Body Composition in Pediatric Patients With Thalassemia Major Attending Assiut University Children's Hospital

Thalassemias are severe inherited anemias characterized by microcytic, hypochromic, and short-lived red blood cells due to defective hemoglobin synthesis(1) β-thalassemias are a heterogeneous group of hereditary hemoglobinopathies characterized by defects in the β-globin chain of hemoglobin and belong to autosomal recessive disorders(2) Three clinical and hematological conditions of increasing severity are recognized and include beta-thalassemia carrier state, thalassemia intermedia, and thalassemia major (3, 4).

Regular blood transfusion is a vital treatment in β-thalassemia major patients to prevent the consequences of anemia. Iron overloading is frequently observed in β-thalassemia major patients with transfusion therapy . Excessive iron can cause multiple organ damage (5, 6, 7, 8).

Patients with β-thalassemia major (BTM) suffer from several patterns of growth retardation including short stature, underweight, wasting, and decreased bone density(9, 10).

Impaired nutritional status has been commonly observed in patients with thalassemia major. Specific nutritional deficiencies, such as vitamin D deficiency, are also often observed due to increased losses of micronutrients or increased endogenous requirements (11).

Patients suffering from thalassemia major have impaired body composition, especially whole-body lean mass and bone mineral density(12, 13). Body composition is influenced by many parameters including age, gender, endocrine system status, nutrition, and exercise (14). All the above parameters are impaired in patients with thalassemia major in addition to hyperactivity of bone marrow, iron overload, and diminished bone mineral density (15, 16).