EFFICACY OF COMBINATION THERAPY WITH ORAL IRON CHELATORS ON ENDOCRINE COMPLICATIONS IN CHILDREN WITH TRANSFUSION DEPENDENT B-THALASSEMIA MAJOR - A ONE YEAR HOSPITAL BASED INTERVENTIONAL STUDY

Abstract

Background and objectives The thalassemias are hereditary anaemias characterized by reduced synthesis of one or more of the globins that form the haemoglobin tetramer. Iron overload in β thalassemia major usually results in iron-induced cardiomyopathy, liver disease, and endocrine complications. Various authors have reported a high incidence of growth retardation, delayed puberty and endocrine dysfunction in poly transfused thalassemic patients. Iron chelation therapy effectively acts to prevent long-term complications of iron overload,- in transfusion-dependent thalassemia major patients. This study intends to see the incidence of endocrine disorders (hypothyroidism, diabetes, hypogonadism, GH deficiency), growth and pubertal delay and also to assess the efficacy of combined oral chelation therapy on these endocrine complications