Assessment of Sexual Maturation in β-Thalassemia Major Patients Receiving Iron Chelation Therapy in Assiut University Hospital

Thalassemia refers to a group of inherited diseases characterized by decreased or absent synthesis of normal globin chains . The direct consequence is an imbalance of the alpha and beta globin chain synthesis that results in anemia from ineffective erythropoiesis and hemolysis.

The term thalassemia major refers to the severe form that is often associated with life-long transfusion dependent anemia.

Hypogonadism is the most frequently reported endocrine complication, affecting 70-80% of thalassemia major patients. Hypogonadism is likely to be caused by iron deposits in the gonads, pituitary gland or both.

Hypogonadotropic hypogonadism resulting from iron deposition in the pituitary gonadotrope is more commonly found than gonadal iron deposition in ovaries or testes occurs.

Iron deposition in the anterior pituitary gland can be demonstrated beginning in the first decade of life, but clinical manifestations are usually not evident until the onset of puberty. At the earlier stage, only a diminished gonadotropin reserve with intact gonadotropin pulse was observed .