Werther G A, Matthews R, Burger H G, Herington A C
Arch Dis Child. 1981 Nov;56(11):855-9. doi: 10.1136/adc.56.11.855.
The commonly occurring short stature in the condition of thalassaemia major was investigated with respect to the possible role of the somatomedin growth factor low molecular weight non-suppressible insulin-like activity (NSILAs). Nineteen affected patients (12 boys, 7 girls) aged between 2 and 21 years were studied. Twelve of them were on or below the 10th centile for height of whom 7 were on or below the 3rd centile for height. Serum immunoreactive growth hormone responses to exercise were normal in 9 of 11 subjects tested. Using an isolated fat cell bioassay NSILAs was undetectable in 10 and was more than 2 SD below the normal mean value in the other 9 subjects. High molecular weight NSILA (not a growth factor) was very low or undetectable in all 9 subjects tested. Low molecular weight NSILAs did not show the normal correlation with age in childhood, nor was there any correlation with height, height velocity, or bone age. The 2 children above the 50th centile for height had undetectable NSILAs. There was no evidence of iron or ferritin interfering in the bioassay, and mixing experiments showed no evidence of inhibitory activity towards NSILAs in thalassaemic sera. Low circulating levels of the somatomedin NSILAs may contribute to the short stature in thalassaemia major, but other factors may permit normal growth in some affected children.
针对地中海贫血重型患者中常见的身材矮小情况，研究了生长调节素生长因子低分子量非抑制性胰岛素样活性（NSILAs）可能发挥的作用。对19例年龄在2至21岁之间的受影响患者（12名男孩，7名女孩）进行了研究。其中12人的身高处于或低于第10百分位，其中7人的身高处于或低于第3百分位。在11名接受测试的受试者中，有9人运动后血清免疫反应性生长激素反应正常。使用分离脂肪细胞生物测定法，10名受试者未检测到NSILAs，另外9名受试者的NSILAs比正常平均值低2个标准差以上。在所有9名接受测试的受试者中，高分子量NSILA（不是生长因子）非常低或未检测到。低分子量NSILAs在儿童期与年龄未显示出正常相关性，与身高、身高增长速度或骨龄也无相关性。身高处于第50百分位以上的2名儿童未检测到NSILAs。没有证据表明铁或铁蛋白干扰生物测定，混合实验也未显示地中海贫血血清中存在对NSILAs的抑制活性。生长调节素NSILAs的循环水平较低可能导致地中海贫血重型患者身材矮小，但其他因素可能使一些受影响儿童实现正常生长。
