Beta Thalassemia is a genetic disorder inherited by autosomal recessive. According to epidemiological studies in Indonesia, beta-thalassemia genes are found with a frequency ranging from 3-10%. The prevalence of vitamin D deficiency was reported to be high in thalassemia patients (2-87.5%). A report from North India showed the prevalence of vitamin D deficiency in about 80% of thalassemia patients. Another study from Thailand showed vitamin D deficiency in 90% of thalassemia patients.
The cause of vitamin D deficiency in thalassemia is not known with certainty. However, this is thought to be the result of an increase in liver iron due to repeated transfusions. Iron deposition in the liver causes disruption of the enzyme 25-hydroxylase in the liver, which is required for the hydroxylation of vitamin D. In addition, hyperpigmentation is also common in patients with thalassemia can inhibit the conversion of vitamin D by sunlight.
Beta thalassemia requires lifelong transfusions, which can cause an accumulation of iron in the skin, liver, and kidneys, resulting in a decrease in vitamin D synthesis.
A cross-sectional study was conducted to analyze the levels of 25(OH)D in children with beta-thalassemia compared with non-thalassemia, conducted at Wahidin Sudirohusodo Hospital Makassar from April to July 2021. This study involved 60 children aged six months to 18 years, consisting of 30 beta-thalassemia and 30 non-thalassemic children.
The results showed that the levels of 25-OHVitamin D were lower in beta-thalassemia children than non-thalassemic children, with a p-value =0.012. Children with beta-thalassemia have a 4.33 times higher vitamin D deficiency risk than nonthalassemic children.
It was concluded that levels of 25-OH-Vitamin D in beta-thalassemia children were lower than in non-thalassemic children. Levels of 25-OH-Vitamin D in children with beta HbE thalassemia are lower than in children with beta-thalassemia major.
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References:
1. Ridha NR, Gautama J, Ganda IJ. Analysis of vitamin d levels in children with thalassemia beta. International Journal of Health Science & Medical Research 2022;1(1): 46-60.
2. Albayrak C, Albayrak D. Vitamin D deficiency in children with beta thalassemia major and intermedia. Turkiye Klin J Med Sci. 2013;33(4):1058–63.
3. Agrawal A, Garg M, Singh J, Mathur P, Khan K. A comparative study of 25 hydroxy vitamin D levels in patients of thalassemia and healthy children. Pediatr Rev Int J Pediatr Res.2016;3(09):652-6.
