Deranged Serum Fasting Lipid Profile in Children and Adolescent with Thalassemia Major

Introduction: Serum fasting lipid profile has been studied in various clinical spectrum of β-thalassemia syndrome. Premature cardiac impairment in thalassemia major (TM) appears primarily due to iron accumulation and oxidative injury; however it might be a sequel of abnormal lipoprotein concentrations. Recent studies revealed impaired lipid profile and atherosclerosis in TM as an emerging complication in patients with prolonged survival. As these children are at risk of premature atherosclerosis, sub clinical atherosclerosis probably begins early in their life might evolve into coronary heart diseases in later life.

The rational of this prospective cross sectional study is to analyze the serum fasting lipid profile in cardiovascular disease free thalassemia major patients and to establish any correlation with age, gender, hematological parameters, liver enzymes and serum ferritin.

Method: The study group comprises of thirty six patients with β-thalassemia major, who were enrolled from March 2012 to March 2014. Fasting blood lipid levels (total lipid, cholesterol, triglycerides, HDL and LDL-cholesterol), liver function test and serum ferritin were measured in all study participants on Hitachi 912 through photometric assay (for lipid profile and liver function test) and by immunoturbidity methodology for serumferritin. Hematological parameters were determined by Cell Dyne Ruby (Abbot, Architect, USA). Patients were stratified in two groups, age ≤ 15 years and >15 years, to determine the possible lipid profile distinction in relation to increasing age.

Results: Of the 36 patients, 17 were males (15.5±5.9 years) and 19 were females (10.1±4.5 years) with the mean age of 12.69±5.85 (range 5-24) years. Mean hemoglobin was 7.4±1.9gm/dl with the hematocrit of 23.1±5.7% while serum ferritin was markedly elevated 4699.7±3089ng/ml. The mean total lipid, cholesterol and triglycerides levels were 494.7 ± 114.5, 90.3 ±23.9 and 150.5±95.1 mg/dl respectively. High triglycerides were detected in 36.1%. While HDL cholesterol and LDL cholesterol were markedly low 17.7±9.2 and 42.3±22.0 mg/dl respectively. The mean total cholesterol to HDL ratio was significantly elevated, 6.21± 3.06 (normal <5.0). In addition, total-cholesterol to HDL-cholesterol ratios were increased irrespective of gender, that is 6.0±2.0 and 6.4± 3.8 in males and females respectively (P=0.6). Data analysis revealed that 22.2% females and 13.8% males had high triglyceride levels (P=0.05). No statistically significant difference was noted in two stratified age groups in respect to lipid profile (table-1).

We established positive correlation of elevated total bilirubin with cholesterol, HDL and LDL cholesterol (P<0.05). Cholesterol ratio was also significantly high in patients with hemoglobin > 8gm/dl (P<0.05). Low HDL cholesterol was correlated positively with LDL cholesterol levels. While no significant correlation of serum lipid could be established with age, gender, high ferritins and with liver enzymes.

Conclusion: Our study revealed that lipid profile impairment is not unusual in β-thalassemia major patients irrespective of age. Considerable deficiency of high density cholesterol and significantly high total-cholesterol to HDL-cholesterol ratio may strongly be associated with occurrence of coronary heart disease in later life. We propose that lipid profile should be regularly done in these patients particularly those with hemoglobin > 8gm/dl and high total bilirubin levels. Whilst total to HDL ratio, underlines as a significant diagnostic marker for future cardiac events in these patients.

Keywords: β-thalassemia major, triglyceride, HDL-cholesterol, LDL-cholesterol.

*Not significant