Abstract: Objective To quantitative analyze of the height and weight of patients with severe beta thalassaemia(β-TM) and its related factors. Methods Children with β-TM from the outpatient and inpatient in Zhongshan Boai Hospital during 2012-2016 were enrolled. According to the different genders and age,data of β-TM patients were compared with the standardization of growth curve fitting values of height and weight in Chinese children aged 0 to 18 for the quantitative evaluation of the change rule of the height and weight. The standard deviation score was calculated,while the regression model was established to analyze its correlation with gender,age,hemoglobin and serum ferritin (nearly one year′s average level). Results Totally 50 cases were included in the study,including 31 boys and 19 girls,with the average age of (7.09±3.89) years old. The level of hemoglobin(Hb) and serum ferritin(SF) were (78.60±9.85) g/L and (2 450.56±1 684.84) ng/ml respectively. The growth development of the boys,especially in 9 to 10-year-old children,obviously lagged behind normal children. The regression model revealed that children with older age(β=5.301,P<0.001) and higher level of serum ferritin (β=0.002,P=0.003) had larger gap on height with theexpected value with the standard deviation score as the dependent variable. In children who were boys (β=0.405,P=0.006),older (β=0.114,P＜ 0.001),and with higher the serum ferritin (β=0.000 1,P=0.011),the greater difference was observed between the weight of the children and the expected value. Conclusions The height and weight of children with β-TM are closely related to gender,age and serum ferritin. Compared with standardized growth curve,quantitative evaluation of the levels are of clinical significance for the prevention and control of β-TM.

Key words: severe beta thalassaemia, growth curve, hemoglobin, serum ferritin

摘要： 目的 定量分析重型β地中海贫血患儿身高和体重,探讨其相关因素。 方法 对本院2012-2016年期间门诊和住院接诊的重型β地中海贫血患儿进行资料收集,按照不同性别和年龄,与中国0~18岁儿童、青少年身高、体重的标准化生长曲线拟合值进行比较,定量评价重型β地中海贫血患儿的身高、体重的水平和变化规律。计算标准差分值,建立回归模型,探讨其与性别、年龄、血红蛋白(hemoglobin,Hb)和血清铁蛋白(serum ferritin,SF)(近1年的平均水平)的相关性。结果 共纳入研究对象50例,其中男童31例,女童19例,年龄(7.09±3.89)岁,Hb和SF分别为(78.60±9.85)g/L、(2 450.56±1 684.84)ng/ml。男性重型地贫患儿生长发育明显落后,9～10岁后落后越明显。以标准差分值为因变量建立的回归模型提示,年龄越大(β=5.301,P＜0.001)、SF含量越高(β=0.002,P=0.003),患儿身高与标准值的差距(以标准差为单位)越大;男性(β=0.405,P=0.006)、年龄越大(β=0.114,P＜0.001)、SF含量越高(β=0.000 1,P=0.011),患儿体重与预期值的差距(以标准差为单位)越大。结论 重型β地中海贫血患儿身高和体重落后,与性别、年龄和SF关系密切。与标准化生长曲线比较,定量评估其水平,对重型β地中海贫血生长发育障碍防治具有临床意义。

关键词: 重型β地中海贫血, 生长曲线, 血红蛋白, 血清铁蛋白