Pintor C, Cella S G, Manso P, Corda R, Dessĭ C, Locatelli V, Müller E E
J Clin Endocrinol Metab. 1986 Feb;62(2):263-7. doi: 10.1210/jcem-62-2-263.
The response of GH to acute administration of GH-releasing hormone (GHRH) was evaluated in 19 patients with thalassemia major and 8 normal children. In 13 of the 19 patients, GHRH induced a definite increase (greater than 5 ng/ml) in plasma GH levels, with peaks occurring 5-45 min postinjection. In 6 patients there was little or no GH rise after GHRH treatment. Overall, the mean GH response to GHRH of patients with thalassemia was lower than that of normal children. These data indicate that in thalassemia major, in addition to the described defect at the hepatic GH receptor or postreceptor level which impedes generation of somatomedins, there may be a marked impairment in somatotroph function. In one patient in whom the GH response to GHRH was superimposable on that of normal subjects, there was a blunted GH response to insulin hypoglycemia. This finding indicates that functional damage in hypothalamic structures for GH control can also occur in thalassemic patients.
在19例重型地中海贫血患者和8名正常儿童中评估了生长激素（GH）对急性注射生长激素释放激素（GHRH）的反应。19例患者中有13例，GHRH诱导血浆GH水平明显升高（大于5 ng/ml），注射后5 - 45分钟出现峰值。6例患者在GHRH治疗后GH几乎没有升高或未升高。总体而言，地中海贫血患者对GHRH的平均GH反应低于正常儿童。这些数据表明，在重型地中海贫血中，除了已描述的肝脏GH受体或受体后水平的缺陷阻碍了生长调节素的生成外，生长激素细胞功能可能存在明显损害。在1例对GHRH的GH反应与正常受试者相当的患者中，对胰岛素低血糖的GH反应减弱。这一发现表明，在地中海贫血患者中也可能发生下丘脑控制GH结构的功能损害。
