; Oxidative stress; Pathogenesis; Pediatrics; Statistics; Thiols
• ISSN: 0041-4301; 2791-6421
• DOI: 10.24953/turkjped.2022.73

Background. Experimental studies have addressed the role of oxidant stress in the pathogenesis of Hemophilia A. This study aimed to determine whether dynamic thiol-disulfide exchange, a recently recognized cellular defense system against oxidative stress, is disturbed in children with hemophilia A.
Methods. This prospective case control study included male children with hemophilia A (n=62) and randomly selected healthy age and sex-matched controls (n=62). Serum native thiol, total thiol and disulfide levels were analyzed with a novel spectrophotometric method. Ratios of disulfide/total thiol, disulfide/native thiol, and native/total thiol were calculated. Statistical comparisons were made using the independent samples t-test or the Mann-Whitney U test, according to whether the data were normally distributed or not.
Results. Serum native thiol (385.0 ± 35.9 versus 418.0 ± 44.3, respectively; p < 0.001) and total thiol (424.2 ± 38.7 versus 458.0 ± 46.3, respectively; p > 0.001) levels were significantly lower in children with Hemophilia A compared to controls. Children with hemophilia A had significantly lower serum native thiol to total thiol ratio than controls (p=0.024). Serum disulfide levels of children with hemophilia A were close to controls (19.2 [17.6- 22.1] versus 19.8 [17.8- 21.2]), respectively; p=0.879) whereas disulfide to native thiol ratio (p=0.024) and disulfide to total thiol ratio (p=0.024) were significantly higher.
Conclusions. Decreased antioxidant capacity with levels of serum native thiol and total thiol in children with hemophilia A might be regarded as evidence for the disturbance of thiol/disulfide balance. Antioxidant treatment can be a future target of therapy in children with hemophilia A.