Management of puberty for optimal auxological results in beta-thalassaemiamajor

Short stature is present in a significant percentage of patients affected b y beta -thalas-saemia major. Growth failure of patients with thalassaemia i s multifactorial. The most important contribution is attributed to the toxi c effect of desferrioxamine and to endocrine disorders, due to iron overloa d. The commonest endocrine complication is hypogonadism. The growth pattern of patients with thalassaemia is characterized by normal growth during chi ldhood, a deceleration of growth velocity around age 9-10 years, and a redu ced pubertal growth spurt. In addition, reduced growth of the trunk is ofte n present. Short stature and short trunk are more evident at pubertal age. Hypogonadism is usually considered responsible for the pubertal growth fail ure, as well as the aggravation of body disproportion at pubertal age. Howe ver, data suggest that pubertal height gain and final height are reduced in both patients with spontaneous puberty and patients with induced puberty. It is concluded that several aspects of peripubertal growth in patients wit h thalassaemia remain to be clarified.