Metabolic Profile & Body Composition in Egyptian Children with Transfusion-Dependent Egyptian B-Thalassemia

Background: Metabolic challenges in beta thalassemia major (BTM) carry an additional burden to both chronic anemia and iron overload. Although optimal nutritional support for children with BTM is mandatory, health insurance cover only blood transfusion and chelation therapy. Nutrition support remains subtle for both the family and governmental interest especially in developing countries. Aim: To assess growth, total body composition, lipid and glucose hemostasis in Egyptian children with BTM in relation to iron overload and state of chronic hypoxemia. Subjects and methods: 200 Egyptian children with BTM were recruited, they were 99 females (49.5%) and 101 male (50.5%) and their mean age was 12.15 ± 4.23 years. 50 age- and sex-matched healthy children with same socio-economic background were also enrolled. All of them subjected to history taking; thorough clinical examination, anthropometric measurements (weight, height, mid arm circumference, skin fold thickness and body mass index), body composition analysis (using bioelectric impedance analysis device) and dietetic assessment (using 24-hours dietetic recall). Serum lipid profile (high-density lipoprotein (HDL), low-density lipoprotein (LDL), cholesterol and triglyceride), C-peptide and serum insulin were assessed. Results: Most children with BTM had families with high birth order and nearly half of them had positive family history, in addition consanguinity was reported to be 1.5 times in BTM compared to what was reported in controls. Almost half of the patients were from Cairo, the other half were from the economically less privileged Upper Egypt. Most of patients were diagnosed presented in 1st two years of life at mean age of 1.52 ± 1.66 and 1^st transfusion was initiated within first four months after initial diagnosis. Most of patient transfused on low mean pre-transfusion hemoglobin of 8.4± 2.6 g/dl. Most studied BTM had iron overload, median (IQR) of serum ferritin was 1800 (1149 - 3100) ng/ml in the last 2 years, with median (IQR) duration on regular iron chelation was 4 (2-6) years and 12.5% of patients were non-compliant. Children with BTM had a significantly lower nutritional intake (kilocalories, protein, carbohydrate, calcium, and phosphorus) than controls with negative impact on anthropometric parameters and body composition parameters both in pubertal and pre-pubertal stage. There was significant increase in body fat value and percent in female BTM compared to male BTM. While there was significant increase in muscle mass % and body water % in males BTM. Children with BTM had significantly higher triglycerides levels however lower cholesterol, LDL, HDL, insulin and C-peptide compared to control. Factors including age, gender, caloric intake, mean pre-transfusion hemoglobin, transfusion burden, splenectomy, viral hepatitis comorbidity, mean serum ferritin, serum insulin, and HDL have impact on body composition in children with BTM.

Conclusion: Poor caloric intake with low pre-transfusion hemoglobin and higher serum ferritin were the main risk factors for poor anthropometric parameters in all ages. Regular assessment of nutrition is crucial for the health of children with thalassemia.

Keywords: Thalassemia, Body composition; Nutritional deficiency; Diabetes; Lipid