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血清铁蛋白水平与重型β-地中海贫血患儿血清hepcidin 和岩藻糖水平的关系。

Relation of Serum Ferritin Level with Serum Hepcidin and Fucose Levels in Children with β-Thalassemia Major.

机构信息

Department of Pediatrics, College of Medicine, University of Misan, Amarah, Misan, Iraq.

Department of Biochemistry, College of Medicine, University of Misan, Amarah, Misan, Iraq.

出版信息

Hemoglobin. 2021 Jan;45(1):69-73. doi: 10.1080/03630269.2021.1898419. Epub 2021 Mar 18.

DOI:10.1080/03630269.2021.1898419
PMID:33733981
Abstract

The aim of this study was to assess serum ferritin, hepcidin, L-fucose, and protein binding fucose levels in β-thalassemia major (β-TM) patients and to correlate the serum ferritin level with hepcidin and fucose levels. A total 70 (26 males and 44 females) children with β-TM, ages ranging from 5 to 16 years (mean age 8.3 ± 2.7 years) and 50 (25 males and 25 females) apparently healthy subjects with matching age and sex were included as a control group. An especially designed questionnaire was used to collect age, gender, body mass index (BMI), hemoglobin (Hb), serum ferritin, hepcidin-25 peptide, α-L-fucose, and protein binding fucose (PBF) levels. β-Thalassemia major patients had significantly ( < 0.05) higher serum ferritin, fucose and PBF levels, but the serum hepcidin level was significantly ( < 0.05) lower when compared to the controls, and their levels were affected by the gender of the β-TM patients, as it was significantly ( < 0.05) higher in female in comparison to male patients. There was no significant ( > 0.05) correlation between serum ferritin with hepcidin and fucose levels as a marker of iron overload in β-TM. The regulation of hepcidin, and L-fucose levels in patients with β-TM is more affected by erythropoeitic activity than by iron overload, as there was no significant correlation between serum ferritin with hepcidin and fucose levels as a marker of iron overload in β-TM.

摘要

本研究旨在评估β-地中海贫血(β-TM)患者的血清铁蛋白、hepcidin、L-岩藻糖和蛋白结合岩藻糖水平,并将血清铁蛋白水平与 hepcidin 和岩藻糖水平相关联。共纳入 70 名(26 名男性和 44 名女性)年龄 5 至 16 岁(平均年龄 8.3±2.7 岁)的β-TM 患儿和 50 名(25 名男性和 25 名女性)匹配年龄和性别的健康对照者。使用专门设计的问卷收集年龄、性别、体重指数(BMI)、血红蛋白(Hb)、血清铁蛋白、hepcidin-25 肽、α-L-岩藻糖和蛋白结合岩藻糖(PBF)水平。β-TM 患者的血清铁蛋白、岩藻糖和 PBF 水平显著( < 0.05)升高,而血清 hepcidin 水平显著( < 0.05)降低,与对照组相比,且其水平受β-TM 患者性别影响,女性显著( < 0.05)高于男性患者。血清铁蛋白与 hepcidin 和岩藻糖水平作为铁过载标志物之间无显著( > 0.05)相关性。β-TM 患者 hepcidin 和 L-岩藻糖水平的调节更多地受红细胞生成活性的影响,而不是铁过载,因为血清铁蛋白与 hepcidin 和岩藻糖水平作为铁过载标志物之间无显著( > 0.05)相关性。

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