重型β-地中海贫血患儿内分泌功能及生长发育监测与分析  

The monitoring and analysis of the endocrine function and growth of patient with β-thalassemia major

作　　者：张旸[1] 陈书远[1] 方敏超 廖影金 

机构地区：[1]惠州市第一人民医院儿科,广东惠州516000 [2]惠州市博罗县人民医院儿科,广东惠州516100

出　　处：《吉林医学》2017年第12期2239-2241,共3页Jilin Medical Journal

摘　　要：目的:探究重型地中海贫血(TM)内分泌功能及生长发育情况。方法:对108例TM者开展身高、体重检测,计算z值。同时开展内分泌激素项目测定,分析结果。结果:在108例病患中,HAZ<-2者占37.04%,WHZ<-2者占7.41%,上述两值均<-2者占6.48%。随着年龄增长,患儿发育迟缓愈加显著,低铁负荷及规范输血者表现较轻。10岁以上者检测甲状腺功能,异常者共计2例。10岁以上男性者中,6例促黄体生成素(LH)、卵胞刺激素(FSH)以及睾酮(T)值低于正常值,占66.67%(6/9);10岁以上女性者中,5例病患的LH、雌二醇(E2)、FSH以及孕酮(P)值低于正常值,占50.00%(5/10)。随着年龄增加,患儿发育迟缓现象愈加显著。相关性分析证实,年龄和HAZ呈现负相关(r=-0.426,P<0.05)。10周岁以上者共计19例,其中12例病患发育迟滞,血清蛋白(SF)值为(4 040±3 008)ng/ml,10岁以下者(2 271±1 087)ng/ml,组间数据差异有统计学意义(P<0.05)。输血之前,SF、血红蛋白(Hb)机体内含量均会对病患的生长发育造成影响。Hb值越低,SF越高,发育越为迟滞。相关性分析表明,血清铁蛋白和HAZ呈负相关(r=-0.261,P<0.05)。HAZ和输血前Hb呈现正相关(r=0.478,P<0.05)。结论:重型地贫患儿通常存在发育迟滞,与不规范治疗及铁过载存在相关性,积极开展内分泌项目和生长发育检查,有助于及早干预及改善患儿预后,提高其生活质量。Objective To investigate the endocrine function and growth of patient with β-thalassemia major. Method-Height and weight were measured in 108 patients with TM,and the 2 value was calculated. Meanwhile,the determination of endocrine hormone was carried out and the results were analyzed. Results Among the 108 patients,HAZ was lower than-2 standard,accounting for 37. 04%,WHZ was lower than-2,accounting for 7. 41%,and both of the above two values were lower than-2,accounting for 6. 48%. With the increase of age,children with growth retardation was more remarkable,patients with lower iron load and regulate blood transfusion. Thyroid function was detected in patients over the age of 10 and 2 of them were abnormal; Among patient of male at the age over 10,6 cases were found that LH,FSH and T were lower than normal,accounting for 66. 67%（6/9）; in patient of female at the age over 10,5 cases of patients with LH,E2,FSH and P values were lower than normal,accounting for 50. 00%（5/10）. With the increase of age,growth retardation in patients was more obvious. Correlation analysis showed that the negative correlation of age and HAZ（r =-0. 426,P〈0. 05）. Among 19 cases of patients at the age over 10,12 cases of them with growth retardation,SF =（4 040 ± 3 008） ng/ml,SF of patients younger than 10 was（2 271 ± 1 087） ng/ml,there were obvious differences between the data of the 2 groups above,P〈0. 05. Before blood transfusion,patient＇s growth was relate to SF and Hb. Growth retardation were more remarkable in patient with lower Hb and higher SF. The correlation analysis showed a negative correlation of serum ferritin and HAZ（r =-0. 261,P〈0. 05）. HAZ and Hb before blood transfusion showed positive correlation（r = 0. 478,P〈0. 05）. Conclusion Children with β-thalassemia major usually have retardation,which is relate to nonstandard treatment and iron overload. Monitoring of endocrine function and growth of these patients is helpful in early intervention and improve their quality of life.

关 键 词：重症地中海贫血 输血 去铁治疗 生长发育 内分泌功能 

分 类 号：R725.5[医药卫生—儿科]