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Reactive oxygen metabolites and prooxidant status in children with Down's syndrome
M Carratelli1, L Porcaro, M Ruscica
1Diacron S.r.l., Diagnostic Division, Grosseto, Italy.
Insights
Children with Down syndrome exhibit elevated reactive oxygen species and reduced antioxidant capacity, indicating a prooxidant state. This oxidative stress may contribute to various health issues associated with the syndrome.
Area of Science:
- Biochemistry
- Pediatrics
- Genetics
Background:
- Down syndrome is associated with numerous health complications, including cardiovascular diseases and immune defects.
- The pathogenesis of Down syndrome may involve increased reactive oxygen species (ROS) due to elevated superoxide dismutase activity.
- Understanding the oxidative status in children with Down syndrome is crucial for managing associated health risks.
Purpose of the Study:
- To evaluate reactive oxygen species (ROS) and total antioxidant capacity (TAC) in children with Down syndrome.
- To investigate the potential role of oxidative stress in the pathophysiology of Down syndrome.
- To introduce new spectrophotometric methods for measuring oxidative status in serum.
Main Methods:
- Spectrophotometric analysis of reactive oxygen species (ROS) and total antioxidant capacity (TAC).
- Comparison of oxidative markers between 40 children with Down syndrome and 20 healthy controls.
- Measurement of thiol groups (sulfhydryl) as an indicator of antioxidant defense.
Main Results:
- Children with Down syndrome showed significantly higher ROS levels (452 U.Carr) compared to controls (270 U.Carr).
- Total antioxidant capacity (TAC) was significantly lower in children with Down syndrome (281 micromol HCLO/ml) than in controls (380 micromol HCLO/ml).
- Thiol group levels were significantly lower in children with Down syndrome (462 micromol/l) compared to controls (644 micromol/l).
Conclusions:
- Children with Down syndrome experience a prooxidant environment due to elevated ROS and reduced antioxidant defenses.
- Oxidative stress is implicated in the development of complications like atherosclerosis, early aging, and neurological disorders in Down syndrome.
- The developed spectrophotometric methods are reliable for assessing oxidative status and can serve as early markers of tissue damage in Down syndrome.
Abstract:
Children with Down's syndrome suffer many diseases among which cardiovascular diseases, increased susceptibility to infections, leukemia, endocrine alterations, immune defects, nutritional disturbance and mental retardation have clinical relevance. It has been suggested that the pathogenesis of Down's syndrome involves reactive oxygen species arising from a mutation in gene encoding, which disproportionately elevates superoxide dismutase activity. Reactive oxygen species and total antioxidant capacity were evaluated using two new spectrophotometric methods in a selected group of 40 children with Down's syndrome and in 20 apparently healthy children used as controls. Reactive oxygen species were significantly higher (p <0.05) in children with Down's syndrome than in controls: 452 (+/- 72) U.Carr vs. 270 (+/- 66) U.Carr respectively. Total antioxidant capacity was significantly higher (p <0.05) in controls than in children with Down's syndrome: 380 (+/- 52) micromol hypochlorous acid (HCLO)/ml vs. 281 (+/- 33) micromol HCLO/ml, respectively. In fact, thiol groups (sulfhydryl) were significantly higher (p <0.05) in controls than in children with Down's syndrome: 644 (+/- 78) micromol/l vs. 462 (+/- 54) micromol/l, respectively Our data show how to simply measure chemical indices of oxidative status in serum samples from children with Down's syndrome. We determined the plasmatic activities of reactive oxygen metabolites and oxidative defense molecules. Accumulated macromolecular damage may be one of the causes of some of the abnormalities that are considered part of the syndrome. Therefore, children with Down's syndrome have to cope with a significant prooxidant environment. Oxidative stress causes alterations such as atherosclerosis, early aging, immunological default and neurologic disorders in Down's syndrome patients. The new test available for measuring reactive oxygen species in serum proved to be reliable and useful as an early marker of tissue damage.