Comparison of Oral Iron Chelation Therapy Versus the Injectable Once for the Decrease Some Endocrinopathy in β-Thalassemia Major Patients

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β-thalassemiamajor is a hereditary hemoglobinopathy due to defect in the production of β-globulin chain. The most common manifestations of disease are anemia and hepatospelenomegaly (due to extramedullary hematopoiesis). The mainstay of treatment of the β-major thalassemia is frequent blood transfusion that leads to iron overload in the critical organs include; liver, heart and endocrine glands. Iron chelators for attenuated of iron overload in body are used in two ways: injectable (subcutaneous deferoxamine) and recently oral once (Deferiprone; L1). Deferiprone is well inserted into the cell and removes iron, so is more effective than defroxamine in reducing endocrine complications and cardiac iron overload. Endocrinopatheis is one of the most common complications due to iron overload and approximately, 60% patients have at least one endocrine organ involvement.There are a few studies compare efficacy of oral and injectable iron chelator for decrease endocrine complications in β-thalassemia major patients. Here, we compared some endocrinopathies include: IGT (impared glucose tolerance), DM (diabetes mellitus), hypoparathyroidism (clinical and subclinical) and hypothyroidism in the patients with β-thalassemia major who received oral chelators against the injectable once.

Seventytwo patients with homozygote β-thalassemia major were included to this study. The patients were treated at the Children’s Medical Center Hospital, Tehran, Iran, from April 1997 to August 2017. Β-thalassemia major was diagnosed in the early of life by standard methods of peripheral blood smear and hemoglobin electrophoresis. Individual characteristics and type of used iron chelator were collected in the specific questionnaire. All of the patients were divided into two group 1(received oral iron chelator; Deferiprone or L1) include; 39 patients (38.46% male and 61.53% female) with age average of 19.5 years and group 2 (received injectable iron chelator; deferoxamine) include; 33 patients (60.6% male and 39.3% female) with mean age of 21.3 years. Deferiprone therapy was started 6 to 65 months (mean 29.9 ± 11.2 months) after receiving deferoxamine therapy from early age. The endocrine functions were evaluated for all the patients before and after of Deferiprone therapy. Fasting plasma glucose levels 110-125 mg/dl or 140-199 mg/dl after OGTT (oral glucose tolerance test 2 hours) was considered IGT (impaired glucose tolerance). Fasting blood glucose levels greater than 126 mg/dL or OGTT more than 200 mg/dl or presence of the symptoms of diabetes and plasma glucose concentrations greater than 200 mg/dL, were regarded to have diabetes mellitus. Hypoparathyroidism was defined as low serum calcium, high serum phosphate and low PTH. Clinical or subclinical hypothyroidism was defined according to Evered criteria.