埃及β-地中海贫血儿童血清锌和铜水平的评估

K. Salama, M. Kamel, S. E. Sayed, S. Hassanein
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摘要

地中海贫血是一种严重的遗传性贫血,由血红蛋白合成失败引起,每年约有6万人出生。目的探讨两种不同口服铁螯合剂对埃及β-地中海贫血患儿血清锌、铜水平的影响及其与临床和常规实验室参数的关系。材料与方法本横断面研究从新儿童医院血科门诊招募60例埃及β-地中海贫血患儿。他们都未满18岁。每个病人的血红蛋白电泳被修改为一种诊断调查。患者分为两组:I组30例口服去铁宁,II组30例口服去铁素。30名年龄和性别匹配的儿童被纳入研究作为对照。病例和对照组均进行了实验室检查,包括全血细胞计数、胆红素(总胆红素和直接胆红素)、丙氨酸转氨酶、天冬氨酸转氨酶、碱性磷酸酶、血清钙和磷、锌和铜水平。样品采用Beckman AU 680 (Beckman Coulter International, Nyon, Switzerland)分光光度法分析。采用原子吸收光谱法测定血清锌和铜水平(Perkin Elmer, Buckinghamshire, United Kingdom)。结果研究病例中有13%的儿童血清锌水平较低,而对照组中没有儿童血清锌水平较低。只有1例患者血清锌水平较高。35%的研究病例血清铜水平高,而对照组没有一例血清铜水平高,只有一例血清铜水平低。比较两种口服螯合剂,锌和铜的含量没有显著差异。结论地中海贫血患者血清锌、铜水平明显高于对照组。这些水平与所使用的口服螯合剂的类型或血清铁蛋白水平无关。
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Assessment of serum zinc and copper levels in Egyptian children with β-thalassemia major
Background Thalassemia, with about 60,000 individuals born annually, is a severe inherited anemia, arising from the failure of hemoglobin synthesis. Objective This cross-sectional study aimed at evaluating the levels of serum zinc and copper in Egyptian children with β-thalassemia major on two different oral iron chelators, and its relation to clinical and routine laboratory parameters. Materials and Methods This cross-sectional study was conducted on sixty Egyptian children, with β-thalassemia major, who were recruited from the Hematology Clinic in New Children Hospital. All of them were below 18 years old. The hemoglobin electrophoresis of each patient was revised as a diagnostic investigation. The patients were classified into two groups: Group I included 30 patients on oral deferasirox, while group II included 30 patients on oral deferiprone. Thirty, age-and-sex-matched children were included in the study as controls. Both cases and controls were subjected to laboratory investigations, which are complete blood count, bilirubin (total and direct), alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, calcium and phosphorous, and zinc and copper levels in serum. Samples were analyzed spectrophotometrically on Beckman AU 680 (Beckman Coulter International, Nyon, Switzerland) using dedicated manufacturer reagents. Serum zinc and copper levels were measured by atomic absorption spectrometry (Perkin Elmer, Buckinghamshire, United Kingdom). Results Thirteen percent of the studied cases had low serum zinc level, while none of the control children had low serum zinc level. There was only one case that had high serum zinc level. Thirty five percent of the studied cases had high serum copper level, while none of the controls had high serum copper level, and only one case had low serum copper level. Comparing both types of oral chelators, there was no significant difference in them, regarding the zinc and copper levels. Conclusions Serum levels of zinc and copper were significantly altered in the thalassemic cases more than the controls. These levels were not related to the type of oral chelator used or their serum ferritin levels.
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