Huang Yong-Lan, Liu Sha, Xia Ting, Hao Wen-Ge, Liang Wei, Sun Xin
Department of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou 510180, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2008 Oct;10(5):603-6.
To study the status of growth and development and the relationship between growth disorders and iron overload in children with beta-thalassemia major.
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.
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 (8239.2+/-5865.5 mg/L vs 5028.1+/-3885.7 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 (9165.5+/-6042.5 mg/L vs 5567.3+/-4447.3 mg/L; P<0.05).
Short stature, low weight and sex development delay are common in children with beta-thalassemia major. This may be related to iron overload.
研究重型β地中海贫血患儿的生长发育状况以及生长障碍与铁过载之间的关系。
纳入50例定期接受输血治疗的重型β地中海贫血患儿（年龄：9个月至17岁）。对他们进行全面的病史采集、临床检查和实验室检查，包括全血细胞计数、丙氨酸转氨酶（ALT）和血清铁蛋白。将身高和体重等身体生长参数与中国儿童的参考值进行比较。
24例患者（48%）身材矮小，身高低于第3百分位数。其中，15例患者的身高和体重均低于第3百分位数。21例10岁以上患者中有7例出现自发性性发育。8例14岁以上患者中有4例未发现性发育。身高低于第10百分位数的患者（n = 31）与身高高于第10百分位数的患者（n = 19）相比，血清铁蛋白水平更高（8239.2±5865.5 mg/L对5028.1±3885.7 mg/L；P<0.05），血红蛋白水平更低（68.2±12.3 g/L对79.7±14.5 g/L；P<0.05），且肝肿大。20例体重低于第10百分位数的患者血清铁蛋白水平显著高于30例身高高于第10百分位数的患者（9165.5±6042.5 mg/L对5567.3±4447.3 mg/L；P<0.05）。
重型β地中海贫血患儿常见身材矮小、体重低和性发育延迟。这可能与铁过载有关。
