小儿重型地中海贫血内分泌功能与生长发育情况分析

Analysis of endocrine function and growth and development in children with thalassemia major

目的探讨小儿重型地中海贫血内分泌功能与生长发育情况。方法选取2018年5月至2021年1月惠州市第一人民医院收治的62例重型地中海贫血患儿为研究对象。测量患儿的身高及体质量,并以此计算年龄别身高Z值(HAZ)及年龄别体质量Z值(WAZ);检测患儿的血红蛋白(Hb)、血清铁蛋白(SF)、甲状腺功能[包括超敏促甲状腺素(s-TSH)、游离甲状腺素(FT4),游离三碘甲状腺素(FT3)]及性激素水平[包括促黄体生成素(LH)、雌二醇(E2)、促卵泡激素(FSH)、睾酮(T)、孕酮(P)],并进行Pearson相关性分析。结果62例重型地中海贫血患儿的平均HAZ值为-1.69±1.37,平均WAZ值为-0.77±0.86;37.10%(23/62)的患儿HAZ值<-2,8.06%(5/62)的患儿WAZ值<-2,6.45%(4/62)的患儿HAZ、WAZ值均<-2;Pearson相关性分析结果显示,重型地中海贫血患儿的年龄、SF与HAZ值呈负相关(r=-0.430,P<0.05),Hb与HAZ值呈正相关(r=0.483,P<0.05);7例10周岁以上的患儿中,14.29%(1/7)的患儿存在甲状腺功能减退,即s-TSH、FT4水平降低,FT3水平正常;7例10周岁以上的患儿中,57.14%(4/7)的患儿存在性发育不良,即LH、E2、FSH、T及P水平低于正常值。结论重型地中海贫血患儿往往存在内分泌功能异常、生长发育迟缓,且生长发育迟缓与患儿的年龄、铁过载及不规范输血治疗有一定相关性,临床上应积极检测患儿的内分泌功能及生长发育情况。

Objective To investigate the endocrine function and growth and development of children with thalassemia major.Methods A total of 62 children with thalassemia major admitted to Huizhou First People's Hospital from May 2018 to January 2021 were selected as the research subjects.The height and body mass of the children were measured,and age-specific height Z value(HAZ)and age-specific body mass Z value(WAZ)were calculated accordingly.The hemoglobin(Hb),serum ferritin(SF),thyroid function[including hypersensitive thyrotropin(s-TSH),free thyroxine(FT4),free triiodothyroxine(FT3)],and sex hormone levels(including luteinizing hormone(LH),estradiol(E2),follicle stimulating hormone(FSH),testosterone(T),and progesterone(P)]of the children were detected.Pearson correlation analysis was conducted.Results The average HAZ value of 62 children with thalassemia major was-1.69±1.37,and the average WAZ value was-0.77±0.86.Among the 62 children,there were 37.10%(23/62)with HAZ value<-2,8.06%(5/62)with WAZ value<-2,6.45%(4/62)with HAZ and WAZ value<-2.Pearson correlation analysis showed that age and SF of children with thalassemia major were negatively correlated with HAZ values(r=-0.430,P<0.05),and Hb was positively correlated with HAZ values(r=0.483,P<0.05).Among the 7 children over 10 years old,14.29%(1/7)had hypothyroidism,that is,the levels of s-TSH and FT4 were reduced and the levels of FT3 were normal;57.14%(4/7)had sexual dysplasia,that is,the levels of LH,E2,FSH,T and P were lower than normal.Conclusion Children with thalassemia major tend to have endocrine dysfunction and growth retardation,and growth retardation has certain relevance with the patient's age,iron overload,and non-standard transfusion therapy.The endocrine function and growth and development of children should be actively detected clinically.