Department of Pediatrics, College of Medicine, University of Misan, Amarah, Misan, Iraq.
Department of Biochemistry, College of Medicine, University of Misan, Amarah, Misan, Iraq.
Hemoglobin. 2021 Jan;45(1):69-73. doi: 10.1080/03630269.2021.1898419. Epub 2021 Mar 18.
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.
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。