Do Endocrinopathies Differ in Most Prevalent Hemoglobinopathy of Middle East: Beta-thalassemia?

S. Ahi, Mohsen Adelpour, Bahareh Haghdoost, A. Jaberi
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Abstract

Repeated blood transfusions in thalassemia patients is followed by endocrinopathies as diabetes, hypothyroidism, hypogonadism, hypoparathyroidism, and disorders in calcium and vitamin D homeostasis. The aimof this study was to evaluate the association of beta-thalassemia patientsendocrinopathies and osteoporosis. Serum level of some factors related tothe function of gonads, thyroid, adrenal, and pancreas along with serumlevels of calcium, phosphate, albumin, vitamin D, and iron were measured.Bone marrow density was tested via dual-energy x-ray absorptiometry(DXA densitometry). In this study, 56 patients with major thalassemia wereinvestigated. Paraclinical analysis indicated osteopenia in 17 (30.4%) andosteoporosis in 39 patients (69.6%) in addition to other types of endocrinedisorders, such as hypogonadism in 29 (51.8%), hypothyroidism in 13(23.2%), hypoparathyroidism in 1 (1.8%), hypocortisolism in 2 (3.6%), anddiabetes in 9 (16.1%) patients. Endocrinopathies had no significant relationship with osteoporosis and osteopenia in men. However, hypogonadismhad a significant relationship with osteoporosis and osteopenia in womenwith thalassemia. Estradiol level was lower in women with osteoporosisin comparison with women with osteopenia. Ferritin levels had neitherassociation with osteoporosis nor with LH levels (P>0.05). Secondary hypogonadism disorders are the main causes of osteoporosis and osteopeniain female beta-thalassemia patients.
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中东地区最普遍的血红蛋白病:地中海贫血是否与内分泌疾病不同?
地中海贫血患者反复输血后会出现内分泌疾病,如糖尿病、甲状腺功能减退、性腺功能减退、甲状旁腺功能减退以及钙和维生素D稳态紊乱。本研究的目的是评估-地中海贫血患者内分泌病变和骨质疏松症的关系。测定血清中与性腺、甲状腺、肾上腺和胰腺功能有关的一些因子的水平,以及血清钙、磷酸盐、白蛋白、维生素D和铁的水平。采用双能x线骨密度仪(DXA)测定骨髓密度。本研究对56例重度地中海贫血患者进行了调查。临床旁分析显示,17例患者骨质减少(30.4%),39例患者骨质疏松(69.6%),此外还有其他类型的内分泌疾病,如性腺功能减退29例(51.8%),甲状腺功能减退13例(23.2%),甲状旁腺功能减退1例(1.8%),低皮质醇2例(3.6%),糖尿病9例(16.1%)。内分泌疾病与男性骨质疏松、骨质减少无显著关系。然而,性腺功能减退与地中海贫血妇女骨质疏松和骨质减少有显著关系。骨质疏松症患者的雌二醇水平低于骨质减少症患者。铁蛋白水平与骨质疏松和LH水平均无相关性(P>0.05)。继发性性腺功能减退是女性-地中海贫血患者骨质疏松和骨质减少的主要原因。
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