重型β地中海贫血患儿的性发育状况分析

Analysis of Sexual Development in Children with Severe Beta Thalassemia

目的探究重型β地中海贫血患儿的性发育状况。方法取2017年3月—2018年3月间在惠州市博罗县人民医院及惠州市第一人民医院诊治的重型β地中海贫血患儿50例开展此次研究,应用化学发光法,对所有患儿的性激素进行检测,同时检测所有患儿的血红蛋白水平和铁蛋白水平,分析对比不规则祛铁与规则祛铁、不规则输血与规则输血患儿性发育状况等情况。结果 50例患儿中,男性24例、女性26例,其中男性患儿发育者10例、未发育者14例;女性患儿月经者4例、彩超提示未发育者22例。血红蛋白水平发育患儿高于未发育患儿,且铁蛋白水平发育患儿低于未发育患儿,差异有统计学意义(P<0.05);性发育率相比较规则输血、规则祛铁者明显高于不规则输血、不规规则祛铁者,差异有统计学意义;性激素水平相比,未发育者低于发育者,差异有统计学意义(P<0.05)。血红蛋白水平与性激素水平呈正相关,且铁蛋白水平与性激素水平呈负相关,差异有统计学意义(P<0.05)。结论重型β地中海贫血患儿均会出现性发育延迟现象,当铁蛋白水平较低和血红蛋白较高水平者性发育较好,通过规则输血治疗、规则祛铁后,可有效降低铁过载现象发生几率,有效促使重型β地中海贫血患儿性发育,故临床应重型β地中海贫血患儿临床治疗措施的研究,使患儿可以尽早接受治疗,促使正常的性发育。

Objective To explore the sexual development of children with severe beta thalassemia. Methods 50 children with se? vere beta-thalassemia admitted to the hospital from March, 2017 to March, 2018 were selected. The levels of sex hormore were detected by chemiluminescence. The levels of hemoglobin and ferritin were also detected in all the children. Analysis and comparison of irregular iron removal and regular iron removal,irregular blood transfusion and regular blood transfusion in children with sexual development were done. Results Among 50 patients,24 were male and 26 were female. Among them,10 were matwer,14 were im? mature,4 were matue,22 were immatue by color Doppler sonography. The level of hemoglobin in children with developmental hemo? globin was higher than that in children with undeveloped hemoglobin,and the level of ferritin in children with developmental hemoglo? bin was lower than that in children with undeveloped hemoglobin(P<0.05). The level of development was lower than that of the devel? oping population,and the difference was statistically significant(P<0.05). Hemoglobin level was positively correlated with sex hor? mone level,and ferritin level was negatively correlated with sex hormone level,the difference was statistically significant(P<0.05). Conclusion Sexual development delay may occur in children with severe beta-thalassemia. Sexual development is better in children with low level of ferritin and high level of red hemoglobin. Regular blood transfusion and regular iron removal can effectively reduce the incidence of iron overload and promote sexual development in children with severe beta-thalassemia. Attention should be paid to the clinical treatment of children with severe beta-thalassemia,so that children can receive treatment as soon as possible,to promote normal sexual development.