Bone mineral status in the thalassemic children

Abstract

Thalassemia is a group of inherited hemoglobin disorder characterized by reduced synthesis of one or more of the globin chains leading to imbalanced a /non-a globin synthesis with secondary hemolytic anemia. In Egypt, P-thalassemia presents a major public health problem, as it constitutes about 85% of the chronic haemolytic anemias, with a gene frequency of0.03% Repeated transfusions unaccompanied by 1ron chelation cause hemosiderosis with secondary functional impairment of most body organs including endocrine glands, liver, heart and bones. On the other hand, iron- chelation therapy with desferal (DFO) prevents hemosiderosis but causes •1 skeletal dysplasia. Bone disease is known as a major cause of morbidity in thalassemia and is caused by many factors mainly the low hemoglobin level and the toxic effect of deposited iron on the endocrine glands and bone cells. It usually takes the form of decrease in bone mass (osteopenia or osteoporosis) with bone ache, deformity, short stature or fracture. Bone mass is measured by bone mineral density (BMD) that can be w, assessed by DEXA .Each patient's BMD is compared with the mean BMD .of age- and sex-matched reference population BMD (Z score).