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成人β地中海贫血患者的生长激素储备

Growth hormone reserve in adult beta thalassemia patients.

作者信息

Vidergor Guy, Goldfarb Ada W, Glaser Benjamin, Dresner-Pollak Rivka

机构信息

Hebrew University-Hadassah Medical School, Jerusalem, 91120, Israel.

出版信息

Endocrine. 2007 Feb;31(1):33-7. doi: 10.1007/s12020-007-0018-7.

Abstract

Reduced serum insulin-like growth factor-1 (IGF-1) and hypogonadotrophic hypogonadism are common features of adult beta-thalassemia, and warrant evaluation of the growth hormone (GH)-IGF-1 axis. The aim of this study was to determine GH reserve in beta-thalassemia patients (9 females, 7 males, 15 major, 1 intermedia), age 29.3 +/- 6.9 years, BMI 21.3 +/- 1.9 kg/m2, and in 20 age, sex and BMI-matched healthy controls, using the GH-releasing hormone (GHRH)-arginine test. The associations between peak GH response and hormonal and biochemical indices were evaluated. Using BMI-related cut-off limits for peak GH response in the GHRH-arginine test, 4/16 beta-thalassemia patients had peak GH lower than 11.5 microg/l, the cut-off limit suggested for lean subjects, and were diagnosed as GH deficient (GHD). Using 9 microg/l as the cut-off limit 2/16 patients were GHD. Reduced serum IGF-1 and IGFBP-3 were present in 69% and 19% of the patients, respectively. Peak GH did not correlate with serum IGF-1, TSH, and fT4 levels or gonadal status. Neither peak GH nor IGF-1 correlated with serum ferritin. Our findings suggest that GHD is present in up to a quarter of adult beta-thalassemia patients. The clinical benefits of GH therapy need to be determined. GHD alone does not account for the high prevalence of reduced IGF-1 in adult beta-thalassemia.

摘要

血清胰岛素样生长因子-1(IGF-1)降低和低促性腺激素性性腺功能减退是成人β地中海贫血的常见特征,因此有必要评估生长激素(GH)-IGF-1轴。本研究的目的是使用生长激素释放激素(GHRH)-精氨酸试验,测定29.3±6.9岁、BMI 21.3±1.9kg/m²的β地中海贫血患者(9名女性,7名男性,15名重型,1名中间型)以及20名年龄、性别和BMI匹配的健康对照者的GH储备。评估了峰值GH反应与激素和生化指标之间的关联。根据GHRH-精氨酸试验中峰值GH反应的BMI相关临界值,16例β地中海贫血患者中有4例的峰值GH低于11.5μg/l(这是建议用于瘦人的临界值),被诊断为生长激素缺乏(GHD)。以9μg/l作为临界值时,16例患者中有2例为GHD。分别有69%和19%的患者血清IGF-1和IGFBP-3降低。峰值GH与血清IGF-1、促甲状腺激素(TSH)、游离甲状腺素(fT4)水平或性腺状态无关。峰值GH和IGF-1均与血清铁蛋白无关。我们的研究结果表明,高达四分之一的成人β地中海贫血患者存在GHD。生长激素治疗的临床益处有待确定。单纯GHD并不能解释成人β地中海贫血中IGF-1降低的高患病率。

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