摘要
目的了解重型β珠蛋白生成障碍性贫血(简称β地贫)患儿生长发育指标落后情况,以及血清铁蛋白(SF)定量、每次输血前Hb量对其生长发育指标的关系和意义。方法对59例重型β地贫(地贫组)患儿和48例同龄健康儿童(健康对照组)检测生长参数:身高[计算身高标准差分值(SDS)]、体质量[计算体质量指数(BMI)],Hb量、SF定量,青春发育分期采用Tanner分期方法分期。应用SPSS11.0软件进行统计学分析。结果与对照组比较,重型β地贫患儿组身高SDS、Hb量、SF定量水平均显著降低(t=-7.084,-25.771,11.928Pa<0.01),而BMI无显著性差异(t=-0.326P>0.05)。青春期发育程度中,在重型β地贫组(10～14岁)21例患儿中仅2例开始发育,且均为Tanner2期,而对照组(10～14岁)24例中全部开始正常发育,重型β地贫患儿发育显著落后。患儿身高SDS与年龄呈显著负相关(r=-0.588P<0.01),与每次输血前Hb呈正相关(r=0.219P<0.05),与SF无相关性(r=0.033P>0.05)。结论重型β地贫的生长和发育指标均落后于正常同龄儿,年龄越大,落后越明显。其生长发育异常与患儿体内铁超负荷有关。患儿平时保持Hb水平相对高值,正规采用去铁胺等祛铁治疗将有助于改善患儿最终身高和发育水平。
Objective To investigate the relationship and significance of serum ferritin（ SF）, pretransfusion mean hemoglobin （Hb） and upgrowth index in children with β thalassemia major. Methods Fifty - nine children with β - thalassemia major and 48 healthy children （ control group）, ranging in age from 2 years and 9 months to 14 years were studied. Height and weight were measured and standard deviation scores （SDS） and body mass index （BMI） were calculated according to local standards. SF and pretransfusion mean Hb were measured. The stages of adolescence was used by stages as Tanner method. The correlation analysis was used as statistical method with SPSS 11.0 software. Results Height SDS, pretransfusion mean Hb and SF in children with β - thalassemia major were significantly lower than those in control group（t= -7.084, -25.7.71,11.928 Pa 〈0.01）. While BMI had no significance （t = -0. 326 P〉0.05）. Only 2 children with β - thalassemia major （aged 10 - 14 years, n = 21 ）were pubescent. The control group （aged 10 - 14 years,n = 24）were pubescent. The upgrowth of children was significantly behinder than in control group. Height SDS was negative correlated with age （ r = - 0. 588 P 〈 0. 01 ）. Height SDS was positive correlated with mean pretransfusion Hb （ r = 0. 219 P 〈 0.05 ）. There was no significant correlation between height SDS and ferritin levels （ r = 0. 033 P 〉 0.05 ）. Conclusions The index and development of upgrowth in children with β thalassemie children were lower than normal children. These changes were much more obvious with ages. This may be related with iron overload. Maintaining the higher Hb lever and alleviating iron overload may be required to obtain an improvement in upgrowth in β - thalassemie children.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2009年第3期196-197,222,共3页
Journal of Applied Clinical Pediatrics
