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Reduced antioxidant capacity in paediatric patients with homozygous sickle cell disease
D A Adelekan1, D I Thurnham, A D Adekile
1Department of Paediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Nigeria.
Insights
Children with sickle cell disease (SS) have lower antioxidant levels, particularly alpha-tocopherol. This suggests a potential role for antioxidant supplementation in managing sickle cell disease complications.
Area of Science:
- Biochemistry
- Hematology
- Nutritional Science
Background:
- Sickled erythrocytes are prone to lipid peroxidation.
- Antioxidants may play a role in mitigating this process.
Purpose of the Study:
- To investigate antioxidant vitamin levels in children with sickle cell disease (SS).
- To compare antioxidant status between SS patients and healthy controls.
Main Methods:
- Measured plasma levels of tocopherol, retinol, carotenes, and ascorbic acid in 22 SS patients and 9 controls.
- Utilized HPLC for tocopherol, carotenes, and retinol; colorimetry for ascorbic acid.
- Assessed riboflavin status using the glutathione reductase activation test.
Main Results:
- Antioxidant levels, except ascorbate, were reduced in SS patients.
- Significantly lower plasma alpha-tocopherol concentrations were observed in SS patients compared to controls.
- Poorer vitamin A status (plasma retinol) was noted in SS patients.
Conclusions:
- Children with sickle cell disease exhibit reduced levels of key antioxidant vitamins.
- Significantly lower alpha-tocopherol levels in SS patients warrant further investigation.
- These findings highlight potential nutritional deficiencies in sickle cell disease management.
Abstract:
The sickled erythrocyte has been shown to be susceptible to lipid peroxidation and a role has been suggested for antioxidants in this process. The present study was undertaken in 22 children, aged 5-18 years with homozygous sickle cell disease (SS) and 9 HbAA controls (AA) of similar age. All the SS patients were in steady state ie, not in crisis or any acute illness at the time of the study. Levels of plasma tocopherol, retinol, carotenes and ascorbic acid (antioxidant vitamins of major nutritional importance) were measured. Plasma tocopherol carotenes and retinol were measured by HPLC after extraction into heptane. Total ascorbic acid (in trichloroacetic acid extracts of plasma) was measured colorimetrically following reaction with 2,4-dinitrophenylhydrazine. Riboflavin status was measured by the glutathione reductase activation test. Levels of all the measured antioxidants except ascorbate were reduced in SS patients compared with control children but only plasma alpha-tocopherol concentration was significantly different between the patients and controls. The median tocopherol level in SS patients (11.32 mumol/l) was significantly lower (P less than 0.02 Mann-Whitney) than that in control children (18.02 mumol/l) when measured directly or when calculated from tocopherol: cholesterol ratio, 4.55 mumol/mmol in SS patients and 7.50 mumol/mmol in control children. The median concentration of total plasma carotenes of SS patients (5.67 mumol/l) was lower than that of control children (12.14 mumol/l). Similarly, plasma beta-carotene concentration of SS patients was lower than that of control children but the difference in each case was not significant. Despite this, the vitamin A status (plasma retinol concentration) of SS patients was poorer than that of control children.(ABSTRACT TRUNCATED AT 250 WORDS)