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Dynamic Thiol-Disulfide Homeostasis in Children With β-Thalassemia Trait

Burçak Kurucu1, Ali Fettah1, Emre Çapkınoğlu1

  • 1Department of Pediatric Hematology and Oncology, University of Healthy Sciences, Ankara Dr. Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Hemoglobin
|May 11, 2022
PubMed

Insights

Children with beta-thalassemia trait exhibit increased oxidative stress, indicated by higher native thiol, total thiol, and disulfide levels. This suggests a compensatory response to combat reactive oxygen species (ROS) production.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Hematology

Background:

  • Oxidative stress and reactive oxygen species (ROS) production contribute to tissue damage in children with beta-thalassemia trait.
  • Dynamic thiol-disulfide homeostasis (DTDH) is a key indicator of the body's pro-oxidant/antioxidant balance.

Purpose of the Study:

  • To investigate the DTDH status in children with beta-thalassemia trait.
  • To compare native thiol, disulfide, and total thiol levels between children with beta-thalassemia trait and healthy controls.

Main Methods:

  • Study included 40 children with beta-thalassemia trait and 30 age- and gender-matched healthy controls.
  • DTDH parameters (native thiol, disulfide, total thiol) were measured using an automated method.
  • Statistical comparison of DTDH parameters between the groups was performed.

Main Results:

  • Children with beta-thalassemia trait showed significantly higher levels of native thiol, total thiol, and disulfide compared to controls (p < 0.001).
  • No significant differences were observed in the ratios of disulfide/native thiol, disulfide/total thiol, or native thiol/total thiol between the groups.
  • Hemoglobin and serum ferritin levels did not correlate with DTDH markers in children with beta-thalassemia trait.

Conclusions:

  • Children with beta-thalassemia trait exhibit elevated native thiol, total thiol, and disulfide levels, indicating increased oxidative stress.
  • The higher disulfide levels suggest significant oxidative stress in beta-thalassemia trait.
  • Increased native and total thiol levels appear to be a compensatory mechanism against oxidative stress in this population.

Keywords:
disulfideoxidative stressthiolβ-Thalassemia (β-thal) trait

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