Assessment of growth and body composition in patients with thalassemia

Abstract

Thalassemia is an inherited hemoglobin disorder that is becoming a major health problem in the world, especially in Mediterranean region. Children with thalassemia appear healthy at birth, but develop anemia that becomes progressively worse due to the partial or total absence of hemoglobin. Because of this condition, they need regular blood transfusions for the rest of their lives to manage chronic anemia, regular blood transfusions lead to iron accumulation in organs and may disrupt organ function and/or damage organs. Iron overload causes most of the mortality and morbidity associated with thalassemia. Long term transfusions should be accompanied by therapy with iron chelating agents. Growth retardation in thalassemia patients almost invariably present. Before the introduction of hypertransfusion and chelating therapy in the management of patients with B thalassemia, it was attributed to the chronic tissue hypoxia associated with severe anemia. Now iron toxicity from transfusion hemosidrosis have been implicated as a major cause of growth retardation. Without chelation, hemosidrosis can lead to tissue damage due to free radical formation. Key Words: Growth and body composition in patients with thalassemia