Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study
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 hemophi...
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Hacettepe University Institute of Child Health
2022-10-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/220 |
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| author | Serçin Taşar Ayşe Gül Güven Asburçe Olgaç Salim Neşelioğlu Özcan Erel Bülent Alioğlu |
| author_facet | Serçin Taşar Ayşe Gül Güven Asburçe Olgaç Salim Neşelioğlu Özcan Erel Bülent Alioğlu |
| author_sort | Serçin Taşar |
| collection | DOAJ |
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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.
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| format | Article |
| id | doaj-art-0d4bb37655ac4c21bcc2bd71815c81a4 |
| institution | DOAJ |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2022-10-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-0d4bb37655ac4c21bcc2bd71815c81a42025-08-20T03:01:10ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212022-10-0164510.24953/turkjped.2022.73Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control studySerçin Taşar0Ayşe Gül Güven1Asburçe Olgaç2Salim Neşelioğlu3Özcan Erel4Bülent Alioğlu5Department of Pediatrics, Ankara Training and Research Hospital, Ankara.Department of Pediatrics, Ankara Training and Research Hospital, Ankara.Department of Pediatric Metabolic Diseases, Dr.Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara.Department of Clinical Biochemistry, Ankara Yıldırım Beyazıt University Faculty of Medicine, Ankara.Department of Clinical Biochemistry, Ankara Yıldırım Beyazıt University Faculty of Medicine, Ankara.Department of Pediatric Hematology, Ankara Training and Research Hospital, Ankara, Türkiye. 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. https://turkjpediatr.org/article/view/220Hemophilia Achildrenoxidative stressthiol |
| spellingShingle | Serçin Taşar Ayşe Gül Güven Asburçe Olgaç Salim Neşelioğlu Özcan Erel Bülent Alioğlu Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study The Turkish Journal of Pediatrics Hemophilia A children oxidative stress thiol |
| title | Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study |
| title_full | Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study |
| title_fullStr | Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study |
| title_full_unstemmed | Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study |
| title_short | Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study |
| title_sort | decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia a a prospective case control study |
| topic | Hemophilia A children oxidative stress thiol |
| url | https://turkjpediatr.org/article/view/220 |
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