Abstract
Endocrine function was investigated in 44(22 female, 22 male) transfusion dependent and deferoxamine treated thalassemic patients between 2.9 and 21.3 years(mean: 10.9 yrs) of age. Pretransfusion hemoglobin ranged from 6.5 to 9.2 g/dl and serum ferritin from 375 to 11000 ng/ml. 17 patients were growth retarded and 25 of 38 patients had delayed bone age. Of the 16 patients (11F, 5M) over 13 years of age, 14 had delayed puberty (in onset or progression). Hypoparathyroidism was present in two patients and diabetes in one patient. All patients had normal basal serum T4 and cortisol levels. Basal serum TSH was elevated in one subject. TSH response to TRH was exaggerated in 7 of 21 patients. GH response to stimulation (insulin and L-dopa) was subnormal in 4 of 20 cases. While 13 of 16 patients had decreased cortisol response to hypoglycemia, only 1 of 6 patients had decreased response to ACTH. Three of 9 patients over 13 years and with delayed puberty had no LH, FSH response to GHRH. Oral GTT showed chemical diabetes in one of 8 cases. The results suggest that endocrine abnormalities are common in patients with thalassemia major treated with frequent transfusion and chelating therapy.
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