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重型地中海贫血患儿血管内皮生长因子。

Vascular endothelial growth factor in children with thalassemia major.

机构信息

Pediatrics Department, Beni Suef University.

出版信息

Mediterr J Hematol Infect Dis. 2013 Jun 5;5(1):e2013044. doi: 10.4084/MJHID.2013.044. Print 2013.

Abstract

BACKGROUND

The β-Thalassemia syndromes are the most common hereditary chronic hemolytic anemia due to impaired globin chain synthesis. Vascular endothelial growth factor (VEGF) plays several roles in angiogenesis which is a crucial process in the pathogenesis of several inflammatory, autoimmune and malignant diseases. Endothelial damage and inflammation make a significant contribution to the pathophysiology of β-thalassemia.

PURPOSE

: The aim of the study was to assess serum VEGF level in children with beta-thalassemia major as a marker of angiogenesis.

METHODS

A total of 50 children entered the study, 40 patients with thalassemia major and 10 healthy controls. We used enzyme-linked immunosorbent assay for quantitative evaluation of VEGF.

RESULTS

VEGF level was significantly higher in patients with β-thalassemia major than healthy controls (p=0.001). VEGF level was also higher in splenectomised thalassemic patients than non splenectomised ones (p=0.001). There were a positive correlation between VEGF and chelation starting age (p=0.008), and a negative correlation between VEGF and frequency of blood transfusion (p=0.002).

CONCLUSION

Thalassemia patients, especially splenectomized, have elevated serum levels of VEGF. Early chelation and regular blood transfusion help to decrease serum VEGF and the risk of angiogenesis.

摘要

背景

β-地中海贫血症是由于珠蛋白链合成受损导致的最常见遗传性慢性溶血性贫血。血管内皮生长因子(VEGF)在血管生成中发挥着多种作用,而血管生成是多种炎症、自身免疫和恶性疾病发病机制中的关键过程。内皮损伤和炎症对β-地中海贫血的病理生理学有重要贡献。

目的

本研究旨在评估重型β-地中海贫血患儿血清 VEGF 水平,作为血管生成的标志物。

方法

共纳入 50 名儿童入组研究,其中 40 名重型地中海贫血患者和 10 名健康对照者。我们使用酶联免疫吸附试验对 VEGF 进行定量评估。

结果

重型β-地中海贫血患者的 VEGF 水平明显高于健康对照组(p=0.001)。脾切除的地中海贫血患者的 VEGF 水平也高于未行脾切除术的患者(p=0.001)。VEGF 与螯合治疗起始年龄呈正相关(p=0.008),与输血频率呈负相关(p=0.002)。

结论

地中海贫血患者,尤其是脾切除的患者,血清 VEGF 水平升高。早期螯合治疗和定期输血有助于降低血清 VEGF 水平,从而降低血管生成风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b020/3684348/b5f257d0736e/mjhid-5-1-e2013044f1.jpg

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