地中海贫血儿童甲状旁腺功能与铁负荷的关系

A. Hagag, M. El-Shanshory, A. M. A. El-enein
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引用次数: 14

摘要

背景:地中海贫血患者存在严重贫血,需要定期输血。这可能导致铁超载及其相关并发症,包括内分泌系统紊乱。本研究的目的是研究重度地中海贫血儿童甲状旁腺功能与铁负荷的关系。方法:60例重型-地中海贫血患者。该队列包括年龄在6-10岁之间的32名男性和28名女性,以及30名年龄和性别匹配的健康儿童作为对照组。所有患者均进行全血细胞计数、Hb电泳、血清铁状态、甲状旁腺激素(PTH)水平、血清离子钙、磷和碱性磷酸酶以及骨矿物质密度评估。结果:β -地中海贫血患儿血清铁蛋白、铁、磷、碱性磷酸酶水平显著高于对照组,血清总铁结合力、甲状旁腺素、离子钙水平显著低于对照组。血清甲状旁腺激素水平与铁蛋白呈显著负相关。33例(55%)患者骨密度降低,21例(35%)患者骨质疏松,12例(20%)患者骨质减少。结论:地中海贫血患者甲状旁腺激素水平明显降低,且与血清铁蛋白呈显著负相关。建议定期和持续随访甲状旁腺激素、钙、磷、碱性磷酸酶和25-羟基维生素D水平,以早期发现地中海贫血患者甲状旁腺功能低下。定期和持续的骨密度评估也被推荐用于早期发现骨质疏松症或骨质减少症。
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Parathyroid function in children with beta thalassemia and correlation with iron load
Background: Patients with beta-thalassemia present with severe anemia requiring regular red blood cell transfusions. This can lead to iron overload and its related complications including disorders of the endocrine systems. The aim of this work was to study parathyroid function in children with beta-thalassemia major in correlation with iron load. Methods: 60 patients with beta-thalassemia major were included. The cohort included 32 males and 28 females with an age range of 6-10 years and a control group of 30 healthy children of matched age and sex. All patients underwent complete blood count, Hb electrophoresis, serum iron status, parathyroid hormone (PTH) levels, serum ionized calcium, phosphorus and alkaline phosphatase, and assessment of bone mineral density. Results: Serum ferritin, iron, phosphorus and alkaline phosphatase were significantly higher in children with beta-thalassemia, while serum total iron binding capacity, PTH and ionized calcium were significantly lower in these patients compared to controls. A significant negative correlation was found between serum parathyroid hormone levels and ferritin. Reduced bone mineral density was present in 33 patients (55%), with osteoporosis in 21 patients (35%) and osteopenia in 12 patients (20%). Conclusions: Parathyroid hormone levels are significantly lower in thalassemic patients, with a significant negative correlation with serum ferritin. Regular and continuous follow up of PTH, calcium, phosphorus, alkaline phosphatase, and 25-hydroxy vitamin D levels is recommended for early detection of hypoparathyrodism in thalassemic patients. Regular and continuous bone mineral density assessment is also recommended for early detection of osteoporosis or osteopenia.
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