不同基因型地中海贫血患儿铁代谢和红细胞系造血状况研究

摘要
图/表
参考文献(8)
相关文章 (15)
点击分布统计
下载分布统计

全文: PDF (963 KB)
输出: BibTeX | EndNote (RIS) 背景资料

摘要目的：探讨不同基因型地中海贫血（地贫）患儿体内铁代谢和红细胞系造血状况。方法：对158例确诊地贫患儿进行血清铁蛋白（SF）、血清转铁蛋白受体（sTfR）、促红细胞生成素（EPO）检测，比较不同基因型地贫患儿之间的差异，并分析其与血红蛋白水平的相关性。结果：158例地贫患儿中，轻型α地贫52例（32.9％），血红蛋白H病（HbH病）27例（17.1％），轻型β地贫59例（37.4％），重型β地贫13例（8.2％），α复合β地贫7例（4.4％）。HbH病及重型β地贫患儿的SF水平较其他各组明显升高，差异有统计学意义（P＜0.01）；重型β地贫患儿sTfR水平较其他各组升高，差异有统计学意义（P<0.05）。重型β地贫患儿EPO水平较其他各组明显升高，差异有统计学意义（P＜0.01）；HbH病和重型β地贫患儿Hb水平和EPO水平呈负相关，分别为γ=-0.656（P＜0.01）和γ=-0.641（P＜0.05）。结论：不同基因型地贫患儿体内铁代谢和红细胞系造血状况不同，联合SF、sTfR和EPO检测可反映造血状况，指导临床治疗。［中国当代儿科杂志，2010，12（8）：602-604］

	服务

	把本文推荐给朋友
	加入我的书架
	加入引用管理器
	E-mail Alert
	RSS
	作者相关文章
	麦惠容
	李长钢
	王缨
	石红松
	赵维玲
	陈运生
	陈小文

关键词 ：地中海贫血, 血清铁蛋白, 血清转铁蛋白受体, 促红细胞生成素, 儿童

Abstract：OBJECTIVE: To study the status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia. METHODS: Serum concentrations of ferritin (SF), transferrin receptor (sTfR) and erythropoietin (EPO) were measured in 158 children with thalassemia. The differences in the concentrations of the three indices among children with different genotypes of thalassemia were compared. The correlations of the hemoglobin level with sereum SF, sTfR and EPO levels were assessed. RESULTS: Among the 158 children with thalassemia, 52(32.9%) were diagnosed with alpha-thalassemia minor, 27(17.1%) with HbH disease, 59(37.4%) with beta-thalassemia minor, 13(8.2%) with beta-thalassemia major, and 7(4.4%) with combining alpha beta thalassemia. The SF levels in children with HbH disease or beta-thalassemia major were significantly higher than those in the other thalassemia groups (P<0.01). The sTfR levels in children with beta-thalassemia major were the highest when compared with those in the other thalassemia groups (P<0.05). The EPO levels in children with beta-thalassemia major were also the highest when compared with those in the other thalassemia groups (P<0.01). There was a negative correlation between hemoglobin and EPO levels in children with HbH disease (r=-0.656, P<0.01) and beta-thalassemia major (r=-0.641; P<0.05). CONCLUSIONS: The status of iron metabolism and erythropoietic proliferation is different in children with different genotypes of thalassemia. A combined measurement of SF, sTfR and EPO may reflect the status of erythropoietic proliferation.［Chin J Contemp Pediatr, 2010, 12 (8):602-604］

Key words： Thalassemia Serum ferritin Serum transferrin receptor Erythropoietin Child

PACS:

R725

引用本文:

麦惠容,李长钢,王缨等. 不同基因型地中海贫血患儿铁代谢和红细胞系造血状况研究[J]. 中国当代儿科杂志, 2010, 12(08): 602-604.

MAI Hui-Rong,LI Chang-Gang,WANG Ying et al. Status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia[J]. CJCP, 2010, 12(08): 602-604.

链接本文:

http://www.zgddek.com/CN/ 或 http://www.zgddek.com/CN/Y2010/V12/I08/602

	［1］陈华琼，邓兵，游亚萍. 不同基因型地中海贫血患儿铁负荷情况的分析［J］. 第三军医大学学报，2005，27（20）：2080-2082.
	［2］黄钰君，伍绍国，区小冰，张力. 不同基因型地中海贫血患儿血清铁代谢指标的研究［J］. 中国当代儿科杂志，2010，12（2）：85-88.
	［3］胡若愚，叶飞，詹乾钢，丁建人，周国忠. 血清铁蛋白检测的临床价值［J］. 中国误诊学杂志，2001，1（7）：1089-1090.
	［4］黄永兰，刘莎，夏婷，郝文革，梁薇，孙新. 重型β-地中海贫血患儿生长发育异常及其与铁超负荷的关系［J］. 中国当代儿科杂志，2008，10（2）：603-606.
	［5］Beguin Y. Soluble transferrin receptor for the evaluation of erythropoiesis and iron status［J］. Clin Chem, 2003, 329(1):9-22.
	［6］Dimitriou H, Stiakaki E, Markaki EA, Bolonaki L, Giannakopoulou C, Kalmanti M. Soluble transferrin receptor levels and soluble transferrin receptor/log ferritin index in the evaluation of erythropoietic status in childhood infections and malignancy［J］.Acta Paediatr, 2000, 89(10):1169-1173.
	［7］Gunga HC, Frommhold M, Hildebrandt W, Kirsch K, R-cker L. Erythropoietin production during flights with pressure aircrafts［J］. Lancet, 1996, 348(9024):416.
	［8］Paritpokee N, Wiwanitkit V, Bhonkaisawan N, Boonchalermvichian C, Nuchprayoon L. Serum erythropoietin levels in pediatric patients with beta-thalassemia/hemoglobin E［J］. Clin Lab, 2002, 48(11-12):631-634.