GROWTH AND PUBERTY IN THALASSEMIA MAJOR

Growth and sexual development were evaluated in 54 (29 female, 25 male ) patients with beta-thalassemia major aged 2.7-21.3 years (mean 10.4 yr). Mean pretransfusion hemoglobin concentration was 7.8 +/- 0.7 mg/d l. All patients except 6 were on desferrioxamine. Age of starting of t herapy was 6.8 +/- 3.9 years, Mean SDS values for height, weight and s itting height were significantly lower (p<0.001) than control cases of similar age, Height deficiency exceeded -2 SD in 18 patients and a de lay in bone age (>2 SD below the mean) was observed in 36 out of 54 pa tients. Among 11 patients over 14 years, 9 showed delay in onset or pr ogression of puberty and 10 had growth retardation. Height SDS were ne gatively correlated with chronological age, age of onset of desferriox amine and present serum ferritin levels (p<0.001). These findings indi cate that abnormal growth and delayed puberty are frequent in transfus ion dependent thalassemics. These can be partly overcome by early onse t of chelating therapy.