【摘要】 目的探讨重型β-地中海贫血患儿生长发育状态及其与铁超负荷的关系。方法于2007年7～8月对50例长期在该院儿科定期输血的重型β-地中海贫血患儿进行身高、体重和性发育评价,并与1995年中国0～18岁儿童体重、身高百分位数参考值比较,同时检测输血前血常规、肝功能和血清铁蛋白水平。结果24例(48%)重型β-地中海贫血患儿表现身材矮小,其中15例同时伴体重低下。≥10岁者21例,仅7例出现自发性青春期发育,TannerII～III期;≥14岁者8例,其中4例尚无性征发育。身高低于第10百分位者(n=31)与身高高于第10百分位者(n=19)分组比较,前者血清铁蛋白水平显著增高(8239.2±5865.5vs5028.1±3885.7mg/L,P<0.05),输血前Hb水平显著降低(68.2±12.3vs79.7±14.5g/L,P<0.05),肝脏显著增大(P<0.05)。而体重低于第10百分位者(n=20)与体重高于第10百分位者(n=30)分组比较,前者仅血清铁蛋白水平差异具有显著意义(9165.5±6042.5vs5567.3±4447.3mg/L,P<0.05)。结论接受中等量输血和不正规除铁治疗的重型β-地中海贫血患儿常伴有身材矮小、体重低下和性发育迟缓,其生长发育异常与体内铁严重超负荷有关。更多还原

【Abstract】 Objective To study the status of growth and development and the relationship between growth disorders and iron overload in children with beta-thalassemia major.Methods Fifty children with beta-thalassemia major and who received blood transfusion therapy regularly(age:9 months-17 years)were enrolled.They were subjected to a thorough history taking,clinical examinations,and laboratory examinations,including complete blood count,alanine transferasa(ALT)and serum ferritin.The physical growth parameters,such as height and weight,were compared with the reference values of Chinese children.Results Twenty-four patients(48%)were of short stature with height under the 3th percentile.Among them,15 cases presented with their height and weight both under the 3th percentile.Spontaneous sex development was seen in 7 cases out of 21 over 10-year-old patients.No sex development was found in 4 out of 8 patients who were over 14 years old.The patients with a height under the 10th percentile(n=31)had higher serum ferritin levels(8 239.2±5 865.5 mg/L vs 5 028.1±3 885.7 mg/L;P<0.05)and lower hemoglobin levels(68.2±12.3 g/L vs 79.7±14.5 g/L;P<0.05)as well as hepatomegaly when compared with those patients with a height over the 10th percentile(n=19).Serum ferritin levels in 20 patients with a weight under the 10th percentile were significantly higher than those in 30 patients with a height over the 10th percentile(9 165.5±6 042.5 mg/L vs 5 567.3±4 447.3 mg/L;P<0.05).Conclusions Short stature,low weight and sex development delay are common in children with beta-thalassemia major.This may be related to iron overload.更多还原