title: Endocrine function in thalassemia intermedia.
abstract: Thalassemias are the most common genetic disorder on a wordwide basis. β-thalassemia is a severe hemolytic anemia which results from genetic defects in the synthesis of the hemoglobin β-chain. Various endocrine abnormalities have been described in patients with thalassemia major. Endocrine disturbances have also been observed in patients with thalassemia intermedia (TI). In this study endocrine functions were investigated in TI and here the frequency of different abnormalities is reported. Ninety-three patients (40 males, 53 females) with thalassemia intermedia, 11-40 years old (mean 19.4 yr) were studied. Medical history was obtained and a complete physical examination was done for each patient. The age, sex, weight, height and serum ferritin levels were recorded using a questionnaire. Growth Hormone (GH) secretion, thyrotropin (TSH), T(4), parathyroid hormone (PTH) and cortisole levels were determined in these patients. The mean ± Standard Deviation (SD) serum ferritin level was 452.4 ± 312.60 μg/L. Mean ± SD hemoglobin concentration was 9 ± 1 g/dl. Short stature was present in 46% of patients. Growth hormone deficiency was one of the most frequent (31%) endocrine abnormalities in these patients. Primary hypothyroidism was observed in 21.5% of patients. Hypoparathyroidism was found in one patient (1%). Failure of puberty was present in 2% of patients, secondary ammenorrhea was observed in 6.4% of patients and diabetes mellitus (DM) in 2% of patients.
  
  
  CONCLUSIONS
  Growth retardation and GH deficiency should be considered as common finding in TI. Therefore endocrine evaluation of these patients is suggested to prevent complications and to improve the overall quality of life.
authors.affiliation: Division of Endocrinology and Metabolism, Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran;
authors.forename: H
authors.initials: H
authors.lastname: Karamifar
authors.forename: M
authors.initials: M
authors.lastname: Karimi
authors.forename: G H
authors.initials: GH
authors.lastname: Amirhakimi
authors.forename: M
authors.initials: M
authors.lastname: Badiei
keywords: endocrinopathies; thalassemia intermedia