Final height and endocrine function in thalassaemia intermedia

We present data of a detailed study of endocrine function in 50 patients (2 1 males, 29 females) with thalassaemia intermedia, 15-46 years old (mean ag e 28.7 yr), with raised serum ferritin levels (mean 1540 mu g/l), Mean haem oglobin concentration was 8.1 g/dl, Half of them had had more than 50 trans fusions in their life and had received irregular intramuscular or subcutane ous chelation therapy, Delayed puberty was one of the most frequent (36%) c linical endocrine abnormalities found in our patients, Primary amenorrhea w as observed in two patients and secondary amenorrhea in four patients, Two males, aged 19 and 36 years, had hypogonadism, A poor response to GnRH, fou nd in three females and in both males tested, suggested that pituitary dysf unction was wholly or partially responsible for hypogonadism. Gonadal funct ion was normal in all patients studied. Glucose intolerance and primary hyp othyroidism were less frequent (24 and 5.7%, respectively) and milder than in thalassaemia major patients. Two patients had low T-3 and T-4 and normal basal and stimulated response of TSH to TRH, This condition has been found in euthyroid sick syndrome and it is likely that it represents an adaptive response by the body to minimize catabolism when undergoing major stress, As a consequence, we believe that periodic endocrine evaluation should be c arried out in subjects with beta-thalassaemia intermedia, particularly in t hose over 14 years old, in order to detect and to treat endocrine dysfuncti on.