Abstract
Endocrine function was evaluated in 16 patients 5-28 yrs old on high transfusion therapy for 1-7 yrs. Growth hormone responses to insulin-induced hypoglycemia (ITT) and/or arginine were normal in 13/14. Serum TSH ranged from <1.7-8.2 μU/ml (normal <5); Adj T4 (RIA) was low in one and normal in the others; T3 (RIA) was elevated in 2 and normal in 8/10. Morning ACTH ranged from 21-118 pg/ml (normal 15-100) and cortisol (F) from 7.2-27.8 ug/dl. Peak F level after ITT and Cosyntropin (0.25 mg i.m) were normal (> 18 μg/dl) in 8/13 and 16/16 respectively. Urinary 17 ketogenic steroids after Metyrapone were normal in 10/13. In 5 patients puberty occurred between 16-20 yrs; 1/5 developed secondary hypogonadism (LH and FSH < 2.0 mIU/ml, testosterone 50 ng/dl). In 5 patients, 18-26 yrs old with bone age > 14, LH and FSH were < 2.0 mIU/ml; 2/5 had a normal rise in gonadal steroids following human chorionic gonadotropin (1000 U × 10). Parathyroid hormone (PTH) ranged from 29-89 μlEq/ml, calcium and phosphorus from 8-10 and 2.8-5.6 mg/dl respectively. One patient had hypocalcemia and required vit D (PTH not measured). Glucose tolerance (OGTT) was normal in 6 patients and abnormal in 9. Of those with abnormal OGTT 4 developed insulin-dependent diabetes.
The data suggest that patients with thalassemia have: 1) pituitary/vs hypothalamic dysfunction with gonadotropin deficiency and possibly reduced ACTH reserve 2) primary gonadal failure and 3) increased incidence of chemical and insulin-dependent diabetes.
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Costin, G., Ortega, J., Hyman, C. et al. ENDOCRINE FUNCTION IN THALASSEMIA. Pediatr Res 11, 429 (1977). https://doi.org/10.1203/00006450-197704000-00361
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DOI: https://doi.org/10.1203/00006450-197704000-00361
