关键词: 重型β地中海贫血, 生长曲线, 血红蛋白, 血清铁蛋白

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