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Abstract Background: Beta thalassemia major is associated with serum lipids abnormalities, presented as hypocholesterolemia, decreased serum low-density lipoproteins (LDL) level and high-density lipoprotein (HDL) level, and hypertriglyceridemia, increasing risk for premature atherosclerosis and other cardiovascular complications. Some previous studies revealed that vitamin D is associated with favorable lipid profile in pediatrics. However, its role needs to be determined in a high-risk group of children with transfusion dependent thalassemia (TDT). Aim: To investigate the correlation between serum lipid profile and dyslipidemia with serum vitamin D level in TDT patients. Methods: A cross sectional observational study was conducted at Pediatrics Hematology Unit of Cairo University Children Hospital from January to April 2021. Subjects were classified into two groups 1st group composing of 50 children suffering from TDT. Another group of 30 apparent healthy children matched for age and sex were included in the study as a control group. The measurement of vitamin D (25-OHD) level was done using HPLC-UV method and 10-12 hour overnight fasting serum lipid profile including total cholesterol, HDL, LDL, and triglyceride were performed. The analysis was done using automated chemistry analyzer Cobas C 311. P value < 0.05 was considered significant. Results: Fifty TDT patients were included in this study, with serum ferritin level ranged from 326 ng/mL to 8698 ng/mL. TDT patients had significant lower cholesterol, HDL and LDL besides higher level of TGs. The prevalence of vitamin D deficiency (a 25-OH D3 level less than 20 ng/mL) in this study was 20%, with mean value at 33.91 ng/mL. It was found that the median for Atherogenic Index Plasma was 0.36 (Atherogenic Index Plasma value at more than 0.24 is a significant predictor for cardiovascular diseases). Statistical analysis revealed that Vitamin D had negative correlation with total cholesterol (r = -0.288, p = 0.010), LDL (r = -0.287, p = 0.010), and HDL (r = -0.280, p = 0.012). There was no significant correlation between vitamin D and triglyceride (p = 0.379). Conclusion: Measurement of serum lipid profile may help in early detection of cardiovascular complications such as premature atherosclerosis among these patients. AIP may serve as an additional indicator of dyslipidemia. In addition, Our results reinforce previously published reports of vitamin D deficiency in TDT patients and demonstrated its correlation with dyslipidemia |