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Biochemical Markers of Bone Turnover in Patients with β-Thalassemia Major: A Single Center Study from Southern Pakistan

机译:β-地中海贫血专业患者骨转换的生化标志物:来自巴基斯坦南部的一项单中心研究

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摘要

Objectives. Skeletal complications in β-homozygous thalassemic patients are uncommon but often debilitating, even amongst children and adolescent patients with well maintained transfusion and chelation therapy. The aim is to evaluate the biochemical markers of bone turnover in regularly transfused thalassemic patients and its possible correlations with demographic data and hematological and biochemical markers. Methods. In this prospective cross-sectional study, 36 β-thalassemia major patients were enrolled from March 2012 to March 2014. All patients underwent complete blood counts, LFTs, serum ferritin, serum calcium, phosphorus, serum albumin, alkaline phosphatase, 25-OH vitamin D, and parathormone (PTH) levels. Results. There were 17 males and 19 females with mean age of 12.56 ± 5.9 years. Hypocalcemia and hypophosphatemia were seen in 66.6% and 19.4%, respectively, while 25-OH vitamin D deficiency was present in 72.2% of thalassemic children and adolescents. Hypoparathyroidism was seen in 13.8% while hyperparathyroidism was detected in 8.3% of patients. There was direct correlation between serum phosphorus and ferritin levels (P < 0.05). No correlation was found between indirect bilirubin and skeletal parameters, calcium and parathyroid hormone (P > 0.05). Conclusions. Biochemical profile is significantly altered in patients with β-thalassemia major and bone associated biochemical abnormalities like hypocalcaemia, 25-OH vitamin D deficiency, and hypophosphatemia are not uncommon in Pakistani patients with thalassemia major.
机译:目标。 β-纯合地中海贫血患者的骨骼并发症很少见,但常常使人衰弱,即使是在输血和螯合疗法维持良好的儿童和青少年患者中也是如此。目的是评估定期输注地中海贫血患者的骨转换的生化标志物及其与人口统计学数据以及血液学和生化标志物的可能相关性。方法。在这项前瞻性横断面研究中,纳入了2012年3月至2014年3月的36位地中海贫血重症患者。所有患者均进行了全血细胞计数,LFT,血清铁蛋白,血清钙,磷,血清白蛋白,碱性磷酸酶,25-OH维生素D和副激素(PTH)水平。结果。男17例,女19例,平均年龄为12.56±5.9岁。低钙血症和低磷酸盐血症的发生率分别为66.6%和19.4%,而72.2%的地中海贫血儿童和青少年中存在25-OH维生素D缺乏症。甲状旁腺功能减退症占13.8%,甲状旁腺功能亢进症占8.3%。血清磷和铁蛋白水平之间存在直接相关性(P <0.05)。间接胆红素与骨骼参数,钙和甲状旁腺激素之间没有相关性(P> 0.05)。结论。在重型地中海贫血患者中,生化特征发生了显着变化,而在重型重型地中海贫血患者中,与骨相关的生化异常,如低血钙症,25-OH维生素D缺乏症和低血磷症并不罕见。

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